Yearlong gap in care linked to decreased lung function in cystic fibrosis
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Key takeaways:
- Gaps in cystic fibrosis care negatively impacted lung function.
- Between continuous and discontinuous care, the difference in lung function was large in adolescent and young adult F508del homozygotes.
Patients with cystic fibrosis had reduced lung function if they had at least one 12-month or longer gap in receiving care, according to findings published in Annals of the American Thoracic Society.
“Our analysis of encounters recorded in the [Cystic Fibrosis Foundation Patient Registry] shows that 35% of patients analyzed had at least one significant gap in care and that there was a pattern of gaps in care that increased with age, despite long-standing recommendations by the [Cystic Fibrosis Foundation] for visits four times per year and intense nationwide quality improvement monitoring of contact frequency of [people with cystic fibrosis] at accredited centers,” Edmund H. Sears Jr., MD, director of respiratory care and assistant director of the adult cystic fibrosis program at Maine Medical Center, and colleagues wrote.
Using Cystic Fibrosis Foundation Patient Registry data from 2004 to 2016, Sears and colleagues assessed 24,328 individuals with cystic fibrosis to find out how a 12-month or longer gap in receiving care impacts lung function.
A larger number of patients had continuous care (n = 15,915; 65%; 51% females; 95% white) rather than discontinuous care (n = 8,413; 35%; 43% females; 93% white), which was identified through the presence of at least one gap in care.
Based on age, discontinuous care occurred in more older adults (aged older than 30 years) vs. young adults (aged 18-21 years) and children/adolescents (aged 6-17 years; 30% vs. 15% vs. 6%-9%).
Researchers assessed 1,082,899 encounters from this entire cohort, and adults vs. those aged younger than 18 years had more encounters preceded by a 12-month gap (75.8%).
When evaluating lung function, researchers adjusted for sex, cystic fibrosis transmembrane conductance regular (CFTR) genotype, race and ethnicity and found that follow-up percentage predicted FEV1 at the index visit was lower in patients with discontinuous vs. continuous care (absolute difference, –0.81; 95% CI, –1 to –0.61).
This model also considered four time-varying covariates, and researchers observed a significant link between reduced lung function and each of them: insurance coverage (absolute difference, –1.02; 95% CI, –1.08 to –0.95), underweight BMI (–8.2; 95% CI, –8.29 to –8.11), CF-related diabetes (–3.12; 95% CI, –3.21 to –3.03) and chronic infections of methicillin-resistant Staphylococcus aureus (–1.19; 95% CI, –1.28 to –1.09) and Burkholderia spp (–1.98; 95% CI, –2.19 to –1.76).
Comparing lung function at specific ages from each care group demonstrated that differences between continuous and discontinuous care were larger at younger ages, such as 16 years (1.2%) and 18 years (1.5%), rather than at 28 years (0.7%).
Notably, when researchers further divided patients according to CFTR genotype, they continued to find significant differences in lung function between both types of care, specifically in patients homozygous for F508del vs. patients heterozygous for F508del at age 10 (–1.7%) and age 18 (–2.1%).
“The effect is most pronounced in late adolescence and early adulthood in those who are homozygous for the F508del CFTR mutation,” Sears and colleagues wrote. “Our findings ... warrant reassessment as we enter the era of advanced therapies for the disease.”
In addition to considering advanced therapies in future studies, Allison J. Love, MD, of the division of respirology at the University of Toronto, and Bradley S. Quon, MD, MSc, associate professor of medicine and principal investigator at the University of British Columbia Centre for Heart Lung Innovation, believe that researchers should investigate why lung function decline in older adults with gaps in care was not as large as it was in adolescents and young adults.
“There is a need for further research in this area, as our aging CF population may have different follow-up requirements with the development of new comorbidities and complex care needs,” Love and Quon wrote in an accompanying editorial. “It is possible that future guideline-based standards of care may involve less frequent clinic visits in the age of disease-modifying [highly effective CFTR modulator therapy] in CF.”