Trends in cystic fibrosis survival over 40 years in South Africa: An observational cohort study. Issue 4 (11th January 2022)

• Record Type: Journal Article
• Authors: Zampoli, Marco; Kassanjee, Reshma; Verstraete, Janine; Westwood, Anthony; Zar, Heather J.; Morrow, Brenda M.
• Is Part Of: Pediatric pulmonology. Volume 57:Issue 4(2022)
• Page Start: 908
• Page End: 918
• Publication Date: 2022-01-11
• Subjects: cystic fibrosis -- South Africa -- low‐middle income -- survival; Pediatric respiratory diseases -- Periodicals; Pediatrics -- Periodicals; 618.922
• DOI: 10.1002/ppul.25810
• Languages: English
• ISSNs: 8755-6863

Abstract

Abstract: Introduction: Temporal trends in cystic fibrosis (CF) survival from low‐middle‐income settings is poorly reported. We describe changes in CF survival after diagnosis over 40 years from a South African (SA) CF center. Methods: An observational cohort study of people diagnosed with CF from 1974 to 2019. Changes in age‐specific mortality rates from 2000 (vs. before 2000) were estimated using multivariable Poisson regression. Data were stratified by current age < or ≥10 years and models controlled for diagnosis age, sex, ethnicity, genotype, and Pseudomonas aeruginosa ( PA ) infection. A second analysis explored the association of mortality with weight and forced expiratory volume in 1 s reported as z‐scores (FEV1z‐scores) at age 5–8 years. Results: A total of 288 people (52% male; 57% Caucasian; 44% p.Phe508del homozygous) were included (median diagnosis age 0.5 years: Q1, Q3: 0.2, 2.5); 100 (35%) died and 30 (10%) lost to follow‐up. Among age >10 years, age‐specific mortality from 2000 was significantly lower (adjusted hazard ratio [aHR]: 0.14; 95% confidence interval [CI]: 0.06, 0.29; p < 0.001), but not among age <10 years (aHR: 0.67; 95% CI: 0.28, 1.64; p = 0.383). In children <10 years, Caucasian ethnicity was associated with lower mortality (aHR 0.17; 95% CI: 0.05, 0.63), and longer times since first PA infection with higher mortality (aHR: 1.31; 95% CI: 1.01, 1.68). Mortality was sevenfold higher if FEV1z was <−2.0 at age 5–8 years (aHR: 7.64; 95% CI: 2.58, 22.59). Conclusion: Overall, CF survival has significantly improved in SA from 2000 in people older than 10 years. However, increased risk of mortality persists in young non‐Caucasian children, and with FEV1z <−2.0 at age 5–8 years.