There is no other condition quite like cystic fibrosis (CF). Faulty genes that the parents usually did not know they carried have caused it. The newborn baby will usually be free of any problems, but the young child is likely to have problems with weight gain and with frequent respiratory infections. Once the diagnosis has been made, the family must learn a complex new set of rules: medication needs to be given several times every day, and chest physiotherapy must be learned and performed twice a day, sometimes more. The family and later the child need to become medical experts: they will need to recognise when they need to seek extra help from their CF team—how do they know when the child is coughing enough to need another appointment? They will need to learn to work with a whole range of willing and enthusiastic practitioners including doctors, nurses, physiotherapists, dieticians, psychologists, social workers and pharmacists. Not to mention the additional worries of school absences for inpatient and outpatient consultations. Then on top of that there are the family crises—not just the normal ones that all families will go through, but those when the child may grow Pseudomonas aeruginosa for the first time and a 2-week hospital admission is required, often with minimal advance notice. As the child grows up, they will be expected to learn more about their health and how to maintain it, with a gradual reduction in responsibility from their parents and the expectation that they will become largely independent of parental input.

There is convincing evidence in favour of improving survival for patients with CF, despite the fact that there is as yet no definitive treatment to cure the defect at a cellular level.1 2 This improved survival is a reflection in part of the improving and pro-active care provided during childhood. Continuous improvements in health and survival suggest that mean survival of recently born infants may be greater than 50 years (fig 1).2 New treatments are being developed that are expected to modify the course of the disease and further improve survival. The CF community eagerly awaits the results from these studies. Both paediatric and adult care is likely to become even more pro-active with improving health surveillance and aggressive early management of complications. This should lead to further improvements in CF survival.

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Figure 1 UK cystic fibrosis population. Proportion of (A) males and (B) females of each 3-year cohort surviving until 2003.

It is now estimated that more than half of the current patients are over 16 years of age. Whereas the numbers of children are expected to remain static, numbers of patients in adult clinics will continue to climb (fig 2).

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Figure 2 Actual (up to 2005) and projected numbers of adult and paediatric patients with cystic fibrosis (CF) registered on the South West CF database, Bath, UK.

CHANGING INCIDENCE OF PROBLEMS AND COMPLICATIONS

Older patients with CF have a different spectrum of pulmonary and non-pulmonary complications from their younger counterparts. Pulmonary complications more commonly encountered in adult patients include infection with unusual and multi-resistant organisms, haemoptysis, and respiratory failure that may require support such as non-invasive ventilation and transplantation. There is an age-related increasing incidence of CF-related diabetes3 and recognition of microvascular effects especially retinopathy,4 5 of osteoporosis especially in older patients with more severe disease and following prolonged exposure to corticosteroids,6 and of other emerging conditions including unusual cancers such as those of the small intestine, colon and biliary tract.7^–9 In contrast, CF-related liver disease and cirrhosis occur mainly in the first decade of life, and incidence probably does not increase significantly thereafter.10 11 It is appropriate for care provision to move from a paediatric to an adult base. It is not appropriate for paediatricians to care for adult patients nor for the care setting of adult patients to be a paediatric one.

The teenage years are a time when young people explore and challenge their world. It is a time when personal CF care may suffer. Families and carers may have to watch as a young person’s health deteriorates. The person with CF may face different challenges with their health. All this at a time when changing priorities for the adolescent and adult patients will make them think more about the concerns they share with their peer group—friendships, higher education, work, finances, as well as relationships and in some cases starting a family of their own. Although male patients are usually infertile, assisted reproductive techniques such as intracytoplasmic sperm injection can be used to assist fertility and enable them to father children of their own.12 Knowledge of infertility is low among adolescents.13 Female CF fertility is usually unaffected by the condition, and obstetric services work alongside adolescent and adult services to provide appropriate maternity care. Appropriate genetic counselling will be helpful because of the increased risk of parents with CF having children with CF. In some adolescent patients, there will be serious complications of deteriorating health, with a massive increase in the utilisation of and dependence on healthcare.

MODELS OF TRANSITION

Children with CF in the UK are cared for in both large regional centres, typically looking after 100 or more children, and smaller specialist clinics in district general hospitals, usually with formal shared care arrangements with a larger regional centre. Adult care arrangements are generally through large regional centres. For a large number of adolescents, moving from a paediatric to an adult clinic may involve a geographical shift in the care setting away from the hospital most local to the home, making the preparation for transition even more critical.

