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The role of the cystic fibrosis nurse specialist

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VOL: 98, ISSUE: 12, PAGE NO: 62

Jacqui Cowlard, BSc, RGN, RSCN, is paediatric home care sister for children with cystic fibrosis, Royal Brompton Hospital, London

The Clinical Guidelines for Cystic Fibrosis Care (Cystic Fibrosis Trust/British Paediatric Association/ British Thoracic Society, 1996) advised that a comprehensive cystic fibrosis (CF) service (level 1 or 2) should ensure ‘smooth transition of patients from paediatric to adult care’. Until the Standards for the Clinical Care of Children and Adults with Cystic Fibrosis in the UK (Cystic Fibrosis Trust, 2001a) there were no national recommendations or models to outline this process of transfer. The role of coordinating this move between services has largely fallen to the each centre’s CF nurse specialist.


The current median age of survival for a person with CF is reported to be 32 years (Epidemiologic Registry of Cystic Fibrosis, 1998). The prognosis has increased greatly over the past decade, with advancing research and treatments together with the development of specialised CF centres.

Betz (1998) states that ‘advances in medical treatments and technologies have led to improved long-term outcomes for children diagnosed with chronic conditions’. Thus there is an increased demand for services to meet the needs of this group through adolescence into adulthood. The increasing adult population with CF has driven our interest in transition planning and service provision for this group.

What is transition?

The American Society for Adolescent Medicine has defined good transition as ‘the purposeful, planned movement of adolescents and young adults with chronic physical and medical conditions from child-centred to adult-oriented health care systems’. It is commonly recognised that adolescence is a difficult time, with many changes (leaving school, starting work, entering relationships) occurring in close succession, and the impact of living with a chronic illness rests heavily on top of everything else. The topic of transition should be introduced to the family at least a year before transfer is expected, allowing them time to explore their feelings and resolve any concerns they may have.

Many young people with CF have seen their peers die as a result of their disease, and it can be difficult to move into the unfamiliar territory of an adult service. For patients graduating from paediatric care there should be a clear indication that there is a positive future ahead, and this can be highlighted to patients and their family by planning for their progression to adult services (Conway, 1998).

Centres have developed multidisciplinary expertise in the management of CF, and the organised delivery of care can now meet the needs of the ever increasing adult population of patients with CF (Elborn, 1998).

The National Consensus Standards for the Nursing Management of Cystic Fibrosis (Cystic Fibrosis Trust, 2001b) recognise that adolescence and young adulthood is a difficult time for those dealing with both the psychological and practical burden of CF, and that the roles of the CF nurse specialist at this time include the following:

- Promoting self-care and responsibility in young adults and offering advice and support to their parents;

- Liaising with schools and colleges to support continuing education;

- Working in liaison with colleagues to ensure that adolescents receive appropriate knowledge regarding issues such as fertility, pregnancy, contraception, safe sex, cross infection, further education/employment and smoking/substance abuse;

- Using specialist expertise and knowledge to advise on the appropriate time for transition and transfer to adult care for each patient.

It is generally accepted that the process of transition should be gradual, with team planning and coordination, but there has been little research examining what services are being provided at present and what makes transition effective from both the professionals’ and the family’s perspective.

Elements of the CF nurse specialist’s role during the transition process

The roles of the nurse seem to fall into three main categories: family support and provision of information, coordination of the transition process and communication.

Family support and provision of information

This begins from the time of diagnosis but has a different emphasis during adolescence and starts to focus and prepare the family for the move into adulthood and adult care. Nurses are in a prime position to support and help adolescents in being responsible for managing their own care as much as possible. They aim to minimise the family’s anxiety and help guide them through the transition period. The team needs to discuss with young clients their understanding of their disease and the rationale for therapy and enable them to understand their symptoms, recognise deterioration and when to respond and to know how and when to access medical resources.

Young people should also start to be seen in their consultations with the doctor on their own and take more responsibility for ordering and preparing their medications. Families will need time to adjust and become familiar with the new team and environment.

There are now many tools to help with the provision of information to both young people and their parents; these include written and electronic information, audio and videotapes. The Cystic Fibrosis Trust website has chat rooms for children, young people and parents to discuss difficult issues and obtain peer support. Patients and their parents need to be made aware of all the available options and be guided through decision-making, ideally by someone who knows them well.

Coordination of the transition process

For any transition programme to be successful it requires the active interest and participation of both the paediatric and adult teams and a defined process individualised to individual patient needs. Viner and Keane (1998) highlight five key elements of an effective transition programme:

- A policy on timing of transfer;

- A preparation and education programme;

- A coordinated transfer process;

- Administrative support;

- Primary care involvement.

Each CF centre runs its transition programme differently, and many provide joint clinics where the young person and his or her family can meet the paediatrician and adult physician together.

An additional issue for patients with CF is that of cross infection, highlighted by Boyle et al (2001), which affects the mixing of the patient populations with different bacteriology. This has also stopped most patients socialising outside of the hospital environment - for instance, in support groups or in meetings.


Effective and open communication channels make the transition process easier to plan, coordinate and evaluate. Communication with families during the period of adolescence must not always be channelled through the parents, and one of the key roles of the nurse is to keep all involved informed.

Where possible the paediatric CF nurse specialist should arrange an early introduction to their adult counterpart, who will be a central person to the patient’s care over future years. Both teams also need to remain aware of the differences between paediatric and adult services, including the approach to care and the care packages provided.

The focus moves from decision-making with the family about their dependent child to decisions made with the independent adult patient, the adolescent or young person being caught midway.

From our experience, integrating some of the therapies through both the paediatric and adult services has been of benefit to both the patient and family. For example, we now use the same home intravenous antibiotic teaching package for parents and adult patients. Regular team meetings and reassessment of the transition programme from both the team and the family viewpoints can help us improve on the current services in place.


According to Harvey (1998), clinical governance ‘includes strategies which allow good practice to be shared and encourages input from patients’. Thus, in an area such as transition, where the patient is the centre of the process, we can reflect on and develop current practice. The CF nurse specialist is integral to the planning and coordinating of this service and can guide others to help provide effective adolescent care, enabling young people to take responsibility for managing their own illness.

To improve transition programmes, clinical services need to be better linked and new services developed that look specifically at the right time for transition and transfer, incorporating the skills of good communication and effective liaison. Perhaps as health professionals we could look at the information that schools and businesses use when people change from one environment to another and use their ideas alongside our own. Ultimately, with effective planning and coordination of services we are more likely to succeed in transition.


Department of Paediatric Respiratory Medicine

Royal Brompton Hospital

Sydney Street



Tel: 020 7352 8121 (for CF guidelines)

The Royal Brompton Hospital and Great Ormond Street Hospital are currently running a two-week course entitled ‘Managing the care of children and adults with cystic fibrosis’ on a biannual basis. The course is accredited at level 3 and MSc. Further course information can be obtained from Fran Duncan-Skingle via bleep 6002 at the Royal Brompton Hospital.

Cystic Fibrosis Trust

11 London Road




Tel: 020 8464 7211


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