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					                                            Starship Children’s Health Clinical Guideline
                   Note: The electronic version of this guideline is the version currently in use. Any printed version can
                         not be assumed to be current. Please remember to read our disclaimer.



                                            CYSTIC FIBROSIS
DISCLAIMER: ALTHOUGH DRUG DOSES ARE GIVEN IN PARTS OF THESE GUIDELINES, THEY
HAVE NOT ALL BEEN CHECKED IN AN AUTHORITATIVE TEXT AND THEY HAVE NOT YET BEEN
CHECKED BY ADHB PHARMACY. DO NOT PRESCRIBE ANY TREATMENTS BASED SOLELY ON THE
DOSES GIVEN IN THIS DOCUMENT. ALWAYS CHECK DOSES IN AN ACKNOWLEDGED SOURCE (e.g.
CHILDREN’S BNF, RCH PHARMACOPOEIA)

    1. Introduction                                                   6.11 Bronchoscopy
    2. Departmental Staff & Contact Numbers                           6.12 Home oxygen
        - Referrals to other specialists                              6.13 Palliative BiPAP support
    3. How the Service Runs                                       7. Chest physiotherapy
       3.1 Clinic                                                 8. Nutritional care
       3.2 Clinic procedures                                      9. Gastrointestinal care
       3.3 Annual review                                              9.1 DIOS & constipation
       3.4 Transition to adult services &                             9.2 Liver disease
       transfer clinic                                                9.3 Iron status
       3.5 Nurse specialist                                       10. Endocrine complications
       3.6 Consult liaison team                                       10.1 CF-related diabetes (CFRD)
       3.7 Social work support                                        10.2 Growth & Puberty
    4. Admission to hospital                                          10.3 Bone metabolism
       4.1 Admitting the child                                    11. Other non-pulmonary complications
       4.2 Investigations                                             11.1 ENT
       4.3 Venous access                                              11.2 Arthropathy
       4.4 Procedural distress                                        11.3 Pseudo-Bartter’s syndrome
       4.5 Discharge                                                  11.4 Infertility
       4.6 Infection control                                          11.5 Stress incontinence
       4.7 Specific Organisms                                     12. Transplant Assessment
    5. Making the Diagnosis                                           12.1 Criteria for assessment
       5.1 Sweat testing                                              12.2 Contra-indications
       5.2 Genetic analysis                                       13. Miscellaneous
       5.3 Other tests                                                13.1 Preparation for surgery
       5.4 Routine inpatient investigations for                       13.2 Immunisation
       newly diagnosed patients                                       13.3 Chicken-pox
    6. Respiratory Care                                               13.4 Overseas travel
       6.1 Respiratory exacerbations                              14. Terminal Care
       6.2 Microbiology                                           15. Drug Formulary
           6.2.1 Cultures                                             15.1 Drugs for the Respiratory tract
           6.2.2 Specific organisms                                   15.2 Drugs for the gastrointestinal tract
           6.2.3 Choice of Intravenous                            16. References
           antibiotics                                            17. Appendices
           6.2.4 Allergic reactions to antibiotics                    I     Annual Review Check List
           6.2.5 Home IV antibiotics                                  II    Brasefield score (CXR score)
           6.2.6 Portacath (TIVAD)                                    III CF annual review template letter
       6.3 Use of steroids                                            IV Nursing transition document
       6.4 rhDNase (pulmozyme)                                        V     Transfer to adult care (pro forma)
       6.5 Hypertonic saline                                          VI ABPA results sheet
       6.6 Long term azithromycin                                     VII Lung function summary sheet
       6.7 Aspergillus lung disease                                   VIII Discharge summary
       6.8 Haemoptysis                                                IX Financial Support
       6.9 Pneumothorax                                               X     Schwachmann-Kulczycki score
       6.10 Intractable wheezing / severe small
       airways disease

Author:           Drs Vyas, Byrne, Jaksic & Jan Tate               Service:             Paediatric CF Team
Editor:           Dr Raewyn Gavin                                  Date Issued:         August 2008
Cystic Fibrosis   Page:                                            1 of 100
                                            Starship Children’s Health Clinical Guideline
                   Note: The electronic version of this guideline is the version currently in use. Any printed version can
                         not be assumed to be current. Please remember to read our disclaimer.



                                            CYSTIC FIBROSIS



These guidelines were developed by Cass Byrnes, Mirjana Jaksic, Julian Vyas, and Jan Tate.
They are based on the Paediatric CF guidelines from the Royal Brompton Hospital, London. We
have also adopted parts of guidelines from the Manchester Children’s Hospitals, and the UK CF
Trust. Furthermore, certain sections have been reviewed, and amended, by colleagues with
specific CF expertise.




Introduction
Death in childhood from CF is now rare, and children born today are likely to have a mean life
expectancy of approximately 40 years. There are over 400 people with CF in New Zealand and just
under half are children. The paediatric team normally sees children and adolescents until they
reach between 16 and 18 years of age when they are transferred to adult CF services. We believe
that with careful attention to detail, children with CF can expect to have a much longer life
expectancy, and a far better quality of life than previously. The forthcoming CFNZ Standards of
Care document is intended to set out certain tenets of clinical practise in CF to ensure that all
those with CF have equitable access to specialist care. This document is written with these goals
in mind. Its purpose is to set out guidelines to ensure a uniformity of clinical care for children with
cystic fibrosis who are looked after by the Paediatric Respiratory and Cystic Fibrosis team at
Starship Children’s Hospital. They should be used as a guide only.

Our philosophy of care for patients with cystic fibrosis is based on current guidelines laid down by
the Royal College of Physicians, Royal College of Paediatrics & Child Health (formerly British
Paediatric Association), CF Trust and the British Thoracic Society, and the impending New
Zealand CF Standards of Care (which are based on the European, and Australian Standards of
Care documents). Many of these have identified significant advantages in terms of survival and
morbidity for patients receiving care from specialist centres. Specialist centres offer valuable
access to comprehensive care from a multidisciplinary team consisting of consultants with a
special interest in CF, junior doctors, nurse specialist, dietitian, physiotherapist, clinical
psychologist, pharmacist and social worker. We are also responsible for producing and distributing
educational material and carrying out research to improve knowledge about this disease. Special
procedures and investigations are referred to that are often not available at district hospitals (such
as formal lung function and bronchoscopy). However, it must be stated that it is not our aim to
belittle the vital CF care done by our colleagues in district hospitals, nor to seek to monopolise
patient care. We prefer to establish a shared care policy, so that patients remain primarily under
the care of their local consultant. We have an existing model of providing outreach support through
annual or biannual outpatient clinics. We are very happy to extend this support to other colleagues
working elsewhere in New Zealand, should they wish it.

Please note that instructions in these guidelines to “discuss with a CF consultant” apply to junior
staff at Starship, and are not intended to dictate the actions of consultant colleagues in other
hospitals. We welcome comments or questions on these guidelines from our colleagues.




Author:           Drs Vyas, Byrne, Jaksic & Jan Tate               Service:             Paediatric CF Team
Editor:           Dr Raewyn Gavin                                  Date Issued:         August 2008
Cystic Fibrosis   Page:                                            2 of 100
                                            Starship Children’s Health Clinical Guideline
                   Note: The electronic version of this guideline is the version currently in use. Any printed version can
                         not be assumed to be current. Please remember to read our disclaimer.



                                            CYSTIC FIBROSIS

                              Details
2. Department Staff & Contact Details

Department of Respiratory Medicine,
Starship Children’s Health,
Private Bag 92024,
Auckland Mail Centre,
AUCKLAND 1142
New Zealand

(09) 307 4949 extn 5471

                              Cass Byrnes
                           Senior Lecturer/Hon            09 307 4949         c.byrnes@auckland.ac.nz
      Doctors
                          Consultant in Paediatric         extn 5471
                           Respiratory Medicine
                               Julian Vyas
                                                          09 307 4949
                          Consultant Respiratory                                  julianv@adhb.govt.nz
                                                           extn 5471
                              Paediatrician
                             Mirjana Jaksic               09 307 4949
                                                                              m.jaksic@auckland.ac.nz
                           CF Clinic Consultant            extn 5471

                                                          09 307 4949
  Team Support                 Dani Ta’ase
                                                           extn 5471

                                                          09 307 4949
                                 Jan Tate                                          jant@adhb.govt.nz
                                                           extn 6556
    CF Nurse
    Specialists
                                                          09 307 4949
                             Catherine Lambe                                      clambe@adhb.govt.nz
                                                           extn 6556


   CF Dietitian               Jenny Heyward                                   jheyward@adhb.govt.nz


                               Sian Beattie                                        sian@adhb.govt.nz
      CF
Physiotherapists
                              Vicky MacBean                                   VMacBean@adhb.govt.nz


   Respiratory                Cathy Douglas
                                                                              ssresplab@adhb.govt.nz
  Physiologists                Sheri Green

                                                          09 307-4949
     Ward 26B
                                                           ext 25760



Author:           Drs Vyas, Byrne, Jaksic & Jan Tate               Service:             Paediatric CF Team
Editor:           Dr Raewyn Gavin                                  Date Issued:         August 2008
Cystic Fibrosis   Page:                                            3 of 100
                                             Starship Children’s Health Clinical Guideline
                     Note: The electronic version of this guideline is the version currently in use. Any printed version can
                           not be assumed to be current. Please remember to read our disclaimer.



                                             CYSTIC FIBROSIS

Referral to other specialists

At times we request other consultants to see the children, and this is often done in conjunction with
the shared-care consultants. Registrars must not make referrals without prior discussion with Dr
Byrnes, Dr Jaksic or Dr Vyas. Our practice is to consult the following:


                                                                     Mr Colin Barber,
            Ear, Nose & Throat
                                                                  Mr Murali Murhadaven

                  Endocrinology                                      Dr Craig Jefferies

                                                            Dr Helen Evans, Dr Simon Chin,
      Gastroenterology & Hepatology
                                                                  Dr Stephen Mouat

                    Genetics                                 Genetic Counselling Services

              Transplantation                                        Dr Mark O’Carroll

             Paediatric Surgery                           Mr James Hamill, Mr Phil Morreau

                                                                   Dr Russell Metcalfe,
                   Radiology
                                                                  Dr David Davis-Payne


                                                                  Professor John Kolbe
             Adult CF Services,                                      Dr Mark O’Carroll
          Auckland City Hospital                                   Dr Margaret Wilsher
                                                           Linda Thrift (CF Nurse Specialist)




Author:            Drs Vyas, Byrne, Jaksic & Jan Tate                Service:             Paediatric CF Team
Editor:            Dr Raewyn Gavin                                   Date Issued:         August 2008
Cystic Fibrosis    Page:                                             4 of 100
                                             Starship Children’s Health Clinical Guideline
                    Note: The electronic version of this guideline is the version currently in use. Any printed version can
                          not be assumed to be current. Please remember to read our disclaimer.



                                             CYSTIC FIBROSIS

3. How the Service Runs

3.1 Clinic

There is one clinic per week, held on Wednesday mornings, between 9am and 11:30 am. All
patients are separated from each other and wait in different areas in the clinic. Those patients with
MRSA, Burkholderia, Mycobacterial infection or multi resistant organisms will be seen last at clinic
and wait in different areas from the usual waiting area. Patients identified by the newborn
screening programme should also be seen in the clinic, although this may not be possible, and
they can be seen at other times with prior arrangement by the nurse specialist.

Newly diagnosed patients will be seen weekly at first. This is partly to allow the parent’s access to
all the members of the team and partly to ensure that the initial care is adequate. As the child
makes clinical progress, and the parents become more accustomed to the diagnosis of CF, so the
clinic intervals can be lengthened. In general, no-one should go longer than 3 months between a
review at clinic. There is some evidence that patients are generally better if seen more frequently,
and the UK CF trust recommends review every 6-8 weeks. Those in whom the diagnosis is still
relatively recent (in the first 6-12 months) could perhaps benefit from being seen 1-2 monthly.
Similarly, those who have severe disease will also likely need to be seen more often than 3
monthly.

Children who receive shared care should ideally be seen by a CF specialist every 6 months.
Whether this occurs at the local district hospital or in Auckland is debatable. The benefits of being
seen in a Specialist CF centre derive from review by all members of the Specialist team, who will
have particular expertise in CF. For this reason we would encourage all children under shared care
to attend the CF clinic in Auckland at least once per year.

The families should be able to see the following team members at the clinic:
    •     Doctor - This may be a consultant or a specialist registrar. Parents may request to see a
          specific doctor, but this may mean they have to wait longer to be seen. All children should
          see a CF Consultant at least once in every three visits.

    •     CF Nurse Specialist - Will measure weight and height and provide general information,
          education and support.

    •     Physiotherapist - Will review techniques and obtain sputum specimens or nasopharyngeal
          aspirates. All children should be seen at least twice year by a physiotherapist. This is
          usually in one of the outpatient rooms, but sometimes the children are seen in the
          Physiotherapy Department.

    •     Dietician - Will review nutrition particularly if there is a problem with weight gain. She is
          always available in the Clinic.

    •     Respiratory Physiologists - Will measure lung function in the Respiratory Lab from about 5
          years of age. Parents are expected to stay outside the Respiratory Lab when the child is
          performing lung function testing.



Author:            Drs Vyas, Byrne, Jaksic & Jan Tate               Service:             Paediatric CF Team
Editor:            Dr Raewyn Gavin                                  Date Issued:         August 2008
Cystic Fibrosis    Page:                                            5 of 100
                                             Starship Children’s Health Clinical Guideline
                    Note: The electronic version of this guideline is the version currently in use. Any printed version can
                          not be assumed to be current. Please remember to read our disclaimer.



                                             CYSTIC FIBROSIS
    •     Consult Liaison Team -Child psychology support is available, although often separate
          appointments are made away from clinic times.

    •     Community Field Worker - Sally Carron is employed by the Auckland branch of CFNZ and
          is present on most CF clinic days. She provides advice and support to families and helps
          with claiming allowances from MOH, WINZ & CF Assn. she can be contacted via the
          Auckland branch of CFNZ outside of clinic hours.


3.2 Clinic procedures

    •     Children are always weighed without shoes and heavy clothing and have their height
          measured on a stadiometer.
    •     Babies are weighed naked up to the age of 1 year.
    •     Head circumference should be measured in children less than 1 year of age.
    •     Children over 5 years have lung function measured on a standard spirometer.
    •     All children have oxygen saturation measured on a pulse oximeter.
    •     Urine is tested for glucose if the child has lost weight or if they are receiving oral steroids, in
          which case blood pressure is also measured.
    •     Sputum, cough suction or cough swabs are always collected for microbiology.

3.3 Annual Review

All patients are seen around the time of their birthday for a full clinical review of progress over the
last year, which takes place in the normal CF Clinic. The results and the whole clinical picture are
then reviewed by a consultant, who will decide on any management changes, or whether there are
any issues to be particularly vigilant for in the coming year. For those children from outside of the
Auckland area, who are seen 6 monthly to yearly, the review is carried out by the consultant when
possible. Occasionally, at the end of the annual review initial visit (done by the Registrar), the
consultant will come in to see them as well for a brief discussion. All families will receive the
Consultant’s opinions & summary of review by letter once all the results are known. Wherever
possible the annual chest XRay and blood testing will occur 2 weeks before the Annual Review
appointment to enable discussion around the results. The nurse specialists will contact families to
come to Starship for this to happen.

The children will be seen for the following:
    •     Discussion with and examination by one of the clinic doctors. This will include the number
          of IV and oral antibiotic courses, usual symptoms and microbiology. There will also be an
          emphasis on growth and puberty. The doctor will also ensure that the issue of fertility has
          been discussed with children at the appropriate age. The doctor will discuss results of lung
          function and chest x-ray (XRay scoring will be done by the Radiology Department). Blood is
          taken (if not taken before appointment)

    •     Discussion with CF nurse specialist and educational information given. Home nebuliser
          equipment, inhaled medications, Special Authority numbers and gastrostomy equipment
          reviewed. Electrical Equipment may require an annual service to be arranged. Visits to
          schools / kindergartens by nurse specialists to discuss CF can be arranged.




Author:            Drs Vyas, Byrne, Jaksic & Jan Tate               Service:             Paediatric CF Team
Editor:            Dr Raewyn Gavin                                  Date Issued:         August 2008
Cystic Fibrosis    Page:                                            6 of 100
                                             Starship Children’s Health Clinical Guideline
                    Note: The electronic version of this guideline is the version currently in use. Any printed version can
                          not be assumed to be current. Please remember to read our disclaimer.



                                             CYSTIC FIBROSIS
    •     Dietary assessment - including written evaluation of nutritional intake by the dietitian. Height
          & weight, growth velocity and BMI charts will be filled in. The relevant section on the A/R
          proforma is filled in.

    •     Physiotherapy review of airway clearance techniques, exercise and inhaled medication
          regimens. Exercise testing will not be routinely carried out for the moment.

    •     The following investigations are carried out (See Checklist, Appendix I) :

             o    Full lung function (including plethysmography) for children over 6 years.
                  Bronchodilator responsiveness will be carried out for specific patients only by
                  request. This is done in the Lung Function Laboratory.

             o    Chest x-ray is scored using Brasfield Scores (see Appendix II).

             o    Ultrasound liver and spleen. Liver ultrasound is recommended by the hepatologists
                  at 3, 6, 9years, and annually after 12 years of age. In those with previous U/S
                  abnormality, hepato-splenomegaly, or abnormal enzymes, liver U/S should be done
                  annually regardless of age

             o    Bone densitometry (DEXA scans) will be measured in patients considered to be at
                  increased risk of developing osteoporosis (section 10.3), and if normal repeated
                  every 2 years. These would include those who have frequent oral steroids
                  (particularly those with chronic ABPA), those on high dose inhaled corticosteroids,
                  diabetics and those with FEV1<50% predicted. Children who may be approaching a
                  time where referral for transplant assessment is appropriate, will need a DEXA scan
                  as part of their pre-transplant “work up”.

             o    Oral glucose tolerance tests are done at annual review in those 12 years and over,
                  or in whom there may be concern about poor nutritional status, deteriorating chest
                  symptoms, several (>3 courses) of prednisolone in the preceding year. It is not done
                  in those already known to have diabetes. See section 8.1.

             o    Sputum, cough suction or cough swab for microbiology including non-tuberculous
                  mycobacteria.


Blood is taken by the phlebotomist at the Laboratory, 3rd floor, Starship. For children under the age
of 5yrs a finger prick is taken for the following:
             •    Full blood count (with WBC differential)
             •    ESR
             •    Electrolytes and creatinine
             •    C-reactive protein
             •    Calcium, magnesium and phosphate
             •    Liver Function & Enzymes tests
             •    Random glucose and glycosylated Hb

Author:            Drs Vyas, Byrne, Jaksic & Jan Tate               Service:             Paediatric CF Team
Editor:            Dr Raewyn Gavin                                  Date Issued:         August 2008
Cystic Fibrosis    Page:                                            7 of 100
                                             Starship Children’s Health Clinical Guideline
                    Note: The electronic version of this guideline is the version currently in use. Any printed version can
                          not be assumed to be current. Please remember to read our disclaimer.



                                             CYSTIC FIBROSIS
             •    Vitamins A, D and E
             •    Total Protein & Albumin
             •    (Genotype if not known)

For children 5 years and over, a venepuncture is done at the Starship Laboratory for the following:
             •    Full blood count (with WBC differential)
             •    ESR
             •    Electrolytes and creatinine
             •    C-reactive protein
             •    Calcium, magnesium and phosphate
             •    Liver Function & Enzymes tests
             •    Random glucose and glycosylated Hb
             •    Vitamins A, D and E
             •    Vitamin K if clotting or LFTs previously abnormal
             •    Total Protein & Albumin
             •    Total IgE
             •    Aspergillus RAST (specific IgE)
             •    Aspergillus precipitins
             •    PR/INR
             •    (Genotype if not known)
Other tests are not done routinely on all children with CF, but on those who fit certain clinical
criteria:
    •     Patients over 10 years should have their Tanner pubertal status assessed as part of the
          annual review.
    •     Liver U/S are done at 3, 6, 9 and 12 years, and annually thereafter. They should also be
          done annually on a child with a previously abnormal U/S scan; with liver enzymes > x2
          normal; a palpable liver or spleen.
    •     Those with CFRD are recommended (by the UK CF trust) to have the following tests:
             o    Fasting lipids
             o    Blood pressure
             o    Urine microalbuminuria
             o    Retinopathy screening: slit lamp and digital photography
             o    Sensory and vibration sense assessment




Author:            Drs Vyas, Byrne, Jaksic & Jan Tate               Service:             Paediatric CF Team
Editor:            Dr Raewyn Gavin                                  Date Issued:         August 2008
Cystic Fibrosis    Page:                                            8 of 100
                                             Starship Children’s Health Clinical Guideline
                    Note: The electronic version of this guideline is the version currently in use. Any printed version can
                          not be assumed to be current. Please remember to read our disclaimer.



                                             CYSTIC FIBROSIS
    •     If there have been difficulties with nutrition in the past 12 months the following are
          recommended (if the were not already done:
             o    Faecal elastase or chymotrypsin (whether child pancreatic sufficient, or insufficient)
             o    Urinary sodium
             o    Coeliac antibodies
             o    OGTT
    •     Nephropathy screening is not mandatory, but may be prudent in children who fit the follwing
          clinical criteria:
             o    CFRD
             o    >2 course i/v aminoglycosides or polymixins in last 12 months [regardless of
                  whether drug levels OK or not]
             o    long term NSAID use

A letter is written by the doctor who saw the patient (Appendix III). One of the consultants
responsible for that clinic is given all the results and a letter is then sent to the GP, shared care
consultant and parents. For children over the age of 13, we send a copy letter to the patient as well
(see Transition). When the child is reviewed by the consultant in the next clinic, a letter is sent in
the usual way, highlighting the discussion over any changes made as a result of the annual review.

3.4 Transition to Adult Services and transfer to the Adult Clinic

Transition to adult care is discussed with all patients and their families from an early age. However,
more detailed discussion takes place from about 13 or 14 years onwards when the adolescent is
encouraged to take a greater controlling interest in their CF management. This is done via access
to information about CF and encouraging the patient to take the lead in decision-making around CF
issues. For any young person, this process is difficult and needs to occur at the adolescent’s own
pace. From 13 years of age the adolescent will be encouraged to see the doctor by themselves for
the first part of the appointment without their parents present. A variety of “adult issues” need to be
broached during this time, discussed frankly and possibly returned to on later occasions. It is
helpful to underscore these discussions with printed information as well. We use the CF Trust book
“Growing Older with CF”. This is written by young adults with CF and addresses issues such as
leaving home, going to university, working, relationships, sex, transplant, and dying. We encourage
the patient to read the book and come back to us with any questions they might have. We feel this
allows the patient the opportunity to process the information at their own pace. It can also allow
issues to be raised without the parents having to initiate discussions around difficult topics (which
some are very reluctant to do). Most patients will transfer at some stage after their 16th birthday,
depending on the individual and family circumstances. However we aim to transfer all young adults
by their 18th birthday.

The nurse specialists from the paediatric and adult CF service plan a transition “handover” meeting
twice a year to discuss patients transferring within the following 6 months. All members of both
teams are included, with the paediatric team presenting the patients clinical, social and family
history. The transfer clinic is the time when the nurse specialist from the adult team attends the
paediatric clinic and meets the patient and their family. Following the transfer clinic and at a time
suitable, the nurse specialists from both teams accompany the adolescent and family on an
orientation of the adult inpatient and outpatient facilities. During this “walk around” the adolescent
will meet members of the adult team and familiarise themselves with the adult hospital.

Author:            Drs Vyas, Byrne, Jaksic & Jan Tate               Service:             Paediatric CF Team
Editor:            Dr Raewyn Gavin                                  Date Issued:         August 2008
Cystic Fibrosis    Page:                                            9 of 100
                                            Starship Children’s Health Clinical Guideline
                    Note: The electronic version of this guideline is the version currently in use. Any printed version can
                          not be assumed to be current. Please remember to read our disclaimer.



                                            CYSTIC FIBROSIS

A transition document, detailing family, social and clinical history, (completed by the paediatric
nurse specialist) and transfer letter (written by the consultant) are given to the adult team in
preparation for their first clinic appointment with the adult CF Team (see Appendices IV and V).

3.5 CF Nurse Specialist

The CF Nurse Specialist is the key care co-ordinator for the patient and family and is their first
point of contact for CF related issues, referring appropriately to other members of the CF Team if
required. The CF specialist nurses have responsibilities to patients, families, and to the staff
involved in patient care.

The CF Nurse Specialist‘s responsibilities include:
    •     Advocacy for every patient
    •     Be up to date with current treatment practices
    •     Maintaining and teaching clinical skills and practice
    •     Professional development
    •     Support and advice
    •     Education, research and quality issues
    •     Patient and family liaisons
Nurse Specialists offer practical support with: intravenous therapy, venous access devices, oxygen
and nebuliser therapy and enteral tube feeding. Nurse Specialists coordinate care between patient
and family, community services and hospital; both practically and through support and advice.

Liaison occurs regularly with:
    •     Homecare Nursing Teams – Central, Waitemata and Kidzfirst.
    •     School nurses & teachers
    •     Nurse from adult CF service
    •     GPs & Practice Nurses
    •     Social workers
    •     Community physiotherapists & dietitians
    •     Psychology services




Author:           Drs Vyas, Byrne, Jaksic & Jan Tate                Service:             Paediatric CF Team
Editor:           Dr Raewyn Gavin                                   Date Issued:         August 2008
Cystic Fibrosis   Page:                                             10 of 100
                                             Starship Children’s Health Clinical Guideline
                    Note: The electronic version of this guideline is the version currently in use. Any printed version can
                          not be assumed to be current. Please remember to read our disclaimer.



                                             CYSTIC FIBROSIS

3.6 Consult Liaison Team

The Consult Liaison Team (CLT) members can meet with parents, children with CF, their siblings,
family, friends and other carers. We recognise that CF can affect a child and/or family in a variety
of ways. The CLT offer the opportunity to discuss any fears, anxieties or problems that can arise
when a child and family are living with CF. As well as talking and listening, clinical psychologists
can offer suggestions for change and practical ways of coping with difficult situations such as
managing blood tests. Any assessments and interventions carried out will be made sensitive to the
needs of the child and/or family and may include working confidentially if required.
Permission from parents will always be sought prior to a clinical psychologist formally introducing herself to a
child. Sometimes the psychologists will liaise with local services as long term follow up is often better carried
out locally nearer to the family's home. This is always agreed with the family first.


Reasons for referral or consultation include:
    •     Coping with a new diagnosis of CF
    •     Helping a child cooperate with medical treatments
    •     Understanding medical conditions & new CF complications, and considering future
          treatments
    •     Managing invasive procedures including fear of needles
    •     Feeding behaviour
    •     School problems
    •     Changes in behaviour/personality
    •     Considering transplantation
    •     Issues towards the end of life
    •     Any other worries/difficulties

3.7 Social work support

A Starship Social Worker is a member of the multidisciplinary team and will accept referrals and
self-referrals to work with children and families while on Ward 26B. The social worker's role is
primarily to undertake an assessment of the child and families need for support throughout
treatment and on discharge from hospital. This help is only available to families whilst a child is an
inpatient.

The Cystic Fibrosis Association in Auckland employs a Field Worker (Sally Caron) who is based in
the community and supports CF families both in the paediatric and adult settings. She is available
at Starship on clinic days and can be contacted at other times by cell phone by families or health
professionals. She liaises with the Nurse Specialists and gives advice around emotional and
financial support.

See Appendix IX For Financial Assistance Available for CF Families
“Outside & Inside” - Where to go for financial assistance” MAY, 2007


Author:            Drs Vyas, Byrne, Jaksic & Jan Tate               Service:             Paediatric CF Team
Editor:            Dr Raewyn Gavin                                  Date Issued:         August 2008
Cystic Fibrosis    Page:                                            11 of 100
                                             Starship Children’s Health Clinical Guideline
                    Note: The electronic version of this guideline is the version currently in use. Any printed version can
                          not be assumed to be current. Please remember to read our disclaimer.



