interstitial_lung_disease

Document Sample
interstitial_lung_disease Powered By Docstoc
					ILD:DOAS:DRAFTv6.AK.




 PATIENT PATHWAY FOR INTERSTITIAL LUNG DISEASE


                  DO ONCE AND SHARE PROJECT


     This document is work in progress (draft v6 March 2006)

           Project Duration – October 2005 to April 2006




                                                               1
ILD:DOAS:DRAFTv6.AK.

CONTENTS:

Introduction – setting the scene                          4-8
             - aim of the document
             - proposal for new model
             - what is not included in this pathway
             - summary
             - format of patient pathway
             - sources informing patient pathway

Glossary of Key Pathway Terms                             9 - 10

Pathway authors & support                                 11

Patient’s Pathway – High level Flowchart of Key Stages in
Patient’s Journey                                         12

Key Stage Summary A                                      13 - 16

Key Stage Summary B                                      17 - 19

Key Stage Summary C                                      20 - 22

Key Stage Summary D                                      23 - 24

Key Stage Summary E                                      25 - 26

Condition Pathway IPF                                    27 - 30

Condition Pathway Sarcoidosis                           31 - 33

Condition Pathway EAA                                    34 - 35

Key Stage Summary F                                      36 - 37

End of Life Management Pathway                           38 - 41




                                                                   2
ILD:DOAS:DRAFTv6.AK.




APPENDIX


Appendix Number 1 – Role of the ILD Co-ordinator   43 - 44

Appendix Number 2 – First Consultation Letter      45 - 46

Appendix Number 3 – List of Patient Information    47 - 52
  - General Organisations and Websites
  - Benefits Advice
  - Publications

Appendix Number 4 – Patient Management Plan        53

Appendix Number 5 – Patient Information Leaflet
                    for IPF                         54 - 55

Appendix Number 6 – Patient Information Leaflet
                    for Sarcoidosis                56 - 60

Appendix Number 7 – Patient Information Leaflet
                    for EAA                         61 - 63




                                                             3
ILD:DOAS:DRAFTv6.AK.




  PATIENT PATHWAY FOR INTERSTITIAL LUNG DISEASE (ILD)


INTRODUCTION:

Introduction to interstitial lung diseases – setting the scene

The interstitial lung diseases (ILDs, also known as diffuse parenchymal lung
diseases) are a collection of diverse conditions that cause inflammation and/or
scarring within the lungs. Unlike asthma and COPD the ILDs do not generally
affect the airways. Some ILDs have a known aetiology, asbestosis for example,
but in many cases the cause and natural history of the condition is unknown.
Although there are more than 150 individual ILDs many are rare, and in terms of
disease incidence and patient and health service burden, three conditions
dominate – idiopathic pulmonary fibrosis (IPF), also known as cryptogenic
fibrosing alveolitis (CFA), sarcoidosis and extrinsic allergic alveolitis (EAA).



IPF is a chronic fibrotic lung disease which has a median age at diagnosis of 70
years and which is more common in men than women. The prognosis of patients
with IPF is poor and more than 50% of patients will die within three years of
diagnosis, losing an average 7 years of life. There has been a steady increase in
the number of cases of IPF diagnosed each year in England and Wales since the
late 1970s and the most recent estimates suggest that currently about 4000
cases are diagnosed each year. In terms of public health burden, therefore, IPF
is similar to a number of common malignancies, such as ovarian and renal
cancers. Despite the importance of IPF as a cause of morbidity and premature
death, historically IPF has been a neglected disease and has received little
attention in terms of research or health care resources.




                                                                                   4
ILD:DOAS:DRAFTv6.AK.




Sarcoidosis is an inflammatory disease of unknown aetiology that may affect any
organ within the body but which most commonly affects the lung. Sarcoidosis
may occur at any age but is most commonly diagnosed in people between the
ages of 30 to 55 years. In general the prognosis of the disease is good and in
many people the condition will resolve without treatment. A small number of
patients get progressive disease, however, and in these people the mortality rate
is double that seen for people of a similar age in the general population.
Approximately 3000 cases of sarcoidosis are diagnosed in England and Wales
each year and again this is a disease which has been neglected in terms of
research and health service funding.


EAA is an inflammatory condition triggered by sensitisation and then exposure to
an environmental allergen. Examples of EAA include bird fancier’s lung and
occupational conditions such as farmer’s lung. There are probably 200-300 new
cases of EAA each year in the UK. In general if the responsible antigen is
identified and the exposure removed then the prognosis is good. A few people
develop progressive fibrosis, however, and the mortality is increased in these
patients.


In summary, the ILDs are an important cause of illness in the UK and collectively
more than 10,000 cases of ILD are diagnosed each year. Three diseases
dominate in terms of numbers and burden, but these need to be distinguished
from a large number of other rarer diseases. IPF is the most common ILD and
has a prognosis which is worse than many cancers. Sarcoidosis and EAA are
next in terms of disease incidence and tend to have a better prognosis, but can
cause disability and premature death. Historically these diseases have been
rather neglected by the respiratory community both in terms of research and
specific care pathways.




                                                                                  5
ILD:DOAS:DRAFTv6.AK.

Aim of this document

The aim of this document is to improve the quality of care for patients with ILD.
Central to our approach is the suggested patient-led care pathway and changes
to the way in which patients with ILD are looked after in secondary care.
Our care pathway is divided into three sections –
      The first section is generic to all ILDs and is designed to improve the
       diagnostic process.
      The second section focuses on the management of IPF, sarcoidosis and
       EAA – the three commonest ILDs.
      The third section focuses on end of life issues for patients with ILD.


Historically most patients with ILD have been looked after by respiratory
physicians in general respiratory clinics. Within this model each physician looks
after only a few patients with ILD and as a result accrues clinical experience with
ILD slowly. Despite this drawback there has been a feeling in the respiratory
community that, because no treatment for ILDs are available, the patients were
not disadvantaged by this model of care. Our experience with lung cancer care
pathways, however, has shown that grouping patients with specific lung diseases
together into specialised clinics offers real benefits to the patients even in the
absence of life prolonging treatments. Patients attending specialist clinics benefit
from having input from a range of health care professionals with specialised
knowledge about their condition and as a consequence receive better information
about their disease, better advice on the best treatment options available
including palliative care options and the potential to try new experimental
treatments as part of a clinical trial. Given the information now available on the
natural history of ILDs, and the emergence of potentially useful new treatments
for IPF in particular, we believe that it is now in the best interest of patients to
move to a model of care which resemble that successfully employed to manage
patients with lung cancer.




                                                                                       6
ILD:DOAS:DRAFTv6.AK.

Proposal for a new model of care for ILD Patients

We propose, therefore, that each secondary care centre which looks after
patients with ILD should have a nominated respiratory physician and a
nominated ILD Co-ordinator, who will probably be a respiratory nurse, to co-
ordinate the care of these patients. We also suggest that all patients with ILD,
and all health care workers looking after patients with ILD, should have access to
a multidisciplinary team (MDT) meeting to review diagnoses and management
plans. The makeup of the MDT should include respiratory physicians,
radiologists, pathologists and nurse with a specialist interest in ILD. We do not
envisage that all hospitals will have an MDT but rather that a hub and spoke
system will develop, with an MDT developing within a centre with a particular
specialist interest in ILD, and surrounding centres having the opportunity to refer
cases to, or present cases at, the MDT in this centre. Other important aspects of
this new model of care and use of patient led care pathways will be the provision
of specified audit points, the potential to provide systematic training for junior
doctors and nurses in ILD and the opportunity to co-ordinate research into the
causes and in particular the treatment of ILD.

What is not included in this document
A number of important issues are outside the scope of this document. These
include the care of children with ILD, the production of care pathways for the
many specific ILDs other than the three most common, and in particular ILD in
association with connective tissue diseases and the assessment of research and
R&D needs. We have also, at this stage, not worked up a dataset for ILD. We do
acknowledge the importance of these areas however, and plan to address some
of them in future work on ILD.

Summary
ILDs are an important and historically neglected cause of respiratory morbidity
and premature mortality. The aim of this document is to improve the care of
people with ILD by providing a patient-led care pathway and suggesting a new
secondary health care model for looking after these people.


                                                                                      7
ILD:DOAS:DRAFTv6.AK.


Format of Pathway

Outline of Patient’s Pathway identifies a high level flowchart view of the key
stages in the patient’s journey.
- Pathway start - Patient has suspected ILD (2 key routes identified as Patient
seen by GP or Patient referred through secondary care)
- Patient has confirmed diagnosis with specific condition management plan
- Pathway ending – Patient follows the end of life management plan.

Each key stage has a corresponding letter (and hyperlink), which links directly to
a detailed summary of information that supports each key stage.
This summary outline includes
   - key stage purpose summary
   - standards to achieve
   - key audit points
   - identified health professionals
   - identified benefit to patient
   - identified benefit to health professional and organisation
   - source informing key stage

Each stage also includes timing points and documents containing
   - definitions (referral criteria, role clarification)
   - sub-pathways (management and treatment plans)
   - patient information (written information leaflets, letter templates, contact
      lists)

Key audit points have been identified throughout this pathway, from two view
points
   i)     overall pathway standards (i.e. compliance to pathway)
   ii)    condition specific standards


Sources Referenced Informing this Pathway
These are the relevant references for each key stage summary, which link to
literature and Government initiative documents, such as Creating a Patient-led
NHS (2005), 10 high Impact Changes and journals/book references.




                                                                                    8
ILD:DOAS:DRAFTv6.AK.

       PATIENT PATHWAY FOR INTERSTITIAL LUNG DISEASE

                    DO ONCE AND SHARE PROJECT

   GLOSSARY OF KEY TERMS USED WITHIN THIS PATHWAY

Condition Management Pathway
A pathway of care that is specially developed for an identified condition or
disease. The pathway identifies the key steps in the care delivery and is
evidence-based with audit standards where possible.

End of life Management Pathway
A pathway of care that manages the patient requiring palliative care only.
The pathway identifies the key steps in the care delivery and is evidence-based
with audit standards where possible.

ILD
The Interstitial lung Diseases (ILDs also known as diffuse parenchymal lung
diseases) are a collection of diverse conditions that cause inflammation and/or
scarring within the lungs. Unlike asthma and COPD the ILDs do not generally
affect the airways. Some ILDs have a known aetiology, asbestosis for example,
but in many cases the cause and natural history of the condition is unknown.
Although there are more than 150 individual ILDs many are rare, and in terms of
disease incidence and patient and health service burden, three conditions
dominate – idiopathic pulmonary fibrosis (IPF), also known as cryptogenic
fibrosing alveolitis (CFA), sarcoidosis and extrinsic allergic alveolitis (EAA).


