Associations between health literacy and patient outcomes in adolescents and young adults with cystic fibrosis.

BACKGROUND: Interactive health literacy (HL) skills enable individuals to participate more fully in healthcare activities and play a role in improving their outcomes. We examine the associations between HL and cystic fibrosis (CF) outcomes and compare HL in a sample from both the Irish CF and general populations. METHODS: A total of251 CF Registry participants aged 13-30 years completed the HLS-EU-Q16 survey and a disease-specific instrument for measuring quality of life (QoL) in CF. Health outcome and healthcare resource utilization (HCRU) data were sourced from the registry. CF patient outcomes were examined using generalized linear models (GLMs) with interactive HL categorization included as a factor. General population interactive HL data are from the 2011 European HL Survey (HLS-EU). Interactive HL in 180 age-sex matched CF and general population individuals was examined using a GLM with study population, sex and educational level included as factors and age as a covariate. RESULTS: Sufficient interactive HL (total sum score ≥13) was self-reported by 81.7% of individuals with CF. Sufficient HL was associated with fewer outpatient visits [7.02(SD = 6.7: 7.4) vs. 8.74(SD = 7.9: 9.6), P < 0.001], days hospitalized [10.25(SD = 9.8: 10.7) vs. 12.8(SD = 11.8: 13.9), P < 0.001], days on intravenous antibiotics [15.3(SD = 14.7: 15.8) vs. 19.7(18.5: 21.1), P < 0.001], days on oral antibiotics [27.4(SD = 26.7: 28.1) vs. 48.48(38.7: 42.4), P < 0.001] and better QoL [77.1(SD = 75.4-78.9) vs. 64.6(60.8-68.3), P < 0.001]. Mean HL scores in CF and general populations were sufficient, although higher among individuals with CF (14.3 vs. 13.1, P < 0.01). CONCLUSION: CF adolescents and young adults with sufficient levels of HL to obtain, understand, appraise and apply health information have better health-related outcomes.

Longitudinal Trends in Real-World Outcomes after Initiation of Ivacaftor. A Cohort Study from the Cystic Fibrosis Registry of Ireland.

RATIONALE: Patient registries have the potential to collect and analyze high-quality postauthorization data on new medicines. OBJECTIVES: We used cystic fibrosis (CF) registry data to assess outcomes after the initiation of ivacaftor, a CF transmembrane conductance regulator (CFTR) potentiator approved for the treatment of CF with a defective gating CFTR mutation. METHODS: Longitudinal trends were examined using mixed-effects regression analysis in 80 ivacaftor-treated patients with CF aged 6 to 56 years registered with the CF Registry of Ireland with at least 36 months of before and after commencement data. The effects of ivacaftor treatment on forced expiratory volume in 1 second (FEV1) % predicted, body mass index (BMI), hospitalization for pulmonary exacerbation, and oral and intravenous antibiotic use were assessed. RESULTS: In the 36 months after ivacaftor initiation, FEV1% predicted improved by 2.26% per annum (95% confidence interval [CI], 0.2 to 4.3) for patients aged younger than 12 years, remained unchanged for 12- to younger than 18-year-olds (95% CI, -1.9 to 2.9), and declined in adults by 1.74% per annum (95% CI, -3.1 to -0.4). BMI in adults increased 0.28 kg/m2 per annum (95% CI, 0.03 to 0.5), and there was no significant change in BMI z-score in children (95% CI, -0.01 to 0.1). In the year after ivacaftor initiation, intravenous antibiotic treatment reduced by 46% (95% CI, -62.5% to -23.3%, oral antibiotic treatment reduced by 49% (95% CI, -61.1% to -32.1%), and there was no significant reduction in hospitalization (95% CI, -59.2% to 9.7%). CONCLUSIONS: In this study of real-world CF registry data, clinical outcomes improved and healthcare resource utilization decreased after commencing ivacaftor.