Paediatricians have long been aware of the challenges of ensuring that their patients continue to receive appropriate services as they move into adult life. CF paediatricians and doctors have led the way in developing models of transitional care over the course of at least the last 20 years.14 15

There are several different models:

• Paediatricians continuing to provide the service for life: this model is now much less common as the number of adult patients has increased and the number of adult chest physicians with appropriate training is also increasing. (Some CF-trained paediatricians may choose to act jointly with their adult colleagues as “CF physicians”, especially in middle sized clinics.) Paediatricians are usually inexperienced in the management of psychosocial aspects of the adult patient, and the frequency and pattern of complications such as diabetes will be different from their paediatric practice.

• Paediatricians handing over to designated adult physicians at a “CF centre”: arrangements must be made for those who do not live locally to have access to appropriate community services. The CF centre will have developed links with other specialists who the person with CF may need—for example, interventional radiology, hepatology and maternity services. These CF centres may be geographically distant from the local hospital where paediatric care was provided. Transition arrangements will be particularly challenging in these circumstances, but local arrangements will need to be made to address the priorities of a transition clinic outlined below.

• Paediatricians handing over to a joint service between the local chest physician and a designated CF centre. This has the benefit of retaining local links with the expertise of a CF centre.

Whichever model is adopted by CF teams, the transition arrangements must be clear to the patients, and, during the period of transition, care must be provided that is appropriate to their needs and in a care setting appropriate to their age and development.

Evidence exists that CF centres provide better care than smaller clinics.16 Adults have increasingly challenging illness as they grow older, and the expertise of a centre can be invaluable. However, patients with CF may prefer to have a service closer to home. For those reaching the end of their life, a local service that they know and trust is very important. CF centres must continue to work with local and general practitioner services; this is particularly important if palliative care is going to become necessary.

Thinking about transition to adult care starts soon after transfer to secondary school, usually aged 11 years in the UK. This is a time of ensuring young people themselves are informed about their condition. They are often becoming increasingly aware of differences from their peers and have a reluctance to talk about their illness with friends. They need to be offered the opportunity of finding out as much as they need to know about CF; issues around fertility are especially important. A dedicated CF psychologist can be invaluable in helping young people understand these issues. The UK CF Trust provides very helpful age-appropriate literature.17 Children need to be encouraged to share the responsibility of their CF care with their families—gradually taking on more of the burden of remembering medication, carrying out their physiotherapy, reporting their own symptoms of cough and breathlessness. Compliance with treatment can become massively challenged if there is significant adolescent rebellion. Young people can be seen on their own in the paediatric clinic, with their parents being asked to wait outside and brought in towards the middle or end of the consultation. This can almost always be achieved in a non-threatening manner and will show individual patients that they are being listened to. This is often a staged process, the first consultation possibly being rather short but developing a rapport and expectation that this will become the norm for clinic attendances. Parents will often also want to meet the team, and this can be done with the agreement of the patient once they have been seen alone.

Westwood et al18 reported that adolescents and young adults value some autonomy in healthcare and prefer a long transition process ending with a transfer from paediatric to adult care at age 16–18 years. Over 90% felt that a transition clinic would be useful. Older patients (with a mean age of 34.5 years) questioned by Anderson et al19 showed a much lower level of concern about transfer from paediatric to adult services than did their parents and CF team members,20 suggesting that, at least for many patients, where their care is delivered is not very important as long as it is of high quality. Parents and care givers can certainly sabotage the transition process if they express any lack of confidence in the adult team.21

CF services may be provided on the same site as adult services, but often it involves a trip to a different hospital. Multidisciplinary paediatric and adult teams can have joint clinics in either the paediatric or adult environment so that young people become familiar with the new team. Our own experience of holding transition clinics in either the children’s hospital or the neighbouring adult hospital has been that attendance rates are very high in the former and singularly low in the latter. We have now stopped holding clinics in the adult hospital until after the date of transferred care. It is important that patients are clear about changes to the service—for example, there may be limited community nursing for adult patients, whereas previously a community paediatric nurse could visit at home.

