Identifying the educational needs of lung transplant recipients with cystic fibrosis.

Purpose: To elucidate the education needs of patients who have undergone lung transplant for cystic fibrosis while participating in the development of therapeutic education programs in French transplant centers.

Methods: From January 2009 to March 2012, in-depth educational diagnosis interviews were conducted with 42 adult transplant recipients with cystic fibrosis who were being followed up at 7 French transplant centers. Several areas were explored: health status, social and occupational outcomes, knowledge about the disease and treatments, and experience of the disease.

Reference percentiles for FEV(1) and BMI in European children and adults with cystic fibrosis.

Background: The clinical course of Cystic Fibrosis (CF) is usually measured using the percent predicted FEV(1) and BMI Z-score referenced against a healthy population, since achieving normality is the ultimate goal of CF care. Referencing against age and sex matched CF peers may provide valuable information for patients and for comparison between CF centers or populations. Here, we used a large database of European CF patients to compute CF specific reference equations for FEV(1) and BMI, derived CF-specific percentile charts and compared these European data to their nearest international equivalents.

Mycobacterium avium and Mycobacterium abscessus complex target distinct cystic fibrosis patient subpopulations.

Background: Clinical observations suggest that Mycobacterium avium complex (MAC) and Mycobacterium abscessus complex (MABSC) may affect cystic fibrosis (CF) patients with different characteristics and risk factors, but this has never been demonstrated within a single prospective cohort.

Methods: We studied 50 MABSC-positive and 23 MAC-positive patients from a French prevalence study of non-tuberculous mycobacteria (NTM) in CF. Risk factors specifically associated with MABSC and MAC were analyzed by nested case-control studies, with two NTM-negative controls matched by age, sex and center for each case.

Multicenter study of prevalence of nontuberculous mycobacteria in patients with cystic fibrosis in france.

We performed a multicenter prevalence study of nontuberculous mycobacteria (NTM) involving 1,582 patients (mean age, 18.9 years; male/female ratio, 1.06) with cystic fibrosis in France.

Long-term noninvasive ventilation in patients with cystic fibrosis.

Background: The benefits of long-termnoninvasive positive pressure ventilation (NPPV) have not yet been evaluated in patients with cystic fibrosis (CF).

Objectives: To evaluate the effect of 1 year of NPPV on lung function in patients with advanced CF.

Methods: Data were obtained from the French CF Registry.

Educational practice for inhaled treatments in French cystic fibrosis care centers.

Inhaled treatments are very common in cystic fibrosis (CF) patients. We sent a questionnaire to the 49 French Cystic Fibrosis Care Centers (CFCCs) to see how the inhalation technique of CF patients was initiated and monitored (composition and formation of a potential educational team, use of a manual for use, amount of devices available for demonstration, education to disinfection procedure, realization and frequency of the inhalation technique monitoring, use of the patient's own device, and frequency of checking when a pediatric patient transfers to an adult CFCC). Results were expressed in percent and compared with the test of the difference between two correlated proportions.

Cystic fibrosis mortality trends in France.

Background: In 1992 France set up a national cystic fibrosis observatory (Observatoire national de la mucoviscidose, ONM) to monitor the state of health of patients on an annual basis. Using the ONM data, this study estimates the main indicators for life expectancy and assesses the total number of cystic fibrosis patients.

Methods: The data for the years 1994 to 2003 are divided into 3-year periods.