Introduction
Cystic fibrosis (CF) is one of the most frequent severe genetic
diseases, with 6757 patients included in the French CF Registry in 2017
(1). The prognosis of CF has improved greatly thanks to early diagnosis
and specific treatments and the establishment of specific CF centers.
These improvements have led to significant demographic changes. Since
2013 in France, more than half of the French CF population are adults,
indicating that almost all the pediatric CF patients have gone through
the process of transition from a pediatric to an adult CF center. This
phenomenon will continue to grow in the years to come. Transition
between the pediatric and adult healthcare system is defined as “the
purposeful, planned movement of adolescents and young adults with
chronic physical and medical conditions from child-centered to
adult-oriented health (2). This period is described as a risky period,
with potential complications or clinical degradation, which can include
unsteadiness in young diabetic patients, solid organ transplantation
rejection in young transplanted patients, and loss of follow-up in young
patients with congenital cardiomyopathies or cancer (3). According to
the systematic review of Crowley and al., limitation of transition
complications in adolescents with chronic conditions is linked to
specific transition clinics and the patient’s education.
Currently, in France, there is no recommended national transition
program for CF patients (4). Worldwide, practices for CF patient
transfer are disparate. In Canada, a transition process has existed in
some hospitals since 1982 (5), Australia and the US have been developing
transition procedures for many years, and Australia has dedicated adult
clinics for CF with positive results (6, 7). Focusing on Europe, the
United Kingdom was a pioneer in the transition for patients with CF,
with well-defined NHS protocols for years (8,9). Conversely, Germany and
Denmark have no official transition programs for CF, but some studies on
short-term program transitions have shown encouraging results within the
past few years (11, 12).
Studies on transfer in CF mainly focus on subjective criteria and
psychological criteria. Our study aimed to evaluate a ten-year-old,
formalized transition process from a pediatric to an adult CF center in
Lyon and observed its impact on the patients’ clinical evolution.