ILD Co-ordinator
Key liaison and supportive role, linking directly to patients and healthcare
professionals.
Role will however be locally adapted to service provider’s specification.
Core elements of role to provide and co-ordinate support and information for
patients and act as point of contact for patients and healthcare professionals.
Role may also encompass additional responsibilities such as co-ordinating
diagnostic investigations and MDT.
This is an evolutionary role, that Trusts may decide to be delivered as part of the
Clinical Nurse Specialists role or as an administrative post, dependent on the
roles and responsibilities identified

Nominated ILD Physician
 In any secondary care Trust with a respiratory service there should be at least
one respiratory physician nominated to have an interest in ILD.
Such physicians should develop services for patients with ILD in accordance with
this document.


                                                                                      9
ILD:DOAS:DRAFTv6.AK.


ILD Team
The team should include as a minimum the nominated ILD physician , the ILD
Co-ordinator and a consultant radiologist with an interest in ILD.

Specialist ILD Physician
A Respiratory Physician, usually in a larger centre, with a declared specialism in
ILD.

Specialist ILD Team
Larger centres should develop a specialist ILD Team.
This will comprise one or more Specialist ILD Physicians , the ILD coordinator,
and one or more Consultant Radiologists and Pathologists specialising in ILD.
The specialist ILD team will be responsible for hosting and organising the ILD
MDT meeting.

Multi-disciplinary Team Meeting (MDT)
MDT meeting is a planned formal meeting made up of the identified healthcare
professionals to review and discuss clinical, radiological and pathological data for
ILD patients.
The MDT aims to provide an agreed diagnosis based on policy, guidance,
evidence-based best practice and clinical expertise.
Formal meeting records are available for all MDT meetings.
ILD healthcare professionals identified for MDT are
    Specialist ILD Physicians
    Nominated ILD Physicians
    Other physicians as appropriate e.g. rheumatologist / immunologist
    Radiologist (s) in Specialist Team
    Pathologist (s) in Specialist Team
    ILD Co-ordinators
    Healthcare Trainees in above disciplines.




                                                                                  10
ILD:DOAS:DRAFTv6.AK.

Pathway Authors

Dr Ian Johnston        (Physician and Clinical Lead)
Dr Richard Hubbard     (Physician and Deputy Clinical Lead)
Dr Simon Johnson       (Physician)
Shonagh Cochrane       (Patient’s Representative)
Julie Morgan           (Respiratory Clinical Nurse Specialist)
Maggie Jephcote        (Community Matron)
Angela Clifford        (Knowledge Manager)


DOAS Project Support
Suzanne Brett          (DOAS Project Manager)
Helen Hood             (DOAS Project Lead)
Karen Hillier-Smith    (DOAS Project Lead)


Care Pathway Support
Annette Kitchin


Additional Support
Dr Maruti Kumaran      (Radiologist)
Dr Kate Pointon        (Radiologist)
Dr Keston Challen      (GP)




                                                                 11
ILD:DOAS:DRAFTv6.AK.

     PATIENT PATHWAY FOR INTERSTITIAL LUNG DISEASE


                          Patient has Suspected ILD


      Patient Assessed by GP                A       Patient Assessed within
                                                        Secondary Care



   Patient                  GP Treats &
  Requires                  Determines
 No Further                  Referral
 treatment                   pathway



         Rapid Access                     Fully Booked
           Referral                         Referral



                        Patient Referred to ILD Team


           B
                         Patient Consultation with ILD Team
                       - Assessment & Investigation Planning
                                                                    C

          Patient Discussed at ILD Multidisciplinary Team Meeting


               D        Patient Consultation
                          with ILD Team –
                   Diagnosis Confirmation/Support                       F


                              Patient follows                      End of Life
                                                                  Management
                           Condition Management                     Pathway
               E
                                  Pathway

                           Ei         Eii          Eiii



                                                                            12
ILD:DOAS:DRAFTv6.AK.

       PATIENT PATHWAY FOR INTERSTITIAL LUNG DISEASE

                          KEY STAGE SUMMARY                    A

Purpose of Key Stage:

          To ensure appropriate clinical assessment of patient is achieved
          To ensure most appropriate referral pathway of care is identified
           based on patient’s clinical need
          To ensure referral pathway is achieved within identified time
           frame

Supportive Information:


   Majority of referrals received through GPs, often ongoing condition in
   the older patient –
    Patient seen by GP and assessed on clinical and/or radiological grounds.

      GP establishes common symptoms such as shortness of breath and other
       possible common causes.

      GP will assess and determine patient’s pathway
         - suspected ILD – patient follows the ‘rapid access referral’. This is
              an urgent referral for the patient to be seen by ILD team in less
              than 14 days (2 week standard)
         - suspected ILD – patient follows the ‘fully booked referral’. This is a
              routine referral for the patient to be seen by the ILD team in less
              than 42 days (6 week standard)

   Referrals from other medical disciplines within hospital and tertiary care
   e.g. rheumatology and health care of the elderly.

Standards to Achieve:


Refer to ILD Team –
    Suspected ILD patient should be referred to the ILD Team and referrals
      managed by ‘pooled waiting list’ management

      When ILD Team not available in local centre then patient should have
       choice of 3 other centres with a Nominated ILD Physician or with a
       specialist ILD team




                                                                                13
ILD:DOAS:DRAFTv6.AK.

Fully Booked Referral – Routine –pooled waiting list management –

GP Referral –
   For appropriate clinical assessment from GP
         - suspected ILD on clinical and/or radiological grounds
         - gradually progressive or no symptoms
         - co-existent malignant disease not suspected
     Chest X-ray desirable, no other investigations essential prior to referral.

Hospital/Tertiary Referral –
   For appropriate clinical assessment from Hospital/Tertiary care
         - suspected ILD on clinical and radiological grounds
         - gradually progressive or no symptoms
         - co-existent malignant disease not suspected
      Full pulmonary function testing to be preferably performed prior to
      ILD clinic
         - spirometry
         - lung volumes
         - gas transfer
         - oxygen saturation
         - high resolution CT thorax

Rapid Access Referral – Urgent – By GP phone call or via electronic referral
directly to ILD Co-ordinator

GP Referral –
   For appropriate clinical assessment from GP as routine plus any of the
     following
         - rapidly progressive dyspnoea
         - acute flare / exacerbation of disease
         - atypical features e.g. fever, rash, haematuria, haemoptysis

Hospital/Tertiary Referral –
   For appropriate clinical assessment from Hospital/Tertiary care as routine
      plus any of the following
         - rapidly progressive dyspnoea
         - acute flare / exacerbation of disease
         - atypical features
         - suspected acute hypersensitivity, vasculitis, pulmonary
             haemorrhage

Role of the ILD Co-ordinator at referral stage – see Appendix No 1.
          - point of contact for GP, hospital and tertiary referrals
          - ensure appropriate referral for routine & urgent appointments
          - point of contact for patients and carers (all patients have access to
             the ILD Co-ordinator whilst waiting for routine and urgent
             appointments)



                                                                                   14
ILD:DOAS:DRAFTv6.AK.

Key Audit Points:


   Overall Pathway –
    Routine referral – measure actual time taken against 42 day (<6 weeks)
     standard
    Urgent referral – measure actual time taken against 14 day (<2 weeks)
     standard
    Proportion of patients referred appropriately

   Remember national targets in 2008 will see referral to treatment standard
   within a 0-18 week time frame.

   Patient Satisfaction Questionnaire –
    Patient made aware of identified time-frames
    Patient made aware of ILD Co-ordinator role
    Patient receives ILD Co-ordinator’s contact details


Identified Key Health Professionals:

      Referring Doctor
      ILD Team / Nominated ILD Physician
      ILD Co-ordinator


Identified Benefit to Patient:

      Appropriate referral pathway based on evidence-based clinical
       assessment
      Speciality care accessed in identified time frame – equality of access
      Direct access to ILD Co-Co-ordinator for information and support



Identified Benefit to Health Professional and Organisation:

      Appropriate referral and prioritisation of patient care achieved
      Standardisation of assessment and referral process
      Relevant investigations and patient information available, reducing delays
       in patient’s journey later in pathway
      Identified health professional (ILD Co-ordinator) to liaise and respond
       directly with health professional and patient/carer re. referral enquiry,
       waiting list management and supportive information
      Cost and time effective management




                                                                                15
ILD:DOAS:DRAFTv6.AK.

Source(s) Informing Key Stage:

   




                                 16
ILD:DOAS:DRAFTv6.AK.

       PATIENT PATHWAY FOR INTERSTITIAL LUNG DISEASE

                           KEY STAGE SUMMARY                     B

Purpose of Key Stage:


          To ensure patient is informed and has an understanding of what
           will happen at first consultation with ILD Team
          To ensure effective and efficient consultation for patient and carer
           at first consultation with ILD Team
          To ensure patient/carer meets the ILD Co-ordinator and is aware
           of role and how to contact
          To ensure diagnostic tests are co-ordinated and where possible
           with planning and patient agreement, are achieved at first
           consultation appointment - one-stop approach

Supportive Information:


   Planning and co-ordinating first consultation with diagnostic testing –

      Patient receives detailed letter outlining first consultation details – see
       Appendix No 2 for written information letter.
      ILD Co-ordinator is clearly identified to patient/carer to access prior to and
       during consultation. See Appendix No 1 for ILD Co-ordinator role.
      Predicted diagnostic testing is required. Where possible there should be
       planned, availability of diagnostic facilities e.g. blood tests, lung function
       testing and chest X-rays, at designated clinic time.
      Same day diagnostics should be individualised to patient’s capacity to
       manage multi-investigation and patient preference. However patient/carer
       are made aware of this possible plan (written information letter) and
       extended length of consultation needed to achieve this.
      If same day diagnostic testing cannot be achieved, patients should receive
       planned dates for testing from ILD at first consultation.
      Patient/carer are made aware that from first consultation to second
       consultation for diagnosis and commencement of either treatment or
       decision to observe, the standard time frame identified is 42 days (<6
       weeks).
      Patient/carer are informed at consultation that clinical decisions regarding
       their care, diagnosis and treatment/management pathways may be
       discussed at the ILD Multidisciplinary Team meeting, prior to them
       returning for their second consultation.




