Background: While historically considered a pediatric disease, the life expectancy for patients with cystic fibrosis (pwCF) is improving. As such, pwCF will likely develop age-related comorbidities and require additional pharmacotherapies, increasing the complexity of an already burdensome medication regimen. Clinical characterization of adult pwCF will help inform the unique challenges faced by this population and identify potential gaps in care.
Objectives: To characterize the adult pwCF population, including the prevalence of chronic conditions common to the general aging population and non-CF medication use, and to compare these characteristics to the general healthcare-seeking adult population.
Methods: This was a retrospective database study of patients aged ≥40 with a CF diagnosis record/claim (index date) between 01/01/2019-12/31/2024 in the TriNetX Linked network, a US-based electronic health record (EHR) research network comprised of de-identified EHR data linked to closed claims and mortality data through tokenization. Patients must have had at least one encounter >1 year prior to index. Patient characteristics, including diagnoses and medications, were described for pwCF and descriptively compared to all patients aged ≥40 in the TriNetX Linked network who had an encounter during the study period and at least one encounter >1 year prior to index.
Results: Among adult pwCF (n=3259) vs. all adult patients ≥40 years, mean (SD) age [61.1 (12.0) vs. 60.5 (11.7) years] and sex (53 vs. 54% female) were similar between groups. For race, pwCF had a greater proportion who identify as white (68 vs. 61%) and Black (20 vs. 17%), and a lower proportion Asian (2 vs. 4%). The proportion of patients of Hispanic/Latino ethnicity was similar between groups (7 vs. 8%).
Among selected chronic conditions, generally a higher proportion of pwCF were diagnosed with age-related comorbidities compared to all adults: hypertension (65 vs. 48%), hyperlipidemia (82 vs. 47%), CHD (25 vs. 13%), COPD (28 vs. 8%), CKD (22 vs. 9%), T2DM (40 vs. 22%), cancer (39 vs. 10%), depression (42 vs. 21%).
Among selected commonly prescribed medications, the overall medication burden was higher in pwCF compared to all adults for both acute and chronic medications: opioids (68 vs. 43%), non-opioid analgesics (66 vs. 41%), benzodiazepines (54 vs. 30%), antidepressants (42 vs. 29%), antidiabetics (52 vs. 23%), antilipemics (42 vs. 34%), antiarrhythmics (54 vs. 27%), antihypertensives (65 vs. 48%).
Conclusions: Overall, in the TriNetX Linked EHR + Claims network demographic characteristics between adult pwCF and all adult patients were similar; however, burden of chronic disease and medication use in pwCF was higher when compared to the general adult population.
