Cystic Fibrosis Foundation Nebulizer and Compressor Accessibility Survey.

BACKGROUND: Although guidelines for inhaled therapies for individuals with cystic fibrosis (CF) are available, recommendations for compressors/nebulizers to optimize care are lacking. The CF Foundation (CFF) convened a multidisciplinary task force to assess the use, durability, accessibility, and cost burden of compressors/nebulizers. METHODS: Online surveys were developed and distributed to 287 CFF programs and adults with CF and parents of children with CF (adults with CF/parents). RESULTS: Health care providers from 38 states completed the survey (59% response rate). Respiratory therapists were mostly responsible to coordinate ordering nebulizers and compressors. Durable medical equipment companies were the most common source of acquisition of compressors (71.8%) and nebulizers (45.9%). A majority of health care providers did not feel the compressors were durable (51.1%) or that they could get enough nebulizers to their patients (69.2%). Barriers to procure compressors were reported. The survey was completed by 734 adults with CF/parents from 48 states. Most adults with CF/parents rated their compressor as durable (65.8%); however, 85.5% of respondents reported some user-experience problem(s). "Hoses popping off" and "increased nebulization time" were most commonly reported. Almost 20% of respondents did not have access to a compressor at some point in the previous year. Most adults with CF/parents did not change compressor filters per manufacturer's recommendation (40% never). Adults with CF/parents reported performing a median of 4 inhaled treatments per day. Median use of nebulizers was 6 months. Most adults with CF/parents thought they had enough nebulizers (53.7%). Individuals with CF doing more inhaled treatments reported more compressor malfunctions. The median out-of-pocket expense was $75-99 and $50-74 for compressors and nebulizers, respectively. CONCLUSIONS: Although the perceptions of health care providers and adults with CF/parents differed to a certain extent, the surveys uncovered several significant issues that may compromise quality of care. Improvement in access to devices and education are needed.

Blood flow regulation and oxidative stress during submaximal cycling exercise in patients with cystic fibrosis.

BACKGROUND: The impact of blood flow regulation and oxidative stress during exercise in cystic fibrosis (CF) has yet to be investigated. METHODS: A maximal graded exercise test was conducted to determine exercise capacity (VO2 peak) and peak workload in 14 pediatric patients with mild CF (age 14±3y, FEV1 93±16 % predicted) and 14 demographically-matched controls. On a separate visit, participants performed submaximal cycling up to 60% of peak workload where brachial artery blood velocity was determined using Doppler ultrasound. Retrograde and antegrade components were further analyzed as indices of blood flow regulation. RESULTS: The cumulative AUC for retrograde velocity was lower in patients versus controls (1770±554 vs. 3440±522cm, P=0.038). In addition, an exaggerated oxidative stress response during exercise occurred in patients only (P=0.004). CONCLUSION: These data suggest that patients with mild CF exhibit impaired blood flow regulation and an exaggerated oxidative stress response to submaximal exercise.

Evidence of vascular endothelial dysfunction in young patients with cystic fibrosis.

BACKGROUND: Cystic fibrosis (CF) is a genetic disorder that affects not only pulmonary function but also multiple organ systems. The fl ow-mediated dilation (FMD) test is a noninvasive assessment of endothelial function and nitric oxide bioavailability. Thus, the purpose of this study was to determine (1) whether endothelial dysfunction is present in young patients with CF and (2) whether endothelial function is associated with pulmonary function and exercise capacity. METHODS: Fifteen patients with CF and 15 demographically matched control subjects participated in this study. Spirometry, brachial-artery FMD, and a maximal exercise capacity test on a cycle ergometer were performed on all subjects to determine pulmonary function, endothelial function, and exercise capacity, respectively. RESULTS: No differences ( P . .05) in age, height, or BMI were observed between patients with CF and control subjects. FEV 1 (% predicted), FEV 1 /FVC, and forced expiratory fl ow between 25% and 75% of vital capacity were lower in patients with CF. Volume of oxygen consumption peak (absolute and relative) was similar between groups; however, volume of oxygen consumption (% predicted and mL/kg fat-free mass/min) and peak workload were significantly ( P , .05) lower in patients with CF. FMD (4.9% 2.6% vs 7.5% 3.1%; P 5 .018) was lower in patients compared with control subjects, respectively. Relationships between FMD and both pulmonary function and exercise capacity were identified. CONCLUSIONS: For the fi rst time to our knowledge, these data provide evidence of vascular endothelial dysfunction in a fairly healthy cohort of young patients with CF. In addition, our data demonstrate the complex relationships between endothelial function and both pulmonary function and exercise capacity in young patients with CF.