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. The interviews combined open- and closed-ended questions for more systematic exploration of knowledge about the disease and treatments. RESULTS: After receiving the transplant, the patients' health had improved and their lives were returning to normal. They had acquired much usable knowledge about managing their disease. Educational needs regarding transplant-related complications and management of immunosuppressive drugs were evident. The analysis also demonstrated the need to better inform patients about pregnancy and new social rights and to offer them psychological support in adjusting to their new health status. CONCLUSION: Therapeutic education of patients should continue well after transplant to maintain safety knowledge and meet patients' new needs. A comprehensive therapeutic education program for transplant candidates that includes the preparatory, immediate posttransplant, and late posttransplant phases is needed.

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. RESULTS: MAC-positive patients were significantly older than MABSC-positive patients (mean [SD] age, 23.1 [10.2] vs 17.4 [8.3] years, p=0.013), and were also older at CF diagnosis (mean [SD] age, 12.9 [16.1] vs 3.1 [7.7] years, p=0.015); they tended to be less frequent of the ΔF508/ΔF508 genotype (33.3 vs 61.1%, p=0.17) and to use pancreatic extracts less frequently (82.4 vs 97.6%, p=0.07). Risk factors identified by multivariate analysis were: i) in the MAC case-control study, an older age at CF diagnosis (p=0.004); ii) in the MABSC case-control study, at least one course of intravenous antibiotics (p=0.01) and more frequent isolation of Aspergillus (p=0.03). CONCLUSIONS: MAC affects adult patients with a mild form of CF, whereas MABSC affects younger patients with more severe CF and more frequent intravenous antimicrobial treatment.

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. METHODS: 34859 FEV(1) and 40947 BMI observations were used to compute European CF specific percentiles. Quantile regression was applied to raw measurements as a function of sex, age and height. Results were compared with the North American equivalent for FEV(1) and with the WHO 2007 normative values for BMI. RESULTS: FEV(1) and BMI percentiles illustrated the large variability between CF patients receiving the best current care. The European CF specific percentiles for FEV(1) were significantly different from those in the USA from an earlier era, with higher lung function in Europe. The CF specific percentiles for BMI declined relative to the WHO standard in older children. Lung function and BMI were similar in the two largest contributing European Countries (France and Germany). CONCLUSION: The CF specific percentile approach applied to FEV(1) and BMI allows referencing patients with respect to their peers. These data allow peer to peer and population comparisons in CF patients.

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. The overall NTM prevalence (percentage of patients with at least one positive culture) was 6.6% (104/1,582 patients), with prevalences ranging from 3.7% (in the east of France) to 9.6% (in the greater Paris area). Mycobacterium abscessus complex (MABSC; 50 patients) and Mycobacterium avium complex (MAC; 23 patients) species were the most common NTM, and the only ones associated with fulfillment of the American Thoracic Society bacteriological criteria for NTM lung disease. The "new" species, Mycobacterium bolletii and Mycobacterium massiliense, accounted for 40% of MABSC isolates. MABSC species were isolated at all ages, with a prevalence peak between 11 and 15 years of age (5.8%), while MAC species reached their highest prevalence value among patients over 25 years of age (2.2%).

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. Patients who started NPPV (ventilated group, n = 41) were compared to matched controls (control group, n = 41). Each ventilated patient was matched to a control 1 year before the start of NPPV (year -1) for gender, CFTR genotype, age +/- 5 years and forced expiratory volume in 1 s (FEV(1)) +/- 10%. The ventilated group was compared to the control group at year -1, during the year of NPPV initiation (year 0) and 1 year after NPPV (year +1). RESULTS: At year -1, the two groups were comparable with regard to forced vital capacity (FVC; 43.7 vs. 49.1% in the ventilated group and the control group, respectively) and FEV(1) (28.2 vs. 28.5%). At year 0, the ventilated group had significantly greater declines in FVC (-3.6 +/- 9.2 vs. +0.8 +/- 8.9%, p = 0.03) and in FEV(1) (-3.0 +/- 6.7 vs. +2.6 +/- 4.4, p < 0.0001). At year +1, the decreases in FVC (-2.1 +/- 10.0 vs. -2.2 +/- 9.9%) and in FEV(1) (-2.2 +/- 6.7 vs. -2.3 +/- 6.2%) were similar in both groups. CONCLUSIONS: These data show that NPPV is associated with stabilization of the decrease in lung function in patients with advanced CF.

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. Education for aerosol therapy was not performed in about 20% of the CFCCs. A specific educational member was present in 33 CFCCs (68%). CFCCs owned statistically less nebulizers for demonstration than other devices. Disinfection advices were always given for nebulizers, but less frequently for spacers (80%) and dry powder inhalers (50%). The inhalation technique was monitored less than once a year or never in 22% of the cases, and rarely with the patient's own material. This monitoring was device dependent (56% with nebulizers vs. 76% with the other devices, p < 0.05). For nebulizers, monitoring depended on the presence of an educational member (p < 0.01) and of a manual for use (p = 0.03). When transferring to an adult CFCC, the inhalation technique was checked in less than a third of the patients. The inhalation technique is poorly and insufficiently initiated and monitored in CF patients. Educational teams and programs are urgently required.

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. Life tables are drawn up for these periods, from which mean and median lengths of life are determined. Using the most recent life table, the number of births in 2003 and the incidence of the disease, the total population of patients can be estimated, assuming a stationary population. RESULTS: In 2001-2003, life expectancy at birth of patients registered with the ONM was 39.1 years and median length of life was 36.4 years. These results, substantially better than those of 1994-1996, are linked to improved conditions of patient inclusion in the ONM database, to improvements in their healthcare, but also to the limitations of the life tables. Based on the 2003 data, the total theoretical number of patients is 6490, and coverage by the ONM database is thus 63.2%. CONCLUSIONS: These provisional results demonstrate the need to convert the ONM observatory into a registry providing exhaustive coverage of all patients.