                                                                                  17
ILD:DOAS:DRAFTv6.AK.

Standards to Achieve:

   Detailed written patient information and direct patient access to ILD Co-
    ordinator.
   Same day consultation and diagnostic testing to achieve one stop approach.
   Role of the ILD Co-ordinator at first Consultation -
           - Acts as primary point of contact for patient/carer and all health
               professionals prior to, during and following first consultation
           - Acts as liaison between patient, ILD Team and key diagnostic
               departments
           - Identifies and actions any clinical requirements for patient e.g. lung
               function
           - Provides verbal and written information for patients on diagnostic
               testing and support contact information – see Appendix No 3

Key Audit Points:

    Overall Pathway –
     Measure actual time from referral to patient receiving first consultation and
      achieving diagnostic testing – measure against standard relevant to
      referral type

    Routine referral – measure actual time taken against 42 day (<6 weeks)
    standard

    Urgent referral – measure actual time taken against 14 day (<2 weeks)
    standard

       Patient achieved one-stop first consultation with same day diagnostic
        testing

    Patient Satisfaction Questionnaire –
     Patient made aware of identified time-frames
     Patient made aware that their medical case is discussed at MDT meeting
     Patient received explanation of MDT meeting purpose
     Patient meets ILD Co-ordinator contact details at first consultation
     Patient received List of Patient Information at first consultation

Identified Key Health Professionals:

       ILD Co-ordinator
       Specialist or Nominated ILD Physician
       Outpatient Clinic staff
       Diagnostic Department staff




                                                                                 18
ILD:DOAS:DRAFTv6.AK.

Identified Benefit to Patient:

      Patient has appropriate expectations of first consultation and information
       to help answer questions re. diagnostic testing
      Patient allows appropriate time and plans appropriately for first
       consultation
      Patient has identified health professional to contact from point of referral,
       prior to, during and following first consultation
      Speciality care accessed in identified time frame – equality of access
      Direct access to ILD Co-Co-ordinator for information and support

Identified Benefit to Health Professional and Organisation:

      Clearly identified role and responsibility of ILD Co-ordinator at first
       consultation
      Clearly identified evidence-based testing and access to tests
      Agreed results reporting process, reducing unnecessary steps and
       variations in testing
      Co-ordinated availability of results reporting for planned MDT meeting
      Identified health professional (ILD Co-ordinator) to liaise and respond
       directly with health professional and patient/carer
      Defined testing and consultation activity for tariff setting
      Cost and time effective management

Source(s) Informing Key Stage:

   




                                                                                   19
ILD:DOAS:DRAFTv6.AK.

       PATIENT PATHWAY FOR INTERSTITIAL LUNG DISEASE

                          KEY STAGE SUMMARY                     C

Purpose of Key Stage:

          To review clinical, radiological and pathological data to reach an
           agreed diagnosis
          To plan team based management and treatment of individual
           patients
          To review clinical, radiological and pathological data in patients
           with poor and/or unexpected response to treatment or unusual
           clinical course
          To provide training in ILD to healthcare trainees

Supportive Information:

      ILD MDT meeting is vital in facilitating key healthcare professionals to
       review and discuss clinical, radiological and pathological data, aiming
       provide an agreed diagnosis for patients which is based on policy,
       guidance, evidence-based best practice and clinical expertise.
      Suggested meeting format
           - To review all patients with ILD in the area covered
           - Trust running a specialist ILD service / multidisciplinary ILD meeting
              may act as a ‘hub’ for patients seen and treated at surrounding
              hospitals where such a meeting may not run
           - To take place monthly or more frequently according to caseload /
              waiting list issues
           - To take place in conjunction with specialist ILD clinic
           - Written combined opinion to be made in the patient’s notes and
              conveyed to patient and GP at next clinic visit or otherwise as
              appropriate

Standards to Achieve:

      Patients aware of MDT meeting to discuss outcome of diagnostic testing
       and to define diagnosis
      MDT meeting attended by identified health professionals. These are
          - Specialist ILD Physician(s)
          - Nominated ILD Physicians
          - Other physicians as appropriate e.g. rheumatologist / immunologist
          - Radiologist(s) in Specialist ILD Team
          - Pathologist (s) in Specialist ILD Team where appropriate
          - ILD Co-ordinator(s)
          - Healthcare Trainees in above disciplines



                                                                                20
ILD:DOAS:DRAFTv6.AK.

   Role of the ILD Co-ordinator -
           - Co-ordinates MDT meeting
           - Ensures patient’s medical notes and diagnostic results are
              available for reviewing
           - Ensures current concerns of patients discussed
           - Records and liaises as appropriate outcome of MDT discussion
     Resources that should be available at MDT e.g. patient’s X-ray films for
       review
     All patients have a defined treatment and management plan to meet their
       individual needs

Key Audit Points:


    Overall Pathway –
     All patients seen at first consultation by ILD Team
     All patients to be discussed at MDT meeting
     All identified health professionals in attendance at MDT meeting
     Outcome of MDT meeting forwarded to identified health professionals in
      written document and copy to patient/carer

    Patient Satisfaction Questionnaire –
     Patient receives diagnosis within timeframe – patient chooses from
          - 0 – 6 weeks         - 6-12 weeks
          - 12-18 weeks         - >18 weeks

Identified Key Health Professionals:


       Members of MDT

Identified Benefit to Patient:


       Patient has realistic expectations of availability of diagnosis
       Patient has understanding of diagnosis process and role of the MDT
        meeting

Identified Benefit to Health Professional and Organisation:


       Clearly defined diagnostic process to follow
       Assists meeting national target of 0-18 week referral to treatment standard
       Appropriate use of professional expertise and designated discussion time
       Minimising risk of diagnosis error by multi-disciplinary participation who
        are designated as being key to the diagnostic process


                                                                                 21
ILD:DOAS:DRAFTv6.AK.

      Clearly defines roles and responsibilities of health professionals involved
       in pathway

Source(s) Informing Key Stage:

   




                                                                                 22
ILD:DOAS:DRAFTv6.AK.

       PATIENT PATHWAY FOR INTERSTITIAL LUNG DISEASE

                          KEY STAGE SUMMARY                    D

Purpose of Key Stage:


          To ensure where possible patient receives diagnosis at second
           consultation with ILD Team
          To ensure patient receives written information on their individual
           management plan on receiving diagnosis – work in progress,
           draft available see Appendix No 4

Supportive Information:

          Recommendation that patient receives written information on their
           diagnosis and treatment plan. ILD Team should develop local patient
           held records for lung function and treatment plans
          Treatment Plans are condition specific and individualised to each
           patient’s needs – see Key Summary Stage Ei, Eii to Eiii, for detailed
           information on treatment plan and supporting written patient
           information

Standards to Achieve:

      Patient has realistic expectations of availability of diagnosis
      Patient receives diagnosis at the earliest opportunity post MDT meeting
      ILD Co-ordinator plans for patient to attend next available clinic post MDT
      ILD Co-ordinator aims to achieve diagnosis for patient as soon as possible
       (avoiding any unnecessary delays) and certainly within 12-18 week
       timeframe

Key Audit Points:


   Overall Pathway –
    Patient receives diagnosis within 18 week timeframe
    Patient & GP receive written information following second consultation and
     diagnosis information

   Patient Satisfaction Questionnaire –
    Patient receives written information of consultation and condition
      information
    Patient asked to identify any suggestions to further improve being told
      about diagnosis – free text space


                                                                                   23
ILD:DOAS:DRAFTv6.AK.


      Patient receives diagnosis within timeframe – patient chooses from
          - 0 – 6 weeks           - 6-12 weeks
          - 12-18 weeks           - >18 weeks

Identified Key Health Professionals:

      Referring Doctor
      Patient’s GP/Primary Care Team
      Specialist ILD Physician/Nominated ILD Physician
      ILD Co-ordinator

Identified Benefit to Patient:

      Patient receives written confirmation of diagnosis, management and
       treatment plan following verbal discussion with ILD Team
      Patient avoids unnecessary delays and increased anxiety due to delayed
       diagnosis
      Patient has time to plan attending hospital with +/- supportive
       relative/carer/friend for outcome of diagnostic investigations and MDT

Identified Benefit to Health Professional and Organisation:

      Efficient referral to treatment pathway of care achieved, therefore national
       target 0-18 week standard met
      Clearly identified roles and responsibilities for health professionals from
       referral to diagnosis
      Pre-planning for diagnosis consultations with peer support
      Clear lines of communication between primary and secondary care

Source(s) Informing Key Stage:

   




                                                                                 24
ILD:DOAS:DRAFTv6.AK.

       PATIENT PATHWAY FOR INTERSTITIAL LUNG DISEASE

                           KEY STAGE SUMMARY                         Ei Eii Eiii

Purpose of Key Stage:


          To ensure each Condition Pathway has a management plan
           outlining treatment, follow-up and end of life management
          To ensure patient is informed and has an understanding of what
           will happen with treatments and follow up care throughout the
           condition pathway
          To ensure effective and efficient care is provided by the ILD Team

Supportive Information:


          Condition Pathways are to be individualised to each patient’s needs

Standards to Achieve:


          Condition Pathway to be fully discussed with patient
          Patient to receive condition specific written information leaflet

Key Audit Points:


Overall Pathway –
   See Condition Pathways individualised audit points


Patient Satisfaction Questionnaire –
    Patient receives written condition specific information leaflet
    Patient accesses ILD Co-ordinator as required

Identified Key Health Professionals:


      Patient’s GP/Primary Care Team
      ILD Team




                                                                                   25
ILD:DOAS:DRAFTv6.AK.

Identified Benefit to Patient:


      Condition Pathway informs the patient and assists their knowledge,
       planning and expectations of care and treatments to be provided
      Patient has continuity of care provided by key healthcare professionals
      Patient has access and open communication with healthcare
       professionals

Identified Benefit to Health Professional and Organisation:


      Guidance reduces variation in care and treatment regimes
      Clearly identified roles and responsibilities for health professionals for
       patient’s condition management
      Reduces risk of inappropriate treatments being introduced and as a
       consequence raising patient expectations/risk issues/incidents and
       complaints

Source(s) Informing Key Stage:

   




                                                                                    26
ILD:DOAS:DRAFTv6.AK.

     PATIENT PATHWAY FOR INTERSTITIAL LUNG DISEASE

                   CONDITION PATHWAY FOR
             IDIOPATHIC PULMONARY FIBROSIS (IPF)

Patient Information Leaflet – see Appendix No 5
                       Information on prognosis, symptom control and efficacy
                       and side effects of treatment –
    Breaking               Information at this stage is vital – here ILD Co-ordinator
                             is helpful
       the                 Need a realistic discussion about prognosis and
     News                    symptom control. Introduction to treatments and the
                             limited likelihood of benefit and problems with side
                             effects

                       Action –
                           Clear explanation of disease and natural history
                           Clear outline of prognosis required at first / second visit,
                             depending on patient wishes for this information
                           Written information needed rather than reference to
                             internet
                           Make GP aware of above information
                           See ILD Co-ordinator for discussion / questions /
                             contact point

