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Review
. 2023 Nov;29(6):1410-1418.
doi: 10.1111/hae.14859. Epub 2023 Sep 17.

Racial and ethnic differences in reported haemophilia death rates in the United States

Stacey A Fedewa  1   2 , Amanda B Payne  3 , Duc Tran  1   2 , Lorraine Cafuir  1   2 , Ana Antun  1   2 , Christine L Kempton  1   2
Affiliations
Review

Racial and ethnic differences in reported haemophilia death rates in the United States

Stacey A Fedewa et al. Haemophilia. 2023 Nov.

Abstract

Introduction: People with haemophilia's life expectancies have improved over time. Whether progress has been experienced equitably is unknown.

Aim: To examine recorded haemophilia death (rHD) rates according to race and ethnicity in the United States (US).

Methods: In this cohort study, rHDs were examined with US National Vital Statistics' 1999-2020 Multiple Cause-of-Death data. rHD was defined as having a haemophilia A (D66) or B (D67) ICD-10 code in the death certificate (underlying or multiple causes of death). Age-adjusted rHD rates were compared with age-adjusted rate ratios (aRR) and 95% Confidence Intervals (CI).

Results: There were 3115 rHDs in males with an rHD rate of 0.98 per 1 million males. Between 1999 and 2020, rHD rates declined by 46% in NH (Non-Hispanic) White, 44% in NH Black (aRR = 0.56, 95%CI 0.43, 0.74), and 42% in Hispanic (aRR = 0.58, 95%CI 0.39, 0.88) males. However, rHD rates remained higher and were on average 30% greater in NH Black versus NH White males (aRR = 1.30 95% CI 1.16, 1.46). Among males with rHD, the median age at death rose from 54.5 to 65.5 years between 1999 and 2020 and was 12 years lower in NH Black (56 years) versus NH White (68 years) males in 2010-2020. There were 930 females with rHD, with an age-adjusted rate of 0.22 per 1 million females, which was consistent between 1999 and 2020.

Conclusion: Reported haemophilia-death rates improved in males across all race/ethnicities, but rates were higher Black versus White males. Given the inherent limitations of the current study's data source, further investigation of survival rates and disparities in haemophilia are needed.

Keywords: death; disparities; ethnicity; haemophilia; outcomes; race.

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Conflict of interest statement

CONFLICT OF INTEREST STATEMENT

Christine L. Kempton: Has received honoraria for participation in advisory boards from Biomarin, Pfizer, Genentech, and Spark.

Figures

FIGURE 1
FIGURE 1
Age-adjusted death rates among males according to race and ethnicity, National Vital Statistics 1999−2020. NH, non-Hispanic.
FIGURE 2
FIGURE 2
Age-adjusted death rates among females according to race and ethnicity, National Vital Statistics 1999−2020.
See this image and copyright information in PMC

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