Children and young adults with CF in the USA have better lung function compared with the UK

Abstract

Background People with cystic fibrosis are managed differently in the US and UK providing an opportunity to learn from differences in practice patterns. Objectives To compare cross-sectional demographics, practice patterns and clinical outcomes between US and UK cystic fibrosis patients. Methods This was a cross-sectional study using 2010 data from patients in the US Cystic Fibrosis Foundation and the UK Cystic Fibrosis patient registries. The a priori outcome measures of interest were lung function and nutritional status. Descriptive statistics and two sample comparisons were performed. Stratification and multivariable linear regression was used to adjust for confounding. Results The study cohort included 13,777 children and 11,058 adults from the US and 3,968 children and 3,965 adults from the UK. In children, mean body mass index percentiles were similar. Lung function (FEV1 and FVC% predicted) was significantly higher in US patients ages 6 through 25 years of age. In a regression model adjusted for only age, FEV1% predicted was on average 3.31% of predicted (95% CI: 2.65, 3.96) higher in the US compared to the UK. When adjusted for age, age at diagnosis, gender, pancreatic insufficiency and genotype, FEV1% predicted was on average 3.03% of predicted (95% CI: 2.37, 3.69) higher in the US compared to the UK These differences persisted despite adjustment for possible confounders. Hypertonic saline and dornase alfa were much more commonly prescribed in US children. Conclusions Children and young adults with cystic fibrosis have better lung function in the US compared to the UK despite similar nutritional status.

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“…Nevertheless, there is some hope that DNase enzymes could potentially be used clinically, since there are already precedents for the use of enzymes in human healthcare. For example, recombinant human DNase I (Pulmozyme, or dornase alpha) is used for the treatment of cystic fibrosis [69]. However, mammalian DNase enzymes require glycosylation for activity and therefore cannot be expressed in bacterial production systems.…”

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“…(6-12) Recently, Goss et al showed that pulmonary function among patients ages 6-25 years was higher in CF patients in the United States (US) compared to the United Kingdom (UK), which was associated with differential prescribing of inhaled mucolytic therapies between the two countries. (7)…”

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