                       Information –
                           No proven treatment for IPF that increases survival
                           Indications for treatment in IPF – poor or declining lung
                             function / patient preference.
   Treatment
                           In many cases due to the presence of stable disease,
                             or co morbidity treatment may not be appropriate
                           BTS guidelines suggest initial treatment as oral
                             prednisolone 0.5 mg/kg and azathioprine 2-3 mg/kg(1)
                                  Audit Point – measure against BTS
                                    guidelines
                           Rationale for treating / withholding treatment should
                             always be explained
                           Consider bone protection at an early stage
                           Treatment should be given by physician experienced in
                             the use of these drugs usually chest physician or
                             rheumatologist working with chest physician
                           Full explanation of side effects / risk benefit to be given
                           Contact for further discussion / side effect alerting
                             available
                           Shared care of drug monitoring according to local
                             protocol


                                                                                  27
ILD:DOAS:DRAFTv6.AK.

                       Action –
                       Oxygenation – assess and give palliative short burst
                       oxygen to control symptoms or LTOT if paO2 <8kpa (2)
                           Provide information on flow rates for symptomatic relief
                       BTS guidelines suggest initial treatment as oral
                       prednisolone 0.5 mg/kg and azathioprine 2-3 mg/kg(1)
                                  Audit Point – measure against BTS
                                     guidelines
                       Attempt disease modifying treatment – best choice
                       regime at present is prednisolone, azathioprine and NAC
                       using regime from Demedts et al(4)
                           Main indications for treatment would be progressive
                             disease
                           Patients need to be aware of the limited evidence for
                             treatment efficacy
                           Where ever practical patients should be entered into
                             clinical trials
                                 - Prednisolone dose 0.5 mg/kg for month 1, then
                                     reducing each month as follows - 0.4 mg/kg, 0.3
                                     kg/month, thereafter decrease by 10mg per
                                     month
                                 -   Azathioprine – 2 mg/kg4(5)
                                 -   N-acetyl cysteine 600mg tds
                            Monitor patients monthly
                                  Audit Point – measure against monthly blood
                                    monitoring standard
                       For patients who cannot tolerate above regime the main
                       current alternative is cyclophosphamide(6)
                           Patients need to be aware of the lack of supportive
                             evidence for these treatments
                           Where possible such patients should enter clinical trials




                                                                                 28
ILD:DOAS:DRAFTv6.AK.

                       Action –
                           IPF according to local protocol clinic visit / lung
                             function (chest X-ray where appropriate) at each visit
                             all on the same day. 3 – 6 monthly intervals
   Follow up                      Audit Point – measure against access to
                                     same day testing and timeframe standard
                                     being achieved
                           Access to ILD service during exacerbation / other
                             unexpected event should avoid A&E visit and go
                             through clinical team (consultant / ILD Co-ordinator /
                             registrar according to local arrangements)
                                  Audit Point – achieved and appropriateness


                Information –
                    TLCO < 40% or rapidly progressing disease consider
                      discussion with transplant unit – note most units with
                      have upper end age criteria of 65 years. Again input
     Lung             from respiratory nurse about pros and cons of
Transplantation       transplant may be helpful here

                       Action –
                           Consider for transplant if severe or rapidly progressive
                             disease(3)
                           Timing should be in line with BTS guidelines
                             ‘symptomatic patients with CFA under the age of
                             65 (see below) should be discussed with the transplant
                             centre following a failed trial of corticosteroid therapy
                             and referred in any of the following circumstances,
                             TLCO and/or VC below 50-60%, resting hypoxia,
                             pulmonary hypertension‘.(1)
                           Where appropriate referral should be made after full
                             discussion with the patient to include work up, post
                             operative care and survival figures
                           Access to transplant centre via transplant coordinators
                             in first instance

                       Information –
                           Patients who have severe or progressive symptoms
                             who do not fit criteria for lung transplant should have a
                             full discussion of options including withdrawal of
Palliative Care              treatment, second line immunosuppressive treatment if
                             appropriate (although no evidence exists for these
   End of life               agents in IPF) and palliative care
    Pathway                Palliative care should be administered by the ILD Team
                             preferably in conjunction with a palliative care team
                           Good liaison between primary and secondary care is


                                                                                 29
ILD:DOAS:DRAFTv6.AK.

                              required to provide consistent care to patient and avoid
                              inappropriate hospital referral

                       Action –
                           Discussion of end of life plan and resuscitation /
                             ventilation decision with patient and family
                           To include home and ambulatory oxygen via cylinder or
                             concentrator according to need for dyspnoea
                           Opiates for cough and dyspnoea
                           Social input including domestic assistance where
                             required
                           Ongoing support possibly including domiciliary visits is
                             popular with patients and could be performed by
                             suitably qualified member of clinical team including GP,
                             community matron, respiratory nurse


References:

1. The Diagnosis, Assessment and Treatment of Diffuse Parenchymal Lung
Diseases in Adults. Thorax 1999;54(Suppl 1):S1-30

2. Royal College of Physicians. Domiciliary oxygen therapy services: Clinical
advice for prescribers. 1st ed. London: Royal College of Physicians; 1999

3. The British Thoracic Society in collaboration with the Thoracic Society of
Australia and New Zealand and the Irish Thoracic Society. Diffuse parenchymal
lung disease guideline. Thorax 2006; in press.
.
4. Demedts M, Behr J, Buhl R, Costabel U, Dekhuijzen R, Jansen HM et al. High
dose acetylcysteine in idiopathic pulmonary fibrosis. N Engl J Med 2005;
353(21):2229-2242.

5. Raghu G, Depaso WJ, Cain K, Hammar SP, Wetzel CE, Dreis DF et al.
Azathioprine combined with prednisone in the treatment of idiopathic pulmonary
fibrosis: a prospective double-blind, randomized, placebo-controlled clinical trial.
American Review of Respiratory Disease 1991; 144:291-296.

6. Johnson MA, Kwan S, Snell NJ, Nunn AJ, Darbyshire JH, Turner-Warwick M.
Randomised controlled trial comparing prednisolone alone with
cyclophosphamide and low dose prednisolone in combination in cryptogenic
fibrosing alveolitis. Thorax 1989; 44:280-288.




                                                                                   30
ILD:DOAS:DRAFTv6.AK.

     PATIENT PATHWAY FOR INTERSTITIAL LUNG DISEASE

            CONDITION PATHWAY FOR SARCOIDOSIS

Patient Information Leaflet – see Appendix No 6
                       Information –
                           Most people with pulmonary sarcoidosis will improve
    Breaking                 spontaneously – so an initial period of 6 months
                             observation is recommended (1)
       the                 Spontaneous remission in 55-90% for patients with
     News                    stage 1 disease, 40-70% of stage 2 and 10-20% of
                             stage 3.
                           Most remissions occur in the first 6 months
                           Treatment is not recommended for
                                - Asymptomatic stage 1 disease
                                - Asymptomatic, stable, mild stage 2 and 3
                                   disease

                       Action –
                           Observe 3 monthly with lung function
                                 Audit Point – measure against 3 monthly
                                  monitoring timeframe

                       Information –
                           Where non-pulmonary sarcoidosis is present in some
                             circumstances steroid treatment should be started
                             without delay – this includes neurological or myocardial
                             sarcoidosis, hypercalcaemia and ocular and renal
   Treatment                 sarcoidosis not responding to topical treatment (1)

                       Action –
                           Precise dose of steroid will depend on organ effected
                             and clinical situation – input from the relevant specialty
                             should be sought

                       Information –
                           For patients with persistent or progressive symptoms
                             and lung shadowing oral corticosteroids alone are the
                             first line treatment

                       Action –
                           Starting dose may be as high as 0.5mg per kg
                             prednisolone, but many patients will improve with
                             lower doses. Consider bone protection at an early
                             stage



                                                                                  31
ILD:DOAS:DRAFTv6.AK.


                             Treatment should be monitored using a combination of
                              lung function, symptoms and chest x-rays1;2. In most
                              cases people will need treatment for 12 – 18 months
                              (2) although this should be at the lowest dose of
                              steroids required to control symptoms, lung function
                              and x-ray changes
                             When treatment is withdrawn patients will require
                              monitoring in clinic at intervals for 12 months to ensure
                              no relapse occurs

                       Information –
                           If patients require prolonged high doses of steroids
                             which lead to troublesome side effects consider steroid
                             sparing agent. At present best evidence suggests
                             methotrexate as the best second line agent.(3)

                       Action –
                           Other choices include azathioprine, chloroquine and
                             chlorambucil(3)

                       Information –
                           Severe lung fibrosis with respiratory failure is an
                             indication for lung transplantation(1)
                           Palliative care should be administered by ILD Team
Palliative Care              preferably in conjunction with a palliative care team
                           Good liaison between primary and secondary care is
   End of life               required to provide consistent care to patient and avoid
    Pathway                  inappropriate hospital referral

                       Action –
                           Discussion of end of life plan and resuscitation /
                             ventilation decision with patient and family
                           To include home and ambulatory oxygen via cylinder or
                             concentrator according to need for dyspnoea
                           Opiates for cough and dyspnoea
                           Social input including domestic assistance where
                             required
                           Ongoing support possibly including domiciliary visits is
                             popular with patients and could be performed by
                             suitably qualified member of clinical team including GP,
                             community matron, respiratory nurse




                                                                                  32
ILD:DOAS:DRAFTv6.AK.


References:

      1. The British Thoracic Society in collaboration with the Thoracic Society
      of Australia and New Zealand and the Irish Thoracic Society. Diffuse
      parenchymal lung disease guideline. Thorax 2006; in press.

      2. Gibson GJ, Prescott RJ, Muers MF, Middleton WG, Mitchell DN,
      Connolly CK et al. British Thoracic Society Sarcoidosis study: effects of
      long term corticosteroid treatment. Thorax 1996; 51(3):238-247.

      3. Statement on sarcoidosis. Joint Statement of the American Thoracic
      Society (ATS), the European Respiratory Society (ERS) and the World
      Association of Sarcoidosis and Other Granulomatous Disorders (WASOG)
      adopted by the ATS Board of Directors and by the ERS Executive
      Committee, February 1999. Am J Respir Crit Care Med 1999; 160(2):736-
      755.




