Hemophilia mortality rates in US higher among Black males: Study

Overall rate fell more than 40 percent for all races between 1999-2020

Margarida Maia, PhD avatar

by Margarida Maia, PhD |

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People with hemophilia are living longer, but Black males still are more likely to die from the disease, and at a younger age, compared with white people, a U.S. study finds.

More research is needed to understand the reason for this difference and to improve care and survival rates for people with hemophilia, according to “Racial and ethnic differences in reported haemophilia death rates in the United States,” which was published in Haemophilia by researchers in Atlanta.

In hemophilia, blood fails to clot properly because it lacks a certain clotting factor, which can lead to frequent and prolonged bleeding episodes.

Treating hemophilia usually involves supplying the missing clotting factor to patients either on demand as bleeds occur or as preventive treatment to prevent or reduce the number of episodes.

Life expectancy in hemophilia was low before clotting factor products became available and then declined again as patients became infected with the human immunodeficiency virus (HIV) or hepatitis C virus (HCV) due to contaminated blood-derived products. Survival improved as blood-derived products began to be screened routinely and recombinant (lab-made) clotting factor products were developed.

“However, it is unknown if recent improvements … have been experienced equitably across all race/ethnicities,” the researchers wrote.

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Tracking mortality rates over 20 years

Black and white males had similar death rates in the U.S. between 1979 and 1995, “but it is not known if patterns changed in recent time periods where [clotting] factor products are abundant, and prophylaxis is the standard of care,” wrote the researchers, who searched available death certificates from the Multiple Causes of Death database for deaths from hemophilia from 1999 to 2020 across all U.S. counties.

They identified 3,115 males who died from hemophilia during that time, which translated into a rate of death from hemophilia of 0.98 per 1 million males in the overall U.S. population. Most deaths (76.1%) occurred in white males, followed by Black (13.3%), Hispanic (7.4%), Asian (2.2%), and American Indian or Alaska Native males (0.9%).

With all the races and ethnicities combined, the age-adjusted rate of death from hemophilia nearly halved from 1.37 per 1 million males between 1999-2004 to 0.76 per 1 million males between 2015-2020. Over the full 21-year period, that rate declined by 46% among whites, by 44% among Blacks, and by 42% among Hispanics.

The rate remained on average 30% higher for Black versus white males, and Blacks who were younger than 25 were 2.52 times more likely than whites of the same age to die from the disease. Black males ages 65 or older were 1.33 times more likely to die.

The rate of death from hemophilia was 33% and 50% lower for Hispanic and Asian males versus white males, respectively. Hispanic males younger than 25 were 75% more likely to die than whites of the same age range, however.

Uneven progress

The median age at death rose 11 years, from 54.5 between 1999-2009 to 65.5 between 2010-2020, but it was 12 years lower in Black and Hispanic males than in whites (56 vs. 68) in the most recent period.

“Rates were higher and deaths occurred earlier in life among Black males compared to white males,” the researchers wrote, suggesting “that progress in hemophilia outcomes may not be experienced equitably across minoritized racial and ethnic groups.”

The most common causes of death between 1999 and 2020 were clotting problems (34.6%), followed by HIV infection (13.2%), liver cancer, disease or HCV infection (10%), heart disease (7.8%), and non-liver cancer (6.7%).

Between 1999-2020, 930 females died from hemophilia, which translated into an age-adjusted rate of death of 0.22 per 1 million females, a rate “far lower than what was observed in males.” Black females were 2.46 times more likely to die than white females.

The study had limitations, such as lack of information about diagnosis.

“Those with fewer resources and minoritized racial and ethnic minorities may be less likely to have their milder forms diagnosed, possibly driving down the number of deaths in these groups,” the researchers wrote. “Given the inherent limitations of the current study’s data source, further investigation of survival rates and disparities in haemophilia are needed.”