Cosmos Study

Sickle Cell Patients Are Living a Decade Longer in 2022 Than in 2008

October 5, 2023

Team A:Kersten Bartelt, RNNeil Sandberg

Team B:Blaine Franklin, PT, DPTJoe Deckert, PhD

Key Findings

The median age at death for patients diagnosed with sickle cell disease has increased 20%, from 50 years in 2008 to 60 years in 2022.
In 2022, 50.0% of patients diagnosed with sickle cell disease were 60 years old or older when they died, compared to 38.9% in 2016.

Sickle cell disease (SCD) is an inherited condition in which there is a mutation in the patient’s red blood cells. Patients often develop acute and severe pain episodes, chronic hemolytic anemia, and organ damage.¹ Treatment often targets pain management and prevention or mitigation of vaso-occlusive episodes or organ damage including cardiopulmonary disease, kidney disease, and central nervous system disease.¹ Over the past decade, the FDA approved three new medications for the treatment of SCD: L-glutamine, crizanlizumab, and voxelotor.^1-4 We sought to understand how the median age at death for patients with SCD has changed over time.

We studied 5,954 patients with SCD and 5,171,437 patients without SCD who died between 2008 and 2022 to determine the median age at death for each population. We found that the median age at death for patients with SCD increased 20%, from 50 years of age in 2008 to 60 years of age in 2022. The median age at death for patients without SCD stayed consistent, with a median of 75 years in both 2008 and 2022.

Figure 1

Median Age at Death for Patients with and Without Sickle Cell Disease

Figure 1. Median age at death for patients who died each year with and without sickle cell disease from 2008 to 2022.

We further stratified the age at death for patients with SCD each year to understand how the proportions of deaths in each decade of life have changed over time. In 2016, 38.9% of patients with SCD who died were older than 60. In 2022, that proportion increased to 50.0% of patients diagnosed with SCD who died after age 60.

Figure 2

Proportion of Annual Deaths by Age for Patients with Sickle Cell Disease

Figure 2. Proportion of deaths for patients with sickle cell disease in each age group by year of death from 2016 to 2022.

These data come from Cosmos, a HIPAA-defined Limited Data Set of more than 214 million patients from 218 Epic organizations including 1,235 hospitals and more than 26,800 clinics, serving patients in all 50 states and Lebanon. This study was completed by two teams that worked independently, each composed of a clinician and research scientists. The two teams came to similar conclusions. Graphics by Brian Olson.

References

Brandow, A. M., & Liem, R. I. (2022). Advances in the diagnosis and treatment of sickle cell disease. Journal of Hematology & Oncology, 15(1). https://doi.org/10.1186/s13045-022-01237-z
Center for Drug Evaluation, & Research. (2019, February 9). FDA approved L-glutamine powder for the treatment of sickle cell disease. U.S. Food and Drug Administration; FDA. https://www.fda.gov/drugs/resources-information-approved-drugs/fda-approved-l-glutamine-powder-treatment-sickle-cell-disease
Center for Drug Evaluation, & Research. (2019b, December 20). FDA approves crizanlizumab-tmca for sickle cell disease. U.S. Food and Drug Administration; FDA. https://www.fda.gov/drugs/resources-information-approved-drugs/fda-approves-crizanlizumab-tmca-sickle-cell-disease
Center for Drug Evaluation, & Research. (2019c, December 20). FDA approves voxelotor for sickle cell disease. U.S. Food and Drug Administration; FDA. https://www.fda.gov/drugs/resources-information-approved-drugs/fda-approves-voxelotor-sickle-cell-disease