Cystic Fibrosis

Cystic Fibrosis C ystic fibrosis (CF) is the lifemost common inherited, limiting, incurable condition amongst Caucasians. It mainly affects the digestive system and lungs. There is considerable variation in the severity of symptoms. CF is still a life shortening condition and in the past, most people with CF did not survive childhood. Recent changes in the understanding and management of CF now mean that almost all affected people live into adulthood and lead productive lives. This improvement in outlook and quality of life is expected to continue. In other cases, a sweat test is required. The majority of children with CF are identified through the newborn screening program. Diagram Fibrosis A: Inheritance of Cystic Who is Affected by Cystic Fibrosis? One in 25 people are carriers of the CF gene. Carriers of the CF gene do not have any symptoms of the condition. If two people carry the gene and have a child, for each pregnancy there will be: • • • one in four chance that the child will have CF; two in four chance that the child will not have CF but will be a carrier; and one in four chance that the child will not have CF and will not be a carrier. Respiratory Aspects of Cystic Fibrosis People with CF produce abnormally thick sticky mucus which blocks small air passages and may result in lung damage due to recurrent infections over a period of time. This is the most serious consequence of CF. Physiotherapy and Active Lifestyle Chest physiotherapy (airways clearance techniques) is an important part of daily care for the person with CF. Frequency and duration of treatment sessions will vary from person to person and be dependent upon current health status (increasing to 2-3 times per day when unwell). A number of different techniques are available which provide more independence for the CF person e. g. PEP Mask, Flutter. Others require hands-on assistance e.g. postural drainage and Since 1989 all babies born in Victoria have been screened at birth for CF. Most infants are diagnosed within two months. Testing is carried out using a heel prick blood sample. For half the children with CF, diagnosis is made from the sample. Royal Children’s Hospital - Education Institute Phone: (03) 9322 5100 Fax: (03) 9328 4433 Page 1 This Information Package has been developed in consultation with medical, nursing and allied health professionals at the Royal Children’s Hospital Australia. percussion. Wherever possible, physiotherapy and inhalation therapy sessions are undertaken at home before/after school to ensure minimal interruption to school life. All people with cystic fibrosis are encouraged to be active and participate in physical activities. The hospital or community physiotherapists are happy to be contacted to discuss an individual student’s ability to participate in school sport with parental permission. Positive Expiratory Pressure (PEP) Mask. therapy involves breathing out against some resistance. This is used to expand areas of lung collapse and/or help mobilise secretions towards the upper airways. It may involve use of a mask or mouthpiece attachment. Physical Activity/Sport Young people with CF are encouraged to participate in physical and sporting activity according to their individual interests. Exercise promotes lung health through the clearance of mucus. Students with CF should be encouraged to participate in all games and activities at school. It is important to remember that the condition may limit the extent to which the student is able to participate. This may vary from time to time, especially if the student has an acute infection. At these times it is important for the student to still be included in the group. This may be achieved by being asked to score, record times or assist with the activity in some other way. Rest, coughing and drinks of water may be required. Communication with the young person and family is important in ascertaining changes in the student’s health status that may impact on his/her participation in school activities. Diagram B: PEP Therapy Cross Infection Lung disease is a major manifestation of cystic fibrosis. The underlying basis is chronic bacterial infections in the lower airways. One particularly troublesome organism is called Pseudomonas. There is evidence that patients with CF who have Pseudomonas in their lower airways are able to pass this infection on to patients with CF who are free of Pseudomonas. As lung disease tends to be more symptomatic in the presence of Pseudomonas, standard medical practice is to prevent contact between these two groups of CF patients. It is important to remember that Pseudomonas is not of any danger to people who do not have CF. Possible routes of cross infection include: • CF patients coughing on another CF patient; • CF patients sharing medical devices such as nebulisers or aerosols (puffers); • CF patients sharing non-medical devices such as drinking containers or musical instruments; and Inhalation Therapy Some people with CF need to undertake inhalation therapy before or after chest physiotherapy. This may involve the use of a puffer and spacer or clinical air pump to deliver medication. Diagram C: Inhalation Therapy Royal Children’s Hospital - Education Institute Phone: (03) 9322 