Care and Mobility considerations
Care
Care needs in children with cystic fibrosis relate to the amount and complexity of medical management of their condition. Older children are likely to require more complex and time consuming treatments as lung damage progresses due to repeated infections. Older children may carry out much of their care themselves but may require prompting and close supervision. Care of their chronic illness together with other life changes i.e. adolescence, school changes, examinations can be over whelming for the young person. Close supervision of treatment by a parent may extend beyond 16.
For all children the main component of care will be related to chest disease. Care needs relate to twice daily physiotherapy, inhaled, nebulised and oral drug treatments. Most children will have pancreatic insufficiency requiring enzyme replacement therapy with meals and snacks and some may have additional care needs related to artificial feeding. Some children may have needs related to both chest disease, pancreatic insufficiency and other complications of cystic fibrosis such as cystic fibrosis related diabetes. Chest disease and dietary management are the most time consuming aspects of care and should be the focus of needs assessment.
The extra care required can make it difficult to get out of the house in time to attend groups and parents of non CF children do not all ways understand because people with CF usually look well. This can leave a family feeling isolated.
Lung and respiratory system
Mildly affected babies and very young children are unlikely to have significant care needs in excess of a normal child although these babies do require extra care. They will be on prophylactic oral antibiotics and need chest physiotherapy.
Some babies may need changes of oral antibiotics, pancreatic enzyme supplements and high calorie supplementary feeding to keep them well. The aim is to closely monitor and respond promptly to slight changes in their baby’s condition in order to keep their baby’s lungs free from changes due to infection. Such changes include development of a cough, changes in appetite, changes in bowel movements and lethargy. The parent carer will have been taught how to assess their baby’s chest and to how to carry out physiotherapy at diagnosis.
Care needs often develop over time as a result of repeated chest infections. The main component of care in the early years is physiotherapy. Children also need appropriate antibiotic prophylaxis, salt supplements, vitamin A, D & E, plus a second antibiotic as soon as there is a slight change in symptoms. Parents need to make sure all medicines are taken. This is difficult when babies dislike the taste and spit them out.
Initially they have 2 weekly appointments, then monthly for the 1st year and then every 2 months and also in between when necessary. They may travel a long way to a regional centre for some of their care.
Days out need to be planned to ensure there is time for physiotherapy and all of the medications. Ensuring enzymes and appropriate feeds/snack are available. Everything is more time consuming and fiddly away from home.
When going on holiday an adequate supply of everything needs to be ordered in advance, there needs to be thought as to where holidays are taken, adequate travel insurance for example.
Parents are advised to avoid people with colds/coughs/runny noses this may mean cancelling family events.
The role of physiotherapy is to remove mucus from the chest; the amount of mucus produced is variable between children. Babies and young children usually do physiotherapy every day from diagnosis, rarely once a day usually twice daily. This takes around 20mins each time if they don’t object and wriggle too much. Physiotherapy is usually done about an hour before feeds to lessen the possibility of vomiting.
For school age children the minimum is around 15 minutes twice a day, duration and frequency is variable depending upon the mood of the child and how well they are, if they are coughing, have thick secretions or are wheezy it will take longer. Specific physiotherapy techniques and the use of positive expiratory pressure (PEP) devices do not indicate prolonged physiotherapy is required. As lung function deteriorates physiotherapy takes longer. Care needs are likely if nebulised drug treatments are used. These have to be spaced out around the physiotherapy. Use of these drugs indicates that lung complications have occurred such as:
- Colonisation with bacteria such as Pseudomonas aeruginosa
- Difficulties expectorating sputum
- FEV1 is 70% or less of normal or evidence of small airways obstruction
These problems often mean that more than 15 minutes of physiotherapy is prescribed It is convenient to set aside a time twice a day to do everything at once particularly if several drugs have to be given spaced around physiotherapy. Some nebulised drugs must be given at least an hour before physiotherapy, some just before and some are best given when the lungs are clear. The nebulised drug/ physiotherapy routine is a significant time commitment each day and may take up to an hour and a half. Children are encouraged to learn about and do their own physiotherapy and nebulisers under close supervision from the age of 8. The need for prompting and supervision reduces over the years and children with mild disease could be expected to manage their nebulisers and physiotherapy substantially themselves from the age of 14. Some young people require close supervision beyond age 16 and some will require supervision and help because of the severity of their condition. Even when close supervision is not required parents are usually still involved and provide encouragement and prompting.
Note that nebulised drugs cannot be mixed together and they may not be given at the same time. Depending on the brand of nebuliser and mood of the child each nebulised drug my take 3-4 minutes or 15 minutes to give twice a day for example:
- rhDNase Pulmozyme) –at least 1 hour prior to physiotherapy
- Bronchodilator (Salbutamol) – 10-15 minutes prior
- Hypertonic saline – immediately prior
- Steroids and antibiotics - after physiotherapy
Children will most likely also take regular oral antibiotics to prevent infection, these do not add significantly to care needs. During exacerbations children may take intravenous antibiotic treatment, this is used intermittently for 2 week courses and therefore does not add to care needs on an average day. If long term antibiotic treatment is required these will be given as nebulisers or orally. The exception to this is in end stage disease, IV antibiotics may be given at home for a 3 month course. This adds significantly to care needs as doses are typically 3 times a day and must be given at regular intervals e.g. 8 hourly at 0800, 1600 and 0000.