                                             CYSTIC FIBROSIS

4. Admission to Hospital

There are several reasons why a child with cystic fibrosis may require to be admitted to hospital:
   • Any deterioration in clinical condition that fails to respond to out-patient measures e.g.
       chest exacerbation, DIOS, IDDM, nutritional intervention
   • Elective 3 monthly admissions for IV antibiotics (usually 2 weeks).
   • Elective operations e.g. portacath or gastrostomy insertion, ENT or dental operation.

4.1 Admitting the Child

History

On admission, the reason for hospital attendance must be identified (and documented clearly in
the notes). Clerking should then follow in the standardised way with emphasis on the following:-
    •     Respiratory system: cough, wheeze, sputum production (quantity, frequency, colour,
          consistency), haemoptysis, chest pain/tightness, dyspnoea, exercise tolerance.
    •     Gastrointestinal system: appetite, heartburn, funny taste in mouth, nausea, vomiting,
          frequency of bowel opening, quality of stool, abdominal pain, rectal bleeding, weight loss,
          calorie supplements, gastrostomy/NG tube feeds (amount, type, nights per week).
    •     Genito-urinary system: thirst, urinary frequency, polyuria, nocturia, incontinence
    •     ENT: nasal obstruction, epistaxis, rhinitis, sense of smell & taste.
    •     Neuromuscular: headache, paraesthesia, muscle weakness, joint pains, backache.
    •     Pain.
Past history of ABPA (if applicable) should be recorded with most recent IgE & Aspergillus RASTs,
together with maximum values in the past year for comparison. (see Appendix VI for ABPA Results
Sheet)
A full drug history including the types of inhaler used is mandatory. Inhaler technique must always
be checked. Drug doses are often recorded in the last clinic letter but do not rely on these. All drug
doses should be checked directly with the patient or their parents before recording and prescribing
them. This must include doses of inhaled medication – write inhaled steroids in mcg not number of
puffs. If a patient is on oral steroids, record the starting date and dose/kg/day.
Check whether there have been problems with aminoglycoside levels in the past.
Any allergies, particularly to drugs should be recorded both in the notes and on the drug chart.
Check it is also written on the front cover of the notes.
Nebulised antibiotics are usually discontinued when a child is receiving intravenous antibiotics, but
may continue depending on antibiotic choice.
A full social history should be taken paying particular attention to school attendance, housing, pets
and active/passive smoking




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Editor:            Dr Raewyn Gavin                                  Date Issued:         August 2008
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The Lung Function Summary Sheet is filled out (Appendix VII) with:
   • The most recent positive sputum culture result with full sensitivities. Certain bacteria like B
      cepacia and MRSA require specific action with regards to therapy and isolation from other
      CF patients.
   • Sputum samples are obtained at the beginning and after 1 week of the IV course
   • The most recent & patient’s best (within the last year) pulmonary function tests (FEV1,
      FVC) must be recorded.
   • Overnight oximetry from last admission
   • Choice of IV antibiotic for admission

Examination

Examination findings should be recorded in the standard way according to systems. Do not forget
the ENT system, particularly nasal polyps. Blood pressure is mandatory on all patients, with
particular attention paid to those on oral steroids. Check presence of glycosuria on all patients.
All children should have the following observations recorded:
     • Weight (kg & centiles) in light clothing, shoes removed. Children < 1 year of age are
         weighed naked
     • Height (cms & centiles).
     • Head circumference in <1 year olds.
     • Temperature, pulse and respiration
     • Oxygen saturation in air or oxygen (include O2 requirement).

4.2 Investigations

All children old enough will have pulmonary function tests (spirometry) performed following
admission. If the child has been admitted from clinic, these will already have been performed and
do not need repeating.

An overnight SaO2 is done on the first night of admission and if abnormal repeated during the last
week

All children require admission bloods and these can be taken when the PICC is inserted in theatre.
Blood tubes and forms are put into patient notes for this to happen. If the child is due an annual
review (usually around the time of their birthday) within the next 3 months, make sure all annual
review bloods are taken at the end of an admission. See section 3.2.

The list of blood tests (with the appropriate bottles) required on admission is given below-

      •   Full blood count (FBC) EDTA                  Use paediatric size blood tubes with
      •   CRP / ESR                                    separate red top tubes for the aspergillus
      •   Coagulation studies                          measurements
      •   Urea & electrolytes
      •   Liver function tests
      •   Calcium, magnesium, phosphate
      •   Glucose (preferably fasting)
      •   Total IgE
      •   Aspergillus RAST
      •   Aspergillus precipitins
Author:           Drs Vyas, Byrne, Jaksic & Jan Tate               Service:             Paediatric CF Team
Editor:           Dr Raewyn Gavin                                  Date Issued:         August 2008
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    •     A chest x-ray is only performed if clinically indicated e.g. to exclude pneumothorax.
    •     Sputum/cough swab must be sent to microbiology within 24 hours of admission.
    •     Nasopharyngeal aspirate for viral immunofluorescence is sometimes indicated (usually
          under 1 year old). Send sample on ice to Lab and must be done before 12 midday
    •     Urinalysis must be performed on admission especially if the child is on oral steroids or there
          is a recent history of weight loss

Further investigations during admission:
    •     Three times weekly weight – Monday, Wednesday, Friday
    •     Weekly spirometry.
    •     Daily BP and urinalysis if on oral steroids.
    •     Aminoglycoside levels 23 hours after 2nd dose (i.e. before 3rd dose), and if in desired
          range, repeat 1 week later. Peak & Trough if on 8hrly, trough only if once daily regimen.
    •     Twice weekly U + Es if on IV colomycin.
    •     Fortnightly WBC if on chloramphenicol, so not routinely required unless having >2 weeks
          course
    •     Weekly sputum / cough swab.
    •     Daily SaO2 unless initial one >95%.

4.3 Venous Access

All children will require venous access for administration of IV antibiotics. If they have a portacath
in-situ, the nursing staff will insert the portacath gripper needle and follow Starship Central Venous
Line management policy. Children without a Port will require a Peripherally Inserted Central
Catheter (PICC). At Starship, this is inserted in theatre by anaesthetist staff. Young children require
a light general anaesthetic and adolescents have the option of sedation and local anaesthetic. The
child will need to be booked onto the acute surgical theatre list, come into hospital nil by mouth 6
hours prior and be admitted to the ward before 0800 on the day of insertion.

Thrombophlebitis - there is some anecdotal evidence for the use of hydrocortisone in PICCs
complicated by thrombophlebitis. It is NOT suitable for blocked lines. It appears to be safe and can
be repeated as necessary. The steroid dose is minimal so there should not be any steroid adverse
effects. If it is going to work it will usually do so after 24 hours

1. Give IV antibiotics in the usual way.
2. Use 5 mg hydrocortisone made up to 5 mls (with 0.9% normal saline) into PICC line.
3. Leave in line until next dose of IV antibiotic.
4. Flush line prior to IV antibiotic in usual way.
5. Concurrently use 0.5% or 1 % hydrocortisone cream topically on arm (over erythematous area).




4.4 Procedural Distress




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Editor:           Dr Raewyn Gavin                                   Date Issued:         August 2008
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                                            CYSTIC FIBROSIS




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Editor:           Dr Raewyn Gavin                                  Date Issued:         August 2008
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4.5 Discharge

Families are given a discharge summary sheet before leaving Starship – these are written by the
house officer or Registrar and are a legal requirement at Starship. The Concerto system will be
alerted if not completed. The CF Registrar has to complete a discharge letter – this must be done if
the patient is from out of Auckland and sent to the referring Paediatrician. The following is recorded
in the discharge summary / letter at discharge (appendix VIII):
    •     Admission & discharge dates, days of IV course and Home IV days
    •     Reason for admission: acute or planned
    •     General conclusions about the admission
    •     Procedures during admission
    •     IV Antibiotics given
    •     Results awaited
    •     Weight on admission & discharge
    •     Spirometry results (FEV1, FVC) on admission & discharge
    •     Overnight SaO2
    •     Drugs on discharge (including any weaning)
    •     Plan for review - when / where
    •     Plan for tests necessary at home (e.g. WBC after 2 weeks if still on chloramphenicol)
    •     Date of next admission if elective (3 monthly IVABs)

4.6 Infection Control

There are increasing worries about cross-infection between children with CF and these dictate that
certain precautions need to be adhered to for all CF children. The concern is that some clinics in
USA, UK and Australia have reported epidemics of resistant organisms occur due to unchecked
mixing between patients. Segregation has been introduced in out patients (see below) and stricter
rules are now applied on the ward, school rooms etc to minimise contact between CF patients.
Although our ward staff will support and reinforce these measures, we will have to rely on the
parents/carers helping to ensure the children stick to the rules. Generally, personal hygiene is
emphasised and children are encouraged to put their hands in front of their mouths when
coughing. Hands should be washed regularly and patients must not share cups, cutlery etc with
other CF children. Specific recommendations are:

4.6.1 On the ward

    •     Each patient will not share a bedroom with another CF patient
    •     Children with CF should not enter any other CF child’s room.
    •     We separate children with CF and try to separate those with non-CF bronchiectasis
          although this is not always possible.
    •     We want to discourage waiting around in corridors on the ward.
    •     No sitting or waiting around the nurses station, including during the evenings.
    •     Disinfectant hand rub dispensers are inside each bedroom for use by staff, all children,
          families and visitors.
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Editor:            Dr Raewyn Gavin                                  Date Issued:         August 2008
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                                            CYSTIC FIBROSIS
    •     Doctors must clean stethoscopes between patients.
    •     There are 2 communal bathrooms on the ward – we try to separate the CF patients use but
          this may not be possible.
    •     Physiotherapy is carried out in the children’s own rooms only. When coughing up sputum,
          sputum pots with covers should be used, but if tissues are preferred, these should be
          disposed of immediately.
    •     When siblings (both with CF) are sharing the same room, physiotherapy must be carried
          out on the patient’s own bed without the other sibling present.
    •     Children infected with MRSA or Burholderia cepacia will stay inside their bedrooms for the
          whole admission, although may spend time off the ward.
    •     Patients with the different organisms in their sputum will not be looked after by the same
          nurse where possible.

4.6.2 Daily Plan

The daily plan is an integrated plan to be used by the whole multidisciplinary team to timetable in
appointments, investigations, treatments and school. By timetabling clinical activities, the risk of
cross infection can be reduced. The Nurse Specialist will fill out the plan each week. This will help
the children and family know what is planned for the admission and when they are able to go away
from the ward each day. The plan will be kept by the patient’s bed.

4.6.3 School Room

School is compulsory (by law) and children are enrolled at the Northern Hospital School when they
are admitted to Starship. The teachers can liaise with the child’s own school teacher for
schoolwork.
There will be one CF child in the schoolroom at any time.
They will also be provided with schoolwork from the teachers so they can continue with at their bed
space.

4.6.4 Play Room

The Play Specialist manages the playroom on the ward and she is available during working hours.
Rules for the playroom are similar to school rules.
There will be one CF child in the area only at any time. CF children will have access to the
playroom according to their daily plan.
The play specialist will arrange play sessions at the bedside at times when another CF child is
having their turn in the playroom.
The children will not be able to eat in there, but have meals at their bedside.

4.7 Specific Organisms

Particular care is necessary for children who are infected with:
   • Burkholderia cepacia complex organisms
   • MRSA
   • Multi-resistant organisms
   • Respiratory viruses e.g. RSV or Influenza
   • Atypical mycobacterium




Author:           Drs Vyas, Byrne, Jaksic & Jan Tate               Service:             Paediatric CF Team
Editor:           Dr Raewyn Gavin                                  Date Issued:         August 2008
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                                            CYSTIC FIBROSIS

The risk of transmission is related to the level of intimacy of contact. The child is put into a room
with private washing and toilet facilities. Items including toys and TVs should be kept in the room
and washed when taken out, before use by another child (this includes a stethoscope). Hands are
washed and rubbed with Sterigel before entering and leaving the room. Socialising with other
children is discouraged and visiting other children in their rooms or being visited by other patients
is not allowed. It is important not to stigmatise patients and the reasons for their relative isolation
must be carefully explained. It is also important that children with B cepacia realise that they do not
pose an infection risk for healthy school friends. Relatives of patients colonised with MRSA may
also carry the organism. Nasal swabs will confirm this. Bactroban (mupirocin) nasal ointment may
eliminate the organism but recolonisation frequently occurs. Staff who have nursed such patients
should also be screened (check with Infection Control Team).

CF children with the above organisms are seen at the end of the CF clinic and are seen last in the
Respiratory Laboratory. They sit in a clinic room away from the other CF patients and the staff go
and see the child in that room.




5. Making the Diagnosis

New Born Screening:

The NZ National Testing Laboratory has been screening all newborn infants for CF since mid-
1981. The National Testing Centre notifies the Lead Maternity Carer (LMC) and the local
nominated CF paediatrician. The LMC receives the notification letter and pamphlets about CF from
the National Testing centre, takes pamphlets to the family and then refers to the local CF
paediatrician. At Starship, the Nurse Specialist will liaise with the LMC, receive the referral from the
LMC, contact the family and organise two sweat tests, CF genetic mutations, faecal chymotrypsin
level and review with CF consultant. The time the family are notified by the LMC and the time the
testing is done should be no more than a few days to overcome parental anxiety. Results from the
sweat test are normally known in 24 hours. Regardless of outcome the parents are referred to
Genetic Services for counselling and parental testing.




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Editor:           Dr Raewyn Gavin                                  Date Issued:         August 2008
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For children missed on newborn screening, or those born outside New Zealand:

The history and/or examination will usually raise suspicions of the CF diagnosis. Common features
are recurrent respiratory infections and failure to thrive with steatorrhoea (but do not be fooled by
the thriving infant). Other features in a baby that mean CF must be excluded include meconium
ileus, rectal prolapse, salty tasting skin, prolonged obstructive jaundice, and unexplained
haemolytic anaemia with hypoalbuminaemia. Finger clubbing and nasal polyps in an older child are
also important, as is isolation of S aureus or P aeruginosa from the respiratory tract. Confirmatory
investigations are outlined below. If in any doubt, or if anyone at all (including the parents) is
worried about CF, do a sweat test.

5.1 Sweat testing

Sweat testing will reliably make the diagnosis in 98% of patients. Despite the availability of
genotyping (and because of its limitations) the majority of children in whom CF needs to be
excluded will undergo sweat testing. This group will include the following:
   • Child with suggestive history / symptoms/ examination.
   • Sibling of a known case (even if asymptomatic).
   • More distant relative of known case if clinical suspicion.

100mg of sweat is not essential. However, caution is advised in interpreting results with a raised
Cl- concentration if less than 100mg was obtained. The result of a sweat test must always be
discussed with a consultant before telling a family. Minimum of 20 minutes and maximum of 30
minutes is the recommended testing time. Sweat testing can be performed once a baby is > 48
hours old although often inadequate samples are obtained in the first few weeks. As with any of
these techniques, it is extremely important that they are performed by personnel who are
experienced. The sweat is analysed by the Biochemistry lab and results include sweat volume,


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Editor:           Dr Raewyn Gavin                                  Date Issued:         August 2008
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Na+ and Cl- levels. National UK Guidelines for sweat testing have been finalised and are available
on http://www.acb.org.uk/.

Results must be interpreted in the clinical context

          Result           Interpretation                              Comments

     -
  Cl <40mmol/l         normal range              if phenotype v suggestive of CF, obtain genotype

     -
  Cl 40-60 mmol/l      indeterminate             in infants it is more likely this reflects CF

     -
  Cl > 60mmol/l        consistent with CF        beware false positives (see below)


Recent data from Australia have led to a suggestion that there should be a decrease in the cut-off
for ‘normal’ values in screened infants from 40 to 30 mmol/l, as the latter is 4 standard deviations
above the mean (Farrell PM et al. Pediatrics. 1996;97:524–8). Although we have not adopted
these values, it would seem sensible to be cautious about ruling out the diagnosis in an infant with
a high index of suspicion and such intermediate sweat electrolyte values. Chloride is the primary
ion measured; sodium should not be measured alone. We do not measure conductivity and its use
is still under review. In normal health, sweat Na+ is usually higher than Cl-. This ratio is often (but
not always) reversed in CF. This may be helpful, but is certainly not diagnostic. Those with milder
CF (children who get to adolescence before the diagnosis is considered) may have a sweat [Na+]
> [Cl- ]. The diagnosis of CF should be made on the basis of 2 sweat test results not one. If there is
any doubt over a result, repeat the test or discuss it with a consultant. Flucloxacillin has no effect
on a sweat test result.

False negative results
Cases are increasingly recognised where the clinical picture of CF is supported by genotyping, but
in the presence of a normal sweat test (<1% CF patients). Beware therefore of excluding the
diagnosis (in highly suggestive cases) on the basis of a normal sweat test alone. Genetic testing
would be the appropriate next step (see below).

False positive results
Many theoretical causes are listed in textbooks, most of which do not appear to cause problems in
routine clinical practice. Those which may be encountered include malnutrition or skin disorders
such as severe dermatitis/eczema. Transient increases in sweat electrolytes have also been
reported in young patients with immunodeficiency states. A more common cause of a spuriously
raised sweat [Cl-] is due to evaporation of water form the sweat sample. This is usually due to
relative unfamiliarity with the technique. If the sweat weight is less than 100mg it would be prudent
not to put any significance to a raised [Cl-], and to repeat the test.

Fludrocortisone suppression test
In grey cases with repeat levels in the 40-60 mmol/l range, this test may help differentiate CF from
normal. Oral fludrocortisone is administered at a dose of 3 mg/m2, 48 and 24 hours before a repeat
sweat test. In normal subjects, the sweat electrolyte values will fall, but this does not happen if the
patient has CF. It should be a consultant decision to perform this test.


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Editor:            Dr Raewyn Gavin                                  Date Issued:         August 2008
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5.2 Genetic Analysis
There are currently at least 1200 mutations in the CFTR gene which are associated with the
clinical picture of CF. There are 5 different classes (I-V), with commonest in the Caucasian
population being a class II mutation, δF508.

Indications for genotyping include the following:
    •     Any child diagnosed with CF on sweat test.

    •     Facilitate screening for other family members.

    •     Allow prenatal diagnosis of future pregnancies.

    •     Siblings of affected children, or more distant relatives with symptoms suggestive of the
          disease. In the case of a newborn sibling, cord blood should be taken at the time of birth.

    •     To aid confirmation of diagnosis in case of borderline sweat test.

If the sibling of an affected child, has an entirely normal sweat test, and is clinically well, it is
debatable whether that child should be subjected to a genetic test for which they cannot give
informed consent. A child who is pre-pubertal will not be in a position to start a family, and so the
benefit to the child of knowing if they are a CF carrier or not (when they are pre-pubertal) is
unclear. CF carrier status can be determined very quickly and easily (via a blood test) once the
sibling gives informed consent. It is recognised that some parents may not understand the ethical
nuances of this, and a consensus for the final course of action may need to be reached negotiated
between the child’s consultant, and the parents.

Based on current knowledge, genotype analysis must not be used to guide prognosis in an
individual child, except rarely (and cautiously) in the case of mutations usually associated with
pancreatic sufficiency (e.g. R117H). Pancreatic status should be confirmed with a faecal elastase
or chymotrypsin in all cases. Although studies have shown a milder lung phenotype in certain
groups such as these, patients with typical, severe lung disease have also been described, hence
it is best not to prognosticate in individual cases. There can also be problems occasionally with a
genetic diagnosis of CF in a patient who is asymptomatic with no apparent CF phenotype.

Limitations of mutation analysis
Due to the large number of identified mutations, and the extreme rarity of many of these, it is only
practical to screen for a few on a routine basis. This is usually 5, 12 or 31 mutations, and therefore
failure to detect mutations does not exclude the diagnosis. The above is of particular importance in
a child of non-Caucasian origin. There is now a specific panel of mutations which are common in
the Asian community. It is therefore CRITICALLY IMPORTANT that in every case the child’s ethnic
origin is included on the request form so that the most likely mutations can be looked for. Extended
screens can be performed if specifically requested but are expensive




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Editor:           Dr Raewyn Gavin                                   Date Issued:         August 2008
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Antenatal Screening
Carrier parents contemplating another pregnancy should be referred for genetic counselling in
order to decide whether they would like antenatal screening (CVS, which can be performed around
10-12 weeks gestation or amniocentesis which is usually slightly later). Because of the
approximately 1% chance of miscarriage, this is thought by most to be appropriate only for those
parents who are considering termination of an affected fetus. On the basis of the limited number of
mutations screened for, some CF children will be, for example, δF508/-, meaning one detected and
one undetected allele. Failure to detect both mutations in the proband does not rule out the
possibility of antenatal or sibling diagnosis, as linkage analysis based on Restriction Fragment
Length Polymorphisms (RFLP) may be possible. Parental blood samples are required.

5.3 Other tests

These may support a diagnosis of CF.

Serum immunoreactive trypsin (IRT):
Only useful in about the first 6 weeks of life, 2-5x higher in neonates with CF. The test is unreliable
in neonates with meconium ileus after surgery. In general, a level >900 mcg/l means CF needs
excluding.

Stool elastase/chymotrypsin:
Low in CF with pancreatic insufficiency. Normal levels are expected by day 3 in term infants and by
2 weeks of age in those born less than 28 weeks gestation, so tests should not be performed
before this time. See section 8 on pancreatic enzyme replacement therapy.

Nasal potential difference:
At present this test is not available in New Zealand.
Difficult in small children as requires co-operation, but may be useful in older indeterminate cases
(over 8-10 years). Can be done easily on young children whilst under general anaesthetic, e.g. for
bronchoscopy. Unusual to get useful readings if nasal polyps or previous nasal surgery, and
should be postponed if the child has a cold. It is a difficult and time-consuming investigation and
will therefore usually only be done once all other CF investigations complete.

5.4 Inpatient investigations for the newly diagnosed patient

There are no “routine” inpatient tests for a newly diagnosed child. Some children may require
admission at the time of their diagnosis to initiate acute treatments, or for further testing. The need
to do this is determined on the basis of the child’s clinical picture.

Newly diagnosed children are seen weekly in the CF clinic. As time goes on, and the child is
observed to be well, and the family are coming to terms with the fact their child has CF and the
treatments we have then started, so the interval can be extended. It is generally the case that a
child is seen weekly for the first month after diagnosis and then fortnightly for another month or so.
The eventual interval between clinic appointments should never be greater than 3 monthly, expect
in the most unusual circumstances.




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Editor:           Dr Raewyn Gavin                                  Date Issued:         August 2008
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6. Respiratory Care
6.1 Respiratory Exacerbations

If the family is worried they will usually phone the CF nurse specialist or the ward out of hours.
Sometimes telephone advice can be given (by nurse specialist or senior doctor only) but often the
patient will need to be seen. Options are to be seen in the next clinic, Day Stay Unit or Children’s
Emergency Department.

Some indications of respiratory exacerbation are:
    •     Changes in sputum production (volume, colour, consistency).
    •     Haemoptysis.
    •     Increased cough.
    •     Increased dyspnoea.
    •     Chest pain or tightness.
    •     Malaise, fatigue and lethargy.
    •     Fever > 38º C. Note that most CF chest exacerbations are not accompanied by fever.
    •     Loss of appetite or weight loss.
    •     Drop in FEV1 or FVC >10% from previous recording.
    •     Changes in chest sounds on auscultation (crackles, wheeze).

NOTE: clear chest on auscultation does not exclude an infective exacerbation and crackles and
wheeze in isolation do not confirm, if that is usual for the child.

If the situation is dealt with over the telephone, it is essential that the CF nurse specialist is
informed; so appropriate follow up can be arranged. It is important to send sputum or a cough
swab to microbiology (this can be done at Starship or in the community – a Repeat Card can be
set up at the community Laboratory); an NPA may be performed in infants. A chest x-ray is only
occasionally useful. Oral antibiotics should be prescribed. If IV required, discuss with consultant on
call. Ensure appropriate documentation in e-note (in CRIS Notes), CED record or clinical record.

6.2 Microbiology

6.2.1 Cultures

Sputum cultures should be done regularly to monitor background flora, and at times of patient
being clinical unwell. It is possible to acquire a new organism (including Pseudomonas) without
there being an obvious change in clinical wellbeing. Knowledge of current sputum flora will
influence treatment. Similarly, knowledge of when a child stops growing an organism may also
influence long term treatments (e.g. stopping nebulised antibiotics).




Author:           Drs Vyas, Byrne, Jaksic & Jan Tate                Service:             Paediatric CF Team
Editor:           Dr Raewyn Gavin                                   Date Issued:         August 2008
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                                             CYSTIC FIBROSIS

    •     Surveillance Cultures

Cough swabs/sputum samples should be taken at the very least every visit. For those whose CF is
more severe, interval samples may also be helpful. Treatment may be indicated, as discussed
below. Culture for non-tuberculous mycobacteria on annual assessment visit on sputum
(preferably) or cough swabs. Remember to write ‘Cystic Fibrosis’ as the diagnosis so the
laboratory can also look for Pseudomonas and Burkholderia species.

    •     Viral Infections

Viral Infections/Colds at home or in the clinic – with no or minor chest symptoms (i.e. not major
exacerbation). It is not necessary to treat a viral infection with antibiotics. However, it can often be
difficult to distinguish a viral infection from a bacterial one; especially in the early stages. It may be
appropriate to treat a child with antibiotics, in spite of a clinical diagnosis of a viral infection.
Circumstances where this may be necessary include if influenza is suspected (risk of secondary
bacterial infection), in a child with severe CF, or if there has been contact with a known bacterial
pathogen (if a sibling with CF has grown the pathogen in their most recent sputum). Always inform
the CF nurse specialist to arrange at least telephone follow up or GP as appropriate. It is
particularly important that this happens for ‘out of hours’ calls. Discuss with Consultant if
concerned.

    •     Positive surveillance cultures

If the child is well and asymptomatic but has a positive routine swab for S aureus or H influenzae
treatment will still be considered. The decision not to treat MUST be discussed with the Consultant.

A new isolate of P aeruginosa in a child not on treatment for this organism is always treated.
However, if Pseudomonas is cultured and the child is known to be chronically infected (& on
nebulised antibiotics), but is well, it may well be correct to offer no additional treatment.

If the child is symptomatic, then the positive culture will guide choice of antibiotic treatment.

Isolations of P aeruginosa after six months or more of clear cultures are always treated. It is worth
trying ‘re-eradication’ although likely the organism will persist.
    •     1st line is oral ciprofloxacin for 3 weeks & nebulised aminoglycoside for 3 months.
    •     If unwell, a 2-week course of intravenous antibiotics should be offered.

It is important to arrange a follow up culture at the end of the course.

Note that if the patient is still symptomatic or has a positive culture after an appropriate course of
antibiotics, admission should be discussed with a consultant. After the use of more than two
successive courses of oral antibiotics for the same exacerbation the patient must be discussed
with the consultant. This is a different situation from the child who gets completely better and a few
weeks later has a 2nd oral course, from which they get better again.




Author:            Drs Vyas, Byrne, Jaksic & Jan Tate               Service:             Paediatric CF Team
Editor:            Dr Raewyn Gavin                                  Date Issued:         August 2008
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                                            CYSTIC FIBROSIS

6.2.2 Specific organisms

    • Haemophilus influenzae
Treatment dose augmentin for 1 month. One further course of a cephalosporin or macrolide can be
given if no eradication / persistent symptoms.

    • Staphylococcus aureus
NB: We do not advocate anti-Staphylococcus prophylaxis at this time but this policy will be
reviewed during 2007
The question of staphylococcal prophylaxis is based on a few studies only and evidence for benefit
is weak. Our current policy is to not use anti-Staph treatment in this way.

Treatment dose of flucloxacillin can go as high as 2g bd in older children (consultant decision). For
those repeatedly culturing Staph despite regular high dose flucloxacillin, consider other treatments,
especially in older children. For example augmentin, azithromycin, fusidic acid or even rifampicin if
this persists. MRSA is increasingly common. There may be some antibiotic sensitivities to guide
you but in resistant cases, consider linezolid. This is a consultant decision, and we would usually
involve the consultant microbiologist as well.

    • Pseudomonas aeruginosa
1st isolation – 3 weeks oral ciprofloxacin (or dual therapy intravenous antibiotics if unwell) plus 3
months nebulised aminoglycoside (Gentamycin, Tobramycin or Colistin - in that order) twice daily.