5100 Fax: (03) 9328 4433 Page 2 This Information Package has been developed in consultation with medical, nursing and allied health professionals at the Royal Children’s Hospital Australia. • due to the risk of cross-infection, it is recommended that schools in which there are two or more students with CF should discuss specific details of these risks with medical members of the CF team. The risk of cross infection between people with CF is directly related to proximity of contact between them. Thus the buddying system is not recommended between CF students. However, joint participation in events such as a school outdoor excursion should generally not pose a problem. effective for 30 minutes. If a meal is longer than 30 minutes then additional enzymes must be taken. Some students will take half their enzymes at the beginning of the meal and half in the middle. The dose of enzymes required depends on the individual and the type of food eaten. The Royal Children’s Hospital uses four different types of enzymes which vary in concentration. These are Pancrease, Cotazyme, Creon and Creon Forte. Nutrition People with CF have special nutritional needs. For most people this includes a highenergy diet, taking enzyme capsules with all meals and snacks and salt replacement. Why is enzyme replacement needed? Many people with CF have problems with the digestion and absorption of food. During digestion, food moves through the digestive system to the small intestine. It is here that the majority of foods we eat are broken down ready for absorption. Enzymes released by the pancreas are passed into the small intestine and break down foods so nutrients can be absorbed. With cystic fibrosis, the tubes of the pancreas are blocked with mucus and this prevents the enzymes from passing into the small intestine. Without enzymes, food is not digested properly and cannot be utilised by the body. Enzyme replacement is necessary. These enzymes consist of lipase (breaks down fat), protease (breaks down protein) and amylases (breaks down carbohydrate). What happens if enzymes are not taken? If replacement enzymes are not taken, the immediate consequence is reduced absorption of food, stomach pains, wind and diarrhoea. At school, students can feel singled out taking enzymes in front of their classmates and friends. They may miss meals rather than take enzymes. Sometimes they simply forget. It is important to encourage students to take their enzymes by giving ready access to them. They are not a drug and will not harm other students if taken accidentally. Students may also need drinks to swallow capsules. Younger students’ parents may wrap the appropriate number of enzymes with each food item or use pictures to indicate the number of enzymes required. Older students may just carry a container in their bag or pocket. What type of food should students with CF eat? People with CF require a high-energy diet. Their energy requirements can be 20-50% more than people of the same age to achieve normal growth. To meet this high-energy requirement, people with CF need substantial snacks and meals, not only ‘junk’ foods high in energy, but nutritious foods high in energy. In contrast to a healthy diet with low fat, low sugar and low salt, students with CF need a high fat, high sugar, high salt diet. How do people with CF take their enzymes? Enzymes must be taken with almost all foods except watery salad vegetables, some fruits and sugary foods such as lollies, soft drink or cordial. Enzymes must be taken prior to meals and snacks and are only Fat: Increasing fat is the easiest way to increase energy as it is a very concentrated source of energy. People with CF are encouraged to have Royal Children’s Hospital - Education Institute Phone: (03) 9322 5100 Fax: (03) 9328 4433 Page 3 This Information Package has been developed in consultation with medical, nursing and allied health professionals at the Royal Children’s Hospital Australia. full cream dairy products and to add margarine, cheese and salad dressings liberally to foods. Sugar: Sugar is another concentrated source of energy and its use is encouraged on cereals. Cordial and soft drinks are recommended rather than water. Encouraging care of teeth is very important. Salt: People with CF lose extra salt in their sweat particularly in hot weather or when playing sport (sometimes up to ten times as much as people without CF). This can cause dehydration. To prevent dehydration, salt and fluid replacement is important. Salt is replaced using foods high in salt such as Vegemite, salted nuts, cheese and savoury snacks, adding salt to foods or using salt tablets or electrolyte powder. Nuts are not recommended for children under 7 years of age. The diet guidelines for diet for people with CF are different to those recommended for other people. People with CF need a nutritious highenergy diet in conjunction with the appropriate enzymes to stay well and achieve adequate growth and development. Daily Schedule This is an example of a typical day in the life of a young person with CF. The daily regimen will vary from one young person to another. 6am First Wake-up Call! Second Wake-up Call! Inhalation Therapy Chest Physiotherapy Dress/Shower Pre-breakfast Medication Breakfast Post-breakfast Medication Inhalation Therapy (if required) 8. 