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Published on: September 13, 2018
Children and young adults with CF in the USA have better lung function compared with the UK
Christopher H Goss1, Stephanie J MacNeill2, Hebe B Quinton3
1Department of Medicine, University of Washington, Seattle, Washington, USA.
Insights
US cystic fibrosis (CF) patients aged 6-25 exhibit superior lung function compared to UK counterparts. This difference persists across various adjusted models, suggesting variations in care may impact outcomes for CF patients.
Area of Science:
- Pulmonology
- Clinical Medicine
- Epidemiology
Background:
- Cystic Fibrosis (CF) management varies between the USA and UK.
- Comparative analysis of different healthcare practices can reveal insights into patient outcomes.
Purpose of the Study:
- To compare demographics, clinical practices, and health outcomes of CF patients in the USA and UK.
- To identify potential differences in care that may influence CF patient health.
Main Methods:
- Cross-sectional study utilizing 2010 patient registry data from the US and UK.
- Key outcome measures included lung function (FEV1, FVC) and nutritional status (BMI).
- Statistical analyses involved descriptive statistics, two-sample comparisons, stratification, and multivariable regression.
Main Results:
- The study included over 13,000 children and 11,000 adults from the USA, and nearly 4,000 children and 4,000 adults from the UK.
- While nutritional status was similar, US children and young adults (ages 6-25) demonstrated significantly better lung function (FEV1% predicted).
- Specific CF therapies like hypertonic saline and dornase alfa were more prevalent in US children.
Conclusions:
- US children and young adults with CF show better lung function than their UK counterparts.
- Despite similar nutritional status, differences in management or treatment protocols may contribute to improved lung function in the US.
- Further research is warranted to explore the specific factors driving these observed clinical outcome disparities.
Background:
People with cystic fibrosis (CF) are managed differently in the USA 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 CF 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 were used to adjust for confounding.
Results:
The study cohort included 13 777 children and 11 058 adults from the USA and 3968 children and 3965 adults from the UK. In children, mean body mass index centiles were similar. Lung function (FEV1 and FVC% predicted) was significantly higher in US patients ages 6-25 years of age. In a regression model adjusted for only age, FEV1% predicted was on average 3.31% of predicted (95% CI 2.65 to 3.96) higher in the USA compared with 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 to 3.69) higher in the USA compared with 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 CF have better lung function in the USA compared with the UK despite similar nutritional status.