Once Pseudomonas is present in repeated cultures regular (i.e. daily) nebulised treatment should
be instigated. There is some evidence that in those who regularly grow Pseudomonas, three
monthly intravenous antibiotics will slow the decline in lung function.
NOTE: if unusual organism is present, particularly Pseudomonas resistant to Colistin, sample to be
sent to ESR for confirmation, as a high index of suspicion for Burkholderia cepacia is needed. All
positive Burkholderia cepacia cultures should be sent to the Microbiology Laboratory in Brisbane
for genotyping of the Burkholderia–like organism.

    • Stenotrophomonas maltophilia
This can clear spontaneously and may not be pathogenic. However if symptomatic, treat with an
oral antibiotic, Cotrimoxazole may be the best option, in light of its typical resistance pattern.

   • Burkholderia cepacia
This must be discussed with the CF consultant and will depend on sensitivities. Patients who
become infected with B cepacia need to be notified to the CF Nurse Specialist for isolation in clinic,
they need to do lung function testing last in the CF clinic, and need to be isolated on the ward
when an inpatient. Stop Colistin nebuliser.

    • Multi Resistant Organism
Get extended sensitivities and start long term oral antibiotic to which it is sensitive, e.g.
trimethoprim, minocycline, doxycycline (if appropriate age). Choice of IV therapy should be
discussed with CF consultant.




Author:           Drs Vyas, Byrne, Jaksic & Jan Tate               Service:             Paediatric CF Team
Editor:           Dr Raewyn Gavin                                  Date Issued:         August 2008
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                                            CYSTIC FIBROSIS

    • 6.2.2.7 Non-tuberculous mycobacteria (NTM)
This includes a large number of species and the commonest to affect the lungs are M avium
complex and M abscessus; others found include M kansasii, M xenopi, and M malmoense. When
grown in the sputum of children with CF, they are usually there as commensals and usually have
no significant effect on respiratory function or nutritional status. A single isolate is NEVER treated,
and even a child with multiple isolates has a 50% chance of not being infected. Occasionally
treatment is required (CF & ID consultant decision). Antibiotic sensitivity testing is critical.
Treatment necessitates 3 drugs for 12-18 months from the following:
rifampicin or rifabutin
clarithromycin
ethambutol
ciprofloxacin

Regimen may be decided after discussion with a Consultant in Paediatric Infectious Disease. (BTS
guidelines for treatment (mostly adult-based) are in Thorax 2000;55:210-218). Note there is never
any rush to initiate treatment for NTM. A CT scan may help the decision and should be carried out
with repeated positive cultures (nodules in parenchyma indicate likely infection).

    • 6.2.2.8 Candida
When grown in sputum it is almost inevitably from the mouth itself. Local treatment will be given if
the child is symptomatic i.e. sore mouth, visible white plaques.

   • 6.2.2.9 Influenza
Annual influenza vaccine is recommended for all CF patients.

6.2.3 Choice of intravenous antibiotics.

This will depend on previous sputum results.
No previous P aeruginosa - must cover common pathogens including S aureus, H influenzae,
Moraxella catarrhalis as well as possible first isolate of P aeruginosa (especially young infants).
Start with cefuroxime + / - flucloxacillin

Chronic infection with P aeruginosa – ceftazidime & tobramycin is 1st line unless previous
sensitivities suggest otherwise. We routinely add oral flucloxacillin if S aureus is isolated within the
last year. Flucloxacillin is usually given orally as it causes problems with IV lines and may cause
backache.

When to change antibiotics – there is no evidence that in vitro sensitivities correlate with in vivo
outcome. Therefore, if the child is improving on ‘best guess’ antibiotics, but the Pseudomonas
comes back ‘resistant’, DO NOT CHANGE drugs without first discussing with the CF consultant. If
the child is not responding, a change may be indicated whatever the sensitivities – again, discuss
with the consultant.

MRSA - Discuss with CF consultant and ID consultant.




Author:           Drs Vyas, Byrne, Jaksic & Jan Tate               Service:             Paediatric CF Team
Editor:           Dr Raewyn Gavin                                  Date Issued:         August 2008
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                                               CYSTIC FIBROSIS
6.2.3.1 Aminoglycosides


We have changed our practice so that tobramycin is now our 1st line aminoglycoside (replacing
gentamicin), assuming the organisms are not resistant to it. This is based on its superior MIC and
data suggesting that P aeruginosa is more often resistant to gentamicin than tobramycin.

Aminoglycosides can be delivered once daily or three times daily. There is evidence that once-
daily dosing of aminoglycosides is less toxic and results in more effective bacterial killing than
conventional three-times daily dosing. There is also evidence that the incidence of P aeruginosa
resistance to aminoglycosides may decrease with once daily rather than three-times daily
administration. In addition the child need less blood testing as only a trough level is required.

However once daily dosing requires the child to have an infusion pump or syringe driver for
administration, so for Home IVs we currently continue to use three times a day dosing via a “push”
(bolus).

Aminoglycoside regimen
                                            10-12 mg/kg once daily over 30 minutes
        Tobramycin
                                               10mg/kg/day in three divided doses


                                            10-12 mg/kg once daily over 30 minutes
        Gentamycin
                                               10mg/kg/day in three divided doses


          Amikacin                            30 mg/kg once daily over 30 minutes


NB: The aminoglycoside should be administered in the morning or early afternoon because there is
a circadian variation in renal toxicity, and also so blood levels can be done at social hours.

We would suggest that you start with the dose that the patient had last time and pay particular
attention if there has been a high trough level. If there has, the dose should be reduced by 20%
from the outset, and ensure the renal function is measured alongside any trough doses.

Measurement of trough levels

Once Daily Dose

    •     Serum aminoglycoside levels should be measured 23 hours after administration of the
          second dose (i.e. shortly before 3rd dose), 24 hours after any adjustment and weekly
          thereafter.
    •     Levels should NEVER be taken through the same line that the antibiotic was given and that
          includes Portacath / PICCs
    •     Label blood form – “TROUGH”


Author:              Drs Vyas, Byrne, Jaksic & Jan Tate               Service:             Paediatric CF Team
Editor:              Dr Raewyn Gavin                                  Date Issued:         August 2008
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                                             CYSTIC FIBROSIS
    •     Aim for trough < 1mg/l for gentamicin and tobramycin, and trough < 3mg/l for amikacin. The
          result must be written on the “Lung Function Summary Sheet” (green summary sheet)
    •     The fourth dose will not be given unless the levels are known.
    •     If the trough is >1mg/l (or >3mg/l for amikacin) omit the next dose and check the trough
          level 24 hours after the omitted dose. Only once the trough level has fallen to below 1mg/l
          (3mg/l amikacin) can the patient be re-dosed, reducing the dose by 20%, and the trough
          level re-checked after 24 hours. Wait for this level to come back and only continue if level is
          <1mg/l (<3mg/l amikacin).
    •     Peak levels are not done routinely but may be taken if there is concern about clinical
          progress on a reduced dose. This should be taken 30 minutes after the end of the infusion.
          Aim 5-10 mg/l for gentamicin or tobramycin.

    •     Each time levels are done, document in the notes:
             o    Date/time blood taken
             o    Dosage regimen
             o    Results
             o    Any change to dosage
             o    Any other action taken


Thrice Daily dose
    •     Measure trough and peak on the 3rd dose – the trough immediately before and the peak 30
          minutes after the dose given – specify the time interval between dose and blood taken.

    •     Levels should NEVER be taken through the same line that the antibiotic was given and that
          includes Portacaths / PICCs

    •     Label blood form – “TROUGH” or “PEAK”

    •     Aim for trough < 1mg/l for gentamicin and tobramycin, and trough < 3mg/l for amikacin. The
          result must be written on the “Lung Function Summary Sheet” (green summary sheet)

    •     The fourth dose will not be given unless the levels are known.

    •     If the trough is >1mg/l (or >3mg/l for amikacin) omit the next dose and check the trough
          level 24 hours after the omitted dose. Only once the trough level has fallen to below 1mg/l
          (3mg/l amikacin) can the patient be re-dosed, reducing the dose by 20%, and the trough
          level re-checked after 24 hours. Wait for this level to come back and only continue if level is
          <1mg/l (<3mg/l amikacin).

    •     Peak levels are not done routinely but may be taken if there is concern about clinical
          progress on a reduced dose. This should be taken 30 minutes after the end of the infusion.
          Aim 5-10 mg/l for gentamicin or tobramycin.

    •     Each time levels are done, document in the notes:

Author:            Drs Vyas, Byrne, Jaksic & Jan Tate               Service:             Paediatric CF Team
Editor:            Dr Raewyn Gavin                                  Date Issued:         August 2008
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                                             CYSTIC FIBROSIS
             o    Date/time blood taken
             o    Dosage regimen
             o    Results
             o    Any change to dosage
             o    Any other action taken

NB: Once steady levels are achieved, repeat weekly

    • 6.2.3.2 IV Colistin.
Occasionally we have used twice daily IV Colistin (colomycin) for children. This is a consultant
decision based on severity illness of the child, reponse to treatment, sensitivity profile. See
formulary for the dose - the usual total daily dose divided into 2 doses.

     • 6.2.3.3 Dosage.
In general, high doses are required because of high renal clearance and also to ensure high levels
of tissue and sputum penetration. Use the serious infection doses, and round up not down. The
aim of therapy is to push antibiotic doses to the upper therapeutic range.

When results of sputum culture are available, confirm that all organisms are covered by the chosen
regime. However, if the child is improving clinically on antibiotics to which the organisms exhibit in
vitro resistance, do not automatically change them (but discuss with consultant).

6.2.4 Allergic Reactions to Antibiotics

Recent studies would suggest that allergy in CF is more common than previously appreciated; but
that documentation of drug reactions is generally poor.

In acute reactions, stop the infusion & give:
    •     IM adrenaline (0.01 ml/kg of 1 in 1000, max 0.5 ml – doses repeated if necessary at 5
          minute intervals according to blood pressure, pulse and respiratory function).
    •     IV chlorpheniramine (2.5-10mg IV, continued orally at usual doses for 24-48 hours to
          prevent relapse).
    •     Oral loratadine or promethazine may be useful for itch and angioedema.
    •     IV hydrocortisone (4-8mg/kg, max 300mg).
    •     Monitor BP/HR/SaO2/RR.
    •     Listen to the chest.
    •     Consider giving oxygen and a plasma expander.
    •     Document event clearly in the notes.
    •     Inform consultant.
    •     Make sure child and family know which is the offending antibiotic




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Editor:            Dr Raewyn Gavin                                  Date Issued:         August 2008
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                                            CYSTIC FIBROSIS

The majority of allergic reactions are ‘late onset’ occurring many days after the antibiotic course
starts; rather than a more immediate allergic reaction, which can take place within minutes of
taking a drug. The late reactions may present in a variety of ways, often with non-specific features,
including rashes, unexplained fevers, nausea, vomiting, diarrhoea, joint pain, muscle pain,
lethargy, abnormal liver function results and abnormal haematological results. Management of
these reactions is essentially to recognize them early and to stop the relevant drug, if it can be
worked out which drug is causing the reaction. Improvement in symptoms should be seen within a
few days.
Antibiotic desensitisation may be considered if the child has multiple antibiotic allergies. This can
be undertaken with incremental introduction of the antibiotic at low dose, usually with prior
treatment with systemic corticosteroids and antihistamines.

Piperacillin/tazobactam (Tazocin, piptazobactam) is rarely used because there is a high incidence
of allergy, and because of cross reactivity, patients may become hypersensitive to other
antipseudomonal penicillins. It has also been recorded to cause reversible bone marrow
suppression – thrombocytopaenia, neutropaenia.

6.2.5 Home I/V Antibiotics

Home IVs are convenient for families of children receiving planned, regular antibiotics, for their CF.
Children who are admitted for an acute (i.e. unplanned) course of IVs are probably not well enough
to be at home on IV treatment.

Home IVs are not suitable for all children and families. Likewise families do not have a right to
receive them. The decision to sanction them needs to be made when the CF team is confident the
family will be able to administer the home IVs safely and reliably.

Parents must not be pressurised into undertaking them (even if the child is anxious to go home).

Parents must be competent and confident to undertake the task. They should be signed off as
competent by a designated member of staff before going home.

Parents must be able to continue with other aspects of the treatment i.e. extra physiotherapy and
attention to diet.

Lack of bed space is not an indication for Home IV antibiotics.

The first 5 – 7 days of the IV course should be given in hospital to allow child to have intensive
physiotherapy and nutrition review, also to allow parents to learn how to give IV antibiotics and
watch for potential problems.

The CF Nurse Specialist and ward nurses will train the family following the Home IV Programme
Booklet – “Home Care of Central Venous Catheters.”




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Editor:           Dr Raewyn Gavin                                  Date Issued:         August 2008
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                                             CYSTIC FIBROSIS

Parents/carers to be trained in the following:
    •     IV line - to look for leaks and signs of infection / thrombosis.
    •     Allergic reactions - what to look for and to stop drug immediately and seek medical advice.
          Access to transport and ambulance if needed.
    •     Adhere to timetable for drug administration (esp. aminoglycosides).
    •     Care in giving antibiotics as bolus dose with correct amount of Normal Saline flush in
          between doses using sterile procedures.

The CFNS to be responsible for:
    •     Familiarising family with booklet “Home Care of Central Venous Catheters” , ensuring
          contact phone numbers, antibiotic doses and times stated.
    •     Assessment of the parent / carer for competency before discharge home.
    •     Completion of Home IV Training record form (Bar Code Number: CR3784) - to be signed by
          CFNS and parent/carer and placed in the patient’s clinical notes.
    •     Sending referral to Homecare Team for: ongoing supplies of equipment support to family,
          change of luer plug after 3 days and maybe dressing change if needed.
    •     Ordering antibiotics, water for injection, Normal Saline 0.9% (10ml vials) and heparinised
          saline (50units/5mls) from Retail Pharmacy at Auckland City Hospital using SO12 and
          script – send originals. Drugs to be ordered day before intended discharge to ensure
          pharmacy has time to make up order.
    •     Give family enough equipment for the first 2 days.
    •     Arrange for weekly review in OPD – to include lung function (book time with lung function
          physiologists), weight, history, weekly aminoglycoside levels (providing previous level
          satisfactory), overnight oximetry at end of course if needed.
    •     Completion of Lung Function Summary Sheet at end of IV course.


6.2.6 Portacath (Totally Implantable Venous Access Device; TIVAD)

     • Indications:
Recurrent problems with venous access in the setting of need for recurrent courses of IV
antibiotics. It is not a solution for needle phobia because needle insertion is still required monthly
for flushing. Careful discussion of the advantages and disadvantages with the child and family is
essential prior to insertion.

     • Site of insertion:
Usually via a subclavian vein into the SVC. The final decision should be left to the surgeon who
will take a variety of factors into consideration (gender, pubertal status, previous Ports, etc). If the
child has had previous central neck lines, imaging of the neck (doppler ultrasound or MR
venogram) may be required to identify a suitable site for insertion. Portacath longevity is best
achieved by a surgeon who regularly inserts them. It is rare for a decision to insert a Portacath to
have to be taken as an emergency.


Author:            Drs Vyas, Byrne, Jaksic & Jan Tate               Service:             Paediatric CF Team
Editor:            Dr Raewyn Gavin                                  Date Issued:         August 2008
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                                            CYSTIC FIBROSIS

    • Protocol for insertion:
Surgeons will take Consent. Investigations: CXR, full blood count, coagulation including
thrombophilia screen, U&E, group & save. If the thrombophilia screen is abnormal, discuss with
Paediatric Consultant and Haematologist.

    • Post insertion:
Chest x-ray done for line position and pneumothorax – this is usually done in theatre.
Analgesia - Regular paracetamol 20mg/kg (max 1 gram) 6 hourly +/- Ibuprofen 5mg/kg (max
400mg) 8 hourly or Diclofenac 1mg/kg (max 50mg) 8 hourly. Be wary of using ibuprofen when
patients are taking aminoglycosides.
Opiate analgesics may be used during the first day, and a laxative maybe needed at the same
time. If further concerns exist about adequate pain control, discuss with Pain Team.

Physiotherapy and early mobilisation are important.

Antibiotics continued for a minimum of 48 hours post-procedure, and until patient is pain free and
back to usual respiratory status.

Portacath may be used from the time of insertion and the needle should be left in by the surgeons.

Usually dissolvable sutures are used - check before patient goes home.

Heparinised Saline (100units/ml) placed in line using Positive Pressure Luer plug before de-
accessing the needle. This dose is sometimes called “Strong Heparin”


Subsequent management:
   • This is the responsibility of CFNS. Monthly accessing of Portacath using Gripper needle
      and flushing with 3mls of heparinised saline (100 units/ml) using sterile technique
   • Local anaesthetic cream is used
   • Gripper needle only to be used
   • Always use a sterile technique, following Starship Central Line Guidelines.
   • Only to be accessed by experienced nurses.
   • Always use Positive Pressure luer plug and use “push / pause” method of flushing line.

    •     6.2.6.1 Complications:

Blockage
Consider Urokinase 5,000 units in 3ml 0.9% saline instilled into port. Use with caution if there is a
history of bleeding or significant haemoptysis. Try flushing port gently after 2-3 hours. If there is
concern over line blockage time is of the essence to prevent thrombus. We would encourage
discussion with the CF Team or Ward 26B.

Port leak
May occur if a forceful flush is attempted when the line is blocked or if the wrong type of needle is
repeatedly used damaging the diaphragm. Diagnosis is with a contrast venogram




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Editor:           Dr Raewyn Gavin                                  Date Issued:         August 2008
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                                            CYSTIC FIBROSIS

Local infection around the port
Clean area, if device is visible then it needs removing but if the inflammation is superficial then
treat with systemic antibiotics after swabs and blood cultures have been taken. Antibiotics should
be administered via another line.

Line infection
This usually demands surgical removal. After cultures have been taken, systemic antibiotics via
another route and possibly injecting vancomycin or teicoplanin into the system may work.
Thrombus may form which may lead to septic pulmonary emboli. Blood cultures and an
echocardiogram may help the diagnosis and sometimes radio-opaque contrast can collect in a
thrombus if injected down the line. Beware of injecting into a line that may have thrombus around it
- you may cause pulmonary embolism, so think first and be careful. Consider anti-coagulation.

Catheter fracture ± embolisation
Fragments should be retrieved at cardiac catheterisation. Refer immediately to on-call consultant in
paediatric cardiology. Remember that one of the commonest causes of pulmonary emboli in
children is an endovascular foreign body. In a CF child with pleuritic pain and/or breathlessness
and/or haemoptysis at least consider this diagnosis. V:Q scanning is a waste of time (the pre-
existing bronchitis/bronchiectasis make it impossible to distinguish acute from chronic V/Q
mismatch. Consider spiral CT with contrast or even angiography if this is a real possibility.

Tinnitus
At the time of antibiotic administration may indicate line migration into the neck veins passing
cranially.


6.3 Use of steroids

Indications for oral steroids:
(Long term steroid use must be discussed with consultants prior to commencement)
    •     Allergic bronchopulmonary aspergillosis (ABPA).

    •     Severe intractable bronchospasm / severe small airways disease.

Long term use as an anti- inflammatory agent is contraindicated in most cases due to the adverse
risk-benefit ratio.

We tend to use prednisone which must not be enteric–coated (specify on prescription) otherwise
absorption is poor in CF (i.e. use white, not pink tablets).

Dexamethasone may also be used and anecdotally may be better for those whose
behaviour/mood is adversely affected by prednisone (NB prednisone 5 mg = dexamethasone 0.75
mg). Dose regimen for ABPA is in section 6.7.

For severe bronchospasm, dose is 2 mg/kg prednisone administered in the morning after food,
which will be reduced as soon as possible, depending on the response.




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Editor:           Dr Raewyn Gavin                                  Date Issued:         August 2008
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                                            CYSTIC FIBROSIS
Attention must be paid to potential adverse effects, particularly glucose intolerance as sometimes
overt CF-related diabetes is precipitated. Patients must be told to report polyuria & polydypsia.
Regular urinalysis for glycosuria is important, particularly in older children.

Other problems are growth failure and hypertension (measure BP in clinic), less commonly oral
candidiasis, cataracts and osteoporosis.

Those on long term steroids will have DEXA scans at annual review – more than a total of 3
months in the last 12 months. Review at Annual Review time.

Exposure to chicken-pox in a child, who has not yet had it, may require varicella-zoster
immunoglobulin (see section on immunisations).

If there is a possibility of aspergillus exposure (in those not being treated for ABPA), Itraconazole
will usually be given to those on long term oral steroids.

Terminal care – steroids may act as general ‘tonic’.

6.3.1 Indications for inhaled steroids

Symptomatic wheezing that require regular bronchodilators, in a similar regimen to local asthma
guidelines. Acute bronchodilator reversibility should be documented. Long term use as an anti-
inflammatory agent in an asymptomatic child is not indicated.

We use fluticasone or budesonide Devices used depend on the age of the child, but nebulised
steroids are rarely used. In older children, at low or moderate doses (<400 mcg/day budesonide,
<200 mcg/day fluticasone) dry-powder inhalers (turbuhaler or accuhaler) are most suitable. Higher
doses of inhaled steroids should be given via a spacer device to reduce mouth deposition and
potential systemic side effects.

However there will be some older children for whom a spacer is unacceptable and then a DPI
should be used. Use of a standard metered dose inhaler alone is inadequate. Children taking
inhaled steroids should rinse their mouth out following inhalation. Side effects include slowing of
growth although the destined final height is usually unaffected. Inadequate CF treatment will also
slow growth. Oral candidiasis and rarely a hoarse voice can also occur.

Always consider whether the dose can be reduced, or indeed stopped whenever the child is seen
in clinic.

Children with wheezing that does not respond to inhaled steroid prophylaxis should be started on a
twice daily long-acting 2-agonist. Use either salmeterol (25-50 mcg bd via accuhaler or
MDI/volumatic) or eformoterol (6-12 mcg bd via turbohaler). The patient must be taking an inhaled
steroid as well.


6.4 Use of rhDNase (Pulmozyme)

rhDNase (Pulmozyme) is a synthetic enzyme that cleaves neutrophil derived DNA in sputum to
reduce viscosity thus aids sputum removal. Cohort studies demonstrate an average 5-8% overall
improvement in FEV1. However response in individual patients may vary from deterioration to
marked improvement (20%+).
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Editor:           Dr Raewyn Gavin                                  Date Issued:         August 2008
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                                             CYSTIC FIBROSIS

Indications:
Traditionally an FEV1 persistently <70% predicted after optimisation of other treatments in a
sputum producing patient.

Prior to commencing rhDNase:

    •     Check compliance, especially with physiotherapy; easy to say, difficult to do.
    •     Exclude new infections e.g. P aeruginosa, B cepacia, S maltophilia, Aspergillus etc.
    •     Exclude or treat allergic bronchopulmonary aspergillosis.
    •     Ensure that nutrition is adequate, and there is no CFRD / impared glucose tolerance.
Other indications may include:

    •     Difficulty in expectorating sputum because of a perceived severe ‘stickiness’.
    •     Children who hardly expectorate at all but have symptoms.
    •     Persistent wheezing.
    •     Persistent or recurrent focal x-ray changes e.g. consolidation in a lobe or part thereof
          (consider bronchoscopy with instillation under direct vision – see section 6.11).
There is some evidence for prophylactic benefit as a trial of use in 6-10 year olds with near normal
lung function showed a reduction in exacerbation rate and a halt in deterioration of lung function. A
recent study showed no clinical difference in those receiving the drug daily or on alternate days.

We have used Pulmozyme in other settings and would be willing to discuss this as an option in any
patient particularly those with:

    •     Resistant mucous plugging in hospital patients.
    •     Persisting lobar collapse (can be used during bronchoscopy).
    •     Severe respiratory disease maximal medical treatment, not improving.
Dose
Trade name: Pulmozyme 2.5mg / 2.5mls by appropriate compressor and nebuliser. Currently we
use the Pari LC Plus but the faster e-Flow Rapid (ultrasonic nebuliser) can be used but requires
the family to purchase it themselves. Given at the same time each day and allowing at least 1 hour
(ideally longer) before the next physiotherapy session to allow time to work.

Remember it must be kept in the fridge

Side effects:
Rare and mild. Hoarse voice occasionally. Rash sometimes seen. There is no need to stop its use
in patients with haemoptysis or pneumothorax.




Author:            Drs Vyas, Byrne, Jaksic & Jan Tate               Service:             Paediatric CF Team
Editor:            Dr Raewyn Gavin                                  Date Issued:         August 2008
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                                            CYSTIC FIBROSIS

6.5 Hypertonic Saline

Induced Sputum:
Hypertonic saline (HS) can be used to encourage sputum production in patients >6yrs. HS can
cause bronchoconstriction, so pre-treatment with bronchodilator is advised. In all cases, HS is
followed by physiotherapy (the opposite for rhDNase). For sputum induction we use 4 - 6% saline
(‘normal’ saline is 0.9%). This should be combined with vigorous physiotherapy.

Maintenance therapy:
When HS is to be used as an adjunct to physiotherapy, 7% should be used (as top of the dose
curve). In those with severe airflow obstruction, or marked peak flow variability, it is wise to start
with 7% but consider reduction to 4% if airway obstruction is a problem. There is no benefit going
to concentration higher than 7%. If HS is being contemplated for a ward patient, discuss with
physiotherapy first.
Response to HS and rhDNase is idiosyncratic. Those who respond to one are not guaranteed to
respond to the other. The longer term Australian study showed clinically trivial improvements in
lung function, but fewer infective exacerbations with HS. The down side is two more nebulisers per
day

We currently recommend that HS is not mixed with any other medication.

6.6 Long term azithromycin

This can be considered as an option in those with severe respiratory disease despite optimisation
of all other treatment. Recently its effects when used long term (250mg/day if <40kg, 500mg/day
>40kg for 6 months) have been studied [Equi et al, Lancet 2002;360:978-84 ]. It was beneficial for
lung function (FEV1 increased by median 5.5%) and reduction of oral antibiotic usage. Its
mechanism remains unknown but could be antibacterial, anti-inflammatory or both. It requires
special application to PHARMAC here in New Zealand but is a standard treatment in other
countries.
Judgement of response: Onset of action is slow (at least 2 months) and a minimum 4, preferably 6
month trial is required. If there has been a beneficial response then one could consider reducing
the dosing frequency to alternate days or 3 times per week as in the US study.

Side Effects:
Theoretically liver function abnormalities and reversible tinnitus. Liver function tests should be
performed at any time blood is being taken for other reasons and at annual assessment. Use of
only one macrolide at any one time.
Await for Pharmac decision to make this available

NB: before starting Azithromycin it is essential that three sputum samples are clear of non-
tuberculous mycobacteria.

6.7 Aspergillus Lung disease

See ABPA Results Sheet – Appendix VI…

Aspergillus fumigatus is a fungus that grows at 37degC. and the spores are of a size that they are
deposited in the distal airways. The fungus produces a large number of toxic and allergenic
exoproducts. There are a large number of manifestations in CF. In general; children are advised to
avoid soiled straw (e.g. stables, pets etc).
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Editor:           Dr Raewyn Gavin                                  Date Issued:         August 2008
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                                              CYSTIC FIBROSIS

6.7.1. Allergic Bronchopulmonary Aspergillosis (ABPA)
This is a serious potential cause of lung damage and is not uncommon in CF (typical prevalence is
10% of the clinic). Early pick-up depends on screening and high clinical suspicion.

Diagnostic criteria
This can be a very difficult diagnosis to make.

Clinical
    •      Increased wheezing / obstructive PFT.
    •      Chest symptoms failing to respond to antibiotics and inhaled medications.
    •      Fever and malaise.
    •      Thick sputum with brown or black bronchial plugs (casts).
    •      Other parameters (continued weight loss, indolent deterioration).
Investigations

Major Criteria
    •      CXR pulmonary infiltrates > 1cm diameter and segmental collapse.
    •      High serum IgE - especially an abrupt recent 4-fold rise to above 500 iu/ml (normal < 50
           iu/ml), that falls with prednisolone therapy.
    •      High specific aspergillus IgE RAST. The normal value <0.35 iu/ml may rise 10-100x in
           ABPA.
    •      Positive IgG aspergillus precipitins using Bencard standard antigens 1 & 2.
    •      Eosinophilia (> 0.4 x 109/l).
    •      Positive skin prick test to aspergillus antigen – (3mm > control) – not used at Starship.
    •      Reversible bronchoconstriction.
    •      Central bronchiectasis.


