Transition from paediatric to adult care in cystic fibrosis.

In the decades since cystic fibrosis (CF) was first clinically defined in the 1930s, there have been many advancements in the treatment and management of this disease. Initially it was considered a disease of childhood where the majority of those affected died before reaching adolescence. Now, through advancements in management and treatment, the vast majority of those affected will live into adulthood. Therefore, paediatric and adult CF services must collaborate to ensure that young people and their families experience a positive and supportive transition into adult services. Key aspects of transition will be discussed, including when to begin the transition process, who should coordinate this and how the transition process should be structured. Challenges of the transition process and potential pitfalls when transition does not run smoothly will also be discussed, as well as tools that may be used to support a positive transition for young people and their families. Educational aims: To familiarise readers with factors that make the transition process positive.To make suggestions regarding the application of the transition process.To highlight factors which may impact on the success of the transition process and the risks associated with disengagement at the point of transition.To discuss tools which can be used by care teams to ensure a smooth transition process.

Controlled evaluation of a transition clinic for Dutch young people with cystic fibrosis.

BACKGROUND: Transition clinics (TCs) are advocated as best practice to support young people with cystic fibrosis (CF) during transition to adulthood and adult care. We aimed to research the functioning of a TC for young people with CF compared with direct hand-over care and to evaluate whether those treated at the TC have better transfer experiences and outcomes compared with the control group. METHODS: Mixed-methods retrospective controlled design, including interviews with professionals, observations of clinics, chart reviews (at four measurement moments), and patient surveys. Qualitative data analysis focused on organization and daily routines, and barriers and facilitators experienced. Young people's transfer experiences, self-management, health care use, and clinical outcomes were assessed quantitatively. RESULTS: The most notable feature distinguishing the TC and direct hand-over care comprised joint consultations between pediatric and adult care professionals in the former. A transition coordinator was considered essential for the success of the TC. The main barriers were lack of time, planning, and reimbursement issues. Young people treated at the TC tended to have better transfer experiences and were more satisfied. They reported significantly more trust in their adult care professionals. Their self-management-related outcomes were less favorable. CONCLUSIONS: The TC had several perceived benefits and showed positive trends in transfer experiences and satisfaction, but no differences in health-related outcomes. Structured preparation of young people, joint consultations with pediatric and adult care professionals, and better coordination were perceived as facilitating elements. Further improvement demands solutions for organizational and financial barriers, and better embedding of self-management interventions in CF care.

Daily Observations of Nebuliser Use and Technique (DONUT) in children with cystic fibrosis.

BACKGROUND: Cystic fibrosis (CF) caregivers focus on correct inhalation technique for nebulisers as this is essential to optimize efficacy of inhaled drugs. However, little is known on this nebuliser technique of patients at home. METHODS: Three "hidden" video registrations were made of 32 children with CF (6-18years) nebulising at home. Videos were randomly scored on inhalation technique items using nebuliser-specific checklists and a total score was calculated. RESULTS: Median nebuliser technique was 91.9% of max score. Nebuliser technique was perfect (score 100%) in 23.3% of the patients and incorrect (score 0%) in 13.3%. Most mistakes were made in the required optimal breathing pattern. CONCLUSION: Most CF patients had good nebuliser technique on a day-to-day basis. However, errors observed likely resulted in reduced treatment efficacy and, in 13%, no treatment at all. Regular "real life" evaluation by the CF-team can improve inhaled therapy substantially.