The parent /carer will monitor their child carefully for small changes in secretions, cough and general health in order to catch any exacerbations early. Care needs increase during chest infections in all children whether mildly or severely affected.
Asthma and severe small airways disease
Mild asthma is common and does not significantly increase care needs because prophylactic treatment takes minimal time to administer but never the less these are additional treatments to an already busy treatment regimen. Asthma on step 4 or step 5 of the asthma guideline or a diagnosis of severe small airways disease are likely to significantly increase care needs because the time spent on physiotherapy increases and medication needs are complex. The use of nebulised treatments significantly adds to the time commitment.
Home oxygen
Home oxygen is given at night via concentrator. Home oxygen does not indicate night needs as the parent does not need to stay awake to monitor oxygen levels. However it does indicate severe chest disease. Such children are likely to have a time consuming regimen to follow during the day including more than 15 minutes of physiotherapy and time consuming nebulised drugs. Even if they are over 16 fatigue and breathlessness may mean that they need help from a parent/carer with all aspects of this. Children in the home oxygen group are likely to have mobility needs.
Lung transplant
Lung transplant is not a cure for cystic fibrosis. However, if transplant is successful walking ability, fatigue and breathlessness can be expected to significantly improve for some years. After successful transplant care in terms of physiotherapy and medication is required but often a young person will be able to do the majority, if not all of this for themselves. Reassessment of needs after lung transplant should be delayed until after rehabilitation. It takes time to recover from surgery, settle down on anti-rejection drugs and build physical stamina. At 3-6 months post transplant longer term prognosis of disability should be clear although there will be a subsequent gradual decline in lung function due to chronic rejection.
Gut and digestive organs
Babies
Enzyme replacement is required for nearly all babies with CF, it is fiddly to mix the enzymes with a spoonful of milk and give it to the baby at the beginning of a feed. It is difficult to get enzymes levels right as it depends on the individual baby and how much milk they take. It is more difficult for breast feeding babies as the amount of milk consumed cannot be measured. This means malabsorption is not always well controlled. Breast or bottle fed babies struggling to gain weight may need additional standard or nutritionally enhanced formula milk after feeds.
Some babies also need Gaviscon after feeds if vomiting frequently. Stools need to be monitored for frequency, colour, odour and consistency to assess the correct dose of enzyme replacement. Increased amounts of vomiting and loose stools mean more time is spent cleaning and washing these babies and their clothes.
Children
90% of children have pancreatic insufficiency which is treated with pancreatic enzyme replacement therapy. Enzymes are taken whenever food is eaten and this can prolong meal times. Salt tablets, vitamin supplements and sometimes dietary supplements are also taken daily by mouth. Enzyme doses have to be adjusted depending upon the fat content of the meal. It is important to get the doses as correct as possible to ensure maximum utilisation of calories and nutrients, which maximises growth and protects against infection. Too much Creon can result in abdominal pain, constipation and an obstruction; too little Creon results in increased bowel action, abdominal pain and weight loss.
Nutrition is very important; children should have 3 meals and 1-3 snacks daily together with their enzyme replacement. Good nutrition improves life expectancy and quality of life.
It is very difficult for some children to achieve this and very often fortified drinks are included to ensure sufficient calories are eaten each day. Daily encouragement to eat enough may be hard work and an endless battle.
Artificial feeding
Care needs in excess are likely when artificial feeding is used. Typically feeds are run overnight though a gastrostomy or PEG feeding tube. The tube and tube site needs to be kept clean and some tubes need turning every day. This takes a few minutes and can be done at bath time. The tube may need to be dressed during the day if the port site is infected.
The feeds are set up at bedtime, some feeds require mixing and preparing and others are ready made and can simply be attached to run through the feed pump. Monitoring during the night is not required and older children e.g. 8 and over should be able to take the feed pump to the bathroom if they go to the toilet during the night. Parents will need to disconnect the feeding tube in the morning and make sure the tube is clean and securely closed for the day.
Children over 14 will be able to set up and disconnect their feeds themselves and care for the tube site. Children over 14 and young adults with poor lung function are likely to require help with some aspects of artificial feeding due to fatigue, e.g. mixing feeds.
Feeding pumps are very sensitive and alarm during the night if child turns over a lot in their sleep. This may well result in extra time getting the child back to sleep by the parent/carer.
Cystic Fibrosis Related Diabetes
Separate guidance on diabetes and insulin treatment is provided. The child with cystic fibrosis will have multiple things to think about, understand and take responsibility for as they learn to manage their condition. In addition to monitoring blood sugar and calculating insulin requirements they will also need to manage their pancreatic enzyme replacement therapy at meal times and remember to take vitamins, antibiotics and possibly dietary supplements. Artificial overnight feeding may make their diabetes more complicated to manage. Care needs are additive.
Liver disease
Liver disease does not give rise to disabling effects until liver failure has occurred. Liver failure causes fatigue. Walking ability and ability to manage physiotherapy may be reduced.
Mobility
Mobility needs only arise when severe lung damage and respiratory failure are present. This means that the lungs cannot meet the oxygen demands of the body. FEV1 will be 40% or less and the child may be on the waiting list for a lung transplant.