Minor Criteria
    •      Aspergillus fumigatus culture from sputum (NB found in 30% of all CF patients).
    •      Brown/black plugs in sputum.
    •      Late skin test reaction.

Some children can have recurrent episodes of ABPA. IgG preciptins and IgE rast are often
unhelpful in diagnosing repeat episodes since the levels often remain high after the initial episode.
Therefore diagnosis of second or subsequent episodes of ABPA often rest on symptoms, serum
IgE and XRay changes.




Author:             Drs Vyas, Byrne, Jaksic & Jan Tate               Service:             Paediatric CF Team
Editor:             Dr Raewyn Gavin                                  Date Issued:         August 2008
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                                            CYSTIC FIBROSIS

Treatment

Oral corticosteroids:
Prednisone, given in the morning after food (not enteric coated as it is not well absorbed in CF, i.e.
white tablets instead of pink) is normally used at a dose of 2mg/kg/day for 2 – 4 weeks (maximum
of 60mgs), then 1 mg/kg/day for 2 – 4 weeks, then 1 mg/kg/alt day for 2 - 4 weeks. The time for
reduction of steroids will be indicated by improvement of symptoms, improvements of chest x-ray,
and a reduction of the IgE which should be measured at 2 – 4 week intervals until on a low dose of
steroids (less than 0.5 mg/kg alternate days).

Relapse is common within 2-3 years of first episode, and often high doses of steroids are needed
for a long time. Side effects are discussed in section above on use of steroids. An equivalent dose
of dexamethasone may be used instead.

Itraconazole:
This is used routinely for treatment of definite ABPA, in combination with oral corticosteroids. For
patients <12 years give 100mgs BD or >12 years 200 mg BD orally (monitor liver function) and
continue whilst they remain on steroids. The capsules particularly are poorly absorbed so take
these with an acidic liquid (e.g. coca-cola, orange juice) and food. A liquid formulation is available
but not funded. This is absorbed better although it is quite unpalatable. The liquid is taken on an
empty stomach. Stop ranitidine/omeprazole if possible to improve absorption. Liver function tests
should be performed if blood is being taken anyway for repeat ABPA markers, otherwise do them
for prolonged courses e.g. at least after 1-2 months or if there is a history of liver dysfunction.

NB. It should also be given to anyone taking oral steroids (for whatever reason) if there is any
suggestion of concomitant aspergillus infection while they are taking the steroids.
We usually continue Itraconazole for 1 – 3 months after stopping steroids.
Levels: Serum levels can be measured to ensure adequate absorption but they do not always
reflect sputum levels but there is some doubt as to what represents therapeutic levels.

Voriconazole:
This is a newer oral antifungal antibiotic, which has better absorption than itraconazole and is not
affected by gastric pH. This is not currently funded. It may be useful as a 2nd line agent for patients
who have not responded to or cannot tolerate itraconazole.

Nebulised amphotericin (non-liposomal):
This may be used in difficult cases at a dose of 5 -10 mg twice daily after physiotherapy (check for
bronchoconstriction and use bronchodilator predose). The liposomal (very expensive) form would
be considered only for those in whom bronchoconstriction is a severe problem. Again this is not
funded.

6.7.2 Other manifestations of Aspergillus lung disease (rare)

Invasive disease may occur, heralded by worsening of symptoms and progression of x-ray
shadows, sometimes with cavitation, haemoptysis and pleuritic pains. Metastatic fungal spread is
also possible in severely debilitated, immunosuppressed (including steroids) or neutropenic
patients. CT scan is useful to confirm the diagnosis. Such cases warrant treatment with parenteral
liposomal amphotericin (Ambisome) 5 mg/kg/day for 4 to 6 weeks. Oral / IV flucytosine is
sometimes given at the same time.


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Editor:           Dr Raewyn Gavin                                  Date Issued:         August 2008
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                                             CYSTIC FIBROSIS

6.8 Haemoptysis

Streaky haemoptysis is common with chronic infection but often indicates deterioration so sputum
should be cultured and a course of antibiotics considered. Haemoptysis must be differentiated from
haematemesis. The source is usually from areas of chronic airway inflammation. Large
haemoptysis (> 250mls in 24 hours) due to vessel rupture can be life threatening. Management
often involves input from CF Specialists, Interventional Radiologist and Thoracic Surgeons.
Anything over half a cupful in 24 hours or fresh clots, warrants admission and discussion with the
CF team at Starship. This occurs in 1% patients/year. The usual site of bleeding is tortuous
bronchial arteries. In CF haemoptysis, remember the possibility of pulmonary embolism if the child
has a portacath (see above).

The patient may experience a gurgling sensation which is a reliable lateralising symptom indicating
the bleeding site. The patient is likely to be very scared - reassurance is essential. Primary
management is resuscitation if needed (incredibly rare) - lay patient on side (gurgling side down),
give oxygen. There is no evidence to suggest that stopping rhDNase is necessary. Physiotherapy
may have to be adapted - seek advice from the CF Physiotherapist.

Investigations:
    •     Hb & platelets.
    •     Coagulation.
    •     Group & save or cross-match blood.
    •     Sputum culture
    •     CXR can show new infiltrates but may not change and is of little use in localising the
          bleeding source.
    •     If massive the consideration a contrast CT and / or bronchoscopy
    •     Oxygen saturations. Bleeding into the lung can cause peripheral hypoxia/destauration.


Initial management
    •     If the child is likely to be transferred ensure further blood products are available on the
          journey.
    •     Give blood and correct coagulation defects if necessary (IV vitamin K / FFP /
          cryoprecipitate).
    •     Start intravenous antibiotics.
    •     Continue with gentle regular physiotherapy, but omit chest clapping for 24 hours. This is
          essential - contact the Starship CF physiotherapist for advice.
    •     Do not perform Lung Function testing for moderate to severe bleeding. Can precipitate
          further bleeding and does not influence management.
    •     Vitamin K may be needed if the child has liver disease (common in CF), or a vitamin K
          deficiency (rare).



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Editor:            Dr Raewyn Gavin                                  Date Issued:         August 2008
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                                             CYSTIC FIBROSIS

Further management
Most bleeds will cease in response to this approach but if massive bleeding persists, or if repeated
bleeding occurs over a short period (daily for 7 days with >100mls on 3/7 days) consider the
following:
    •     Oral tranexamic acid. Dose is 15-25 mg/kg tds (max 1g/dose).
    •     IV vasopressin (Argipressin) is occasionally useful - the paediatric dose is 0.3 units/kg over
          20 minutes followed by 0.3 units/kg/ hour. The adult dose is 20 units in 100ml 5% dextrose
          given over 15 mins followed by an infusion of 0.2 units/min for 36 hours. It can lead to water
          intoxication and can cause bronchoconstriction.
    •     Bronchoscopy - It is rarely useful in the acutely bleeding child. If you are considering this
          procedure initially try flexible, then consider a rigid, under general anaesthetic. With
          massive haemoptysis, go straight to rigid bronchoscopy. This can be technically very
          difficult but may allow clot removal (beware precipitating further bleeding), tamponade of
          bleeding site using a Fogarty catheter, or haemostasis with thrombin glue or iced saline
          lavage/vasoconstrictor lavage (e.g. adrenaline)
    •     Selective bronchial angiography and embolisation should only be carried out by
          experienced specialists in a tertiary centre. Numerous dilated tortuous bronchial arteries
          are often identified some of which may take origin from aberrant sources. Actual source of
          bleeding is difficult to discern. Great care to avoid spinal artery (with consequent
          paraplegia) and other systemic artery embolisation is necessary. Post embolisation pain
          requiring narcotic analgesia and transient dysphagia are common. This is not a cure and
          many patients develop new vessels within months or years that may bleed and so require
          further embolisation.
    •     Lobectomy may be considered as a last resort.

6.9 Pneumothorax

BTS guidelines at
http://www.brit-thoracic.org.uk/c2/uploads/PleuralDiseaseSpontaneous.pdf

A high index of suspicion is needed - consider the diagnosis if there is unexpected deterioration,
unexplained chest pain, or worsening breathlessness. If in doubt, do a CXR but CT scan may be
needed to detect it or determine optimal site for drain placement. However, a chest drain is not
always mandatory. A small asymptomatic pneumothorax can be managed by observation alone,
for example if the pneumothorax is 20% or less of the thoracic volume. The incidence of
pneumothorax increases with age (overall 8%) and is a marker of severe lung disease. It carries a
bad prognosis, particularly if the chest drain cannot be rapidly removed.

Pneumothorax requires admission, contact Starship Children’s Health or the local team – the
Starship CF Team is happy to be contacted as well.

A tension pneumothorax is an emergency that requires urgent treatment with a chest drain,
regardless of the CF. It is important to remember that tension, and therefore impairment of venous
return can occur in children with CF even when the CXR suggests the pneumothorax is small to
medium sized. Assessment of severity should not be based on XRay appearance of pneumothorax
alone; hypoxia may be the only clue.

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Editor:            Dr Raewyn Gavin                                  Date Issued:         August 2008
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                                             CYSTIC FIBROSIS
If the patient is decompensating or has a large pneumothorax, management includes –

    •     Monitor SaO2 and give oxygen and check for CO2 retention.
    •     Intercostal chest drain under local or general anaesthesia as appropriate.
    •     Oral analgesia as needed post op.
    •     Antibiotics (IV antibiotics are prudent in all but the most trivial pneumothoraces).
    •     Gentle physiotherapy must be continued, techniques may need changing (no PEP masks.
          Deep breathing with inspiratory holds are encouraged.
    •     The Starship CF physiotherapist will advise.

The lung may be slow to re-expand and if after three days there are no signs of resolution with a
continuing air leak, we would advise to consult with Starship CF Team. Surgery should be
considered if no progress is being made. A 50% mortality has been reported in patients with a
chest drain in situ for more than one week. Similarly, recurrences are common (>50% ipsilateral
and up to 40% contralateral). In cases of recurrence further treatment maybe necessary.
Sclerosing pleurodesis or pleurectomy make subsequent transplant very difficult although are not
an absolute contraindication. Localised abrasion pleurodesis +/- surgical resection or
thoracoscopic stapling of blebs lead to less adhesion so are preferable options.

Guidelines from the British Thoracic Society suggest that pressurised aircraft flight should not be
undertaken until a CXR has shown that the pneumothorax has not re-accumulated. They also
recommend not flying for 6 weeks after a pneumothrax. It is recognised, however, that in the
presence of an underlying lung abnormality (e.g CF) there is a risk of a recurrence of the
pneumothorax for up to a year after the event.

6.10 Intractable wheezing/ severe small airways disease

At least 50% of CF patients are atopic on the basis of skin prick testing to common allergens,
although if aspergillus is excluded the prevalence of atopy is the same as that of the non-CF
population. The great majority is well controlled with conventional ‘asthma’ type treatment using
standard guidelines for asthma (See: www.paediatrics.org.nz/documents)

A small group of patients may not respond appropriately to usual asthma medication and these
tend to be characterised by:
    •     Little if any sputum production (despite large amounts in the chest).
    •     Wheezing.
    •     Tight chest.
    •     A severe obstructive lung function pattern.
    •     Little if any bronchiectasis on CT scan.
    •     Often (but not always) IgE >500 iu/l.




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Editor:            Dr Raewyn Gavin                                  Date Issued:         August 2008
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                                             CYSTIC FIBROSIS
In these children consider:
    •     Compliance, no physiotherapy equals no sputum.
    •     Is this ABPA? This is the most common and conventional explanation.
    •     Is there a new bacterium in the sputum?
    •     Is there oesophageal reflux + / - aspiration

Consider testing for ABPA, HRCT scan, bronchoscopy and/or a pH study. Particularly ominous is
the patient who used to be a ‘conventional sputum producer’ who quite suddenly stops producing
and begins to wheeze.

However, if concern continues discussion with Starship CF Team maybe appropriate.

6.11 Bronchoscopy
Indications in CF:
    •     Need for microbiological diagnosis in a non-sputum producing child:
             o    Not responding to IV antibiotics.
             o    Patients not previously infected with P aeruginosa in whom there is clinical concern
                  due to persistent deterioration (do not simply start empirical antipseudomonal
                  therapy).
    •     Therapeutic suctioning:
             o    Persistent focal area of collapse / consolidation on chest x-ray, may also include
                  instillation of rhDNase (2.5 mg in 10 mls normal saline).
             o    It is rarely of value when chest x-ray changes are generalised or the focal changes
                  have persisted for more then 6 weeks.
    •     Other indications include investigation of an atypical course which may include:
             o    Stridor or intractable wheezing to assess for tracheo or bronchomalacia.
             o    To assess for gastroesophageal reflux and aspiration including lavage for fat-laden
                  macrophages to exclude aspiration.
    •     Haemoptysis may occasionally require rigid bronchoscopy.
    •     At the time of a general anaesthesia for another procedure.

6.12 Home Oxygen

All children with CF admitted with a respiratory exacerbation should have a continuous overnight
oxygen saturation performed on the first or second night (especially if FEV1 <50% or resting
SaO2<92%). The minimum is that every child admitted must have a spot oxygen saturation on
admission and during the first night. Oxygen therapy is usually given in hospital if saturations are
<90% for >5% of the time, but this is not evidence-based. If saturations were low and oxygen was
required at the start of the admission then the overnight monitoring should be repeated towards the
end of the admission. If they remain low (saturations <90% for >5% of the time, or 50% of the time
less than 95% saturation), then consideration should be given to providing oxygen at home, almost
always only at night. An oxygen concentrator is preferred to regular use of cylinders. If oxygen is
needed whilst awake then this is a clinical marker for consideration of transplant assessment.
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Editor:            Dr Raewyn Gavin                                   Date Issued:         August 2008
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                                            CYSTIC FIBROSIS

6.13 Palliative BiPAP Support

When a patient develops incipient respiratory failure, and is thought to have reached an end of life
phase to their illness, it may be appropriate to commence them on overnight mask ventilation. The
patient can often experience fear at the degree of dyspnoea, coupled with the knowledge that they
are dying. This often can result in difficulty sleeping, or even a determination not to go to sleep (in
case they don’t wake up). Initiation of mask ventilation can alleviate these concerns, although
clearly this needs to be done in concert with psychological support, and end of life discussions with
the child and family.

Mask ventilation is purely palliative. If a patient is to be started on this, it must be explicitly
discussed prior to commencing that this does not “cure” the CF, nor does it take over the child’s
breathing. Its only role is to support the breathing. It must also be clearly explained to the parents
(and child if appropriate) that to start on BiPAP does not mean that ventilation for acute illness is
implicit. The decision as to whether a child with end stage CF should be ventilated on PICU is one
which must also be discussed with the family (and child), but is one which may require other
circumstances to be considered (e.g. to give an important family member time to attend the child
before dying, etc.).




7. Chest Physiotherapy

Out-Patients
A paediatric physiotherapist is available in clinic to assess all patients and discuss/review their
individual physiotherapy routine. This is carried out annually at annual review or at any other time
at request. The airway clearance techniques used vary within age groups:

Babies and infants
Modified postural drainage and chest percussion is taught to parents/carers. Blowing games can
be introduced and then huffing when able/appropriate.

3-4 years and upwards.
Modified postural drainage with chest percussion. Progression towards active cycle of breathing as
appropriate. Bubble PEP. Exercise encouragement.

8 years and upwards
Encourage independent treatment (with family support).i.e. PEP, ACBT, Flutter, Acapella.
Encourage exercise.
The frequency and duration of treatments will alter depending on infective exacerbations and
severity of disease. The minimum recommended is twice a day for 10-15 minutes. Exercise should
be encouraged daily as able.

Airway clearance techniques taught include:
    •     Active Cycle of Breathing Techniques (ACBT) – Combination of thoracic expansion
          exercises, breathing control, and forced expiration technique. Used in conjunction with
          modified postural drainage.


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Editor:           Dr Raewyn Gavin                                   Date Issued:         August 2008
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                                             CYSTIC FIBROSIS
    •     Positive Expiratory Pressure (PEP) – Provides resistance to expiration through a
          mouthpiece or facemask, followed by forced expirations.
    •     Flutter – Pipe-shaped device that creates oscillation and positive pressure on expiration.
    •     Acapella – oscillating PEP with similar characteristics to Flutter. Is not gravity-dependent.

Other physiotherapy issues that may be discussed are:
    •     Exercise – The importance of regular exercise is encouraged.
    •     Posture correction.
    •     Urinary incontinence – Stress incontinence, usually with coughing, can occur even in young
          children. Please consult the physiotherapist for advice.

Inhaled medication should be co-ordinated with physiotherapy:
    •     Bronchodilators -10-15 mins pre-physiotherapy if necessary, and benefit shown.
    •     Steroids and antibiotics - Post-physiotherapy.
    •     rhDNase – At least 1 hour pre-physiotherapy or after physiotherapy. N.B some children
          take it 1-2 hours pre-physiotherapy, and a few even longer but do not take immediately
          before bed as it can cause coughing through the night.
    •     Hypertonic Saline - Immediately pre-physiotherapy or in conjunction with physiotherapy
          when doing PEP.

For antibiotics and hypertonic saline the child must always be assessed for bronchoconstriction
when the 1st dose is given. This is done in the Lung Function lab with the respiratory technicians.

Nebulisation equipment is organised through the CF nurse specialist and the Home Care nursing
teams. At present, Pari LC Plus nebulising bowls are used in conjunction with a medium output
compressor. These breath activated nebulising bowls can be used with a mouthpiece or a mask
which is attached with an elbow piece. All children over the age of 3 years should be encouraged
to use a mouthpiece. It is advisable to filter the antibiotics using an expiratory filter valve set with
filter pad attached to the Pari LC Plus bowl. This is to prevent other members of the family inhaling
the antibiotic. Nebulise in a large airy room or in a room with the window open.

    •     All Pari nebulising equipment is supplied by Starship and/or the Homecare Nursing teams.
    •     Compressors need to be serviced yearly.
    •     Pari nebulising bowl and filter exhalation set to be changed every six months.
    •     The nebuliser bowl and exhalation filter valve set to be washed with hot, soapy water,
          rinsed well and left to dry in-between treatments. Once a week all the equipment is placed
          into boiling water and boiled for 10 minutes to clean the jets in the base of the nebulising
          bowl. Equipment must be used dry before use.

The E-Flow Rapid is a new nebuliser system that takes a much shorter time to nebulise antibiotics
&/or Pulmozyme. Starship does not supply the E-Flow or ongoing supplies. Families can purchase
them (& 6-monthly change of handset and aerosol head) through the CF Association at a cost of
approx $1,500. Change of equipment pieces are approximately $300/year



Author:            Drs Vyas, Byrne, Jaksic & Jan Tate               Service:             Paediatric CF Team
Editor:            Dr Raewyn Gavin                                  Date Issued:         August 2008
Cystic Fibrosis    Page:                                            45 of 100
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                                            CYSTIC FIBROSIS

In-Patients
All children admitted prior to 3pm will be assessed and physiotherapy requirements established.
After this time any emergency treatment can be provided by the on-call physiotherapist if the
patient meets the urgent on-call criteria, otherwise they will be assessed as soon as able the
following day. Treatment is also continued over the weekend. Children will also be seen pre- and
post-general anaesthesia to ensure they can clear sputum effectively as required. Prior to
discharge the home regimen will be reviewed as well as exercise and progression of treatment
where appropriate.

Inpatients are seen twice daily by the physiotherapist and a third session is encouraged
independently or with family members in the evening. Inpatients are also taken to the gym for
exercise programmes daily (on weekdays) as time allows. This is also something that can be done
with the aid of a therapy assistant once the programme is established.




   Nutritional
8. Nutritional Care

The Dieticians Association of Australia (DAA) CF Interest Group have recently produced their own
set of guidelines for nutritional care in CF. We consider these to be the standard to which all CF
children should be cared for.

The document can be accessed by the web at:
http://www.cysticfibrosis.org.au/pdf/CF_Nutrition_Guidelines.pdf


Pancreatic Enzyme Replacement Therapy (PERT)

    •     Introduce PERT once clinical evidence of pancreatic insufficiency is confirmed by stool
          tests of faecal elastase and/or faecal chymotrypsin.
    •     Due to the progressive nature of CF, individuals who are pancreatic sufficient at diagnosis
          will continue to require periodic assessment of pancreatic function.
    •     When commencing PERT, start with the minimum recommended dosage.
    •     Fat based dosing linking consumption of grams of fat to units of lipase in the capsules will
          assist in optimising absorption. (see following page)




Author:           Drs Vyas, Byrne, Jaksic & Jan Tate                Service:             Paediatric CF Team
Editor:           Dr Raewyn Gavin                                   Date Issued:         August 2008
Cystic Fibrosis   Page:                                             46 of 100
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                                             CYSTIC FIBROSIS

Administration of PERT
•   Pancreatic enzyme capsules should be swallowed whole.

•   For infants and younger children (who cannot swallow capsules) the capsule is opened and
    microspheres sprinkled onto ½ - 1 teaspoon apple puree.

    Apple puree is slightly acidic, and its viscous consistency assists with swallowing the
    microspheres.

•   Enzymes should be taken before and/or during the meal or snack. If breast/formula feeds or
    meals last for longer than 30 minutes, give the enzymes as a divided dose.

    Divide the dose as ½ amount at start of feed and rest of the enzyme half way through the
    feed/meal.

•   Do not mix microspheres with expressed breast milk, formula or other liquids.

Pancreatic Enzyme Preparations Available in New Zealand
                                              Composition per capsule
                                                     BP units
          Product (Distributor)                   Lipase              Protease              Amylase

    Enteric-coated Microspheres
     Creon 10000 (Boehringer-
                                                  10 000
            Ingelheim)                                                   600                  8000
                                                   8000
     Cotazym ECS (Organon)

    High Lipase Enteric-coated
          Microspheres
     Creon Forte (Boehringer-
           Ingelheim)                             25 000                1000                 18 000
 Panzytrat (Technipro/Pharmaco)                   25 000                1256                 22 500




Author:            Drs Vyas, Byrne, Jaksic & Jan Tate               Service:             Paediatric CF Team
Editor:            Dr Raewyn Gavin                                  Date Issued:         August 2008
Cystic Fibrosis    Page:                                            47 of 100
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                                            CYSTIC FIBROSIS

                  Australian Guidelines for Pancreatic Enzyme Replacement Therapy

Infants
• 500-1000 U lipase/g of dietary fat
• Commence with the minimum dose e.g. 2,500 units lipase/breast feed or 120ml
    infant formula
• Titrate dose according to weight gain and bowel signs
Children and Adults
• 500-4000 U lipase/g of dietary fat
• Maximum dose of 10,000 U lipase/kg body weight/d
• Aim for lowest effective dose
• Use individual approach
• Distribute the enzymes throughout the day in alliance with the fat content of food
    and drinks consumed
• Monitor weight gain, growth and bowel signs
• Patients should be encouraged to discuss PERT with clinic staff before increasing
    dose
Before PERT dose is increased evaluate the following:
Distribution
• Check that PERT is correctly distributed over the day’s meals based on the fat
    content of individual meals, snacks and fluids, e.g. milk
Administration
• Capsules should be swallowed whole or the granules inside the capsules mixed
    with an acidic fruit puree e.g. apple. Granules should not be chewed.
• Enzymes should be taken before and/or during meal or snack. Enzymes are
    effective for 30 mins after consumption. Therefore, additional enzymes are
    required of eating or drinking milk (or infant formula) 30 min after the first dose.
Storage
• Store capsules in an airtight container, in a cool, dry place
• Check the enzymes capsules have not exceeded the expiry date
Adherence
• Administer PERT with all meals, snacks and fluid containing fat
• Avoid increasing the dose if adherence is poor
• Develop strategies to improve adherence, particularly at school
Efficacy
Implement and evaluate the following if PERT appears to be ineffective:
• Measure faecal fat excretion (%FFE)
• Increase PERT dose by small increment but avoid exceeding the maximum ‘safe’
    dose of 10,000 U lipase/kg/d
• Consider use of adjunctive therapies e.g. H2 blockers, synthetic prostaglandins
• Refer to gastroenterologist for review of PERT and/or GI investigation
Before using acid suppressing agents
• Document %FFE and usual PERT intake and distribution
• Add adjunctive agent
• Repeat measurement of %FFE and assessment of PERT intake and distribution




Author:           Drs Vyas, Byrne, Jaksic & Jan Tate               Service:             Paediatric CF Team
Editor:           Dr Raewyn Gavin                                  Date Issued:         August 2008
Cystic Fibrosis   Page:                                            48 of 100
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                                             CYSTIC FIBROSIS

    Fat Based Dosing For Pancreatic Enzymes

In New Zealand, a suggested starting dose is 1400 IU Lipase/gram fat in food and beverages.

Pancreatic Enzyme                  Amount of Enzyme                     Grams of Fat
Creon 10000                        1 capsule                            7 grams
Creon 10000                        ½ capsule                            3-4 grams
Creon 10000                        ¼ capsule                            1.8-2.5 grams
Creon Forte                        1 capsule                            18 grams

Education for fat based dosing of PERT must be given by a registered dietitian.

PERT dosage should not exceed 10,000 IU lipase/kg/body weight due to the risk of fibrosing
colonopathy at higher dosage.

For further information and practical administration guidelines refer to:
       Australasian Clinical Practice Guidelines for Nutrition in Cystic Fibrosis, September 2006
       Dietitians Association of Australia National Cystic Fibrosis Interest Group
       www.cysticfibrosis.org.au/books/nutrition

          Anthony H, Collins C, Davidson S et al. Pancreatic enzyme replacement therapy in cystic
          fibrosis: Australian guidelines. Journal of Paediatrics and Child Health, 1999 35(2):125-9.

          A Guide to Giving Paediatric Enzymes for the Infant with Cystic Fibrosis
          Rhonda Akroyd, Paediatric Dietitian, Auckland Healthcare, June 2000
          Order from Boehringer-Ingelheim.




9. Gastrointestinal Care
9.1 DIOS & constipation

Distal Intestinal Obstructive Syndrome (DIOS) is a common complication in CF. The incidence
varies widely but it only affects those with pancreatic insufficiency. The pathophysiology is not fully
understood, but there are often multiple contributory factors including:
    •     Dehydration
    •     Rapid increase in enzyme dosage
    •     Viscid intestinal secretions
    •     Altered gut motility and pH
    •     Poor compliance with enzyme therapy
Viscid muco-faeculent material accumulates in the terminal ileum / caecum leading to partial
obstruction with pain usually in the right lower quadrant, abdominal fullness and a palpable mass in
the right iliac fossa.



Author:            Drs Vyas, Byrne, Jaksic & Jan Tate               Service:             Paediatric CF Team
Editor:            Dr Raewyn Gavin                                  Date Issued:         August 2008
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                                            CYSTIC FIBROSIS

Differential diagnosis:
Constipation, appendicitis, intussusception, volvulus, adhesions post abdominal surgery, fibrosing
colonopathy, biliary tract or gallbladder disease, acute pancreatitis, urinary tract infection.

Investigations:
It is important to find out the reason behind the DIOS to prevent recurrence.
     • WBC, amylase, liver function tests, urinalysis, stool culture.
     • AXR - dilated small bowel loops with “bubbly” ileocaecal mass, classic feature but not
         commonly seen.
     • Abdominal ultrasound.
     • Barium /gastrografin enema - by specialist radiologist can diagnose and help treatment at
         same time.
     • After the acute episode, consider faecal fat study.

Treatment:

Chronic
   • Check dose / compliance / timing of enzyme supplements.
   • Diet – ensure adequate dietary roughage.
   • Ensure adequate fluid intake.
   • Ensure patient has well established toilet routine (try to go after meals), even at school.
   • Laxatives may help eg. lactulose 5-20 mls bd.
   • If ongoing malabsorption is documented consider acid reduction with ranitidine (very bitter
       in liquid form) 2-4mg/kg bd or omeprazole (0.4-0.7 mg/kg bd initially, maximum dose 40
       mg/day. To exceed this dose i.e. up to mg/kg bd, it is a consultant decision).

