ABSTRACT
OBJECTIVES: To describe the current use of airway clearance techniques among people with cystic fibrosis (CF) in the UK, and the baseline characteristics for users of different airway clearance techniques. DESIGN: Analysis of the UK CF Registry 2011 data. SETTING AND PARTICIPANTS: All people with CF in the UK aged ≥11 years (n=6372). RESULTS: Of the 6372 people on the UK CF registry in 2011, 89% used airway clearance techniques. The most commonly used primary techniques were forced expiratory techniques (28%) and oscillating positive expiratory pressure (PEP) (23%). Postural drainage and high-frequency chest wall oscillation were used by 4% and 1% of people with CF, respectively. The male:female ratio of individuals who used exercise as their primary airway clearance technique was 2:1, compared with 1:1 for other techniques. Individuals with more severe lung disease tended to use devices such as non-invasive ventilation or high-frequency chest wall oscillation. CONCLUSIONS: Forced expiratory techniques and oscillating PEP are the most common airway clearance techniques used by people with CF in the UK, and postural drainage and high-frequency chest wall oscillation are the least common techniques. This is significant in terms of planning airway clearance technique trials, where postural drainage has been used traditionally as the comparator. The use of airway clearance techniques varies between countries, but the reasons for these differences are unknown.
Subject(s)
Cystic Fibrosis/therapy , Respiratory Therapy/methods , Adolescent , Adult , Age Factors , Airway Management , Anti-Bacterial Agents/administration & dosage , Chest Wall Oscillation/methods , Child , Cross-Sectional Studies , Drainage, Postural/methods , Exercise , Female , Forced Expiratory Volume , Humans , Male , Sex Factors , Young AdultABSTRACT
BACKGROUND: The widespread availability of genetic testing allowing the identification of "milder" individuals with CF coincided with improvements in CF life expectancy but the relative contribution of case mix to that improved survival is uncertain. METHODS: Patients in the U.K. CF registry were divided into 'mild phenotype' defined as pancreatic sufficient and 'typical CF' defined as pancreatic insufficient. Distributions of age at death were compared with Mann-Whitney test. Temporal trends in incidence and prevalence were described. Jonckheere-Terpstra test was used to compare the trend for median age at death from 2007 to 2010. RESULTS: Patients with 'mild phenotype' had significantly higher age at death (32 years, interquartile range 14 years versus 27 years, interquartile range 29 years; Mann-Whitney test p-value = 0.026). The proportion of patients with 'mild phenotype' appeared to be increasing (0.128 in 2007, 0.144 in 2010). The trend for increasing age at death (from 25 years in 2007 to 29 years in 2010, Jonckheere-Terpstra test p-value = 0.012) was independent of the 'mild phenotype' patients. CONCLUSION: The impact of mild phenotypes on the improvement in the median age at death among people with CF was trivial.