                                                                                   33
ILD:DOAS:DRAFTv6.AK.



      PATIENT PATHWAY FOR INTERSTITIAL LUNG DISEASE

                  CONDITION PATHWAY FOR EAA

Patient Information Leaflet – see Appendix No 7
                            Most people with EAA will improve spontaneously
                             when the responsible antigen is removed – correct
    Breaking                 identification of the antigen and its removal are thus the
                             priority for treatment.(1)
       the                 A small minority of people will have progressive
     News                    disease with fibrosis even when the antigen is removed
                             and so a period of observation to check that the
                             disease resolves completely in all patients is
                             recommended.
                       Action –
                           Observe 3 monthly with lung function
                       Information –
                           For patients with severe or slow resolving disease oral
                             corticosteroids alone are the first line treatment and
                             may speed recovery(2)
   Treatment           Action –
                           Starting dose may be as high as 0.5mg per kg
                             prednisolone, but many patients will improve with lower
                             doses. Consider bone protection at an early stage. In
                             general steroids are used to speed disease recovery
                             and so the duration of steroid use should be as short as
                             possible and total duration of treatment should be
                             weeks rather than months. However treatment should
                             be continued until chest x-ray has cleared and lung
                             function improvement has stabilised. Once treatment
                             has been completed patients should be reviewed at
                             least one further time in clinic to ensure that relapse
                             has not occurred
                       Information –
                           Some patients may relapse when the steroid dose is
                             decreased and require a steroid sparing agent.
                             Potential choices include azathioprine and
                             cyclophosphamide(1)
                       Action –
                           No evidence base to guide treatment
                       Information –
                           Severe lung fibrosis with respiratory failure may occur
                             and this is an indication for lung transplantation(4)
      Lung


                                                                                 34
ILD:DOAS:DRAFTv6.AK.

Transplantation

                       Information –
                           Patients who have severe or progressive symptoms
                             who do not fit criteria for lung transplant should have a
                             full discussion of options including withdrawal of
Palliative Care              treatment, second line immunosuppressive treatment if
                             appropriate (although no evidence exists for these
     End of life             agents in IPF) and palliative care
      Pathway              Palliative care should be administered by ILD Team
                             preferably in conjunction with a palliative care team
                           Good liaison between primary and secondary care is
                             required to provide consistent care to patient and avoid
                             inappropriate hospital referral

                       Action –
                           Discussion of end of life plan and resuscitation /
                             ventilation decision with patient and family
                           To include home and ambulatory oxygen via cylinder or
                             concentrator according to need for dyspnoea
                           Opiates for cough and dyspnoea
                           Social input including domestic assistance where
                             required
                           Ongoing support possibly including domiciliary visits is
                             popular with patients and could be performed by
                             suitably qualified member of clinical team including GP,
                             community matron, respiratory nurse


References:

1.    The British Thoracic Society in collaboration with the Thoracic Society of
      Australia and New Zealand and the Irish Thoracic Society. Diffuse
      parenchymal lung disease guideline. Thorax 2006; in press.

2.    Kokkarinen JI, Tukiainen HO, Terho EO. Effect of corticosteroid treatment
      on the recovery of pulmonary function in farmer's lung. Am Rev Respir Dis
      1992; 145(1):3-5.

3.    King TE, Jr. Clinical advances in the diagnosis and therapy of the interstitial
      lung diseases. Am J Respir Crit Care Med 2005; 172(3):268-279.

4.    Vourlekis JS, Schwartz MI, Cherniack RM, Curran-Everett D, Cool CD,
      Tuder RM et al. The effect of pulmonary fibrosis on survival in patients with
      hypersensitivity pneumonitis. Am J Med 2004; 116:662-668.




                                                                                   35
ILD:DOAS:DRAFTv6.AK.




       PATIENT PATHWAY FOR INTERSTITIAL LUNG DISEASE

                         KEY STAGE SUMMARY                  F

Purpose of Key Stage:

          To ensure full discussion and understanding of decision
           outcome, between patient and ILD Team in planning and
           commencing the End of Life Pathway
          To ensure patient and family/friends are provided with full
           support and care provided as needs indicate and as identified in
           pathway of care

Supportive Information:

   
   


Standards to Achieve:

   
   


Key Audit Points:

   

Identified Key Health Professionals:

      Patient’s GP/Primary Care Team
      Specialist ILD Physician /Nominated ILD Physician
      ILD Co-ordinator
      District Nursing Team
      Community Matron

Identified Benefit to Patient:

      Dignity and respect maintained at all times
      Patient fully involved (as they wish) in decision-making and management
       of care
      Designated skilled carers providing continuity of care and advise


                                                                             36
ILD:DOAS:DRAFTv6.AK.


Identified Benefit to Health Professional and Organisation:

       Patient care individualised and provided as per best practice
      Patient accessing appropriate care provider
      Clearly agreed decision-making process and patient’s management plan
       communicated to key healthcare professionals, preventing unnecessary
       miss communications and extra steps in patient’s pathway

Source(s) Informing Key Stage:

   




                                                                          37
    ILD:DOAS:DRAFTv6.AK.


           PATIENT PATHWAY FOR INTERSTITIAL LUNG DISEASE

                  END OF LIFE MANAGEMENT PATHWAY



                       Decision made by ILD Team
        No further active treatment available - Prognosis 6-12 months
       Decision communicated to patient/carer
       Documented in medical records
       Decision and management plan communicated to GP and Primary Care
        Team

Key Questions for Patient and ILD Team to discuss –
   Have advance Directives been discussed with the patient?
   Has preferred place of care been discussed and documented?



                           Referral made to:


    Community Matron
    If ongoing monitoring and           District Nursing Team
    treatment of symptoms required      For palliative nursing care and
    to prevent unnecessary              support
    admission to hospital and co-
    ordination of services


Consent for referral to Community Services
                            – obtained from patient and carer



                               Assessment and
                      Single Nursing Assessment Process

                    – Should include social care community




                                                                          38
 ILD:DOAS:DRAFTv6.AK.


       PATIENT PATHWAY FOR INTERSTITIAL LUNG DISEASE

         END OF LIFE MANAGEMENT PATHWAY continued




                        Discuss choices         Provide
Check patient is        on access and           information about
receiving ‘benefits’    services available      other services
                                                available



               1 Key worker in        1 Key worker in
               Secondary Care          Primary Care
                                         ?DN Team



        Formulate care package to allow care at home
                                      - Include carers


            Key worker regularly    Reassesses regularly
             reassesses patient        and documents
              and carer needs        patient’s preferred
                                     place of care (PPC)


   Diagnosis 6 months


     Ensure patient registered on Gold Standard Framework
      (GSF), and patient and family aware




                                                               39
 ILD:DOAS:DRAFTv6.AK.



       PATIENT PATHWAY FOR INTERSTITIAL LUNG DISEASE

         END OF LIFE MANAGEMENT PATHWAY continued


                                      Patient regularly reviewed at
                                      PHCT meeting and
Shared care by health                 anticipatory care discussed
and social care teams
     if required                      Key worker facilitates
                                      communication between
                                      primary and secondary care

  Consider carer support -
  Referral to hospice at home for day/night care
  Trent Crossroads



  Is access to             Are specific management         Education
 specialist care        plans required from secondary       of Team
    required            care? Disease specific-control
(Complex Care)                   of symptoms


 Self management promotion              Access to equipment and
                                        AHP services if required



                                      Ongoing patient review of
                                      patient and carer needs
Referral made to May Scheme-
     to access continuing
  care funds to enhance the
    care of existing teams            Consider spiritual care if
                                      required




                                                                   40
ILD:DOAS:DRAFTv6.AK.



      PATIENT PATHWAY FOR INTERSTITIAL LUNG DISEASE

        END OF LIFE MANAGEMENT PATHWAY continued




                        Last few days of Life



     Initiate LCP              Are proactive         Are family aware
                               care plans in          of impending
                                  place?                  death?


        Ensure out of hours            Ensure family have
      teams are informed with          emergency numbers of
       patient’s preferred PPC         who to contact when
                                       patient dies (not 999)


                                Patient dies
                        Verification of Death ?DN
                         teams+ GP



   Bereavement Care                  Bereavement
                                     Support of family
 Communicate death of                Practical information for those
 patient to all parts of             left behind
 NHS/Social Services                 Ensure family have a visit from
                                     PHCT
  Inform secondary
                                     Discuss specialist bereavement
   care key worker
                                     services

                                     Support of staff
                                     Undertake GSF Patient review at
                                     PHCT meeting

                                                                    41
ILD:DOAS:DRAFTv6.AK.




 PATIENT PATHWAY FOR INTERSTITIAL LUNG DISEASE



                  DO ONCE AND SHARE PROJECT




                       APPENDIX




                                              42
ILD:DOAS:DRAFTv6.AK.


        PATIENT PATHWAY FOR INTERSTITIAL LUNG DISEASE

                    DO ONCE AND SHARE PROJECT

                           APPENDIX NUMBER 1

Proposed Role of ILD Co-ordinator
Key liaison and supportive role, linking directly to patients and healthcare
professionals. Will vary initially from one
service provider to the next.

Role will however be locally adapted to service provider’s specification.
Core elements of role to provide and co-ordinate support and information for
patients and act as point of contact for patients and healthcare professionals.
Role may also encompass additional responsibilities such as co-ordinating
diagnostic investigations and MDT.

This is an evolutionary role, that Trusts may decide to be delivered as part of the
Clinical Nurse Specialists role or as an administrative post, dependent on the
roles and responsibilities identified.

Expansion of role could include
         - maintaining ILD patient database
         - introducing and carrying out research initiatives
         - co-ordinating clinical trials
         - planning, implementing & auditing Clinical Nurse Led reviews (out
             patients, home visits etc)

Key          Role
Stage
    A           Point of contact for GP, hospital and tertiary referrals
                Ensure appropriate referral for routine & urgent appointments
                 point of contact for patients and carers (all patients have
                 access to the ILD Co-ordinator whilst waiting for routine and
                 urgent appointments)

    B           Acts as primary point of contact for patient/carer and all health
                 professionals prior to, during and following first consultation
                Acts as liaison between patient, ILD Team and key diagnostic
                 departments
                Identifies and actions any clinical requirements for patient e.g.
                 lung function
                Provides verbal and written information for patients on
                 diagnostic testing and support contact information



                                                                                     43
ILD:DOAS:DRAFTv6.AK.


    C          Co-ordinates MDT meeting
               Ensures patient’s medical notes and diagnostic results are
                available for reviewing
               Ensures current concerns of patients discussed
               Records and liaises as appropriate outcome of MDT discussion

    D          Supports patient and ILD Team at time of diagnosis being given

    E          Supports patient and ILD Team throughout management of
                condition
               Accessible to patients through telephone and clinic support
               Provides links to other social and healthcare professionals as
                required by patient
               Responsibility for monitoring of Steroid Sparing Agents –
                Clinical Nurse Specialist only

    F          Supports patient through decision to commence end of life
                pathway
               Co-ordinates and liaises closely with healthcare professionals




                                                                                 44
ILD:DOAS:DRAFTv6.AK.


       PATIENT PATHWAY FOR INTERSTITIAL LUNG DISEASE

                    DO ONCE AND SHARE PROJECT

                           APPENDIX NUMBER 2

                                                                  [Hospital] LOGO

                                                                 Hospital Address


                                                               Telephone number
                                                                        Minicom
PERSONAL
Patient name                                           Patient Hospital Number
Patient Address Line 1                                 Patient NHS Number
Patient Address Line 2
Patient Address Line 3
Patient Address Line 4                                 Date


Dear [Patient Name]

RESPIRATORY CLINIC APPOINTMENT
Consultant:
On:
Clinic:
Location:

An appointment has been booked for you to attend the above respiratory clinic.
At this first appointment you will see a respiratory physician – a doctor who is an
expert in chest problems.