Acute
Kleen Prep (oral)
     • This should not be used in children <20kg. Gastrografin can be used in those less than
        20kg.
     • The dose of Kleen Prep is 1 sachet in 1 l water. Then 10mls/kg/hour is given for the first 30
        mins, then increase to 20 mls/kg./hour if no result. This can be further increased to
        25mls/kg/hour.
     • Further increases may be necessary, up to a maximum of 100mls/kg/hour for 4 hours or a
        total of 4l on 4 hours, whichever is greater.
     • Patient should be well hydrated before and during KleenPrep, as it is highly osmotic.
     • Further information re Kleen Prep is in the formulary section of this guideline.
     • Admit patient.
     • Aim is to take solution until clear fluid is passed PR.
NG tube is usually required as volume is so large but occasionally some patients will prefer to drink
it (more palatable if cool).
Start regular lactulose and review chronic management.

Constipation

If severe should be considered as part of DIOS spectrum. However beware increasing enzyme
doses when all that is needed is simple constipation treatment. Main differential from DIOS is that
constipation tends to be limited to rectum.


Author:           Drs Vyas, Byrne, Jaksic & Jan Tate               Service:             Paediatric CF Team
Editor:           Dr Raewyn Gavin                                  Date Issued:         August 2008
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                                             CYSTIC FIBROSIS
Treatment:
Ensure adequate fluid intake.
Lactulose 5-15 mls twice daily
Movicol may also be used as 2nd line to lactulose

9.2 Liver Disease

Reports of the prevalence of liver disease in CF vary but cirrhosis has been reported in 24% CF
patients and up to 50% in post mortem findings. However, symptomatic liver disease is
uncommon, being reported as the cause of death in only 2% of CF patients. There is no known
genotype-phenotype correlation but there is a high familial concordance. The characteristic
pathology arises from a diffuse intrahepatic biliary abnormality leading to focal or multilobular
biliary cirrhosis. However in infancy, presentation may be with bile duct obstruction (neonatal
cholestasis) due to inspissated bile or with fatty change. Gallstones and cholecystitis can occur in
later childhood.

Detection of liver disease

There is no single gold standard for the diagnosis of liver disease, but a combination of ultrasound,
physical examination and serum transaminases should identify most patients with significant liver
disease.

An abdominal ultrasound performed at 3, 6 ,9 & 12 years of age, and annually thereafter (at the
time of the annual review).

Annual abdominal ultrasound if:
    •     Palpable liver or spleen
    •     Liver enzymes elevated > 2X normal
    •     Persistent enzyme elevation <2 X normal
    •     Previously abnormal liver ultrasound

When to refer to hepatologist
    •     Signs of significant liver disease
    •     Heterogeneity of hepatic echotexture on ultrasound
    •     Splenomegaly -Clinical or ultrasonographic
    •     Hypersplenism – decreased platelet count, white cell count
    •     AST, ALT, GGT >2X upper limit of normal or persistent elevation <2X normal >6 months
    •     Abnormal Bilirubin
    •     Abnormal INR not responsive to vitamin K

Complications of liver disease
  • Synthetic liver dysfunction (elevated INR, decreased albumin, ascites)
  • Gallstone related disease – right upper quadrant pain, jaundice, pale stool, sudden
      elevation in liver enzymes or bilirubin.
  • Oesophageal varices, upper or lower gastrointestinal bleeding
Author:            Drs Vyas, Byrne, Jaksic & Jan Tate               Service:             Paediatric CF Team
Editor:            Dr Raewyn Gavin                                  Date Issued:         August 2008
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                                            CYSTIC FIBROSIS

Standard treatment

Cirrhosis
    • Assess for other causes of liver disease
    • Alpha-1-antitrypsin deficiency (phenotype),
    • Wilson’s disease (serum copper, caeruloplasmin)
    • Hepatitis B and C (serology)
    • Others as clinically indicated

Prevention of secondary morbidity

Nutrition.
   • Vital to long term prognosis
   • Medium chain triglyceride predominant supplementation may be required
   • Do not restrict protein intake

Fat soluble vitamin deficiency
    • Vitamin K 10mg oral OD for all patients with demonstrated liver disease
    • Patients with coagulopathy (INR> reference range)
    • Consider consumptive coagulopathy (disseminate intravascular coagulopathy) – (increased
       APPT, decreased fibrinogen)
    • Intravenous vitamin K (10mg)
    • Repeat INR at four hours, if INR not normalised further 10mg IV vitamin K and repeat INR.
    • If still abnormal contact on call Paediatric Gastroenterologist immediately

Monitor vitamin A, D and E levels yearly
If deficiency documented:
    • Assess compliance
    • Increase dose of supplement
    • Encourage sun exposure for vitamin D deficiency
    • Document normalisation of vitamin levels

Patients with a large spleen and functional hypersplensim (decreased platelets or white cell count)

Assessment for gastroesophageal varices
   • Patients at risk of having oesophageal varices may require upper endoscopy +-
      oesophageal band ligation to reduce risk of variceal bleeding.
   • Hypersplenism
   • Platelets <100 x106
   • White cell count < 3 x106

Gastrointestinal bleeding (upper or lower GI bleeding)
   • Blood in either vomit or stool (red or black) may be from oesophageal variceal bleeding
   • General concepts of management see GI bleeding guideline
   • Seek expert advice immediately
   • ABC
   • IV access X2 as large a calibre as possible
   • Fluid resuscitation to achieve euvolaemia (hypervolaemia will drive bleeding)

Author:           Drs Vyas, Byrne, Jaksic & Jan Tate               Service:             Paediatric CF Team
Editor:           Dr Raewyn Gavin                                  Date Issued:         August 2008
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                                            CYSTIC FIBROSIS
    •     Bloods including cross match, fbc, liver enzymes and coagulation profile (beware initial
          haemoglobin may be predilutional and artefactualy high
    •     Start Octreotide (500mcg in 40ml normal saline 2ml/hour) no dose adjustment for age or
          weight)
    •     Omeprazole IV 1mg/kg q8hourly
    •     Intravenous antibiotics
    •     Seek urgent surgical/ paediatric gastroenterology/PICU advice
    •     Ongoing bleeding or instability may require PICU, acute endoscopy or Senstaken-
          blakemore tube.

Patients with a spleen palpable on abdominal examination should avoid risk abdominal trauma
(risk of splenic laceration/ rupture)
Avoid contact sports (rugby, boxing, BMX cycling etc)
Advice to be tailored to individual, age of child, size of spleen etc. i.e. a 6 year old with minimal
splenic enlargement is probably safe playing soccer but a 16 year old with a markedly enlarged
spleen and a platelet count of 30x106 cell/ml may not be.

Non steroidal anti-inflammatory drugs (NSAIDs – aspirin ibuprofen, voltaren etc) are
contraindicated in patients with liver disease (risk of gastric / oesophageal bleeding).

Vaccination
Patients with cirrhosis should have immunity documented against Hepatitis A and B and
vaccinated if not protected.

Ursodeoxycholic acid (increases bile flow)
10-15 mg/kg bd. Usually well tolerated. Main side effect is diarrhoea, in which case reduce the
dose. This reverses markers of CF liver disease but it is unclear whether it can delay or reverse
fibrosis. Funding is via exceptional circumstances.

Liver transplant
    • Approximately 1-2% of patients with Cystic Fibrosis will require a liver transplant.
    • Outcomes are comparable with patients transplanted for other reason (85-90% 5 year
        survival).
    • Pulmonary function is usually stable or improved following liver transplantation.
    • Indications for liver transplantation are synthetic liver dysfunction (elevated INR, decreased
        albumin or hepatic encephalopathy).
    • Pulmonary disease must be mild or moderate (not severe).

9.3 Iron status

The quoted incidence of iron deficiency anaemia in CF patients varies markedly. Iron deficiency
anaemia (hypochromic microcytic anaemia with low ferritin) is the extreme end of a spectrum of
iron deficiency. The earliest features are low/deficient iron stores, i.e. low ferritin, which progresses
to iron deficient erythropoiesis i.e. low ferritin, raised TIBC, reduced transferring saturation and
hypochromic red cells. This will progress to anaemia if the iron stores are not restored.

Many are cautious about supplemental iron in CF patients; especially those infected with P
aeruginosa, as the organism requires iron for its growth and has developed iron scavenging
mechanisms. It has also been shown that free iron i.e. that unbound to ferritin, catalyses the
generation of highly reactive hydroxyl radicals and promotes oxidative cell injury. Increased

Author:           Drs Vyas, Byrne, Jaksic & Jan Tate                Service:             Paediatric CF Team
Editor:           Dr Raewyn Gavin                                   Date Issued:         August 2008
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                                             CYSTIC FIBROSIS
concentrations of iron, ferritin and isoferritins have been found in the sputum of adults with stable
CF.

Another important cause of hypochromic microcytic anaemia is anaemia of chronic disease, where
iron is poorly utilised due to the increase in certain cytokines. Here the major differentiator from
iron deficiency anaemia is a normal or raised ferritin. These patients would not benefit from oral
iron supplementation.

It must also be remembered that ferritin is also an acute phase reactant and can go up in acute
infection/inflammation (although this is rarely seen in practice).

Our policy is to treat overt iron deficient anaemia, rarely seen in our patients (1%), but we tend not
to give iron at the earlier stages of reduced stores due to concerns over its potential adverse
effects on lung disease. In addition it is often poorly tolerated with gastrointestinal side effects.
When necessary, we use sodium feredetate (sytron liquid) or if not tolerated ferrous fumarate
liquid, whilst in older children 1st line is ferrous sulphate tablets Bloods should be checked after 3
months of treatment.

                   Transport iron / iron supply                                 Functional iron
                   Serum       Serum        Serum        Hypochromic Hb                      MCV
                   ferritin    TIBC         Transferrin red cells
                                            saturation
                                             Iron deficiency
 Storage                ↓           N              N               N                N               N
 depletion
 Iron deficient         ↓            ↓             ↓               ↓                N               N
 Erthropoiesis
 Iron deficiency        ↓            ↓             ↓               ↓                ↓               ↓
 anaemia
                                           Iron malutilisation
 Anaemia of         N or             ↓             ↓            N or                ↓           N or
 chronic
 disease




Author:            Drs Vyas, Byrne, Jaksic & Jan Tate               Service:             Paediatric CF Team
Editor:            Dr Raewyn Gavin                                  Date Issued:         August 2008
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                                            CYSTIC FIBROSIS

10. Endocrine Complications of CF

10.1 Cystic Fibrosis Related Diabetes (CFRD)

Background

In CF individuals with exocrine pancreatic insufficiency, insulin deficiency develops with increasing
age. Insulin secretion is reduced even in individuals with normal glucose tolerance, and with age
there is an increase in the development of impaired glucose tolerance and diabetes. CF related
diabetes (CFRD) is rare in those under 10 years although up to a third of this age group will
already have impaired glucose tolerance. The reported prevalence of CFRD depends on the
diagnostic criteria used and screening methods, but approximately 50% of individuals with CF will
have CFRD by 30 years of age.


Insulin secretion

In CF the insulin response to a glucose load or a meal is reduced in amplitude and delayed
compared to normal individuals. Basal insulin secretion is usually maintained for some years after
the onset of diabetes. This means that many people with CFRD will have normal glucose levels
after fasting, and in the early stages of developing diabetes glucose levels will only be elevated
after meals. A frequent pattern is to see normal glucose levels on waking which rise during the day
as the individual eats but the pancreas is unable to secrete enough insulin. The problems related
to CFRD and impaired glucose tolerance.

Loss of the anabolic effect of insulin
This is often an important factor in the decision to treat with insulin. Studies in adults have shown a
fall in BMI and lung function for up to 5 years before a diagnosis of CFRD is made. Individuals with
CFRD have lower BMI, worse lung function and decreased life expectancy compared to those with
CF but no diabetes (especially females).

Risk of the microvascular complications of diabetes
This has become a real concern, as individuals with CF are surviving longer. Risk of developing
complications is related to the time over which they have had diabetes and control (HbA1c).

Symptoms of hyperglycaemia
Polyuria and polydypsia can develop insidiously over time.
Hyperglycaemia also results in tiredness, so even if weight and growth are normal, adequate
diabetes control can have a positive impact on quality of life.

Making a diagnosis of CFRD, and when to treat

The agreed criteria for the diagnosis of diabetes are based on cut off values for fasting glucose,
random glucose, and the glucose level at 120 minutes after an oral glucose tolerance test. These
definitions are used for CFRD, with the understanding that the situation is not exactly the same as
in type 1 and type 2 diabetes. The cut off values are based on assessment of cardiovascular risk
and treatment goals may be different in CFRD.



Author:           Drs Vyas, Byrne, Jaksic & Jan Tate               Service:             Paediatric CF Team
Editor:           Dr Raewyn Gavin                                  Date Issued:         August 2008
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                                             CYSTIC FIBROSIS
Glucose levels and the response to GTT can vary with time in CF- a “diabetic” GTT can revert to
normal if infection is treated or steroid treatment stopped. Response to GTT may not fit with
daytime glucose levels. Significantly raised glucoses during the day can sometimes be seen where
the GTT is not diagnostic of diabetes, or a person with a “diabetic” GTT may have normal glucoses
during the day.


              Definition                                       Clinical features
                                                          Symptoms plus
                                             random glucose concentration >11.1 mmol/l
                                                                 or
              Diabetes
                                                   Fasting glucose of >7.0 mmol/l
                                                                 or
                                         2 hour plasma glucose over >11.1 mmol/l after GTT
                                              Fasting plasma glucose <7.0mmol/l, and
   Impaired glucose tolerance            2 hour plasma glucose >7.8 mmol/l to <11.1 mmol/l
                                                            after GTT
     Impaired fasting glucose                Fasting glucose of >6.1 mmol/l to <7.0 mmol/l

Standard criteria for interpretation of oral GTT results

Oral glucose tolerance test
    •     Glucose levels are measured before and after a standard oral glucose load.
    •     Preparation: The child should be fasted from midnight. Drinks of water are allowed.
    •     Dose of glucose: 1.75 g/kg glucose to a maximum of 75 g, as glucose mono hydrate diluted
          in water (200-300 mls).
    •     A glucose drink giving the same dose of glucose can be substituted-
             o    Lucozade Energy “original” contains 17.9g glucose/100ml and the dose of this is
                  9.2ml/kg to a maximum of 394 mls.
             o    Polycal (Nutricia clinical) contains 66.4g glucose/100ml and the dose is 2.6mls
                  perkg to a maximum of 113mls.
    •     Samples: Take blood for glucose at 0 mins (fasting). Give the glucose drink, and take blood
          for glucose at 120 minutes.

Screening for CFRD or impaired glucose tolerance

UK CF Trust guidance is that every child with CF over 12 years has a yearly GTT.
GTT should be considered in the following circumstances, (even if the child is under 12 years of
age), and can be carried out at the local hospital if necessary:
    •     If there are symptoms of hyperglycaemia - polyuria, polydypsia.
    •     If random glucose or HbA1c is elevated, or there is glycosuria.
    •     Unexplained weight loss or growth failure.
    •     Poor lung function (Poor FEV1 <40%) or unexplained deterioration in respiratory function.

Author:            Drs Vyas, Byrne, Jaksic & Jan Tate               Service:             Paediatric CF Team
Editor:            Dr Raewyn Gavin                                  Date Issued:         August 2008
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                                            CYSTIC FIBROSIS
    •     Prior to commencing supplemental enteral tube feeding.
    •     If there are symptoms of hypoglycaemia.
    •     Before major surgery.

GTT is not needed if the diagnosis of diabetes is already established with random glucose levels.

Regular random blood glucose measurements and urine testing for glycosuria should be done
for inpatients:
     • who are receiving high dose steroid treatment
     • are on overnight feeds (test at the end of the feed)

Fasting glucose levels and HbA1c can be normal in established CFRD. An abnormal fasting
glucose, or HbA1c should alert the clinician to the possibility of CFRD. However, normal results do
not exclude CFRD. If any doubt or concern persists, an oral glucose tolerance test should be
performed.

Action on the results of the GTT
    •     Normal glucose- repeat GTT in a year

    •     Impaired glucose tolerance. GTT should be repeated in 6 months. If weight gain or lung
          function are a concern a profile of random glucose levels should be performed.

    •     Diabetic. A profile of random glucose levels should be done.


Profile of random blood glucose levels
If the child is well this can be done at home- give the family a blood glucose meter and let them do
one or two glucose levels a day over 2-3 weeks.

Get them to vary the time of sampling. Remember that glucose is most likely to be high 1-2 hours
after meals or at the end of an overnight feed.

Ensure they do not alter their diet in order to produce normal glucose concentrations.

Tell them to ask for advice at once if they find that a lot of the glucose levels are over 15mmol/l

For inpatients the same rules apply for timing of tests, aim to do 4-5 tests a day in order to get a
clear picture after a few days.


Treatment
Children with symptomatic hyperglycaemia, or who have elevated glucose levels throughout the
day, on most days, should be treated whatever their clinical state. For those with occasional high
glucoses, or those with a diabetic GTT result where there does not seem to be hyperglycaemia,
treatment should be considered if insulin deficiency may be contributing to poor long function or
weight loss. It may be appropriate to wait before treatment if the child is getting steroid therapy or
has an acute infection since glucose tolerance may return to normal.

Author:           Drs Vyas, Byrne, Jaksic & Jan Tate                Service:             Paediatric CF Team
Editor:           Dr Raewyn Gavin                                   Date Issued:         August 2008
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                                            CYSTIC FIBROSIS
CFRD is related to insulin deficiency and insulin is the logical treatment. There is no evidence of
benefit with oral hypoglycaemic agents and they are rarely used now. The insulin given should be
tailored to the glucose levels and the child’s daily routine. Although twice-daily mixtures might
seem easier they frequently do not suit children with CF, who often do not eat breakfast, have
erratic eating habits, and may need to adjust their insulin frequently because of their clinical state.
It is usually easier to give a flexible plan, which covers the child’s actual eating habits than to try to
change things. Younger children may not be able to give an insulin injection at lunchtime, so a
morning mixed insulin may be the answer in this situation, but it is usually better to give this as part
of a 3 times daily regimen.

Choice of insulin is dependant on a number of factors. Insulin therapy (dose, type, and initiation)
will be decided upon by the Paediatric Endocrinologists who support the Starship CF patients.



Evidence for the early use of insulin in CF

In adults with CF, there is evidence that nutritional status and lung function decline as diabetes
develops and improves with insulin treatment, and there is some evidence that insulin treatment is
beneficial in adolescents. The adverse impact of diabetes on CF appears to be related to loss of
the anabolic effect of insulin and also possibly the effect of increased glucose levels in secretions
affecting infection rates. This evidence of the adverse effect of diabetes on CF has resulted in a
change in approach in recent years, with insulin treatment being introduced much earlier.

Starting insulin

Doses required can vary enormously in CF and there is no formula to help with how much insulin
to give. The best plan is to start at a low dose and adjust upwards, to avoid hypoglycaemia. Ensure
that glucoses are checked regularly and at varied times of the day. When starting insulin treatment
it is best to plan that the first injection given is the glargine or levemir because this makes
adjustment easier. When adjusting insulin doses after starting, the dose of levemir or lantus should
be changed first, before adjusting the shorter acting insulin.

Dietary advice

The family should have input from a dietitian familiar with CF and diabetes. It is important that they
understand that the dietary management is not the same as in other forms of diabetes and they do
not need to adopt a “diabetic” diet.

Calorie intake
In CFRD maintaining adequate nutrition remains the priority and a high calorie and high fat diet
must continue. Older children should avoid high sugar snacks and drinks between meals (i.e.
regular fizzy drinks, juices and squashes, jellied sweets etc.) and substitute no-sugar-added drinks
(i.e. diet fizzy drinks and squashes).
Regular-eating
Children who are taking twice daily insulin must avoid skipping meals and snacks because of the
risk of hypoglycaemia. Even on “flexible” insulin regimens, omitting meals and snacks is
discouraged, as this might lead to weight loss. Food intake should not be reduced to try to control
glucose levels; meals and snacks must be given whatever the blood glucose.



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Editor:           Dr Raewyn Gavin                                  Date Issued:         August 2008
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                                            CYSTIC FIBROSIS

Hypoglycaemia

Hypoglycaemia is a blood glucose less than 4.0 mmol/L. Children must be encouraged to identify
the feeling of being low so that treatment can be initiated quickly. Symptoms of hypoglycaemia
include confusion, irritability, pallor, fatigue, dizziness, and a “wobbly” or “funny” feeling. Caregivers
and schools should be given information about hypoglycaemia. Hypoglycaemia can be caused by
a number of factors; too much or wrongly timed insulin dose, insufficient carbohydrate intake,
exercise, missed enzyme doses, diarrhoea and vomiting leading to poor absorption of food, alcohol
consumption.

Treatment of hypoglycaemia
Give a rapidly absorbed carbohydrate followed by a complex carbohydrate snack. There is an
understandable tendency to over treat hypoglycaemia, which can result in hyperglycaemia later on.
Chocolate and sweets are not a good alternative for the initial treatment of hypoglycaemia- they
are not as rapidly absorbed as glucose, and it gives the wrong psychological message to reward
hypoglycaemic episodes.

If hypoglycaemia is suspected test the blood glucose if possible.
Treat hypoglycaemia with 10g of rapidly absorbed carbohydrate (50 ml of Lucozade, 100 ml of
coca-cola, 3 glucose tablets, 2 tsp. of jam/honey/syrup).
Wait 15 minutes and test blood sugars again. If < 4.0 mmol/L, repeat step 2. If > 4.0 mmol/L, give a
complex-carbohydrate snack (such as a sandwich, crisps or 3-4 biscuits).
Think about what caused the hypoglycaemia.

Equipment

Ideally patients should go home with spares of pens and glucose meters. Remember to contact the
GP to make sure that supplies of insulin, pen needles, lancets and strips for the meters are added
to the regular prescription. Most children will need 6-8mm needles for their pens. Never use needle
and syringe for insulin and always use an appropriate device for pricking fingers.

Outpatient follow up

This will usually be in the paediatric diabetes clinic at the Greenlane Clinical Centre.

Monitoring

A realistic plan for monitoring blood glucose levels at home should be discussed. HbA1c should be
checked every 3-4 months. Individuals with CFRD are not at increased risk of thyroid disease or
coeliac disease so this is not screened for, but regular eye screening and checks for urine albumin
should be started in everyone over 12 years.

If a child with diabetes is admitted to the ward –

Please call on the Diabetes cell phone (No. 021974804). A member of the Endocrine team to
review the patient, even if things are going very well. All insulin pens and glucose monitoring
equipment will be held by the ward and always administered under the supervision of nurses.




Author:           Drs Vyas, Byrne, Jaksic & Jan Tate               Service:             Paediatric CF Team
Editor:           Dr Raewyn Gavin                                  Date Issued:         August 2008
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                                            CYSTIC FIBROSIS

Surgery

Prior to any general anaesthetic a plan must be made to reduce or stop the insulin while the child
is fasting (see Diabetes and Surgery guideline in Starship Clinical Guidelines)

Diabetic ketoacidosis (DKA)

DKA is rare in CFRD but it can still happen. DKA should be managed according to Starship Clinical
Guidelines.

Transition

Transfer to the adult services, and the preparation for this transition is done over a number of
years. It coincides with the process of transition which occurs with regard to CF per se.

Other practical aspects

Pharmacy has supplies of most common insulins, pens and needles but not in every presentation
there is available, so check before writing the prescription. Glucose meters are not routinely held
by pharmacy. Insulin prescribing is a frequent source of drug errors. There are a lot of very similar
sounding insulins, make sure you get the right one. Remember to be clear about time- better to
write “before breakfast” than a time if that’s what you mean. Never abbreviate units to u or iu
because this can be misread as 0 or 10 and generate some quite big errors.
If a patient has shared care, there may be a local paediatric diabetes team. In addition many GPs
run diabetes clinics and get all their patients to come. The team at Starship have experience with
CFRD and it is thought preferable for CFRD care to be based there. Families are given all our
contact details and a lot can be done by phone. If a child with diabetes is travelling overseas they
need a letter saying that they are travelling with insulin, needles and glucose testing equipment
(this can be added to the letter about their CF).

There are strict rules covering driving and diabetes. Patients applying for a licence must declare
their diabetes and must get medical confirmation that they are well controlled.

10.2 Growth and puberty

10.2.1 Growth

Average birth weight is slightly reduced in CF and growth rate (weight and length) is reduced in the
first year of life, mainly because of impaired nutrition. Once the diagnosis is made and nutrition is
improved, catch up growth usually occurs. Height velocity in early childhood is normal although CF
individuals are on average 0.5 SD’s shorter than the normal population. In later childhood the
height deficit increases. This is mainly due to delay in puberty, with worsening lung function and
the onset of CF related diabetes contributing. Adult height is usually within the normal range for the
population but reduced compared to mid parental height.

Pituitary function (growth hormone (GH), gonadotrophins, ACTH) is normal in CF. Chronic
infection, nutritional factors and steroid treatment result in GH resistance and can also reduce GH
secretion. Gonadotrophin and sex steroid secretion is normal during puberty in CF but the changes
tend to be delayed compared to the normal population. Boys reach normal testicular volumes in
puberty despite the majority having azoospermia.

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Editor:           Dr Raewyn Gavin                                  Date Issued:         August 2008
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                                              CYSTIC FIBROSIS
Patient monitoring

    •     Height (measured with a stadiometer) and weight should be recorded at every clinic visit
          (minimum every 3 months) and plotted on the standard growth centile charts.
    •     In children under 2 years, head circumference (OFC) should be plotted.
    •     Assess the pubertal stages in older children and adolescents:
    •     Girls:
              o    Ask if pubic hair is present
              o    Is there any breast development (part of chest examination)
              o    Ask whether periods have started
    •     Boys:
              o    Ask if pubic hair is present
              o    Has voice broken

Who to be concerned about
The following groups may need further assessment or investigations:
    •     anyone whose relative position in terms of height or weight is falling (they are not
          maintaining their centile position)
    •     children who are very short (below 0.4th centile) even if they are maintaining their relative
          position
    •     children who seem very short for their midparental height (>2 s.d. below the mid parental
          centile)
    •     delay of puberty. 98% of normal girls have started pubertal development (Tanner breast
          stage 2) by 13.7 years and 98% of boys have started development (testicular volume over
          4 mls) by 14.2 years
Assessment

Look for factors related to CF, which may impact on growth:
    •     nutrition - intake or malabsorption
    •     chronic infection
    •     impaired glucose tolerance or CF related diabetes
    •     steroid treatment
    •     pubertal delay
    •     coeliac disease
Feeding behaviour problems are common in younger children and input from clinical psychologist
may be valuable. Supplemental feeds should be started early.




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Editor:             Dr Raewyn Gavin                                  Date Issued:         August 2008
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                                            CYSTIC FIBROSIS

Consider checking for non-CF related causes:
    •     hypothyroidism
    •     Turner syndrome (this is not always associated with clinical features and it is worth
          checking karyotype if a girl is very short and this has not been done before)

Consider GH deficiency if there is continuing poor height velocity with no other explanation.
Investigation requires stimulation testing and other causes should be excluded before this. There
have been a number of studies of the use of GH in CF patients (without GH deficiency) which have
demonstrated short term anabolic benefits, but there is no evidence for increased adult height.

Referral may be helpful for the following groups:
    •     Reduced height velocity or short stature, which does not seem to be caused be CF related
          problems.
    •     Children who are so short or growing so badly that they seem to be heading for a much
          reduced adult height. An assessment of growth may be helpful even if the clinical issues
          underlying this are clear and cannot be altered.
    •     Concerns by family or child about height.
    •     Pubertal delay. Adolescents may not express their level of concern about this very clearly
          so consider referral even if they do not seem very bothered.


Bone age is a way of looking at how much growth there is still to come. There are ways of
calculating adult height potential from bone age but they are not very accurate and likely to be
overoptimistic in CF patients. Bone age is not likely to be helpful in children under 4 years.
Assessment of bone age is operator dependent and results are more likely to be helpful if the
score is assessed by someone with sufficient experience.

10.2.2 Puberty

Pubertal delay is common in CF and is related to nutritional factors and infection. Pubertal
development will occur eventually but can be very late in those with the most significant medical
issues. Presentation may be with short stature or with concerns about development. There is no
need to wait for an age limit before referring. Delayed puberty does not result in any benefit in
terms of adult height. Delayed pubertal development has been found to contribute significantly to
the psychological problems suffered by adolescents with CF.