15am Leave for School. Recess Remember enzymes with food, extra drinks, salt tablets in summer What about teaching nutrition to people with CF? Some students experience difficulty when nutrition and healthy eating is discussed as part of their classes. Often the diet pyramid is used as a teaching tool. Students with CF can become anxious when recommended foods are described as ‘bad’ foods or ‘occasional’ foods. The Nutrition Department at the Royal Children’s Hospital uses the diagram of the CF diet cube for teaching nutrition to students with CF. 12. 30/1pm Lunch: Remember enzymes with food, extra drinks, salt tablets in summer again! 3. 30pm Home: Homework/Chores/Relax Pre-dinner Medication Evening Royal Children’s Hospital - Education Institute Phone: (03) 9322 5100 Fax: (03) 9328 4433 Page 4 This Information Package has been developed in consultation with medical, nursing and allied health professionals at the Royal Children’s Hospital Australia. Dinner Post-dinner Medication Inhalation Therapy Chest Physiotherapy Bath Bed the hospital to inform teachers about CF and its educational implications. A presentation by the medical team covers all aspects of CF management and related issues. An invitation is sent to the parents of each school-aged student who attends the clinic to pass on to the school. Young people with CF attend an outpatient clinic every two to three months and are reviewed by the CF medical team. During this clinic, an Education Advisor is available to discuss schooling issues with the young person and his or her family. These issues may include difficulty in keeping up with the workload, peer issues, absenteeism and vocational guidance. Absenteeism Children with CF may be absent from school for outpatient visits and hospitalisation. Absences should be monitored carefully so that the child does not have unnecessary absences that are not directly related to CF. Students who attend the Royal Children’s Hospital will need to visit the hospital for outpatient appointments every two to three months. This may take up to half a day and includes review by health professionals in respiratory, gastroenterology, endocrinology, physiotherapy, dietetics, nutrition and lung function. Some students need to be hospitalised for intravenous antibiotics and intensive physiotherapy for approximately 10-14 days. This admission time includes reviews by the multi-disciplinary CF team, health and education professionals. In some circumstances, in consultation with the CF team, a small number of children may complete their intravenous antibiotics through the Hospital in the Home (HITH) program. During this period, the child may be able to attend school for short periods between treatment times. Educational Implications Most students with CF are well and have minimal restriction to their school life. However, occasionally teachers may have contact with a student who has moderate to severe lung disease. The Role of the Education Advisor at the Royal Children’s Hospital If hospital admission is required, the young person is referred to the Royal Children’s Hospital Education Institute. On acceptance of the referral, the young person is allocated an education advisor. Their role is to support the continuity of educational opportunities for the student by maintaining connections with the school of origin, by establishing links with the key contact person at the school. Communication via fax, phone, email and the use of innovative information and communication technologies is established to ensure the young person is well connected with his or her teachers and peers. The Education Advisor contributes to and facilitates: • the establishment of a school based support group; • school based professional development training by the hospital’s multidisciplinary team; • development of school based strategies for inclusion; and • provision of resource information packages. The Department of Respiratory Medicine at the Royal Children’s Hospital conducts an annual teachers’ seminar early in Term 1 each year. This one day meeting is held at Royal Children’s Hospital - Education Institute Phone: (03) 9322 5100 Fax: (03) 9328 4433 Page 5 This Information Package has been developed in consultation with medical, nursing and allied health professionals at the Royal Children’s Hospital Australia. School Re-entry A carefully planned school re-entry program facilitates the successful return of the student. The Royal Children’s Hospital Eduction Institute can assist by: • making phone calls and arranging to provide relevant written information via fax, e-mail or post; making a school visit; giving an on-site presentation; offering professional development programs; providing a video which details the student’s progress in hospital, together with relevant information regarding his or her special needs; arranging desktop videoconferencing to facilitate education connections; arranging a Program Support Group meeting; providing an information package; and providing a transition summary report. • • • • Cystic fibrosis has an impact on the entire family. Siblings can experience a range of emotions including resentment because of the time consuming