At this appointment it might be necessary for you to have some tests to enable
the doctor to assess exactly what is wrong with your lungs. These tests could
include blood tests, lung function tests (breathing in and out through different
machines), and/or a chest x-ray. These tests are all straightforward and routine,
and are nothing to worry about. It may also be necessary for you to have other
tests and therefore you should expect to be at the hospital for at least two hours.
Or, depending on the circumstances, these tests may be arranged for another
day. Remember, the doctor may not be able to tell you exactly what is wrong
until all of these tests have been completed.

Please find a map enclosed showing where the Clinic and other departments that
you may need to visit are located within the hospital. Should you have any
mobility worries (e.g. breathlessness or other problems walking), then please do


                                                                                 45
ILD:DOAS:DRAFTv6.AK.

not hesitate to ask one of the clinic assistants who will arrange wheelchair
assistance between the Clinic and the other departments. Disabled blue badge
parking is also marked on the map.

If you need hospital transport to this appointment then please contact [the
Transport Booking Office] [telephone number] between [opening times] to
arrange this.

If this appointment is unsuitable, please contact the [Appointments
Office/consultant’s secretary/Respiratory Nurse contact???] on [telephone
number] as soon as possible between [opening times].

If you have any further questions or worries regarding this appointment or the
tests mentioned above please feel free to contact [Respiratory Nurse name] on
[telephone number] between [opening times] who will be happy to answer any
queries you might have.

Regards

[Respiratory Clinic Appointments Service]


[Following information in blue could possibly go as generic information
alongside the hospital map (or the relevant section of the hospital map) –
perhaps printed on the reverse of the letter or as a separate enclosed A4
sheet.

Please feel free to bring someone with you to the appointment. There is a
café located near to the Clinic [location of café] which serves drinks and
light refreshments at reasonable prices.

Please note that [name of hospital] is a teaching hospital and you may be
asked if you have any objections to student doctors sitting in on your
appointment.



Enc Map of hospital with out patient clinic, nearest disabled parking, blood
taking room, lung function department, x-ray department and cafe all
highlighted]




                                                                           46
ILD:DOAS:DRAFTv6.AK.




      PATIENT PATHWAY FOR INTERSTITIAL LUNG DISEASE

                   DO ONCE AND SHARE PROJECT

                         APPENDIX NUMBER 3

List of Patient Information

General organisations and websites and contact numbers

British Lung              The British Lung Foundation exists to provide
Foundation (BLF)          information and support for everyone living with a
                          lung disease and for the people who look after
www.lunguk.org            them.
                          This website provides a good point of contact for
www.blf-uk.org            patients with lung disease and their carers and has
                          a wide range of leaflets on lung conditions (see
08458 50 50 20            below).
                          There is also a help line number which is manned
                          by respiratory nurses and benefits advisors.

Breathe Easy       Clubs The British Lung Foundation's Breathe Easy Club is
Support Groups           the only support group throughout the UK for people
                         with any type of lung condition, ranging from
08458 50 50 20
                         asthma and bronchitis to emphysema and lung
                         cancer. The club is completely free to join and aims
                         to provide support and practical information for
                         anybody affected by a disease of the lung - whether
                         they are a sufferer, friend or carer. The Club also
                         works to raise awareness of lung disease and to
                         promote a greater understanding of the problems it
                         can cause.




                                                                                47
ILD:DOAS:DRAFTv6.AK.

Roy Castle Lung             Although aimed at lung cancer patients, this
Cancer Foundation           organisation produces practical information on
                            coping with breathlessness – please see below
www.roycastle.org/patient
/patientinfo.htm

Free Telephone Helpline
    (0800 358 7200)

Macmillan Cancer Relief Again although aimed at lung cancer patients, this
                        organisation produces some good information
www.macmillan.org.uk    booklets – please see below

0808 808 2020

www.direct.gov.uk/disabl    An informative and useful Government website
edpeople                    listing all benefits available to disabled people and
                            how to apply. Gives general advice.
                            See also below




                                                                                    48
ILD:DOAS:DRAFTv6.AK.


                               Benefits advice

www.direct.gov.uk/Disabl     Comprehensive site with advice and information on
edPeople                     entitlements and how and where to apply.
                             This site also contains a list of who does what in
                             relation to benefits, i.e. where to go for which
                             benefit.

Blue Badge Scheme            Contact local council for information and application
                             forms. Also enquire about any other restricted
Contact local council        access permits that might be available to make
(borough and city if         things easier for you.
necessary)
Citizens Advice Bureau       The CAB have trained advisors to help with filling in
                             benefit forms.
http://www.citizensadvice.   Local centres in Phone book or Yellow Pages
org.uk/

Benefits Advice Line         For disabled people – Sends out benefits forms and
                             answers queries. Can arrange for an advisor to
Freephone: 0800 882200       telephone patients back to help with filling in DLA
                             and AA forms in the correct way Advisors will
                             sometimes visit patients at home.

Benefits and                 If you pay to register to this site it provides an
Work.co.uk                   excellent guide on how to fill in the DLA form to
                             achieve results. (currently £15 a year)
http://www.benefitsandwo
rk.co.uk/site.htm

Cinema Exhibitors'           This is a card that can be used to verify that the
Association Card             (disabled) holder is entitled to one free ticket for a
                             person accompanying them to the cinema – can be
www.ceacard.co.uk/           used in most UK cinemas (cost £5 for 3 years)




                                                                                  49
ILD:DOAS:DRAFTv6.AK.


                                Publications
         Title                  Source                     Details
Exercise and the Lungs British Lung Foundation   Very basic explanation
                       (BLF)                     about what happens in the
                                                 body during exercise, and
                                                 how this affects the lungs
                                                 (2pgs)

Exercises for the lungs   BLF                    Very basic advice on how to
                                                 cope with breathlessness.
                                                 (1 pg)

Coping with Breathing     BLF                    Repeat of advice in
Problems                                         ‘Exercises for the Lungs’
                                                 plus more detailed methods
                                                 of breathing control. (2 pgs)

Coping with shortness     Macmillan Cancer       First in series of 3 booklets
of breath (1)             Relief                 based upon the
                                                 experiences of people who
                                                 live with breathlessness –
                                                 detailed advice on
                                                 breathing techniques

Living with               Macmillan Cancer       Second booklet in series of
breathlessness (2)        Relief                 3 centres on adjusting
                          www.macmillan.org.uk   activity levels in order to
                                                 keep breathlessness to a
                                                 minimum – detailed
                                                 practical advice on how to
                                                 cope with daily life

Managing the              Macmillan Cancer       Third booklet in series–
Experience of             Relief                 detailed practical advice on
Breathlessness (3)                               all aspects of
                                                 breathlessness, inc. how it
                                                 affects other areas of your
                                                 life, includes sections on
                                                 depression, eating.

Controlled breathing      Prodigy                Basic advice on breathing
                          www.prodigy.nhs.uk     techniques

A Practical Guide to      Roy Castle Lung        Deals with reasons for
Breathlessness            Cancer Foundation      shortness of breath and


                                                                                 50
ILD:DOAS:DRAFTv6.AK.

             Title              Source                        Details
                         http://www.roycastle.org   ways to deal with this
                         /patient/patientinfo.htm   debilitating and sometimes
                         Free Telephone             frightening problem.
                         Helpline (0800 358         Promotes maximum self-
                         7200)                      control and awareness of
                                                    non-medical treatment
                                                    options.

Take a Breather – A      Roy Castle Lung            A video that demonstrates
Patient’s Guide          Cancer Foundation          practical techniques to help
                                                    people with lung cancer
                                                    cope with breathlessness.

Air travel               BLF                        FAQs about air travel with
                                                    good straightforward
                                                    answers. (2 pgs)

Going on holiday with a BLF                         Detailed information about
lung condition                                      individual travel companies
                                                    and what they provide and
                                                    also about getting oxygen in
                                                    the UK and abroad (11 pgs)

Travelling with          COPD group                 Advice on how to deal with
Respiratory Conditions                              the issues involved in
                                                    travelling with respiratory
                                                    conditions and provides
                                                    company specific info too.

Oxygen                   BLF                        Info about how oxygen
                                                    might help and forms it
                                                    comes in (3 pgs)

Benefits of Stopping     Prodigy
Smoking                  www.prodigy.nhs.uk

Taking antibiotics       BLF                        (3 pgs)

How to get the best      BLF                        Checklist of suggestions
from your doctor                                    (1pg)

Talking to your doctor   BBC website                Advice on how to get the
in hospital              www.bbc.co.uk/health       best from your doctor –
                                                    what questions to ask and
                                                    how to ask them.