Assessment of pubertal delay
    •     Height & weight.
    •     Tanner staging.
    •     Bone age if there are concerns about height.
    •     Gonadotrophin and sex steroid levels may be low even if the child is in puberty already and
          are not likely to help.



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Editor:           Dr Raewyn Gavin                                   Date Issued:         August 2008
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                                              CYSTIC FIBROSIS
Treatment of pubertal delay

Individuals with the most significant medical problems are the most likely to have delayed puberty.
Any nutritional problems should be addressed, and CF related diabetes should be excluded as a
contributory factor. Growth during puberty can be adversely affected by nutritional problems,
infection and steroid treatment; this can reduce the increment in height achieved during this phase
of growth. It may be appropriate to delay treatment if there is a realistic chance that medical status
can be improved so that growth occurs with less adverse factors. Often it is unlikely that any
significant change will occur (and things might get worse) and it is reasonable to go ahead with
treatment to induce puberty even if optimum growth is unlikely.

Potential benefits of treatment

    •     Psychological and social.
    •     Height.
    •     Bone density - Bone density reaches a peak during puberty as a result of sex steroid
          action. CF patients are at increased risk of osteoporosis and it makes sense to optimise
          bone density at this point.

Treatments available

Patients should be referred to Starship Endocrine Team. Treatment to induce puberty mimics the
gradual rise in sex steroids during normal puberty and aims to complete growth and development
over about 2 years. Some individuals start to develop in spontaneous puberty after a few months of
treatment and medication can be stopped. There is no harm in stopping treatment at any point but
if spontaneous puberty does not occur, it usually makes sense to take the individual to nearly adult
height and development before stopping and reassessing endogenous function. Given in these
doses treatment does not have an adverse effect on adult height.

    •     10.2.2.1 Steroid treatment for the induction of puberty

Females
    •     Increasing doses of oral ethinyloestradiol:
    •     2 or 2.5 µg ethinyloestradiol daily for 6 months (either 2µg tablets or one quarter of a 10µg
          tablet)
    •     5 µg ethinyloestradiol daily for 6 month
    •     10 µg ethinyloestradiol daily for 6 months
    •     15 µg ethinyloestradiol daily for 6 months
    •     20 µg ethinyloestradiol daily for 6 months
    •     Adding in progesterone when 15 µg ethinyloestradiol is given or before this if there is
          spotting, using: levonorgestrel 30 µg daily or norethisterone 5mg daily for 7 days out of
          every 28 days
Males
Increasing doses of intramuscular depot testosterone esters as Sustanon 100 (100mg in 1ml)
    •     50mg IM every 4-6 weeks for 6 months
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Editor:             Dr Raewyn Gavin                                  Date Issued:         August 2008
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                                            CYSTIC FIBROSIS
    •     100mg IM every 4 weeks for 6 months
    •     100mg IM every 3 weeks for 6 months
    •     100mg IM every 2 weeks for 6 months


There are currently no reliable data to guide pubertal induction with topical sex steroids in males or
females.

Oxandrolone is a weak androgen with growth promoting effects. It can stimulate growth in boys
who are in early puberty but have not started their pubertal growth spurt. The effect on growth rate
is less than with sex steroids and there is little effect on development or appearance.
Dose is 2.5mg oral, once daily for 3 months.

10.3 Bone Metabolism

Increasing survival of CF adults has thrown up the finding of low bone density in at least one third
of young adults. Clearly nutrition has a crucial role but latterly the impact of other factors has
become apparent. The definitions of ‘osteoporosis’ and use of bone mineral density in children and
adolescents is unclear.

The amount of vitamin D in multivitamin preparations is probably insufficient and whilst
measurements of blood vitamin D levels are not routinely low, the comparison with the correct
normal range is controversial and the biggest variability appears seasonal. We therefore prefer to
use vitamin A + D capsules to ensure appropriate intake.

The role of vitamin K in osteoblastic activity has also become more apparent and indeed may be a
prime factor in osteoporosis. Studies show vitamin K levels may to be low in CF patients, including
those who are pancreatic sufficient. Vitamin K in multivitamin preparations is minimal and so we
have recently begun recommending oral water soluble vitamin K (menadiol) at a dose of 10mg/day
in those able to swallow tablets and certainly over aged 8 years for both males and females. It is
hoped this will lead to stronger bones in our adult patients. Drinking milk (especially adolescent
girls) and weight-bearing exercise should be encouraged.

Bone densitometry (DEXA scans) will be measured in patients considered to be at increased risk
of developing osteoporosis, on alternate years. These would include those who have frequent oral
steroids (particularly those with chronic ABPA), those on high dose inhaled corticosteroids,
diabetics and those with FEV1<50% predicted. An abnormal scan should be repeated in 6-12
months, as the trend is important. A normal one should be repeated in 2 years. Treatment will
include attention to diet, calcium intake, reduction of steroid use and encouragement of
exercise/mobility.




Author:           Drs Vyas, Byrne, Jaksic & Jan Tate               Service:             Paediatric CF Team
Editor:           Dr Raewyn Gavin                                  Date Issued:         August 2008
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                                            CYSTIC FIBROSIS

          Non-
11. Other Non-pulmonary complications of CF
11.1 ENT

11.1.1 Nasal polyps

Occur in about 10% of children and up to 40% of adults with CF.
Uncommon < 5 years and onset is generally between 8-10 years.
Aetiology is uncertain but may be related to infection, allergy, immune factors, altered secretions
and abnormal cilia. There is also an association with chronic sinus infection.
Usually asymptomatic.
Can result in chronic nasal obstruction (which increases airway resistance and may lead to mouth-
breathing).
Can also cause headaches and impair smell and taste.
Chronic rhinitis develops which can increase the incidence of pulmonary infections.
Diagnosis is made by simply looking up the nose with a light but sometimes it is difficult to
differentiate polyps from inflamed turbinates.

If troublesome:
Initial treatment is usually a steroid nasal spray such as fluticasone (Flixonase) or budesonide
(Butacort) Flixonase is not funded, price varies with pharmacies, approx $26 . Butacort 50 is
approx $10 / Butacort 100 has part charge.
Anti- histamines are of limited value.
If unsuccessful, surgery should be considered, but due to the high recurrence rate (60- 90%),
multiple procedures may be necessary.
Oral steroids are occasionally used for severe multiple recurrent polyps.


11.1.2 Sinusitis

Although almost all children with CF have chronic paranasal sinusitis, only 1% are symptomatic.
X-ray of the sinuses is of little value, as over 92% of all children with CF will have opacification of
the maxillary, ethmoid and sphenoid sinuses. Initially, opacity is due to retention of thick secretions
but later it may be due to polyposis within the sinuses. The frontal sinuses rarely develop in
children with CF, probably due to early onset of sinusitis, which prevents pneumatisation.
Chronic sinusitis is commonly associated with nasal polyposis.
Sinusitis may cause headaches, particularly on tilting the head forwards. Other symptoms are
related to chronic nasal obstruction (mouth-breathing, snoring, loss of sense of smell) and purulent
drainage (postnasal drip, constant throat-clearing, halitosis).
Long-term oral antibiotics may be of value (3-6 weeks). Oral metronidazole may improve halitosis.
Sinus washout is rarely successful, as the secretions are thick and tenacious; occasionally, more
radical surgical drainage procedures are necessary to alleviate symptoms.




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Editor:           Dr Raewyn Gavin                                  Date Issued:         August 2008
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                                            CYSTIC FIBROSIS

11.2 Arthropathy

Arthropathy may occur in up to 10% of children with CF and the mean age of onset is 13-20 years.
Cystic fibrosis arthropathy (CFA) is a specific condition, which may be immune-complex, mediated
and related to chronic pulmonary infection and inflammation. Typically, the children have an
episodic arthritis with pain and swelling, usually of large joints such as knees and ankles and
wrists. It is often accompanied by low-grade fever and there may be erythema nodosum or an
erythematous rash or purpura. Joint x-rays are usually normal. Episodes tend to settle
spontaneously after 3-4 days and respond well to non-steroidal anti-inflammatory drugs (e.g.
ibuprofen). Intensification of chest therapy may also help control joint symptoms. Beware renal
toxicity when using IV aminoglycosides in those on regular ibuprofen.

Some of the children with arthritis and advanced lung disease have features of hypertrophic
pulmonary osteoarthropathy (HPOA), this occurs in 2-7% of CF patients with a median age of
onset of 20 years. In this condition, as well as arthritis, which is often accompanied by joint
effusions, there are features of periostitis. The latter consists of tenderness and pain over the long
bones with periosteal elevation on x-ray. Periosteal changes may also be noted on radioisotope
bone scan. HPOA is seen in patients with more severe lung disease and worsens during chest
exacerbations. Anti-inflammatory drugs may be necessary.

Occasionally, sero-positive rheumatoid arthritis occurs co-incidentally in CF. It may require
treatment with anti-inflammatory agents, steroids and regular infusions of immunoglobulin. Finally,
it must be remembered that ciprofloxacin can cause arthropathy in both children and adults with
CF. Onset of symptoms may occur between three weeks and two months but tend to respond
within two weeks once the drug is stopped.

11.3 Pseudo-Bartter’s syndrome

An uncommon cause of metabolic alkalosis that has been seen as a presenting feature of CF as
well as a complication in those with known disease. It is accompanied by chronic salt depletion and
sometimes failure to thrive without severe dehydration. Principal findings are hypokalaemic
hypochloraemic metabolic alkalosis, sometimes with hyponatraemia. Check venous sample in
blood gas machine for bicarbonate. However after salt replacement, the metabolic abnormality
resolves and weight gain follows rapidly. Treatment is with sodium and/or potassium chloride
supplements, which may be required for many months.

Unexplained failure to thrive should always have urinary electrolytes checked, a spot urine Na+
<20 mmol/l indicates low total body sodium that needs correcting. A serum potassium at the lower
end of the normal range may still be associated with body depletion.




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Editor:           Dr Raewyn Gavin                                  Date Issued:         August 2008
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                                             CYSTIC FIBROSIS

11.4 Infertility

“What they don’t tell you – A young person’s guide to sexual and reproductive health issues in CF”.
Professor Susan Sawyer and the team at Adolescent Health, Royal Children’s Hospital,
Melbourne, Australia. This book is a very helpful resource for teenagers with CF.

Although it should be assumed that all males are infertile, this is not necessarily the case and so
male contraception must be strongly encouraged. There is also the risk of sexually transmitted
diseases if condoms are not used. Patients should be counselled about this.

The age at which infertility is discussed will vary according to the patients maturity. Occasionally
parents are reluctant for the issue to be discussed. Research amongst young adult males with CF
suggests that they generally feel that they ought to have been told of their infertility by the age of
15 or so. Parents should be encouraged to discuss this with their sons particularly around the time
of puberty. However we consider it essential that all boys are aware of their infertility prior to
transfer to adult services. The annual review is often a good time to do this. It is important to
stress to them that infertility is not the same as impotence and that sexual performance is
unaffected (although the volume of ejaculate is reduced). There are reports of CF men fathering
children after microsurgical epididymal sperm aspiration and intracytoplasmic sperm injection
(ICSI).

Girls are not infertile. Their fertility, and ability to conceive may be compromised by their degree of
well being. The average duration of survival for a woman with CF, who has given birth is
approximately 10 years. Whether this is related to the pregnancy per se or not is unclear. As with
boys, contraception and sexual health should be discusses.

11.5 Stress Incontinence

The physiotherapist will discuss this issue at time of annual review.

Urinary incontinence is a condition where certain activities (e.g coughing, laughing, jumping) lead
to a leak of urine. This can be anything from a slight dribble to a complete emptying of the bladder.
It is known that many women with CF are affected by urinary incontinence and it has become
increasingly recognised that young girls may also be affected.

A survey found 1 in 3 girls aged 11-17 years answering the survey had a problem at times. For
many (if not all) girls this is rather embarrassing and many do not want to talk to their parents about
it, and especially not to male doctors! It is more likely they will discuss this with female members of
the team. We can arrange for the girls to be seen by a gynaecologist or physiotherapist, as
sometimes simple ‘pelvic floor exercises’ can help.




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Editor:            Dr Raewyn Gavin                                  Date Issued:         August 2008
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                                            CYSTIC FIBROSIS

12. Transplant Assessment

Paediatric lung transplants are performed at the Royal Children’s Hospital. Melbourne. Those over
the age of 16, (even if they are still receiving paediatric care) may be accepted onto the adult lung
transplant programme at Auckland City Hospital.

In the past, most transplants performed in CF children were heart / lung (HLT) with the CF patient’s
heart being used in a domino procedure for another patient. More recently, double lung transplant
(bilateral, sequential lung transplant) are being done more often. Although living lobar transplants
(a lobe each from two relatives, most commonly parents) have been performed in adults their
utilisation in paediatric transplant is not commonplace.

Consideration of a child for HLT assessment should be based on the individual patient, and is best
performed in a multi-disciplinary fashion. The timing for referral for transplant assessment is not
clearly defined. It is guided by a few clinical criteria. If transplant is being considered, we would
suggest discussion with the Starship Paediatric CF team in the first instance, as it may be that
there are other treatments which can be instigated to avoid the need for referral.

12.1 Criteria for transplant assessment

    •     Significantly reduced lung function, usually with FEV1 <30% predicted. May include rapidly
          declining FEV1 even if still >30% predicted.
    •     Severely impaired quality of life.
    •     Daytime Oxygen-dependent (resting SaO2 < 90%).
    •     Child and family committed to the idea.

Children with an FEV1 of 30% predicted have a 50% chance of dying within the next two years.
The risk of dying from a lung transplant is approximately 50% for the first two years after receiving
one. Thus, the time to perform a transplant is when the risk of dying from the complications of a
transplant is less than the risk of dying without one. Transplant can be discussed at any age, but
the timing of referral to a transplant centre is something which should be done before a child
reaches a degree of pulmonary morbidity which necessitates a transplant i.e. this is best done
before the child’s health is very seriously compromised. It is suggested that if a child is considered
as a possible lung transplant recipient, he/she should be discussed with one of the CF consultants
at Starship. A transplant will not be considered until all the other possible causes of failing lung
function (nutrition, physio, CFRD, ABPA, etc) have been excluded or adequately treated. A child’s
suitability for transplant assessment can be discussed with Dr Mark O’Carroll (Adult CF Specialist,
and Transplant Physician, Auckland City Hospital). However, clinical assessment and workup will
then need to be done by the paedatric CF team, and for this reason it is perhaps logical to discuss
the child with them (Paediatric team) initially.




Author:           Drs Vyas, Byrne, Jaksic & Jan Tate                Service:             Paediatric CF Team
Editor:           Dr Raewyn Gavin                                   Date Issued:         August 2008
Cystic Fibrosis   Page:                                             68 of 100
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                                             CYSTIC FIBROSIS

12.2 Contra-indications

The following contra- indications differ between centres, and may be subject to change over time
with the availability of e.g. newer antibiotics and increasing surgical expertise. The decision will be
influenced by the presence of multiple problems within an individual child.

Absolute
    •     Other organ failure (excluding hepatic when a lung/liver transplant could be considered).
    •     Untreated Mycobacteria tuberculosis.
    •     Invasive pulmonary aspergillosis.
    •     Non-pulmonary infections e.g. Hepatitis B or C, HIV.
    •     Malignancy.
    •     Child does not want the procedure.
Relative
    •     Long-term high dose steroid treatment (>5mg/day).
    •     Previous thoracic surgery - pleurodesis will make the procedure more difficult and should
          be discussed with the surgical team.
    •     The presence of multi- resistant organisms e.g. some genomovars of B cepacia complex,
          MRSA, panresistant P aeruginosa.
    •     Severe osteoporosis.
    •     Psychosocial issues/ lack of family support.
    •     Non-adherence to current treatment.

Transplantation is so familiar to many people now from TV, newspapers etc, which tend to be
biased towards successful outcomes, that it is often perceived as a miracle cure. It is therefore
important when discussing the issues with the family and child, that as well as the potential
benefits, the following negative points should be addressed (these will be addressed at the
assessment meetings, but should be raised early with families):
    •     Acceptance onto the waiting list does not guarantee a transplant. Due to a shortage of
          donors about 50% of patients will die before organs become available. The time spent with
          a pager waiting for organs will be extremely stressful (uncertainty, false alarms etc).

    •     Heart/lung or lung transplantation is not a complete cure for CF, it is palliative. After the
          operation, invasive procedures including bronchoscopy and biopsies are likely to be
          required. In addition, unless complete eradication of reservoirs of infection has been
          successful (which almost never occurs due to chronic infection of sinuses), there is
          potential for bacterial infection of the transplanted lungs, which may make ongoing
          antibiotic therapy and physiotherapy necessary.

    •     Transplantation has little impact on the non-pulmonary manifestations of the disease (ie
          enzyme replacement and other therapies need to be continued), although there may be
          nutritional benefits in the medium term. CF-related diabetes may worsen.

Author:            Drs Vyas, Byrne, Jaksic & Jan Tate               Service:             Paediatric CF Team
Editor:            Dr Raewyn Gavin                                  Date Issued:         August 2008
Cystic Fibrosis    Page:                                            69 of 100
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                                             CYSTIC FIBROSIS
    •     Problems associated with transplantation include early rejection, severe sepsis related to
          immunosuppression and later development of obliterative bronchiolitis (OB). OB can
          eventually lead to severe respiratory impairment, and is difficult to treat successfully.

    •     Survival in adults, after a lung transplant, has improved over the last decade. In New
          Zealand, adult lung transplant recipients have an 80% survival at one year, and 66%
          survival at two years (i.e. 1/3 of recipients will die within 2 years of transplant. In contrast
          survival figures for children have not shown the same improvement in survival rates, nor
          have they appreciably altered in the time up to 2001. This means that 50% of children who
          undergo lung transplant will die within 2 years of the operation. More recent data are not
          available.




13. Miscellaneous

13.1 Preparation for surgery

General anaesthesia commonly leads to lung atelectasis (hence post-operative fever), even in
healthy patients, a situation which is exacerbated in children with CF. We therefore routinely give
peri-operative antibiotics to all CF children undergoing general anaesthesia, however good their
lung function. This includes portacath insertion, gastrostomy insertion/changes, ENT surgery such
as polypectomy, tonsillectomy and also gastrointestinal endoscopy. Many of these procedures are
carried out at Starship Children’s Hospital but it is still important to ensure the surgeons and
gastroenterologists are aware of this when arranging the procedure – always give antibiotic
recommendations (IV vs oral, and choice of drug) in the referral letter.

Minimal lung disease - (especially minor surgery) can receive high dose oral antibiotics for 48
hours pre- and 48 hours post-op.

Moderate lung disease - (most children) will receive IV antibiotics for 48 hours pre-op and at least
48 hours post-op. This may be started in shared care hospital.

Severe lung disease may need 7-14 days IV antibiotics pre-surgery and 7 days postoperatively,
and these would be given at Starship. Choice of drug is determined by the latest sputum or cough
swab culture. The CF consultant will advise over the exact choice, which is usually ceftazidime and
tobramycin. It is also important that chest physiotherapy is strictly adhered to during the admission.

Bronchoscopy – no antibiotics beforehand. Consider 48 hours IV ABs post-procedure if significant
volumes of sputum present.


All CF patients undergoing general anaesthesia at Starship must be discussed with on call CF
consultant.




Author:            Drs Vyas, Byrne, Jaksic & Jan Tate               Service:             Paediatric CF Team
Editor:            Dr Raewyn Gavin                                  Date Issued:         August 2008
Cystic Fibrosis    Page:                                            70 of 100
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                                            CYSTIC FIBROSIS

13.2 Immunisation

We recommend that all routine childhood vaccinations are given at the usual times and should be
arranged by the general practitioner.

Influenza immunisation for children over 6 months of age is mandatory and is also arranged by
GPs. However families must be reminded and it is also useful to put a reminder in to the clinic
letters to GPs in early autumn. The vaccines are usually available in April each year. If a child is
receiving it for the first time, a 2nd dose is repeated 4 weeks later; otherwise it is a single injection
each year. The vaccine is inactivated (killed) and it is given by deep subcutaneous or intramuscular
injection. There are several products available, which are licensed for children over 6 months (see
New Zealand Immunisation Handbook 2006). Egg hypersensitivity is a contraindication. Parents
should also receive the vaccine.

Pneumococcal vaccine is not routinely recommended, as Pneumococcus is not an organism
commonly associated with CF. Prevenar is to be made available as part of national immunisation
policy, including catch up for those under 2 years. For older children however, if parents are keen,
we would have no objection (Pneumovax is used for children >5 years). It is of course mandatory
for children who have had a splenectomy.

Palivizumab (Synagis) is a monoclonal antibody available as passive immunisation against
respiratory syncytial virus (RSV). It is given as 5x monthly intramuscular injections. There is
currently no evidence for benefit in CF & there are still no results from US trial, with the company
not planning to alter its license to include CF.

13.3 Chicken Pox
Although the literature is scarce, it has been documented that varicella- zoster infection can lead to
infective pulmonary exacerbations and that early treatment with acyclovir may prevent pulmonary
deterioration.

Children who are not on oral corticosteroids. If the diagnosis of chicken pox is confirmed and we
are contacted early in the course of the illness, we suggest a one week course of oral acyclovir in
those children who are unwell and particularly those who are known to have significant chest
disease.

If however we are informed late in the course of the illness or the child really has mild chicken pox
only with a few spots then acyclovir is not warranted. This is particularly the case in CF children
who are well from the CF point of view.

If children are on oral corticosteroids or have recently been on them, then the Guidelines as
outlined in the BNFc should be followed:

Chicken pox contacts should only receive Varicella-Zoster Immunoglobulin (VZIG) if they have not
had chicken pox previously:
    •     and are currently taking oral steroids
    •     or within the last 3 months have been taking the equivalent of 2 mg/kg/day prednisone for 1
          week
    •     or within the last 3 months have been taking the equivalent of 1 mg/kg/day prednisone for 4
          weeks.
Author:           Drs Vyas, Byrne, Jaksic & Jan Tate                Service:             Paediatric CF Team
Editor:           Dr Raewyn Gavin                                   Date Issued:         August 2008
Cystic Fibrosis   Page:                                             71 of 100
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                                             CYSTIC FIBROSIS
VZIG is given by deep intramuscular injection at the following doses:

                    0-5 years                                                250mg
                    6-10 years                                               500mg
                   11-14 years                                               750 mg
                  over 15 years                                              1000mg

We would also recommend that we see those children and if a chicken pox rash still develops in
these children who are at risk of serious disease, IV acyclovir is indicated.

13.4 Overseas Travel

Before travelling patients and parents are encouraged to plan the necessary arrangements to
accommodate the child’s CF needs within the family holiday. Parents should consider the following
issues (although some may not be relevant to their particular trip)
    •     Travel Insurance: This will cover costs of admission to a hospital if required, but will also
          allow the child to be repatriated for medical care, if the family so desire.

    •     In-flight oxygen: we are not able to perform flight assessments at present. Parents of
          children who have oxygen saturations that are <94% in room air, or who have overnight
          oxygen therapy should make arrangements with the airline they are flying with to provide
          oxygen on board. The airline may require a letter, or form to be filed in by a doctor in the CF
          team. This can be done, but we require at least a months notice.

    •     Consideration of the facilities at the destination is important. Some medicines require to be
          kept in a refrigerator (e.g. Dornase alpha). The family should ensure they will have access
          to clean water, an area for preparing medications, a fridge etc. It is prudent to investigate
          the local medical facilities, and the location of the nearest Paediatric CF centre (if
          applicable). If the child becomes ill on holiday they may be able to be treated by the local
          medical team, or they may require to be transferred to the Paediatric CF centre, which may
          be some way away.

    •     Carry information to be able to contact with the home CF team. Phone number of the CF
          Nurse, and an email address or fax number should the local medical team require it.

    •     Many children with CF use electrical equipment (nebulisers, overnight feeding pumps etc).
          It is important to ensure that items that require an annual service do not require one prior to
          going away. If travel abroad is planned, it is important the family ensure the machine will
          function on the mains electricity supply of the destination. Plug adaptors will likely be
          needed. The machine manufacturers may be able to advise if they have a local branch or
          franchise where a replacement or spare parts can be obtained if the machine breaks down.

    •     The parents will need to calculate the required amount of medications to take with them. It
          is normally suggested that an additional weeks medications be taken in case of travel
          delays etc. If a prolonged trip is planned, it may be appropriate to arrange for regular review
          with the local CF centre. This will need to be arranged beforehand, to allow the Starship
          team to prepare clinical information, and for the local team to find a free clinic appointment.



Author:            Drs Vyas, Byrne, Jaksic & Jan Tate               Service:             Paediatric CF Team
Editor:            Dr Raewyn Gavin                                  Date Issued:         August 2008
Cystic Fibrosis    Page:                                            72 of 100
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                                             CYSTIC FIBROSIS
    •     Some children may require a two week IV antibiotic ‘tune up’ prior to departure. This needs
          to be indicated to the team a week or two before this to allow all the necessary
          arrangements to be made.

    •     If the trip is to a hot location, salt supplements (600-1800mg/day) may be required.

    •     If the destination in sunny, then sun block may be required, especially if the child is
          currently on, or has recently finished a course of ciprofloxacin.

    •     If CF medication, feed and /or equipment are to be taken, letters from the CF team verifying
          the medicines, feed or equipment for the airport security, and for Customs and Excise will
          be required. These need to be requested at least 2 weeks prior to departure to allow time to
          prepare them.

    •     If a specific clinical letter is not requested, the family is encouraged to take the last few
          clinic letters with them – as a source of information for a local team.

    •     Children who require enteral feeds, and who are likely to be in transit for a prolonged period
          of time (>8 hours) will need to have their feed with them on the plane (and not in the hold).
          Insulin (where applicable) also needs to be taken on board, and stored in a cool place. This
          will require further verification for security (esp if flying to/from USA or UK). It is strongly
          suggested that the parents establish if there are any specific items banned by local security
          arrangements at the destination.

    •     It is important to point out that cross infection amongst passengers is a recognized
          complication of airline flight, but mainly with long haul (>6 hours) flights. It is almost
          impossible to ensure that no-one else on a flight does not have a significant respiratory
          pathogen in their sputum. This information should not prevent families from travelling, but it
          is important to relay this, so individual families can reach their own conclusion about
          whether this is an acceptable risk or not.

    •     CF is not a contraindication to any of the vaccinations recommended for travelling. If a child
          is on prednisone, or has recently finished a prolonged course, this may compromise
          vaccine effectiveness. This issue should be discussed with the GP, or other authority on
          vaccination.

    •     For families whose child is going on charity flights (e.g. Starlight Foundation) there is a
          need to inform the charity of any risk of cross infection. This is to prevent their child
          catching a respiratory infection, and also to prevent others acquiring their child’s respiratory
          organisms.

    •     For long haul flights routine precautions about staying well hydrated will apply. Chest
          physiotherapy should not be left for too long. If it is not possible during travel, it should be
          done as soon as possible once the destination is reached.

    •     For children with CFRD, advice about the timing of insulin during long haul travel may be
          needed prior to departure, and should be sought from the Paediatric Diabetes Team.



Author:            Drs Vyas, Byrne, Jaksic & Jan Tate               Service:             Paediatric CF Team
Editor:            Dr Raewyn Gavin                                  Date Issued:         August 2008
Cystic Fibrosis    Page:                                            73 of 100
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                                            CYSTIC FIBROSIS

14. Terminal Care
Fortunately death in childhood is an unusual event amongst our CF population. The few that do
occur tend to happen in the home rather than at hospital. The overriding principal is that the child’s
comfort and wishes must come first followed closely by those of the immediate family. The
management of a dying child needs to be flexible so as to cater for individual family needs and
reviewed frequently. We believe that communication amongst the CF team and health
professionals involved is critical and must be consistent so as not to confuse the family.

The issue of terminal care will be discussed with the parents by the child's consultant. These
discussions will include the child if possible and the family / whanau. We would encourage an
honest and open approach at all times, although we would also consider the wishes of the child
and his or her family / whanau about sharing information.

Information needs to be honest, and simple. It is sometime important to explain the anticipated
time to death, although it is often best to express this in general terms of “a day or two,” within the
next two weeks,” “within the next few months”, or “within this next two years” etc. Parents often
want a more definite timeframe, but the temptation to give one should be avoided. It is often
important to discuss issues around mode of dying, location of dying, and actions after death. The
child’s opinion should be paramount in this (where appropriate). If time allows it is also important to
encourage the child to have a say in preparations to mourn (funeral arrangements etc). This may
include selecting music or readings, location, etc.