nature of CF care and special attention given by family and friends to the student with CF. Teachers also need to be aware of other family and friends who may be picking up siblings or dropping them off if the parents are supporting the CF student. Anxiety can be caused by concern about the deteriorating health of the affected brother or sister. Parents should be encouraged to inform the school if the student with CF is hospitalised or unwell so that support can be offered to the siblings. School Camps With good planning and communication most children and adolescents with cystic fibrosis should be able to attend school camp. Many parents are anxious about sending their child to camp because of concerns about physiotherapy and the administration of medication. It is important for young people with CF to join their peers at camp and not to feel different due to their chronic illness. From an early age, people with CF are taught to be independent with their daily treatment. The level of teacher supervision will vary between individuals. CF is not a condition where the child is well one minute and sick the next. However, the Royal Children’s Hospital should be contacted if the student requires any medical attention whilst on camp. Please phone 9345 5844/5818 during business hours or 03 9345 5522 after hours and ask the switchboard to page the Respiratory Consultant or Fellow on call. Please refer to Appendix 1 – ‘School Camp Checklist’. • • • • Integration Issues Most young people with CF attend school regularly and are supported within their own school environment. Application may be made for integration funding in particular circumstances. Students who experience long and/or frequent absences from school, and require assistance and treatment, may be eligible to receive this funding. Applications are made in conjunction with the Program Support Group guidelines. Siblings Primary School School Commencement Royal Children’s Hospital - Education Institute Phone: (03) 9322 5100 Fax: (03) 9328 4433 Page 6 This Information Package has been developed in consultation with medical, nursing and allied health professionals at the Royal Children’s Hospital Australia. For all young children and their families, the commencement of school life is an exciting and eventful time. It is not surprising that the parents of children with CF anticipate the start of school life with particular apprehension. Handing over the care of their child to someone else will naturally cause some concern for the family. It may also be the first time the child with CF recognises that he or she is different in some way. Common parental concerns include: • • • • • children having time to finish their lunch; taking of enzymes; being teased about taking medication; that their child will be treated like everyone else; and access to toilets. drawn to them. If supervision is required, it is important that someone whom they feel comfortable with accompanies them. Frequent trips to the toilet may be necessary for students with CF. Again, this may cause embarrassment and anxiety. Easy access to toilets should be considered when planning an excursion. Fatigue The student with CF has a hectic daily schedule, which often includes a physiotherapy session prior to school each morning. This may cause students to be tired, especially if they have been feeling unwell. However, students with CF should be able to participate in any activity. Physical activity is strongly encouraged. Special Events Special events at primary school such as school photos, guest speakers, class celebrations, parades or productions are an exciting highlight for all students. It is possible that on the day of a special event the student may be absent due to health care requirements. If this is the case, then it will be important to inform parents or other key contacts (such as an Education Advisor) to discuss any arrangements that may allow this student to participate. Hospital treatment may be re-scheduled to allow the student to attend school for a special event. The Royal Children’s Hospital conducts an annual School Commencement meeting for parents. The aim of the meeting is to discuss the implications for a child with CF starting school. Parents are encouraged to practise the ‘before school’ CF care routine over summer so their child will arrive at school on time. Good communication and careful planning between home, hospital and school will ensure a successful commencement to school life. School Excursions Students with cystic fibrosis are able to attend school outings and excursions. However you may need to take into account the possibility of student fatigue and the need to take enzymes before any food intake. This is to ensure the food is digested properly. If there is going to be an extra ‘treat’ on the day, check with parents to see if the student will require extra enzymes. Parents can also provide extra enzymes to be held at the school for special occasions. They should be replaced each term to ensure their efficacy. Parent/teacher Communication A communication book may be a useful tool for teachers to provide notes to parents about any issues relating to the student’s condition e. g. observation of fatigue. Students with CF may need to