Mortality in adults with sickle cell disease: Results from the sickle cell disease implementation consortium (SCDIC) registry

Franklin Njoku, Norma Pugh, Donald Brambilla, Barbara Kroner, Nirmish Shah, Marsha Treadwell, Robert Gibson, Lewis L. Hsu, Victor R. Gordeuk, Jeffrey Glassberg, Jane S. Hankins, Abdullah Kutlar, Allison A. King, Julie Kanter
University of Illinois at Chicago. Research Triangle International. Duke University. University of California San Francisco. Augusta University. Icahn School of Medicine at Mount Sinai. St. Jude Children’s Research Hospital. Washington University School of Medicine. University of Alabama.
United States

American Journal of Hematology
Am J Hematol 2024;
DOI: 10.1002/ajh.27279

Abstract
The cause of death in people affected by sickle cell disease (SCD) is often challenging to define as prior studies have used retrospective or administrative data for analysis. We used a prospective longitudinal registry to assess mortality and clinical co-morbidities among subjects enrolled in the Sickle Cell Disease Implementation Consortium (SCDIC) registry. At enrollment, we collected the following data: patient-reported demographics, SCD phenotype, baseline laboratory values, comorbidities, and current medications. Subjects were followed for a median of 4.7 years before the present analysis. The relationship of clinical co-morbidities (at time of enrollment) to mortality was determined using survival analysis, adjusting for SCD phenotype and gender. There was a total of 2439 people with SCD enrolled in the SCDIC registry. One hundred and twenty-eight participants (5%) died during the observation period (2017-2022). Six people died from trauma and were excluded from further analysis. Proximate cause of death was unwitnessed in 17% of the deaths, but commonest causes of death include cardiac (18%), acute chest or respiratory failure (11%), sudden unexplained death (8%). Enrollment characteristics of the individuals who died (n = 122) were compared to those of survivors (n = 2317). Several co-morbidities at enrollment increased the odds of death on univariate analysis. All co-morbidities were included in a multivariable model. After backward elimination, iron overload, pulmonary hypertension, and depression, remained statistically significant predictors of the risk of death. SCD reduces life expectancy. Improved comprehensive and supportive care to prevent end-organ damage and address comorbidities is needed for this population.

Category
Class V. Pulmonary Hypertension Associated with Hematological, Systemic, Metabolic and Other Disorders
Symptoms and Findings Associated with Pulmonary Vascular Disease

Age Focus: Pediatric Pulmonary Vascular Disease or Adult Pulmonary Vascular Disease

Fresh or Filed Publication: Fresh (PHresh). Less than 1-2 years since publication

Article Access
Free PDF File or Full Text Article Available Through PubMed or DOI: Yes

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