It is vital not to avoid answering a child’s questions directly. If they sense a degree of “cover-up”
(from staff or parents) they may often tacitly collude with this, even though this is not in their best
interests, nor those of the family, to avoid discussing these painful subjects. Remember, all families
with a child with CF will have been expecting this from almost the time of diagnosis.

Children and families should be given a choice as to where their child receives terminal care. This
should include staying at Starship, at home, or in a hospice. However in New Zealand most
children prefer to be cared for by their families at home.

The Starship Palliative Care Team is available to offer advice and support for professionals and
families. This includes supporting end-of-life care outside hospital, in liaison with community
services.

Discussions and decisions around resuscitation are reviewed regularly with families. As levels of
resuscitation can change depending on the child’s condition and family situation. These
discussions need to focus on what can be done rather than what will be done.

Should the family have decided to care for their child at home, the local paediatric community
team, as well as the general practitioner will take the lead role in the care. The Starship CF Team
would offer our full support, including liaison through the Starship Palliative Care Team.

Starship support services may continue to provide physiotherapy, pharmacological advice and
medications, humidified CPAP etc.

Medication for symptom relief is individualised for each child and consultative support and advice is
available from the Starship Palliative Care Team. Medications may include analgesia, anxiolytics,
and / or antiemetics etc.

Author:           Drs Vyas, Byrne, Jaksic & Jan Tate               Service:             Paediatric CF Team
Editor:           Dr Raewyn Gavin                                  Date Issued:         August 2008
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                                            CYSTIC FIBROSIS

Death of a child
A medical professional will be required to certify the death. A Death Certificate is legally required
to be completed, however this does not need to be done immediately. The family / whanau may
require some time alone with the child before this is done. The family can contact the Funeral
Director when they are ready to do so. This may occur before the child dies.

Post Mortem
Information gained from a post mortem (even if the child‘s death can be certified) may well help
others children in the future. It is important to discuss post mortem with the parents before the child
dies, to allow them time to think about the issue.

Patient dies in Starship
(See also Deaths not reported to a Coroner Guideline)
Inform Duty Manager for access to protocol and if needed contact the Starship Palliative Care
Team for advice. The Respiratory Consultant on call that week should be made aware if a CF
patient is admitted, and is receiving end of life care. They should also be informed when the child
dies.

Patient dies at home
The GP should have been informed if a child has been discharged home for end of life care. The
GP will normally certify death. A multi-disciplinary team meeting (including the family, and patient
where appropriate) should agree a plan for when the child is at home. The community team will be
available to support the family during the time leading up to death.

Palliative Care Support
Any child and family with a palliative care need can be referred to Starship Palliative Care Team.
This is an outreach service. The team can be contacted through the Auckland Hospital switchboard
(09 3797440) and ask for the paediatric palliative care team.

Transport Home of a Child’s Body from Starship
Parents can take their child’s body home after death. A death certificate should be given to the
family before they leave, although the funeral director may be able to pick this up later.

A child’s body can be removed from the hospital at any time unless there is a legal requirement for
a post mortem. The family may wish for the child to go home or to a relative’s house.

Support for child and family / whanau during terminal illness
A child’s death is enormously distressing for their family / whanau who may need a range of
support which includes psychological, emotional, financial, spiritual and cultural. The child and their
family (including siblings) should be offered ongoing support.

Bereavement Counselling
All families are offered an appointment with a CF consultant, and the CF nurse at 2-3 months after
their child has died. This is to allow the parents (or other family members) the opportunity to
discuss any questions they may have about their child’s death. For children who lived outside
Auckland, but who have died at Starship, we would ask our colleagues at the host hospital to do
this. We are always happy to support them in this, and if necessary we will see a family when we
next attend an outreach clinic, or arrange to see a family in Auckland if they wish to travel up.



Author:           Drs Vyas, Byrne, Jaksic & Jan Tate               Service:             Paediatric CF Team
Editor:           Dr Raewyn Gavin                                  Date Issued:         August 2008
Cystic Fibrosis   Page:                                            75 of 100
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                                              CYSTIC FIBROSIS

15. Drug Formulary
NB: ALTHOUGH DRUG DOSES ARE GIVEN IN THIS SECTION, AND IN OTHER PARTS OF
THESE GUIDLEINES, WE HAVE NOT CHECKED ALL DOSES IN AN AUTHORITATIVE TEXT.
DO NOT PRESCRIBE ANY TREATMENTS BASED SOLELY ON THE DOSES GIVEN IN THIS
DOCUMENT. ALWAYS CHECK DOSES IN AN ACKNOWLEDGED SOURCE (E.G. CHILDREN’S
BNF, MELBOURNE PHARMACOPOEIA ETC)

15.1 Drugs for the respiratory tract

Prophylactic antibiotics (Starship does not currently use antibiotic prophylaxis routinely; this may
change in the future).

Oral

                                                                                      Syrup 250 (250mg amoxicillin/62.5 mg
  Augmentin     7.5mg/kg three             Use if Flucloxacillin not tolerated or
                                                                                      clavulanic acid per 5 ml)
                                              regularly grows H influenzae.
 Oral syrup and   times a day                                                         Syrup 125 (125 amoxicllin/31.25mg
                                         Tastes better than flucloxacillin but may
      tablet    (as amoxicillin                                                       clavulanic acid per 5ml)
                                         discolour teeth more than flucloxacillin.
                  component)                                                          Tablet 500 (500mg amoxicillin/125mg
                                                 Clean teeth after dose.
                                                                                      clavulanic acid)
                        15-40kg;                                                        Potential for hepato- and ototoxicity but
                                                                                               usually very well tolerated.
                       250 mg od
   Azithromycin                                     Consultant decision.              Not funded in the community, will need to
                         >40kg                                                           apply for exceptional circumstances
                       500 mg od                                                                        approval.
                                                                                          If S aureus a troublesome, regular
                                                   Give BEFORE meals.
   Flucloxacillin   10-20 mg/kg bd                                                        problem can use up to 2 grm bd –
                                                    Liquid tastes awful.
                                                                                                  Consultant decision.



Nebulised

                                                                                             Consultant decision.
                      <10 years
                                                                                     No need to use expensive liposomal
 Amphotericin B       5 mg bd;        Dilution: 50 mg in 10ml of water. Use
                                                                                      preparation unless cannot tolerate
  (Fungizone)                         1-2ml of this solution & dilute further
                                                                                             standard preparation.
    chronic           >10 years       with 1-2ml of water (min volume 3ml
                                                                                       Consultant will need to apply for
   aspergillus        10 mg bd                   for nebulisation).
                                                                                        exceptional circumstances for
                                                                                                     funding.
                       <8 yrs:         Make up with Normal Saline 0.9% to                 Double dose if given alone.
       Colistin      37.5mgs bd                       4mls                            The lower standard Colistin dose is
                                          Pre-dose with bronchodilator.              required if using the I-Neb nebulisers
  75 mg = 2MU          8 yrs +:           Give 1st dose in hospital with                       (see section 6.12).
                      75mgs bd           spirometry pre - and post-dose.               Volume for nebulisation is 4mls.
                       <2 yrs:
   Gentamicin         20 mg bd
                                          Dose can be doubled in certain
    (from IV           2-8 yrs:
                                                 circumstances.
    solution)         40 mg bd
                                           Blood levels not necessary.
                       >8 yrs:
                      80 mg bd




Author:             Drs Vyas, Byrne, Jaksic & Jan Tate               Service:              Paediatric CF Team
Editor:             Dr Raewyn Gavin                                  Date Issued:          August 2008
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                                             CYSTIC FIBROSIS
                                  Needs to be phenol free to reduce
   Tobramycin                           risk of bronchospasm.
   (IV solution)   80- 160 mg bd     Blood levels not necessary.
                                  Brand from Mayne Pharma/DBL is
                                 phenol free, reducing bronchospasm.
      TOBI           300 mg bd
   Preservative        during
                                                                                   Not currently funded or available in
       free        ALTERNATE
                                                                                                    NZ
   Tobramycin        MONTHS




Antibiotics for treatment of pulmonary exacerbations

Oral

                                Syrup 250 (250mg amoxicillin/62.5 mg
                15mg/kg every 8                                           Use if Flucloxacillin not tolerated or
   Augmentin                    clavulanic acid per 5 ml)
                hours                                                         regularly grows H influenzae.
 Oral syrup and                 Syrup 125 (125 amoxicllin/31.25mg
                (as amoxicillin                                         One month For S aureus, and H
      tablet                    clavulanic acid per 5ml)
                component)                                              influenzae etc.
                                Tablet 500 (500mg amoxicillin/125mg
                                                                        Care with CF liver disease
                                clavulanic acid)
                                Not funded in the community, Consultant
                   10 mg/kg od      will need to apply for exceptional  Ten days gives about 1/12 cover vs S
  Azithromycin
                   max 500 mg            circumstances approval.             aureus, H inf and Mycoplasma

                                        Good for H influenzae, some anti staph
                    15mg/kg/dose
       Cefaclor                                          action                                       One month
                    every 8 hours
                                        Only oral antipseudomonal agent.
                                       Photosensitising so warn patient re
                                     sunlight. High strength sunblock should
                                                                                           Two weeks.
                                         be used for 4 weeks after course
                    15 mg/kg/dose                                               Consultant decision to exceed this
                                                       finished.
                         bd                                                                  period.
  Ciprofloxacin                               Joint pains occasionally.
                   (max 1.5g daily)                                             Care should be taken if previously
                                    Milk will reduce absorption. Avoid milk for
                                                                                 used within previous 3 months
                                     at least 30 mins before and after taking
                                                                                 because of risks of resistance.
                                                    ciprofloxacin.
                                       Tablets are fully funded, liquid only
                                       available from hospital pharmacies.
                       7.5-15             Cheaper alternative to azithromycin.
                                                                                                      One month
  Clarithromycin    mg/kg/dose 12          Available as syrup 125mg/5ml and
                       hourly                 tablets 250mg, fully funded
                                  Use mainly for S maltophilia. Maintain
                   6 weeks–6
                                           adequate fluid intake
                 months: 120 mg
                                  Treatment should be stopped if blood
                        bd
                                   disorders or rashes develop. Advise
                   7 months–6
                                  patient/carer to report all rashes, sore                            One month
 Co-trimoxazole years: 240 mg bd
                                            throats and fevers.
                 7–12 years: 480
                                      Avoid in severe liver disease.
                     mg bd
                                 Available as co-trimoxazole 240mg/5ml
                13–18 years: 960
                                      combined product (8mg/ml of
                     mg bd
                                        trimethoprim component)

Author:            Drs Vyas, Byrne, Jaksic & Jan Tate               Service:             Paediatric CF Team
Editor:            Dr Raewyn Gavin                                  Date Issued:         August 2008
Cystic Fibrosis    Page:                                            77 of 100
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                                              CYSTIC FIBROSIS

                                           For S aureus. Give BEFORE meals.
                     40-50 mg/kg                                                                        One month
   Flucloxacillin                                  Liquid tastes awful.
                     every 6 hours

                                          Last line for MRSA or S aureus where
                                              patients have not responded to
                                           conventional agents e.g. high dose
                                           flucloxacillin, rifampicin, fusidic acid
                                                                                   Consultant will need to apply for
                     <12yr. 10mg/kg             Monitor FBC weekly.
                                                                                    exceptional circumstances for
                    (max 600mg) tds. Courses >28 days risk optic atrophy so
     Linezolid                                                                                 funding.
                                     pt having 4/52 or repeated courses must
                                                                                Liquid and tablets available in NZ but
                    > 12yr 600 mg bd have ophthalmic exam before starting
                                                                                             not funded.
                                     first course and every 2/12 after. Aim for
                                    2-week courses. Where possible patients
                                     warned to immediately report any visual
                                          changes, regardless of treatment
                                                     duration.



Intravenous

NOTE
  i)         Two antipseudomonal antibiotics from different classes (e.g. bacteriostatic and
             bacteriocidal drugs) should always be given. Exceptions to this are consultant
             decisions.
    ii)      High dose flucloxacillin is usually given by mouth as it ruins long lines and is well
             absorbed orally. This is automatically given when S aureus has been grown in the past
             year but for other patients discuss with the consultant on admission.
    iii)     Preferred blind starting combination is ceftazidime (or aztreonam) plus tobramycin (or
             gentamicin) plus oral flucloxacillin/augmentin. (Some evidence that tobramycin is
             superior to gentamicin in terms of MICs).
    iv)      Course length is always a minimum two weeks. Sometimes a third week adds an
             additional benefit.
    v)       Care with first doses as unexpected, severe hypersensitivity really does occur.
    vi)      Round doses up or down for ease of administration, especially for home IVABs.
    vii)     Antibiotics can impair liver and renal function. Care with underlying impairment.
    viii)    Children on home antibiotics should have aminoglycosides three times a day. Once
             daily aminoglycosides would require them to have a syringe driver at home.

                 30 mg/kg od.
                                           Infuse over 30 mins.
                   If previous                                              Only use if resistant to tobramycin or
    Amikacin                   Levels at 23 hours after 2nd dose (ie before
                   course had                                                            gentamicin.
                               3rd dose) must be < 3mg/l. Repeat at least
                 raised trough                                                Dilution: 0.9% sodium chloride.
 aminoglycoside                  every 7 days. If level raised, OMIT next
                  level reduce
                                          dose and remeasure.
                 dose by 20%
                 75 mg/kg tds
   Aztreonam                            No gram-positive activity.                  Usual dilution: water.
                      (Max 8
   Beta lactam
                    gms/day).
                                   Unexpected hypersensitivity on first
   Ceftazidime   50 mg/kg tds                                                       Usual dilution: water.
                                                exposure.
  cephalosporin (Max 9 g/day).



Author:             Drs Vyas, Byrne, Jaksic & Jan Tate                Service:             Paediatric CF Team
Editor:             Dr Raewyn Gavin                                   Date Issued:         August 2008
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                         not be assumed to be current. Please remember to read our disclaimer.



                                            CYSTIC FIBROSIS
                  20,000-25,000
                        U/kg tds
                                    Slow infusion over 30 mins. Measure renal
                  Use above total
                                              function every three days.                  Not a first line agent.
    Colomycin         daily dose
                                                                                  Avoid using with IV aminoglycosides
                   divided into 2
                                  May use up to 2MU tds if desperate in those       and amphotericin (renal failure).
    polymixin            doses
                                                         >40kg                    Usual dilution: 0.9% sodium chloride
                     (i.e.30,000-
                                                 Consultant decision
                    38,000 U/kg
                           bd)
                         10-12
                     mg/kg/day.
                    ONE DOSE Infuse over 30 mins. Levels at 23 hours after
                      Max dose:    2nd dose (ie 1 hour before 3rd dose) - must
   Gentamicin
                   600mg/day. If                      be <1 mg/l.
                                                                                  Usual dilution: 0.9%sodium chloride.
                        previous   Repeat at least every 7 days. If level raised,
 aminoglycoside
                     course had           OMIT next dose and re-measure.
                   raised trough                    See section 6.2a
                    level reduce
                   dose by 20%
                                     Infuse over 30 – 120 mins. Monitor FBC
                                                        weekly.
                                     Consultant decision only as courses >28       Use oral route wherever possible.
                  <12y:10mg/kg
                                      days leads to risk of optic atrophy. Pts     Otherwise convert to oral route as
     Linezolid     (max 600mg)
                                    having alternate monthly drug should have         soon as clinically indicated.
                           tds
                                   ophthalmic exam before starting first course Last line for MRSA or S aureus where
  oxazolidinone
                                    and every 2 months after. Where possible        patients have not responded to
                  12y: 600mg bd
                                    patients should be warned to immediately              conventional agents.
                                     report any visual changes, regardless of
                                                  treatment duration.
                  20 – 40 mg/kg                                                       Use ONLY if ceftazidime or
   Meropenem                                 Headache not uncommon.
                          tds.                                                            aztreonam resistant.
   carbapenem
                     Max 2g tds                                                           Usual dilution: water.
    Timentin =
                   80-100 mg/kg
    (ticarcillin/
                          qds.                                                            Consultant decision.
    Clavulanic                                  Infusion over 30 mins.
                                                                                  Kept for B cepacia and desperation.
        acid)
                   Max 3.2 gms                                                            Usual dilution: water
 carboxypenicilli
                          qds
          n
                         10-12
                   mg/kg/day in
                                  Infuse over 30 mins. Levels at 23 hours after
                    ONE DOSE
                                    2nd dose (ie 1 hour before 3rd dose) must
   Tobramycin         Max dose:
                                                      be <1 mg/l)
                    600mg/day.                                                    Usual dilution: 0.9% sodium chloride.
                                            Repeat at least every 7 days.
 Aminoglycosid        If previous
                                      If level raised, OMIT next dose and re-
          e          course had
                                                       measure.
                   raised trough
                                                    See section 6.2a
                    level reduce
                   dose by 20%




Author:           Drs Vyas, Byrne, Jaksic & Jan Tate               Service:             Paediatric CF Team
Editor:           Dr Raewyn Gavin                                  Date Issued:         August 2008
Cystic Fibrosis   Page:                                            79 of 100
                                            Starship Children’s Health Clinical Guideline
                    Note: The electronic version of this guideline is the version currently in use. Any printed version can
                          not be assumed to be current. Please remember to read our disclaimer.



                                            CYSTIC FIBROSIS
Antifungal therapy

                                        Must be used when treating ABPA with
                                      steroids, when taking steroids for whatever
                                         reason if aspergillus isolated, and for       See section 6.6 for length of
                                        symptomatic aspergillus infection. See        courses Poorly absorbed, use
                      1/12 - 12y:
                                                        section 6.6.                    liquid, on empty stomach if
   Itraconazole   5 mg/kg once daily
                                      Adrenal suppression also been seen when          possible. Take capsules with
                                               combined with budesonide.              acidic liquid e.g. coca-cola and
          Oral      >12yrs 200 mg
                                      Do liver function tests if taken >1-2 months food. Stop antacids if possible.
                       once daily
                                              or if known liver dysfunction.          Headaches seem commonest
                                        Oral liquid is not funded. Consultant will problem but in theory hepatotoxic..
                                     need to apply for exceptional circumstances
                                                        for funding.
                        2 – 12y:     May be used for ABPA where patients have
                      200mg BD            not responded to or are intolerant of
                         >12y:                         itraconazole.
                         <40kg          Consultant decision and microbiologist       Photosensitising so warn patient
                    200mg bd for 1                   approval required.            re sunlight. High strength sunblock
  Voriconazole
                        day then                      See section 6.5                should be used in summer or on
      Oral
                       100mg bd        Take on an empty stomach. Monitor liver holidays for 4 weeks after course
                         >40kg                    function tests monthly.                          finished
                    400mg bd for 1 Voriconazole is not funded. Consultant will
                   day then 200mg need to apply for exceptional circumstances
                          bd.                           for funding.
                                       For invasive fungal infections. Given with
   Flucytosine       50 mg/kg qds
                                     amphotericin. Monitor FBC and liver function           Consultant decision
       IV           (>1 month old)
                                        every 3 days. Given over 20 – 40 mins.
                   Test dose of 100
                  mcg/kg (max 1mg)                                                          Consultant decision.
                  over 10 – 15 mins.                                                  Administer over 30 – 60 mins.
   Liposomal                           For invasive or troublesome aspergillus.
                    Observe for 1hr                                                   Compatible with 5% Dextrose
  Amphotericin                        Check renal/liver function at least 3/week.
                   5 mg/kg od. Start                                                                 only.
  (Ambisome)                               Do not give with other nephrotoxic
                    at 1 mg/kg od,                                                    Flush pre & post dose with 5%
                                     antibiotics e.g. aminoglycosides, colomycin.
                      increase to                                                                 dextrose.
           IV
                   5mg/kg od over 3                                                 Final concentration of the solution
                         days.                                                            should be 0.2 – 2 mg/ml.

                  Test dose: 1mg
 Amphotericin B        /10 mls
 (conventional) (100micrograms/m              Infuse over 6 hours.
            ®
  Fungizone        l) over 1 hour.           Drug is photosensitive.
                      Full dose:   See Starship guidelines for admininistration.
      IV         0.5mg/kg/over 4
                        hours




Author:           Drs Vyas, Byrne, Jaksic & Jan Tate                Service:             Paediatric CF Team
Editor:           Dr Raewyn Gavin                                   Date Issued:         August 2008
Cystic Fibrosis   Page:                                             80 of 100
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                      Note: The electronic version of this guideline is the version currently in use. Any printed version can
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                                               CYSTIC FIBROSIS
Other Respiratory treatments

                                                                                             Potential for hepato- and
                                            Potential long-term treatment as anti-
                      250 mg od, 15-40                                                    ototoxicity but usually very well
                                                         inflammatory.
                            kg                                                                        tolerated.
    Azithromycin
                                                                                        No additional anti-staph prophylaxis
                                               Consultant will need to apply for
                          500 mg od,                                                   needed when maintained on this long
          Oral                                  exceptional circumstances for
                            >40kg                                                        term (unless macrolide resistant).
                                                          funding.
                                                                                        Avoid long term concurrent use with
                                                                                                    erythromycin
                      2.5 mg once daily
                              for
      rhDNase                                 In afternoon, at least 1 hour pre -           3-month trial to assess effect.
                        1st 3 months,
                                                        physiotherapy.                      Occasionally use twice daily –
                         then 2.5 mg
          neb                                                                                   consultant decision.
                        alternate days

                   4 mls up to twice
 Hypertonic saline
                    a day 30 mins                Occasionally very beneficial.               Consider if rhDNase fails.
     4 - 7%
                          pre                    Pre-treat with bronchodilator.          Refer to administration guideline to
                   physiotherapy or                                                          make different strengths.
       neb
                    give with PEP



15.2 Drugs for the Gastro-intestinal tract

Fat soluble vitamins

Recommended starting doses for vitamin supplementation in individuals with CF 1
            Age               Vitamin A (IU)              Vitamin D (IU)          Vitamin E (IU)                    µ
                                                                                                         Vitamin K (µg)
          0-12 mths              1500-2000                  400-1000                  40-80                   150-500
           1-3 yrs               1500-2500                  400-1000                 50-150                   150-500
           4-7 yrs               2500-5000                  400-1000                 150-300                  300-500

          8-18 yrs               2500-5000                  400-1000                 150-500                  300-500
           Adults                2500-5000                  400-1000                 150-500                  300-500
Vitamin A: 1mg retinol = 3.3 IU
Vitamin D: 1 µg = 40 IU ergocalciferol or cholecalciferol
Vitamin E: 1mg RRR- α-tocopherol = 1.5 IU,
1mg all-rac-α-tocopherol = 1 IU

These starting doses are based on international recommendations for vitamin supplementation
and known upper levels for avoiding toxicity.
Fat soluble vitamins should be taken with food and PERT to enhance absorption.




Author:              Drs Vyas, Byrne, Jaksic & Jan Tate               Service:             Paediatric CF Team
Editor:              Dr Raewyn Gavin                                  Date Issued:         August 2008
Cystic Fibrosis      Page:                                            81 of 100
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                    Note: The electronic version of this guideline is the version currently in use. Any printed version can
                          not be assumed to be current. Please remember to read our disclaimer.



                                             CYSTIC FIBROSIS

Vitamin A, D, & E preparations available on prescription in New Zealand
                                          Vitamin content IU per ml/tablet
           Preparation
                                    Vitamin A         Vitamin D       Vitamin E
         Vitadol C*- liquid                    7500                 1400                     -
      Healtheries Multivitamin
                                               2497                  900                     -
             Tablet **
           Micelle E ***                          -                    -                  156 IU
*Vitadol C incurs a part charge of approximately $2.10 per bottle
**Healtheries Multivitamin Tablets and Micelle E are fully subsidised on prescription.
***Micelle E – a Special Authority application for Alpha Tocopheryl Acetate must be made by a
consultant.
Vitamin K :Offered to all children able to swallow tablets and mandatory for those with liver disease
(with or without clotting abnormalities).
Use water-soluble preparation: 10 mg od.


Other Gastro-intestinal drugs

                              Birth – 6months: 1 mg/kg tds
     Ranitidine            >6months: 2 mg/kg bd (max 150 mg                          - small risk of headache
                                          bd
                                                                             Round dose to 10mg/20mg capsule
                                                                             size. Oral suspension can be made
                                                                              by community pharmacy, 2mg/ml,
    Omeprazole              0.4-0.7 mg/kg bd (max 40 mg/day)
                                                                                         fully funded.
                                                                               Doses up to 1.5 mg/kg bd are a
                                                                                     consultant decision.
                                                                             Before 1st and middle feeds of the
   Domperidone                        0.2-0.4 mg/kg tds
                                                                                  day and last thing at night.
                               Birth – 6months: 1 mg/kg tds
     Ranitidine            >6months: 2-4 mg/kg bd (max 150mg
                                             bd)
  Infant gaviscon                   one sachet per feed
   Erythromycin                         3 mg/kg tds                                   dose for gastric stasis
                            <10 kg, 25 ml with 75 ml flavoured
                                        juice / water
                           10-25 kg, 50 ml with 150 ml flavoured             Do NOT give in the presence of bile
Oral Gastrografin
                                        juice / water                       stained vomiting or bowel obstruction
                           >25 kg, 100 ml with 200 ml flavoured
                                        juice / water
                                 Use same doses as oral
                            <5yrs: Dilute to 5 times its volume
      Rectal
                                         with water                                Requires IV line for IV fluids
    Gastrografin
                            >5yrs: Dilute to 4 times the volume
                                         with water
    Klean-prep               Add contents of 1 sachet to 1 litre             Do NOT give in the presence of bile
                                            water                                    stained vomiting
Author:            Drs Vyas, Byrne, Jaksic & Jan Tate               Service:             Paediatric CF Team
Editor:            Dr Raewyn Gavin                                  Date Issued:         August 2008
Cystic Fibrosis    Page:                                            82 of 100
                                              Starship Children’s Health Clinical Guideline
                     Note: The electronic version of this guideline is the version currently in use. Any printed version can
                           not be assumed to be current. Please remember to read our disclaimer.



                                              CYSTIC FIBROSIS
                              Can be given orally or via NG tube             Do not administer just before bedtime
                            Start at 10ml/kg/hour for 30 mins then             due to risk of aspiration Maximum
                                     20 ml/kg/hour for 30 mins                   volume is 100 ml/kg or 4 litres
                             If well tolerated rate can go up to 25           (whichever is smaller) over 4 hours.
                                             ml/kg/hour                      Patients must be reviewed after 1st 4
                              If not passing essentially clear fluid                          hours
                                per rectum then a further 4 hours              Monitor for hypoglycaemia, which
                               treatment can be given. Maximum                    can occur with CF diabetics
                             daily dose should be 200 ml/kg or 8                    undergoing this regimen
                                   litres (whichever is smaller).
                                         1-5 years: 5 ml bd
     Lactulose                          5-10 years: 10 ml bd                     adjust dose according to response
                                       >10 years: 15-20 ml bd
                                 1 - 6 years: 1 sachet of Movicol
                                        Paediatric Plain OD                  Adjust dose accordingly - maximum 4
                               7 - 12 years: 2 sachets of Movicol                       sachets daily.
          Movicol                       Paediatric Plain OD
                            13 - 18 years: Initially 1 - 3 sachets of
                             Movicol per day in divided doses for             Maintenance dose 1-2 sachets daily
                                           up to 2 weeks.
                                                                             Commonest side effect is diarrhoea,
                                                                               in which case, reduce dose. Last
 Ursodeoxycholic
                              10-20mg/kg/day in divided doses                dose should be taken in late evening.
      acid
                                                                              Consultant to apply for exceptional
                                                                                   circumstances for funding.




16. References

Standards of clinical care for children and adults with cystic fibrosis in the UK. Published by Cystic
Fibrosis Trust, 2001.

Australasian Clinical Practice Guidelines for Nutrition in Cystic Fibrosis 2006




Author:             Drs Vyas, Byrne, Jaksic & Jan Tate               Service:             Paediatric CF Team
Editor:             Dr Raewyn Gavin                                  Date Issued:         August 2008
Cystic Fibrosis     Page:                                            83 of 100
                                            Starship Children’s Health Clinical Guideline
                   Note: The electronic version of this guideline is the version currently in use. Any printed version can
                         not be assumed to be current. Please remember to read our disclaimer.