cough regularly. Coughing should not be discouraged. However, paying undue attention to their cough may cause embarrassment and anxiety. If necessary it is important that students with CF are able to leave the room without attention being Royal Children’s Hospital - Education Institute Phone: (03) 9322 5100 Fax: (03) 9328 4433 Page 7 This Information Package has been developed in consultation with medical, nursing and allied health professionals at the Royal Children’s Hospital Australia. Transition between Years 6 and 7 Transition from primary school to secondary school can be an exciting, yet daunting experience. Students need to adjust to a larger number of teachers and may be anxious about such a big change. It may be important for the student with CF that a structured transition plan is implemented prior to commencing secondary school with the family, student, primary and secondary teachers. It is essential that students feel comfortable about sharing information in relation to their condition with secondary school staff. Their decisions should be respected. To ensure that the transition is successful, regular meetings should occur during the transition stage. assist with the early identification of difficulties. Medication and Enzymes Most young people with CF need to take pancreatic enzymes with meals and snacks to ensure their food is digested properly. Enzymes are not medication and are harmless if taken by others. These enzymes help the body to absorb nutrients from food and reduce both the bulk and the number of stools, and the amount of flatulence. Students may still need toilet privileges to be able to leave the room quickly and without embarrassment. Enzymes are usually given by the parents to the student to enable his or her independence with enzyme therapy. Secondary School There are striking variations in the severity of cystic fibrosis and each student should be treated as an individual. The student’s privacy is paramount. Any sharing of information must be done in consultation with the student and with a parent’s permission. Coughing Young people with CF may need to cough regularly. Coughing should not be discouraged. However paying undue attention to their cough may cause embarrassment and anxiety. If students know they can leave the class to get ‘a drink of water’ or to clear their throat and dispose of their tissues without attention being drawn to them, they will feel more comfortable and cause less distraction to the class. Body Image Young people with CF may be underweight. This may impact on their body image and make them sensitive about weight issues. Transition Students with CF who have absences from school may experience a gradual disassociation from school. This can affect their learning, especially in sequential subjects such as mathematics and foreign language study but also in areas such as work experience, vocational training, special activities and subject selection. When students are making a transition within a school with new staff or a different campus, the continuity of their educational opportunities will be enhanced by a comprehensive handover. Transition between Year 10 and the VCE Students moving into their VCE years participate in several workshops informing them about subject selection, study skills, postsecondary options and other relevant topics. Students with CF who have absences from school may not be in attendance for one or more of these vital information sharing sessions. This Psychological CF tends to make students feel different, even though the disorder can be categorised as ‘invisible’. Feeling a part of the peer group is extremely important. Provision of counselling time to address these issues may Royal Children’s Hospital - Education Institute Phone: (03) 9322 5100 Fax: (03) 9328 4433 Page 8 This Information Package has been developed in consultation with medical, nursing and allied health professionals at the Royal Children’s Hospital Australia. can make the process of transition to the VCE years bewildering, uninformed and may consequently lead to a lack of confidence in preparing for the challenges ahead. In some cases, students with cystic fibrosis may be eligible for special provisions when sitting for external examinations. VCE School Assessed Coursework (SACs) and School Assessed Tasks (SATs) may need to be designed or adapted to accommodate any special needs of a student with cystic fibrosis. All students are required to meet course requirements. Schools may be able to re-schedule School Assessed Coursework due to a student’s hospitalisation or recuperation at home. Students can be given an extension of overall time to complete a task and, if necessary, regular breaks can be built into examinations. Teachers should contact the Royal Children’s Hospital Education Institute for further clarification regarding individual students. Teachers can also consult the Victorian Curriculum and Assessment Authority on 9651 4300, or view the website at www.vcaa.vic.edu.au. Carrier A person possessing a single gene for a genetic trait or disorder, such as CF. Carriers show no sign of the trait. In CF, each parent of a CF child is a carrier of a single CF gene. Cyst (as in cystic) A closed sac containing fluid. Enzymes