Original article
Clinical Changes of Patients with Cystic Fibrosis during Transition from Pediatric to Adult Care

https://doi.org/10.1016/j.jadohealth.2008.03.005Get rights and content

Abstract

Purpose

To evaluate the clinical changes of adults with cystic fibrosis (CF) during transition from a pediatric to adult CF center.

Methods

Data were collected at the time of transfer, 1 year earlier and 1 year later, for all patients in our adult CF center arriving from one of the three pediatric CF centers in Paris between January 2001 and June 2004.

Results

Sixty-three of the 68 patients (transferred at a median age of 21.0 years) were regularly attending this adult CF center after 1 year and one had died. The mean number of outpatient visits increased in the year after transfer (5.7 vs. 3.8 in the year before, p < .001). The occurrence of clinical events and the rate of bronchial colonization did not change. Pseudomonas aeruginosa was found in about 60% of patients at any time. Pulmonary function declined regularly with no statistically significant difference in the rate of decline between the 2 years of follow-up (FEV1 was 54.7% predicted at transfer). Nutritional status remained stable (mean body mass index was 19.1 kg/m2). The number and duration of oral and i.v. antibiotic courses did not change, but more patients received them at home (p < .001) and self-administered physiotherapy after transfer (p = .001). The proportion of students decreased from 79.3% to 48.1% (p = .02) and the proportion in the workforce increased from 12.7% to 20.4% after transfer.

Conclusions

Patients with CF remained clinically stable during transition and progressively acquired autonomy.

Section snippets

Patients

Seven CF centers in the Paris, France, area manage more than 1000 patients with CF: four are pediatric centers (Necker, Trousseau, and Robert Debré hospitals inside Paris, and André Mignot hospital in Versailles), one is a mixed pediatric and adult CF center (Créteil), and two are specifically adult CF centers (ours at Cochin hospital, Paris, and another one at Foch hospital, Suresnes). A retrospective survey was performed, including all patients arriving at our center from January 1, 2001 to

Demographic data

Sixty-eight patients were included in the present study. They came from one of the three pediatric CF centers in Paris (35 from Necker [51.5%], 19 from Trousseau [27.9%], and 14 from Robert Debré [20.6%]). Approximately 100 adults with CF had arrived at our CF center between January 1, 2001 and June 30, 2004.

Thirty-six of these 68 patients were women (52.9%) and 32 were men (47.1%). The mean age at diagnosis was 3.8 ± 5.2 years; the mean age at first arrival at the pediatric CF center in Paris

Discussion

Improvement in care of patients with CF has resulted in longer survival. A larger proportion of patients now reach adulthood. These patients require an age-appropriate care and counseling about adult issues, for example, independence acquisition, career choices, and reproduction. In the last 10 years, pediatric and adolescent health professionals have emphasized the need for developmentally appropriate health care and a smooth transition from pediatric care for young adults with chronic

Conclusion

Clinical status and care of young adults with CF did not change after transfer from a pediatric center to our adult CF center. There was no noticeable change in bacterial pulmonary colonization and in the main treatments. Nevertheless, they acquired autonomy in everyday life and in their disease management after transfer. These findings are likely the result of good transition policies and implementation within the various CF centers referred to. In addition to trying to improve survival,

Acknowledgments

We thank the physicians from the pediatric CF centers in Paris, A. Clément, B. Fauroux, J. Just (Hôpital Trousseau), A. Munck (Hôpital Robert Debré), G. Lenoir, J. de Blic, M. Sorin (Hôpital Necker), and from the Cochin adult CF center (D. Dusser, R. Kanaan, P.R. Burgel, and D. Manach) for their care of the patients.

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    This study was supported by Vaincre la Mucoviscidose.

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