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Trends in Cystic Fibrosis survival over 40 years in South Africa: an observational cohort study
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  • Marco Zampoli,
  • Reshma Kassanjee,
  • Janine Verstraete,
  • Anthony Westwood,
  • Heather Zar,
  • Brenda Morrow
Marco Zampoli
University of Cape Town Department of Paediatrics and Child Health

Corresponding Author:[email protected]

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Reshma Kassanjee
University of Cape Town School of Public Health and Family Medicine
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Janine Verstraete
University of Cape Town Department of Paediatrics and Child Health
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Anthony Westwood
University of Cape Town Department of Paediatrics and Child Health
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Heather Zar
University of Cape Town
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Brenda Morrow
University of Cape Town Department of Paediatrics and Child Health
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Abstract

Introduction: Temporal trends in CF survival from low-middle-income settings are 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 the year 2000 (versus 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 P. aeruginosa (PA) infection. A second analysis explored association of mortality with weight and FEV1z-scores at age 5-8 years. Results: 288 people (52% male; 57% Caucasian; 44% p.Phe508del homozygous) were included (median diagnosis age 0.5 years: Q1,Q3: 0.2, 2.5); 58 (35%) died and 30 (10%) lost to follow-up. Among age >10 years, age-specific mortality from year 2000 was significantly lower (adjusted hazard ratio aHR: 0.14; 95% 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 time since first PA infection with higher mortality (aHR 1.31; 95% CI 1.01,1.68). Mortality was 7-fold 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 year 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.
27 Oct 2021Submitted to Pediatric Pulmonology
28 Oct 2021Submission Checks Completed
28 Oct 2021Assigned to Editor
01 Nov 2021Reviewer(s) Assigned
16 Nov 2021Review(s) Completed, Editorial Evaluation Pending
20 Nov 2021Editorial Decision: Revise Major
29 Nov 20211st Revision Received
30 Nov 2021Submission Checks Completed
30 Nov 2021Assigned to Editor
30 Nov 2021Reviewer(s) Assigned
13 Dec 2021Review(s) Completed, Editorial Evaluation Pending
27 Dec 2021Editorial Decision: Accept
Apr 2022Published in Pediatric Pulmonology volume 57 issue 4 on pages 908-918. 10.1002/ppul.25810