                                                                                  51
ILD:DOAS:DRAFTv6.AK.

          Title                  Source                      Details
COPD and your diet,       Respiratory Nurses       Provides basic nutritional
nutritional advice for    QMC Nottingham,          support and advice for
patients with a chronic   Produced By CIG          patients with lung disease
lung disease              (COPD)

Patient information: a    Respiratory Nursing      A guide to nutrition for
guide to healthy eating   Service, Medical         patients with lung disease
for patients with lung    Directorate, James
disease'                  Paget Healthcare Nhs
                          Trust, Lowestoft Road,
                          Gorleston, Norfolk

Nebulisers                BLF                      Explanation of nebulisers
                                                   and how to use them –
                                                   primarily aimed at asthma
                                                   patients (4 pgs)

Lung transplantation      BLF                      Very basic info (2 pgs)

Steroid treatment for     BLF                      Mainly info for asthma
asthma and other lung                              patients (3 pgs)
diseases

Inpatient checklist       BBC website              List of questions to ask
                          www.bbc.co.uk/health     when you stay in hospital (2
                                                   pgs)

Questions for your        BBC website              List of questions for your
specialist                www.bbc.co.uk/health     specialist

Bronchoscopy              BLF                      Well written (3 pgs)

Bronchoscopy              www.patient.co.uk        Well written patient info
                                                   (3 pgs)
Spirometry                www.patient.co.uk        Useful info for patients on
                                                   lung function tests (2 pgs)

CT Scan                   www.patient.co.uk        Useful basic info (2 pgs)

CT scan                   Cancer Research UK       Detailed straightforward info
                          www.cancerhelp.org.uk    (5 pgs)




                                                                                 52
ILD:DOAS:DRAFTv6.AK.

      PATIENT PATHWAY FOR INTERSTITIAL LUNG DISEASE

                  DO ONCE AND SHARE PROJECT

                       APPENDIX NUMBER 4

    Patient’s          The aim of this plan is to record the details of the
   Management          proposed treatment for the next 12 months. We hope
                       that the details recorded here will help both the patient
      Plan
                       and the health care workers looking after the patient

     Diagnosis




    Proposed              o   Wait and see
   Management             o   Trial of steroids
      Plan
                          o   Azathioprine
                          o   Methotrexate
                          o   Cyclophosphamide
                          o   N-acetyl cysteine
                          o   Other



Proposed Duration
   of Treatment



    Next Clinic
   Appointment



  Actions Prior to     Actions to be achieved e.g. lung function/blood tests
    Next Clinic
   Appointment



                                                                               53
ILD:DOAS:DRAFTv6.AK.


       PATIENT PATHWAY FOR INTERSTITIAL LUNG DISEASE

                    DO ONCE AND SHARE PROJECT

                           APPENDIX NUMBER 5


  Patient Information for Idiopathic Pulmonary Fibrosis
                           (IPF)

What is it?
Otherwise known as cryptogenic fibrosing alveolitis or usual interstitial
pneumonia, IPF is a disease which leads to persistent and often progressive
scarring of the lungs. The scar tissue prevents the lungs from working normally
and it becomes more difficult for oxygen to pass from the air sacs in the lungs
into the blood, where it is needed. The amount of scarring usually increases with
time and is generally irreversible. The speed of progression is very variable;
some people may remain stable for several years whilst others deteriorate more
rapidly.

Although IPF is usually thought of as an uncommon disease there are about
4000 new patients diagnosed with IPF each year in the UK, and occurs
throughout the world. Furthermore the disease does seem to be becoming more
common. Despite the efforts of researchers in this area the cause of IPF is not
known.

Symptoms
Most people with IPF develop their symptoms between the ages of 60 to 85
years and the disease is uncommon below the age of 50 years. Men are affected
more commonly than women. The main symptom is usually shortness of breath
on exertion, for example walking up hills or stairs. A cough, which is usually dry,
is also common and finger clubbing, which is a change in shape of the
fingernails, may occur.


How is it diagnosed?
The diagnosis is usually made by a specialist, and so for most patients this
means being referred by their general practitioner to the hospital. Most people
will need to have the following investigations.

   1. A chest x-ray will always be done. It may show signs of the scarring even
      early on, so some people will be diagnosed by an abnormal chest x-ray
      before they develop symptoms.


                                                                                  54
ILD:DOAS:DRAFTv6.AK.

   2. Lung function tests. These are breathing tests to show how well your
      lungs are working. They are also used later on to monitor the severity of
      the disease and how it is progressing.
   3. Blood tests are usually done to exclude other causes of your shortness of
      breath.
   4. CT scan. This is a special x-ray that gives a more detailed picture of your
      lungs. There are characteristic features on the CT scan that can allow
      your doctor to make the diagnosis of IPF.

In addition, some people may have a bronchoscopy which involves passing a
flexible telescope down into the lungs to collect samples. A small number of
patients may also need to have an operation with a general anaesthetic to
remove a piece of their lung for more detailed tests – this procedure is called an
open lung biopsy.

Treatment
The treatments that are currently available for IPF do not work very well and may
have bad side effects. For this reason we do not treat people automatically, but in
general monitor people first.

The treatments which are generally tried for IPF at the moment are steroids and
drugs which suppress the immune system. Unfortunately these drugs do not
work for most patients and the side effects of the drugs will often outweigh the
benefits. For example steroids may cause weight gain, thinning of bones and
diabetes, whilst immunosuppressive drugs may dampen down your immune
response making you more likely to pick up infections Patients on such drugs
need careful supervision with regular blood tests.

New treatments for IPF are becoming available however, and so if your disease
does get worse we will discuss treatment options with you. If you are under the
age of 65 years and otherwise well your doctor may discuss lung transplant with
you.


What will happen if I do not respond to treatment?
Unfortunately most patients with IPF will become more breathless with time and
some will die of the disease. Your doctor will be able to provide medications to
relieve the symptoms and may recommend oxygen therapy if you are very
breathless. You may need to have assistance with mobility or simple changes to
the house to make activities such as showering easier.




                                                                                 55
ILD:DOAS:DRAFTv6.AK.

      PATIENT PATHWAY FOR INTERSTITIAL LUNG DISEASE

                   DO ONCE AND SHARE PROJECT

                          APPENDIX NUMBER 6

               Patient Information for Sarcoidosis
What is sarcoidosis?
      Sarcoidosis is a disease resulting in small nodules (called granulomas)
      throughout the body. Sarcoidosis most commonly affects the lungs and
      skin but most organs can be affected including the eyes, heart, brain, liver,
      spleen, bowels, kidney, nerves, lymph nodes, muscles, nose and sinuses.
      We do not know the cause of sarcoidosis but it probably results from
      something in the environment causing the disease in a patient who is
      susceptible, probably as a result of their genes. Although sarcoidosis is
      not hereditary and cannot be passed on to children, in a small number of
      cases other family members are occasionally affected but there is no
      evidence that sarcoid can be passed on from one person to another.
      Sarcoidosis is a relatively rare disease occurring in 1:10,000 people of the
      population in the UK. The disease affects all races and ages but is most
      commonly found in those between 20-40 years of age and is four times
      more common in people of African descent that Europeans. Sarcoidosis
      very rarely affects children.

What are the different types of sarcoidosis?
      Acute sarcoidosis: Sarcoidosis may flare up rapidly causing enlarged
      lymph glands, a skin rash on the legs, fevers, malaise and often, joint
      pains. In the majority of cases this form of sarcoidosis (sometimes called
      Lofgren’s syndrome) settles down without treatment.

      Chronic sarcoidosis: Sarcoidosis causing progressive scarring (fibrosis)
      of the lungs, chronic skin rashes and affecting other organs including the
      heart and brain is more difficult to treat and although this may stop
      spontaneously, is less likely to do so. Patients with this type of sarcoidosis
      generally require treatment and longer follow-up than those with acute
      sarcoidosis.

What are the symptoms of sarcoidosis?
      Sarcoidosis most commonly affects the lungs and skin but as it can affect
      almost any organ and varies from person to person, a large range of
      symptoms are possible. In some cases sarcoidosis does not give rise to
      symptoms and only comes to light when tests such as chest X-rays are
      performed for other reasons. The list below describes how sarcoidosis
      may affect different organs. Each patient with sarcoidosis is different and



                                                                                 56
ILD:DOAS:DRAFTv6.AK.

        most will only have some (not all) of these symptoms depending on which
        organs are affected.


General symptoms
        Many patients with sarcoidosis experience fatigue or tiredness and
        occasionally have weight loss and fever.

Respiratory (lung)
      Sarcoidosis affecting the lungs may not cause any symptoms but usually
      causes cough and breathlessness. Occasionally chest pain may result
      and most often this is an uncomfortable tight feeling in the centre of the
      chest.

Skin
        Sarcoidosis can affect the skin in many ways. A purple bruise-like rash on
        the front of the shins called erythema nodosum may flare up at the start of
        sarcoidosis and is often associated with feeling unwell and temperatures.
        Sarcoidosis can also cause small bumps (nodules or papules) anywhere
        on the skin or just under the surface of the skin. Occasionally the nose
        and cheeks can be affected by a purplish rash (called lupus pernio).

Lymph gland
     Lymph glands are structures that normally help fight infections and are
     commonly affected in sarcoidosis resulting in swollen glands. This can
     happen at any site and is very common in the centre of the chest (visible
     on an x-ray) but often occurs in the neck and may be the first symptom of
     sarcoidosis.

Eye
        Sarcoidosis can affect the eyes in different ways; inflammation in the front
        of the eye can result in redness, pain, excessive sensitivity to light or dry
        eyes. Sarcoid affecting the back of the eye can cause problems with poor
        vision and it is important that patients with sarcoidosis and changes in
        their eyes or vision are seen by an eye specialist.

Bones and joint
     Sarcoidosis can affect the body’s bones and joints. Most commonly this is
     pain in the joints, often the knees and ankles although any joint may be
     affected. Occasionally sarcoid granulomas in bones can result in pain
     away from the joints.

Heart
        Sarcoidosis can affect the heart in two main ways. Either by reducing the
        heart’s pumping efficiency leading to congestion of the lungs and
        breathlessness (termed cardiomyopathy). The second way is by affecting
        the electrical conducting system resulting in abnormal or slow heart


                                                                                  57
ILD:DOAS:DRAFTv6.AK.

      rhythms. This can often be diagnosed by a standard ECG (heart tracing)
      or 24 hour ECG recording. Sarcoidosis affecting the heart almost always
      requires treatment.

Nervous system and brain
     A small number of patients are affected by sarcoid in the nervous system.
     This can result in weakness, numbness or pins and needles in the arms,
     legs and other parts of the body. The brain and its coverings can be
     affected by sarcoid granulomas which can result in headaches, abnormal
     sensation and loss of function of nerves. Sarcoidosis affecting the brain
     again always requires treatment.

Kidney
     Sarcoidosis can affect the kidneys themselves or cause damage resulting
     in reduced kidney function or cause a raised calcium in the blood stream
     which can lead to kidney stones and other problems. This can be
     checked by a blood test measuring the level of calcium, urea and
     creatinine in the blood.

Liver and spleen
       Sarcoidosis granulomas can affect the liver and spleen. These may
       become enlarged and function less efficiently. Although this seldom
       causes symptoms, abnormalities in the liver can be detected by blood
       tests and an enlarged spleen may occasionally cause problems by
       reducing blood counts.

How is sarcoidosis diagnosed?
      Sarcoidosis is diagnosed in different ways according to which organ is
      affected. In most cases evidence of sarcoidosis is visible on a chest x-ray
      or CT scan of the lungs. In many cases tissue from the affected organs is
      sampled (called a biopsy) and examined under the microscope to confirm
      the presence of sarcoid granulomas. These biopsy samples can be taken
      from the skin, lymph nodes or the lungs (the latter by a procedure called a
      bronchoscopy). In some cases it is important to perform these tests to
      ensure that sarcoidosis is the correct diagnosis rather than diseases which
      can sometimes look like sarcoidosis including tuberculosis.