                                            CYSTIC FIBROSIS

Appendices
Appendix l – Annual Review Assessment/Investigations Check List

                                             All patients


Height (cm)               %’ile                        Weight (kg)                  %’ile


BMI                                                    (Z-score)


Last years best height%                                Last year’s best weight %


FEV1 (l)                  % predicted                  FVC (l)                      % predicted


FEF 25-75% (l/sec)        %predicted


Hb                        WBC                          Neuts                        Plt


INR                       APTT                         ESR/CRP                      eosinophils


Urea                      Na                           K                            Creatinine


ALT                       AST                          Alk Phos                     GGT


Bilirubin                 Ca                           Phosphate                    albumin


Vit A                     Vit D                        Vit E                        Vit K (if Rx’d)


IgE                       Aspergillus RAST             Asp pptns


Fasting glc                                            HbA1C


SPUTUM


MC&S                                                   Non-tuberculous mycobacteria


Fungi                                                  Burkholderia cepacia species


Pseudomonas aeruginosa


Author:           Drs Vyas, Byrne, Jaksic & Jan Tate                 Service:               Paediatric CF Team
Editor:           Dr Raewyn Gavin                                    Date Issued:           August 2008
Cystic Fibrosis   Page:                                              84 of 100
                                            Starship Children’s Health Clinical Guideline
                   Note: The electronic version of this guideline is the version currently in use. Any printed version can
                         not be assumed to be current. Please remember to read our disclaimer.



                                            CYSTIC FIBROSIS

CXR + Brasfield Score




Weight problems in last 12/12


Faecal elastase (or if panc sufficient)                Coeliac antibodies


Urinary Na                                             OGTT


Age related


3years                    Liver U/S


6 years                   Liver U/S


9 years                   Liver U/S


>10 years                 Pubertal status


12 years and above        Annual liver U/S             Annual OGTT


CFRD


Fasting lipids            Blood pressure               Urine microalbuminuria


Retinopathy               Slit lamp                    digital imaging
screening
(>12years)


Sensory and vibration sense


Nephropathy screen

(If CFRD, >2 x i/v aminoglycosides or polymixins in last 12/12, or Rx NSAID’s daily)


Urine microalbuminuria




Author:           Drs Vyas, Byrne, Jaksic & Jan Tate               Service:             Paediatric CF Team
Editor:           Dr Raewyn Gavin                                  Date Issued:         August 2008
Cystic Fibrosis   Page:                                            85 of 100
                                            Starship Children’s Health Clinical Guideline
                    Note: The electronic version of this guideline is the version currently in use. Any printed version can
                          not be assumed to be current. Please remember to read our disclaimer.



                                            CYSTIC FIBROSIS
Appendix II – Brasfield Score (Chest X-Ray score)

This score is used for longitudinal assessment of PA chest x-ray taken at annual review. Score is
obtained by subtracting total points from 25, ie 25 would be best possible score.


                                                                                    0 = absent
                                                                                         1
                                          e.g. depressed
                                                                                         2
            Air trapping              diaphragms, kyphosis,
                                                                                         3
                                                                                         4
                                                                                    5 = severe
                                                                                    0 = absent
                                       Parallel lines, end-on                            1
          Linear markings                     circles                                    2
                                                                                         3
                                                                                    4 = severe
                                                                                    0 = absent
                                                                                         1
    Nodular cystic lesions                0.5 cm or larger                               2
                                                                                         3
                                                                                    4 = severe
                                        Segmental/lobar
                                                                                     0 = absent
                                         atelectasis or
           Large lesions                                                           3 = segmental
                                      consolidation. NB not
                                                                                    4 = multiple
                                       confluent nodules.
                                                                                 0 = normal
                                                                                      1
                                                                                      2
                                       Impression of overall                          3
          General severity                  severity.                                 4
                                                                               5 = severe with
                                                                             complications e.g.
                                                                             pneumothorax, cor
                                                                                 Pulmonale

References
Brasfield D, Hicks G, Soong S, Tiller RE. The chest roentgenogram in cystic fibrosis: a new
scoring system. Pediatrics 1979;63:24-29.

Brasfield D, Hicks G, Soong S, Peters J, Tiller R. Evaluation of scoring system of the chest
radiograph in cystic fibrosis: a collaborative study. Am J Roentgenol 1980;134:1195-1198.




Author:           Drs Vyas, Byrne, Jaksic & Jan Tate                Service:             Paediatric CF Team
Editor:           Dr Raewyn Gavin                                   Date Issued:         August 2008
Cystic Fibrosis   Page:                                             86 of 100
                                             Starship Children’s Health Clinical Guideline
                    Note: The electronic version of this guideline is the version currently in use. Any printed version can
                          not be assumed to be current. Please remember to read our disclaimer.



                                             CYSTIC FIBROSIS
Appendix lII – CF ANNUAL REVIEW TEMPLATE LETTER



GP details
Patient details

Problems:
Cystic fibrosis.
etc…


Known Allergies:


Medications Leaving Clinic:


Hospital Admissions in the Past Year:…….. Total Days IV Therapy:……….
      Growth         Weight                             centile         BMI
                      Height                            centile
   1 Year Ago        Weight                             centile      1 year ago
                      Height                            centile



Lung Function: (today and change in past year)
          FVC                         %predicted                      %change
          FEV1                        %predicted                      %change
    FEF25-75                          %predicted                      %change


Investigations:
  Hb                                                    WCC
  Eosinophils                                           ESR/CRP
  platelets                                             Neutrophils
  urea                                                  Na
  K                                                     creatinine
  ALT (<45)                                             AST (<45)
  ALP(45-300)                                           GGT (0-50)
  SBR (2-20)                                            Ca
  Phosphate                                             Albumin (35-47)
  Vitamin A (0.7-2.8umol/L)                             25 Hydroxy vitamin D (50-100nmol/L)
  Vitamin E (12-46umol/L)                               (Vitamin K)

Author:            Drs Vyas, Byrne, Jaksic & Jan Tate                Service:            Paediatric CF Team
Editor:            Dr Raewyn Gavin                                   Date Issued:        August 2008
Cystic Fibrosis    Page:                                             87 of 100
                                            Starship Children’s Health Clinical Guideline
                   Note: The electronic version of this guideline is the version currently in use. Any printed version can
                         not be assumed to be current. Please remember to read our disclaimer.



                                            CYSTIC FIBROSIS

  Total IgE (<100)                                     Aspergillus RAST
  Aspergillus precipitins
  Glucose (3-5.6 fasting)                              HbA1c
  Faecal elastase                                      Coeliac antibodies
  Urinary Na                                           OGTT: N / Impaired / DM
  Blood pressure                                       Fasting lipids
  Urine microalbuminuria
  Retinopathy screening


Sputum Culture:

Imaging:
      Chest X-ray Report /Brasfield score:
      Liver U/S:

CFRD Review:

DEXA scan results:

Nutritional Report:

Physiotherapy Report:

Nursing Assessment:

Comments:

Recommendations:




Having received this Annual Review letter if there any queries regarding recommendations please
do not hesitate to call the CF Nurse Specialist, pH 3074949 ext 6556




Author:           Drs Vyas, Byrne, Jaksic & Jan Tate               Service:             Paediatric CF Team
Editor:           Dr Raewyn Gavin                                  Date Issued:         August 2008
Cystic Fibrosis   Page:                                            88 of 100
                                            Starship Children’s Health Clinical Guideline
                   Note: The electronic version of this guideline is the version currently in use. Any printed version can
                         not be assumed to be current. Please remember to read our disclaimer.



                                            CYSTIC FIBROSIS
Appendix IV – Nursing Transition Document



                     STARSHIP / ACH CF NURSE SPECIALIST DOCUMENTATION

  PATIENT LABEL                                                           Other Telephone Contacts:

                                                                            ________________________
                                                                            ________________________

  Joint transition meeting: Date: _____________                  Tour of GCC : _________________
  Tour of Ward 72, ACH______________                 Last Starship appt: _______________
  First Green Lane appt: _____________
  Last clinic letter from Starship sent / received: ____________________________

  Parents:_________________________________________________________________________
  Siblings:_________________________________________________________________________
  _________________________________________________________________________________
  Home Life:________________________________________________________________________
  _________________________________________________________________________________
  Work / School: ____________________________________________________________________
  Income Support Benefits: __________________________________________________________
  ________________________________________________________________________________

  DEVICES:
     • Portacath                            Date inserted: __________
     • Gastrostomy                       Date inserted: __________
     • Date of last Portacath Flush _________________
     • Equipment at home:
        __________________________________________________________________________
        __________________________________________________________________________

  Home Care Team: _________________________________________________________________
  Nutritional Supplements:___________________________________________________________
  Type of Physiotherapy: ____________________________________________________________

  ALLERGIES: _____________________________________________________________________

  Special Authority numbers:_________________________________________________________
  _________________________________________________________________________________
  _____________________________________________________

  Complications (eg oxygen, DIOS, haemopytsis, CFRD, liver involvement, etc)
  _________________________________________________________________________________
  _________________________________________________________________________________
  _________________________________________________________________________________
  _________________________________________________________________________________

  Fertility / Contraception discussed_______________

  Jan Tate,
  CFNS
  STARSHIP CHILDREN’S HEALTH


Author:           Drs Vyas, Byrne, Jaksic & Jan Tate               Service:             Paediatric CF Team
Editor:           Dr Raewyn Gavin                                  Date Issued:         August 2008
Cystic Fibrosis   Page:                                            89 of 100
                                            Starship Children’s Health Clinical Guideline
                   Note: The electronic version of this guideline is the version currently in use. Any printed version can
                         not be assumed to be current. Please remember to read our disclaimer.



                                            CYSTIC FIBROSIS
Appendix V - Transfer to Adult care (Desired Information) – From Prof Kolbe


     Genotype_________________________

     Diagnosed at ______ years on the basis of ________________

          Bronchopulmonary Suppuration, HRCT on __________ showed ___________
          Chronic infection by ____________
          (negative) culture for NTM………
          (no) evidence of ABPA, Aspergillus IgE = ______________
          (mild, moderate, severe) impairment of pulmonary function,
           (no) previous pneumothorax, ____________(include management)
          (no) major haemoptysis, ___________(include management)
          ACT in the form of ______________, _____________episodes per day
          (nil) regular inhaled corticosteroids, ____
          (nil) inhaled SABA, LABA, _______________
          use of parentral steroids
          (nil) nebulised bronchodilators, _____________
          (nil) nebulised antibiotic, ________________
          (nil) pulmozyme, (no) previous trials etc
          (nil) azithromycin, in….., ………….response


     Upper Respiratory Tract
       CT scan on _______________, showed__________________
       (previous) ORL review
       (no) Previous upper respiratory tract surgery, _____________________
       (no) intranasal steroids
       (no) saline nose wash


     (Nil) gastro-oesophageal reflux
       [ (no) previous endoscopy
       (nil) proton pump inhibitor]


      Pancreatic Exocrine (in)sufficiency
    [regular pancreatic supplements, _____________
    dietician review on _______________
    on proton inhibitor
    bowel habit
    previous formal assessment of (mal)absorption]


     Pancreatic Exocrine Insufficiency
       (no) evidence of glucose intolerance, last tested with ________________
        on _____________
        HbA1c on ………………
       treatment with______________ insulin _______________
       diabetic complications.

Author:           Drs Vyas, Byrne, Jaksic & Jan Tate               Service:             Paediatric CF Team
Editor:           Dr Raewyn Gavin                                  Date Issued:         August 2008
Cystic Fibrosis   Page:                                            90 of 100
                                               Starship Children’s Health Clinical Guideline
                       Note: The electronic version of this guideline is the version currently in use. Any printed version can
                             not be assumed to be current. Please remember to read our disclaimer.



                                               CYSTIC FIBROSIS
      Hepato-biliary disease
    [(no) evidence of significant liver disease
    liver function tests ……..
    liver synthetic function
    (no) biliary cirrhosis
    (no)portal hypertension/oesophageal varices
    (no) evidence of hypersplenism
      (no) Oesophageal varices, last upper GI endoscopy on, _____________ (next upper GI
      endoscopy) on __________________________________
    (no) follow up by Liver Service]
    (no) evidence of cholelithiasis


     DIOS
     - (no) previous episodes on _________________, treated by ______________
     - (no) regular laxative treatment ________________


     Nutrition
    (mal)nourished, BMI = ______________________, on ____________________
    (nil) supplementation, _____________
    Target weight =
    Presence of PEG


     Vitamins
    Vitamin A = ________________, on __________________
    Vitamin E = ________________, on __________________
    Vitamin D = ________________, on __________________
    Supplements ____________


     Bone disease
    BMD (spine), T score = ____________________ ,on ______________
    Next Dexa scan due on ____________________
    (nil) treatment with  -    Vitamin D supplement, ________________
                             -      Calcium supplements_________________
                             -          Biphosphonate (_______________) next dose due on
                                    ______________
                             -      Other ________________



     (In) fertility
                                      -         tested on ______
                                      -         Conception history
                                      -         Obstetric history


Uro-genital


Author:            Drs Vyas, Byrne, Jaksic & Jan Tate                  Service:             Paediatric CF Team
Editor:            Dr Raewyn Gavin                                     Date Issued:         August 2008
Cystic Fibrosis    Page:                                               91 of 100
                                            Starship Children’s Health Clinical Guideline
                   Note: The electronic version of this guideline is the version currently in use. Any printed version can
                         not be assumed to be current. Please remember to read our disclaimer.



                                            CYSTIC FIBROSIS
Gynaecology (?)


Venous access
                                   -         Type (past and present)
                                   -         Complications


Social               -     family history of CF
                                 -       domicile
                                 -       education/work
                                 -       spouse/partner
                                 -       children
                                 -       social security benefits


Other.



Clinic Data (dd/mm/year) .

          FEV1= ________FVC= _________ on _______


      Weight =                     , BMI =                     .




Author:           Drs Vyas, Byrne, Jaksic & Jan Tate               Service:             Paediatric CF Team
Editor:           Dr Raewyn Gavin                                  Date Issued:         August 2008
Cystic Fibrosis   Page:                                            92 of 100
                                            Starship Children’s Health Clinical Guideline
                   Note: The electronic version of this guideline is the version currently in use. Any printed version can
                         not be assumed to be current. Please remember to read our disclaimer.



                                            CYSTIC FIBROSIS
Appendix Vl – ABPA Results Sheet

                                                                        Patient Label


CRITERIA                             DATE       DATE       DATE        DATE       DATE        DATE       DATE       DATE


Pulmonary infiltrates

Positive Asp skin prick
testing

Late skin reaction

Reversible
bronchoconstriction

Central Bronchiectasis

Brown plugs in sputum

Positive sputum culture

Total IgE (usually x 4)

Positive Aspergillus
RAST

Aspergillus precipitins

Blood eosinophilia

ESR

CRP

Itraconazole serum level
(>250ug)
Prednisone & Dose
Methyl Pred
Inhaled Steroids, Name &
Dose
Other (e.g. Itraconazole)
& Dose




Author:           Drs Vyas, Byrne, Jaksic & Jan Tate               Service:             Paediatric CF Team
Editor:           Dr Raewyn Gavin                                  Date Issued:         August 2008
Cystic Fibrosis   Page:                                            93 of 100
                                            Starship Children’s Health Clinical Guideline
                   Note: The electronic version of this guideline is the version currently in use. Any printed version can
                         not be assumed to be current. Please remember to read our disclaimer.



                                            CYSTIC FIBROSIS
Appendix VII – Lung Function Summary Sheet (1)




Author:           Drs Vyas, Byrne, Jaksic & Jan Tate               Service:             Paediatric CF Team
Editor:           Dr Raewyn Gavin                                  Date Issued:         August 2008
Cystic Fibrosis   Page:                                            94 of 100
                                            Starship Children’s Health Clinical Guideline
                   Note: The electronic version of this guideline is the version currently in use. Any printed version can
                         not be assumed to be current. Please remember to read our disclaimer.



                                            CYSTIC FIBROSIS
Appendix VII – Lung Function Summary Sheet (2)




Author:           Drs Vyas, Byrne, Jaksic & Jan Tate               Service:             Paediatric CF Team
Editor:           Dr Raewyn Gavin                                  Date Issued:         August 2008
Cystic Fibrosis   Page:                                            95 of 100
                                            Starship Children’s Health Clinical Guideline
                    Note: The electronic version of this guideline is the version currently in use. Any printed version can
                          not be assumed to be current. Please remember to read our disclaimer.



                                            CYSTIC FIBROSIS
Appendix VIII – Discharge Summary
                                                                         Paediatric Cystic Fibrosis

INPATIENT SUMMARY WARD 26B for respiratory exacerbation

cc        Parents, Other Paediatricians involved in case, .


Dear GP

Re:

Admitted:                                    IV Course Completed:
Discharged for Home IV:                      Total Days of IV Therapy:


Diagnosis/Problems:

Discharge Medications:


Admission:
 FVC                                          %predicted:
  FEV1                                        %predicted:
  Height:                                     Weight:
  ESR:                                        CRP:
  WBC




Peak at ____ Days of Therapy:
  FVC                                         %predicted:
  FEV1                                        %predicted:
  Height:                                     Weight:
  ESR:                                        CRP:


* ESR, CRP & WBC only when initial values elevated.

Sputum Culture:
Antibiotics Used:
Comments:
Other Treatments:
Results of other investigations:
Recommendations & Follow Up:
Author:           Drs Vyas, Byrne, Jaksic & Jan Tate                Service:             Paediatric CF Team
Editor:           Dr Raewyn Gavin                                   Date Issued:         August 2008
Cystic Fibrosis   Page:                                             96 of 100
                                            Starship Children’s Health Clinical Guideline
                   Note: The electronic version of this guideline is the version currently in use. Any printed version can
                         not be assumed to be current. Please remember to read our disclaimer.



                                            CYSTIC FIBROSIS
Appendix IX – Financial Support

“Outside & Inside” -: Where to go for financial assistance”. May 2007

Work and Income New Zealand (WINZ)
Enquiries : Tel 0800 559 009

Child Disability Allowance (CDA) - $38.46 per week
To get the CDA your child must be under the age of 18 years, have cystic fibrosis and be a New
Zealand resident. The receiver of the benefit must be providing the child with constant care.
The CDA is not income or asset tested. You may be eligible to receive added assistance for
ongoing costs, but this added assistance is income- tested.

Invalid Benefit - $175.91 per week (16 – 18 years old), $217.38 per week (18 years and over)
This is available for New Zealand residents only.
This is an income-tested benefit.

Training Incentive Allowance
Provides financial assistance to patients with CF who are receiving the Invalid Benefit. The amount
varies according to the cost and timeframe of the course. Associated expenses are sometimes
included.

Community Services Card
All Children with Cystic Fibrosis and Adults on the Invalid Benefit or on low income (below
$20,916.00 pa) are eligible for a Community Services Card.

NOTE:
(a) Respond to all mail received from WINZ or your benefit may be affected.
(b) Work with your local office on any changes in your circumstances, i.e. if you leave the country
for more than 42 days or if you are on the Invalid Benefit and you are earning more than the
weekly amount allowed.

Ministry of Health (MOH)
Enquiries: phone 0800 281 222

Mileage / Accommodation
Mileage: To claim MOH Mileage support without a Community Services Card, you need to live
more than 35 km from the hospital, unless you visit a facility more than 25 times in 2 months, OR,
you visit a facility more than 50km away from home, at least 6 times in 6 months.
If you have a Community Services Card and travel more than 25 km one way per visit (for a child),
OR 80 km one way per visit (for an adult), then you can claim MOH Mileage support.
Accommodation: If you are travelling more than 100km one way, this is sometimes paid on a
specialist’s recommendation

NOTE: Anybody who cannot afford to pay up-front can ask the MOH to book travel or
accommodation directly. Overseas travel is not covered.




Author:           Drs Vyas, Byrne, Jaksic & Jan Tate               Service:             Paediatric CF Team
Editor:           Dr Raewyn Gavin                                  Date Issued:         August 2008
Cystic Fibrosis   Page:                                            97 of 100
                                            Starship Children’s Health Clinical Guideline
                   Note: The electronic version of this guideline is the version currently in use. Any printed version can
                         not be assumed to be current. Please remember to read our disclaimer.



                                            CYSTIC FIBROSIS

Carer Support (Respite care)
At present there is no national policy covering the whole of New Zealand - the individual DHBs
have autonomy as to whether or not they allocate funding for respite care, and any support given
varies from one area to another.

Prescription subsidy Card
On reaching 20 prescriptions filled in a year (Feb 1st – January 31st):
Community Service Card holders will get their remaining prescriptions free for the rest of the year.
Non CSC holders already paying $15.00 per item, will only pay a $3.00 charge per item for the rest
of the year.

For information or assistance with WINZ or MOH please contact
Sally Carron, National Fieldworker, on 09 636 0351 or 021 126 1237 or sally@cfnz.org.nz

Cystic Fibrosis Association of New Zealand
Enquiries: phone 0800 651 122

Breath4CF Grants
$300 is available per year to PWCF for exercise related activities or equipment. Breath4CF Extra
Mile Scholarship, worth $1500 each – there are two of these available for PWCF who have
reached the ‘next level’ in their chosen sport, music or recreational activity, and one available for a
PWCF to use to attend an Outward Bound course of their choice.

Chris Howlett Endowment Fund
This fund provides for a payment to be made to each PWCF resident in New Zealand, when they
turn 18 – the payment currently stands at $1700.

Tertiary Study Grants
You may apply for 50% of course costs up to a maximum of $2,500 per annum. Please note that
you may well qualify for the Training Incentive Allowance from WINZ as well, but you must apply
for it BEFORE you pay any money yourself or take out a student loan to cover your course fees –
give Sally a call if you’re not sure how to proceed.

Mark Ashford Memorial Scholarship
$3,000 - awarded annually to one PWCF for academic achievement - the Sponsor of this
scholarship is Winstone Wallboards.

Cystic Fibrosis Achievers Awards
Awarded in four Categories: Leadership, Endeavour in the Arts, Endeavour in Sport, and Tertiary
Education - the Sponsor of these awards is Boehringer Ingelheim.

Hospital Allowance (for treatment associated with cystic fibrosis)
$20.00 per night allowance is available to a maximum of 28 days per admission when on transfer
from your base hospital or admitted to a hospital more than 100 km from your usual place of
residence.

Prescription Refunds
For adults who do not qualify for a Community Services card, or who are not eligible for the
Prescription Subsidy card, the CFA will subsidise 75% of the $15.00 charge for subsidised
medication relating to the treatment of cystic fibrosis.

Author:           Drs Vyas, Byrne, Jaksic & Jan Tate               Service:             Paediatric CF Team
Editor:           Dr Raewyn Gavin                                  Date Issued:         August 2008
Cystic Fibrosis   Page:                                            98 of 100
                                            Starship Children’s Health Clinical Guideline
                   Note: The electronic version of this guideline is the version currently in use. Any printed version can
                         not be assumed to be current. Please remember to read our disclaimer.



                                            CYSTIC FIBROSIS
Fertility Grants
50% of your costs can be applied for to a maximum of $5,000. This service is sometimes paid for
by DHBs – please ask them first.

Organ Transplant Fund
If more than 100km from home, you are eligible for $20.00 per night for up to 90 days in hospital.
Plus, transplant related expenses, food vouchers and approved travel for family members (if
needed above MOH financial support) to a maximum of $5,000.

Support for Palliative Care.
Assistance is available to the family for practical support when high care needs are required for the
person with cystic fibrosis in end stage of the condition.

Funeral Grant
A grant of $2000 can be applied for to assist with funeral costs for a PWCF.

Financial assistance for costs relating to CF
Support funds are given to the branches through the National Office to support their community for
costs relating to cystic fibrosis i.e. inpatients and clinic attendance, medical equipment and other
needs discerned by the branch committee.
Community voucher support distributed from National Office
Food vouchers: Families or PWCF doing IV treatment in the home
Treatment related to weight gain, supported by professional intervention.
Petrol vouchers: Supporting adult PWCF who travel long distances to another hospital and don’t
qualify for MOH support.
Welfare: Food or petrol vouchers in an emergency offering immediate practical support.
NOTE: Conditions may apply to some payments form the Cystic Fibrosis Association

Application forms can be downloaded www.cfnz.org.nz or please contact:
Allison Carding on 03 341 8014 or 0800 6511 22 or email allison@cfnz.org.nz or
Sally Carron on 09 636 0351 or 021 126 1237 sally@cfnz.org.nz




Author:           Drs Vyas, Byrne, Jaksic & Jan Tate               Service:             Paediatric CF Team
Editor:           Dr Raewyn Gavin                                  Date Issued:         August 2008
Cystic Fibrosis   Page:                                            99 of 100
                                            Starship Children’s Health Clinical Guideline
                   Note: The electronic version of this guideline is the version currently in use. Any printed version can
                         not be assumed to be current. Please remember to read our disclaimer.



                                            CYSTIC FIBROSIS
Appendix X - Shwachman-Kulczycki Score

This is a general score of clinical severity, which is assessed at annual review. Score each of the 4
parameters out of 25, and add up the 4 scores to give the total score (out of 100).

                                                Excellent - 86-100
                                                  Good - 71-85
                                                  Mild - 56-70
                                                Moderate - 41-55
                                                  Severe - <40

   POINTS          GENERAL               PHYSICAL
                                                                 NUTRITION           X-RAY FINDINGS
                   ACTIVITY             EXAMINATION
                                                          Weight and height
              Full normal activity. No cough, clear above 25th centile,              Normal, clear lung
      25      Plays ball, goes to lungs, normal HR& Normal stool, good                    fields.
                school regularly.    RR, good posture. muscle mass and
                                                                  tone.
                                                                             th
               Lacks endurance,         Rare cough,       Wt and Ht 15-20                  Minimal
              tires at end of day,      normal HR,       centile, stool slightly        accentuation
      20           good school            minimal            abnormal, fair          of bronchovascular
                   attendance.      hyperinflation, clear muscle tone and              markings, early
                                    lungs, no clubbing.          mass.                  hyperinflation.
                                                          Wt and Ht above
                                                          3rd centile, stools
              May rest voluntarily,     Occasional                                   Mild hyperinflation,
                                                            often abnormal,
                tires easily after    cough/wheeze,                                  patchy atelectasis,
                                                           large and poorly
              exertion, fair school increased RR, mild                                    Increased
      15                                                   formed, minimal
                attendance, tires hyperinflation, early                               bronchovascular
                                                               abdominal
                  after exertion.        clubbing.                                        markings.
                                                         distension,reduced
                                                          muscle mass and
                                                               poor tone.
                                    Frequent cough,    Wt and Ht below
                                                                               Moderate
                                    often productive, 3rd centile, bulky
                                                                             hyperinflation,
                 Home teacher,       clubbing, chest offensive stool, mild
                                                                              widespread
              dyspnoeic after short retraction, mod       to moderate
      10                                                                    atelectasis and
                  walk, rests        hyperinflation,       abdominal
                                                                           areas of infection.
                  frequently.         wheezes and     distension, flabby
                                                                                Minimal
                                     crackles, mod       muscles and
                                                                            bronchiectasis.
                                        clubbing.       reduced mass.

                                                                                  Severe
                                       Tachypnoea,
                                                        Marked malnutrition hyperinflation, lobar
                                    tachycardia, severe
                                                          with protuberant     atelectasis&
                                      coughing spells,
              Orthopnoeic, stays in                       abdomen, rectal     bronchiectasis,
          5                         extensive crackles,
                  chair or bed.                         prolapse, large foul, nodules / cysts
                                     cyanosis, signs of
                                                           frequent, fatty    pneumothorax,
                                        heart failure,
                                                               stools.            cardiac
                                      severe clubbing.
                                                                               enlargement.


Reference
Shwachman H & Kulczycki LL. Long-term study of 105 patients with cystic fibrosis. Am J
Dis Child 1958;96:6-15.

Author:           Drs Vyas, Byrne, Jaksic & Jan Tate               Service:             Paediatric CF Team
Editor:           Dr Raewyn Gavin                                  Date Issued:         August 2008
Cystic Fibrosis   Page:                                            100 of 100

				
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