[Analysis of hospital outpatient care of a rare disease: cystic fibrosis].

Introduction: As neonatal screening has become more widespread, the French CF Care Network has become structured around 45 qualified centers, the French CF Society, 2 national expertise centers, the Patient Registry and the National Protocol on CF Care, in collaboration with the patients' association Vaincre la Mucoviscidose. This organization, and progress in treatment, have resulted in the outpatient follow-up of a growing number of patients. Since 2010, CF Network representatives have been conducting an assessment of outpatient follow-up to identify difficulties in complying with national and international clinical practice guidelines. Methods: Two complementary quantitative and qualitative approaches were used to characterize and quantify the activities carried out by professionals in 8 centers both for outpatient visits and patient care coordination. Results: Two thirds of the 1,475 patients followed-up in the centers were managed over the period, less than half (40%) of them attended outpatient visits, but all of them were affected by care coordination activities, whether or not they were related to the visit. The core team (doctor, nurse, physiotherapist) is not present at each scheduled outpatient visit as  recommended. Professionals devote 40% less time to follow-up in adult centers than in pediatric centers, all activities included. The multidisciplinary outpatient visiting process is complicated by the lack of available resources and the unsuitability of certain premises. Discussion: With a constantly growing number of patients, CF centers are struggling to comply with good clinical practice and meet the specific needs of adult patients and transplant recipients. An upgrade of professional resources and an update of the National Protocol appear to be necessary.