Source: Annual Congress 2009 - Cystic fibrosis: advances in clinical research
Disease area: Paediatric lung diseases

Abstract

Background: Over the last decades, median survival in cystic fibrosis patients (CF) has markedly improved to 40 years and more. This circumstance has led to an increasing need for transition of CF adolescents to adult CF care. Patients, families and paediatric CF teams might have some doubt with regards to a successful transition.
Methods: We reviewed our recent experience, evaluating all CF patients undergoing transition (02/2004-02/2008) regarding their demographics/social status and clinical condition including the Liou Raw-Score (Liou et al, 2001), at time of transition and 1 year later.
Results: 20 CF patients (11 females) underwent transition at a median age of 20.8 years. 19 patients were in full-time employment at transition, 2 of which had to considerably reduce employment by 1 year thereafter. No significant change in mean FEV₁ (2.3±0.8 vs. 2.3±1.0 L), sputum bacteriology, and body mass index (21.3±2.7 vs. 21.4±3.3 kg/m²) was detected over the 12-months period. There was a slight increase of the frequency of chest exacerbations, resulting primarily from significantly more exacerbations in 4 patients chronically infected with Burkholderia species, 1 of which was evaluated for lung transplantation. There was a slight but non-significant change in the Liou Raw-Score (70±35 vs. 65±40) and expected 5-year survival (89±14 vs. 86±18) 1 year after transition.
Conclusion: Our CF cohort underwent successful transition from paediatric to adult care with regards to their social status and clinical condition. An increase in exacerbation frequency per patient years was identified; however, there was no negative impact on expected 5-year patient survival.

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M. Hofer, C. Benden, G. M. Tini, R. Spinas-Haeller, A. Moeller, P. Eng, A. Boehler (Zurich, Aarau, Switzerland). Transition from paediatric to adult care: perspective of an adult CF-centre. Eur Respir J 2009; 34: Suppl. 53, 1253

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