Substances which help produce and/or accelerate certain chemical processes in the body such as breaking down of foods during digestion. Trade names for enzymes include Pancrease, Cotazyme Forte, Creon and Creon Forte. Fibrosis Formation of fibre or scar tissue as a result of structural change or other damage. Flutter A physiotherapy device shaped like a pipe. As one breathes out into it, vibrations and some generated positive pressure in the lungs help mobilise secretions. Frogs A colloquial term for sputum/spit/mucus. Gastrostomy Additional nutrition can be administered directly into the stomach during sleep by a surgically positioned device known as a gastrostomy tube. There are two kinds of tubes: a temporary tube which is in place for approximately six weeks and a low-profile permanent tube which sits flush to the skin. It is also known as button, MicKey™ or PEG. Tertiary Transition Options The Education Advisor working with the young person may discuss career options beyond VCE. Advice is given to complement each student’s personal situation. Young people with CF may be linked to welfare and disability liaison staff in tertiary institutions. Funding options can also be explored e.g. ‘Futures for Young Adults,’ a program for young people with a chronic illness to receive financial support as they progress through their tertiary studies. Glossary of Terms Antis A colloquial term for antibiotics. Temporary Gastrostomy Permanent Low Profile Gastrostomy Royal Children’s Hospital - Education Institute Phone: (03) 9322 5100 Fax: (03) 9328 4433 Page 9 This Information Package has been developed in consultation with medical, nursing and allied health professionals at the Royal Children’s Hospital Australia. Infusaport/Port An infusaport is an internal central venous access device that is placed under the skin. It is also known by its trade name, PORT-A-CATH™. Flushing the PORT-A-CATH™ CF Clinical Nurse Consultant, CF Home Support Team, Royal District Nursing Service, Ms Alex Robinson 0410560336 Permanent PORT-A-CATH™ site is approximately the size of a 20c coin and is a raised area often located below the nipple. Nebuliser A device used for delivering a mist. In the treatment of CF, nebulisers may be used for inhaling aerosol antibiotics or mucolytic drugs. PEP (Positive Expiratory Pressure) PEP therapy involves breathing out against some controlled resistance. This is used in CF to expand areas of lung collapse and/or help mobilise secretions towards the upper airways. It may involve the use of a mask or mouthpiece attachment. Pseudomonas Bacteria which frequently colonise the lungs of people with CF and are a major cause of respiratory infections. Sweat Test The diagnostic test for CF to measure the concentration of salt in sweat. Websites Royal Children’s Hospital www.rch.unimelb.edu.au Royal Children’s Hospital Education Institute www.rch.org.au/edinst/ Research Trust www.cfresearchtrust.asn.au Useful Contact Numbers and Websites RCH Education Institute (03) 9322 5100 CF Clinic Coordinator RCH Department of Respiratory Medicine, Ms Judith Glazner (03) 9345 6259, (03) 9345 5818 Royal Children’s Hospital - Education Institute Phone: (03) 9322 5100 Fax: (03) 9328 4433 Page 10 This Information Package has been developed in consultation with medical, nursing and allied health professionals at the Royal Children’s Hospital Australia. Appendix 1 School Camp Checklist Some simple precautions for camp include: Discussion of specific requirements with the student and his or her family. The provision of a copy of a student’s daily treatment plan (physiotherapy and medication). The family are encouraged to discuss possible variations in the treatment plan with the CF CaTeam. Nominating a teacher (if possible, someone who knows the child and is liked by them) to be the student’s contact person if there are any concerns. Providing a location, if required, where students with CF can conduct their physiotherapy and take their medication in private. Salt supplements as well as fluid replacement when the student with CF is engaged in strenuous activities or during hot weather. The availability of table salt at meal times. Access to pancreatic enzymes during meals and snacks. Dosage will vary between individuals and the fat content of meals. Checking the storage requirements for medication. Providing access to electricity for the use of a nebuliser. Car battery operated nebulisers are also available for camping purposes. Checking the student’s dietary requirements. A diet high in energy and fat is recommended. Contact numbers of the local medical centre closest to the camp location. Camp Location:__________________________________________________________ Medical Centre:__________________________________________________________ Phone Number:__________________________________________________________ For emergencies, please call the Royal Children’s Hospital switchboard (24 hours) on 9345 5522 and ask to page the Respiratory Consultant on call. Royal Children’s Hospital - Education Institute Phone: (03) 9322 5100 Fax: (03) 9328 4433 Page 11 This Information Package has been developed in consultation with medical, nursing and allied health professionals at the Royal Children’s Hospital Australia.