Hemophilia Survival Rates Improve, Black Men Still Fall 10 Years Behind

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A new study investigating recorded hemophilia-related death (rHD) rates in the US revealed an overall improvement in rates among males across all racial and ethnic groups. However, disparities persisted, with higher rates observed in Black males when compared with White males.¹

With advancements in medical care and treatments, the life expectancy of individuals with hemophilia has significantly improved over the years but the extent to which these improvements have been equitable across different racial and ethnic groups warrants further research, according to the study “Racial and ethnic differences in reported hemophilia death rates in the United States”.

In the cohort study, rHDs were analyzed using the US National Vital Statistics' 1999–2020 Multiple Cause-of-Death data. Investigators defined rHD as having a hemophilia A (D66) or B (D67) ICD-10 code in the death certificate, whether it was underlying or multiple causes of death.

From 1999-2020 records investigators identified 3115 males with a hemophilia-related death. Data showed approximately 76.1% of deaths occurred in White men (n = 2370), 13.3% in Black men (n = 413), 7.4% in Hispanic men (n = 233), 2.2% in Asian men (n = 69), and 0.9% in American Indian/Alaska Native men (n = 28). Missing race and ethnicity data were identified in 2 cases.

Age-adjusted rHD rates were compared with age-adjusted rate ratios (aRR) resulting in an rHD rate of 0.98 per 1 million men. Rates decreased between 1999 and 2020 by 46% in White individuals, 44% in Black individuals (aRR, 0.56; 95% CI, 0.43-0.74), and 42% in Hispanic individuals (aRR, 0.58; 95% CI, 0.39-0.88).

During the 21-year study period, the research revealed a significant decline in age-adjusted rates of rHD, and the overall age-adjusted rHD rate reduced by nearly half, decreasing from 1.37 per 1 million males in 1999-2004 to 0.76 per 1 million males in 2015-2020, encompassing all race and ethnic groups.

However, the results also brought to light persistent disparities in these rates across races. Statistically significant differences were observed, with Black males experiencing a 30% higher average rate (aRR, 1.30; 95% CI, 1.16-1.46) compared with White males throughout the study period. Conversely, Hispanic (aRR, 0.67; 95% CI, 0.57-0.78) and Asian males (aRR, 0.50; 95% CI 0.39-0.64) demonstrated lower rates compared with their White counterparts.

A notable shift was seen in the median age at rHD over the study period. From 1999 to 2009, the median age at death stood at 54.5 years and rose by 11 years to 65.5 years during the period of 2010 to 2020. This increase in the median age at rHD was consistent across all racial and ethnic groups examined in the study.

Despite this improvement, the data exhibited a concerning disparity evident in the most recent period (2010–2020), where Hispanic and Black individuals still experienced a median age at rHD of 56 years, falling behind their White counterparts who had a median age of 68 years. This disparity highlights a significant gap in the average age of death between White males and individuals from Black and Hispanic backgrounds.

The average age of death for Black males in the 2010–2020 period, at 56 years, closely resembled the average age observed in White males a decade earlier, during the period of 1999–2009 (56.5 years). Investigators noted throughout the entire study duration, the median age of death remained consistently 10 years lower for Black and Hispanic males in comparison to White males, underscoring a critical area for further attention and targeted interventions to enhance health outcomes for individuals with hemophilia from diverse racial and ethnic backgrounds.

While no association has yet been identified, the study speculated this disparity could be impacted by that seen among black individuals with HIV. According to a previous study evaluating the general population with HIV, when compared with White individuals, Black individuals had reduced rates of HIV management and were less likely to be treated with antiretroviral drugs that suppress HIV replication.²

“Our observation that Black men had a lower median age at death and that HIV continues to be a leading cause of death among Black males with hemophilia listed in their death certificate in both earlier and recent years could signal ongoing survival disparities among Black people with hemophilia and an HIV infection,” investigators wrote.¹

References

1. Fedewa, SA, Payne, AB, Tran, D, Cafuir, L, Antun, A, Kempton, CL. Racial and ethnic differences in reported haemophilia death rates in the United States. Haemophilia. 2023; 1-9. doi:10.1111/hae.14859

2. Crepaz N, Dong X, Wang X, Hernandez AL, Hall HI.Racial and ethnic disparities in sustained viral suppression and transmission risk potential among persons receiving HIV care — United States, 2014. MMWR Morb Mortal Wkly Rep. 2018;67:113–118. doi:10.15585/mmwr.mm6704a2