      Once sarcoidosis is diagnosed, patients may require further investigation
      to see if sarcoidosis is present in different organs and how severely these
      have been affected. This will vary according to your symptoms but often
      includes lung function tests (breathing tests which indicate if the function
      of the lungs has been affected and by how much). Electrocardiograph
      (ECG) and 24 hour tape. These tests record the electrical activity of the
      heart over a few seconds or a 24 hour period and are helpful in telling if
      sarcoidosis has affected the conducting system of the heart. Some
      patients will have an ultrasound scan of the heart (called an
      echocardiograph) which examines if the heart has been enlarged or



                                                                               58
ILD:DOAS:DRAFTv6.AK.

      otherwise affected by sarcoidosis. X-rays are commonly performed of the
      lungs and often a more detailed x-ray called a CT scan will be performed
      of the lungs which is better at showing sarcoid nodules and distinguishing
      them from other diseases. CT scanning may also be performed of the
      brain and occasionally other areas as appropriate. Patients with
      sarcoidosis usually have blood tests to look at blood cells to check the
      liver and kidney function and blood calcium levels.

How is sarcoidosis treated?
      As sarcoidosis may cause very minor to very severe problems, different
      patients may need different treatments. Sarcoidosis may often be active
      for a period of months to years and then cause no further problems.
      Because of this it is common for doctors to observe a patient’s symptoms
      and tests for a period of some months in case the disease becomes
      inactive without needing treatment. In some patients where symptoms are
      severe or an organ is seriously affected and in almost all patients with
      sarcoidosis affecting the brain, heart or eyes, treatment will be started
      straight away.

      Acute sarcoidosis
      Sarcoidosis which flares up rapidly causing enlarged lymph glands, skin
      rash on the legs, fevers, malaise and joint pains dies away without
      treatment in up to 90% of cases. Painkillers such as Paracetamol or
      Ibuprofen are generally all that is required in such cases.

      Chronic sarcoidosis
      Sarcoidosis occurring over longer periods causing progressive scarring
      (fibrosis) of the lungs, hypercalcaemia, chronic skin rashes and affecting
      other organs including the heart and brain is more difficult to treat and
      although this may stop spontaneously, is less likely to do so. Patients with
      this type of sarcoidosis generally require longer follow-up and require
      more treatment than those with acute sarcoidosis.

      Steroid treatment for sarcoidosis
      Patients with progressive symptoms or worsening tests including lung
      function tests, raised blood calcium and patients with sarcoidosis affecting
      the heart or brain usually require treatment with steroids. Prednisolone is
      the most commonly used drug: usually a moderate to high dose is given
      for 6-12 weeks and gradually reduced according to the response.
      Treatment is usually given for between six months and two years and if
      the disease appears inactive after this time may be cautiously withdrawn.
      Steroids have significant side effects including raised blood pressure,
      diabetes, osteoporosis, weight gain, bruising, nausea and mood changes.

      Steroid sparing drugs
      In patients who require high doses of steroids for prolonged periods or
      who develop unacceptable side effects, steroid sparing drugs can be used



                                                                               59
ILD:DOAS:DRAFTv6.AK.

      to try and control the disease without the need for high doses of steroids.
      The evidence that says these drugs are effective is not very strong and
      these drugs may not be effective in all patients. The most commonly used
      is Methotrexate which is given as a tablet once weekly. Other steroid
      sparing drugs used include Azathioprine and Hydroxychloroquine. All of
      these drugs have different types of side effects and your physician will
      discuss these with you in detail should they be required for your condition.

      Other treatments
      Occasionally other treatments are required including treatment of
      advanced lung fibrosis due to sarcoidosis which may need oxygen and in
      rare cases lung transplantation. Damage to the conducting system or
      other parts of the heart can require treatment including a pacemaker or
      other treatments for heart failure. Specific treatments may also be used in
      the eyes and skin.

What is the prognosis for patients with sarcoidosis?
      Whilst the disease is active it is common for patients with sarcoidosis to be
      followed by a specialist in sarcoidosis, often a respiratory physician and
      occasionally by other specialists such as an eye or heart specialist
      depending on which organs are affected.

      Patients with acute sarcoidosis (see above) may be unwell for weeks to
      months and often improve without the need for steroid treatment and only
      require occasional pain killers. Patients with chronic sarcoidosis may have
      the illness for months to years. In many patients the disease may burn out
      and become inactive however scarring of the lungs, skin or other organs
      may remain. Once treatment is stopped you may still need to see your
      specialist to ensure the disease does not come back. Sarcoidosis can
      occasionally persist although this is not typical. Very occasionally
      sarcoidosis may be fatal, usually as the result of severe lung fibrosis or
      heart involvement.

Further information
      This sheet contains general information about sarcoidosis. As the disease
      affects different people in different ways, it is best to discuss your disease
      with your own physician.




                                                                                 60
ILD:DOAS:DRAFTv6.AK.

      PATIENT PATHWAY FOR INTERSTITIAL LUNG DISEASE

                   DO ONCE AND SHARE PROJECT

                           APPENDIX NUMBER 7

    Patient Information for Extrinsic Allergic Alveolitis
                           (EAA)


What is extrinsic allergic alveolitis?
      Extrinsic allergic alveolitis is a disease of the lungs caused by an allergy to
      things we breathe in (called an allergen). Extrinsic allergic alveolitis (also
      called hypersensitivity pneumonitis) can result from breathing in a large
      number of different substances in the home, at work and as a result of
      hobbies. When an allergen is breathed in some people will become
      allergic to it causing inflammation and in some cases eventually lung
      scarring (called fibrosis). Causes of extrinsic allergic alveolitis include
      exposure to mouldy hay in farmers, exposure to pet or farm birds,
      mushroom and other fungal spores and a variety of other occupations
      including organic materials such as woodworking. Birds in the home
      (especially budgies, cockatiels and pigeons) are the most common cause
      for extrinsic allergic alveolitis in the UK and is sometimes called bird or
      pigeon fancier’s lung.

      Only a small number of people exposed to allergens will become allergic
      to them and become ill. Why some people get extrinsic allergic alveolitis
      and some do not is not clear but a person’s genes may make them more
      likely to develop the condition if exposed to the allergen.

What are the symptoms of extrinsic allergic alveolitis?
      Extrinsic allergic alveolitis takes two forms with different symptoms:

      Acute: In early disease the symptoms are always related to exposure to
      the allergen. Usually several hours after exposure to the allergen, fever,
      flu-like symptoms with chest tightness, cough, breathlessness and
      occasionally wheezing occur. This may be associated with tiredness,
      headache, aches and pains. These symptoms last a few hours, completely
      resolve and the patient is usually well between exposures to the allergen.
      People who are exposed to the allergen on a regular basis can have
      symptoms that don’t get better – see next section.

      Chronic: People who are continuously exposed to a small amount of the
      allergen may have less obvious symptoms which build up gradually; this
      can lead to lung scarring and long term lung damage. In the chronic form
      there are often no or only minor symptoms of fever and malaise and the


                                                                                  61
ILD:DOAS:DRAFTv6.AK.

      main symptoms are gradually progressive breathlessness initially on
      exertion. Weight loss may occur. These symptoms can progress to quite
      severe levels before coming to medical attention.

How is extrinsic allergic alveolitis diagnosed?
      Patients with these symptoms are generally referred to a chest specialist.
      Extrinsic allergic alveolitis is usually suspected, by the combination of the
      appropriate symptoms (see above) and exposure to a known cause of
      extrinsic allergic alveolitis such as pet birds.

      The following tests are usually performed. Patients have a chest x-ray
      which may be normal in early disease but may show lung scarring
      (fibrosis) in more advanced disease. A more detailed X-ray scan called a
      CT scan of the lungs is usually performed which will show inflammation in
      the alveolar spaces and air passages and occasionally fibrosis. Blood
      tests are performed to detect the presence of antibodies against the
      allergen, for example bird proteins in bird fancier’s lung or moulds in
      farmer’s lung. If you have been exposed to the allergen it is possible to
      have these antibodies but not become ill, and therefore these tests should
      only be performed in those who have symptoms. To examine how much
      damage the disease has caused to the lungs breathing tests are
      performed.

      Occasionally if the diagnosis is not clear from these tests a biopsy of the
      lung may be required to distinguish extrinsic allergic alveolitis from other
      lung diseases. A lung biopsy can be performed in two ways, firstly under a
      local anaesthetic during a bronchoscopy where a flexible tube is passed
      through the nose into the lungs and a sample of lung tissue taken. This
      test, called a transbronchial biopsy, does not always provide a clear result
      and a lung biopsy may need to be performed by a surgeon through a small
      incision under a general anaesthetic. This procedure requires three to
      four days in hospital.


How is extrinsic allergic alveolitis treated?
      The most important aspect of treatment is to avoid further exposure to the
      allergen. This may require the removal of household pets, a change in
      employment or use of a mask when exposed to the allergen. Failure to
      stop allergen exposure increases the chance that extrinsic allergic
      alveolitis will progress to irreversible lung damage. In patients with severe
      disease, steroid treatment may be prescribed.



      Steroid treatment
      Patients with severe symptoms or severely affected lung function tests
      usually require treatment with steroids. Prednisolone is the most
      commonly used drug with treatment started at a moderate dose for


                                                                                62
ILD:DOAS:DRAFTv6.AK.

      several weeks and gradually reduced according to response. Treatment
      may be required for up to three months and if the disease appears inactive
      after this time, may be cautiously withdrawn. Steroids may have side
      effects including raised blood pressure, diabetes, osteoporosis, weight
      gain, bruising, nausea and mood changes.

      Other treatments
      For severe chronic extrinsic allergic alveolitis oxygen treatment is
      occasionally used.

What happens to patients with extrinsic allergic alveolitis?
      If the causative allergen can be identified and withdrawn, patients with
      early extrinsic allergic alveolitis should make a complete recovery.
      However they will remain allergic and should they be exposed to the
      allergen in the future the symptoms will recur.

      For patients who have chronic extrinsic allergic alveolitis, removal of the
      allergen is likely to stop the disease getting worse although if lung fibrosis
      has occurred, symptoms may not resolve fully. Remaining exposed to the
      allergen will result in continuing and possibly severe lung damage.




                                                                                 63

				
DOCUMENT INFO
Shared By:
Categories:
Tags:
Stats:
views:17
posted:11/11/2011
language:English
pages:63