The priorities for successful transition of patients with CF (and children with other long-term health problems) that centres and clinics should be addressing are:

1. Establish a transition process to start from 11–13 years of age

2. Develop a written transition guideline to be understood and agreed by all the multidisciplinary paediatric and adult teams

3. Provide written guidelines for the young person and family to describe how they will go through the transition process. Consider involvement of representatives of the young people and their families at this stage

4. Establish a transition clinic at which the patient and family will be seen by paediatric and adult multi-professional and possibly multi-agency team. In this clinic, address issues that may be of concern to the young person, such as whether the adult team will have similar guidelines for admission for intravenous antibiotics. Consider, agree on and document how many occasions each young person and family will be seen in this transition clinic

5. Define with the patient and carers the point at which final transfer will take place, leaving as much choice as possible with the young person and establishing a final deadline. This “deadline” should be clearly defined—usually the 17th, 18th or 19th birthday—and this date should be clearly stated in the written transition guideline

6. Check that all correspondence is complete before the point of transfer from paediatric to adult care

7. Request feedback from the young people after their transfer as to how the local process might be further improved

8. Develop regular meetings between the paediatric and adult clinics and discuss and agree the transition process

FUNDING PAEDIATRIC AND ADULT SERVICES

During the last decade there has been a great change in CF care organisation, with the development of clinical networks for both paediatric and adult services. Historically in the UK, CF has been funded through a block contract arrangement in which hospitals are paid to provide care for all patients with CF but without a clear relationship with the number of patients being cared for nor with their disease severity. Rising patient numbers (because of increasing survival) and rising treatment costs mean that these block contracts usually leave the CF service in a position of continual underfunding.

The total cost of CF care is in direct proportion to the number of patients that a centre or clinic is caring for and to the disease severity of these patients, particularly related to their lung function, as expressed by forced expiratory volume in 1 s, and to airway Ps aeruginosa colonisation status.22 23 It makes poor sense to choose any other funding system than an appropriate nationally agreed tariff-based system. Any local funding agreements will remain unsatisfactory because adult services will continue to expand and therefore will require greater funds to be available. There is a real danger that funding of adult units may come at the expense of paediatric units, as funds are diverted to units with greater patient numbers instead of attracting new investment, with consequent weakening of the paediatric centre. Commissioners of healthcare need to understand the implications of fig 2—that rising numbers equates with rising costs and that paediatric clinic sizes will remain constant. Overall, disease severity of paediatric patients is likely to continue to improve, but this is unlikely to deliver a cost saving, as the treatments and monitoring required are likely to become more expensive. The addition of nebulised preservative-free tobramycin (TOBI) costs an additional £8904 (€11 207, US$17 373) per annum, and the nebulised mucolytic dornase alpha (Pulmozyme) an additional £6760 (€8510.5, US$13 196) per annum.24 The only severity-based funding structure available in the UK is that originally described by Robson et al22 and further developed by the UK CF Trust. Table 1 gives the most recently updated 2004 figures available from the CF Trust (shown with permission). It is now time that this or a similar finance structure should be adopted across the UK to allow resources to match patient groups and to allow adolescent and adult services to continue to develop.

CONCLUSION

All CF centres in the UK should by now have arrangements in place for transition from paediatric to adult services, although all centres need not use the same model. There should not be a “one size fits all” solution. However the care is delivered, ongoing audit is imperative. In the UK, this can be done by monitoring outcomes in comparison with standards set nationally with the assistance of the UK CF Trust. The transition model in place should be centred on the optimal care for the patient and should allow the patient to retain a degree of control as to the speed of transition, albeit with some predefined limits. These models of care that are set up for CF services will need to be reproduced for a burgeoning number of other conditions, in particular those in which multidisciplinary teams are involved, such as adolescents with neuromuscular conditions requiring home overnight non-invasive ventilation.25 These conditions were once thought of as being paediatric problems but are now recognised as being of paediatric-onset with their own specific requirements and changing disease processes through adolescence and into adult life. Appropriate adolescent and adult services need to be developed for all these patient groups with appropriate transitional arrangements that may last many years before the date of final transfer of responsibilities to adult care. It has taken 20 years for CF transitional and adult services to develop. The increasing number of patients, the advocacy of the UK CF Trust, and the long history of collaborative team work among CF teams has allowed this to happen. This model needs to be quickly replicated if it or something similar has not already been developed for young people with other conditions to prevent them from languishing in the “black hole” between children’s and adult services.