January 2024

Lara C. Pullen, PhD

Between 1999 and 2020, recorded hemophilia death (rHD) rates in the U.S. improved in males across all racial and ethnic groups. Stacey A. Fedewa, PhD, MPH, of Emory University School of Medicine in Atlanta, and colleagues found, however, that death rates remained higher in Black versus white males. They reported their results in Haemophilia and call for further investigation of survival rates and disparities in hemophilia.

Using U.S. death certificate data, the researchers identified 3,115 rHDs in males and calculated an rHD rate of 0.98 per 1 million males in the U.S. general population. Underlying causes of death included coagulation defects (34.0%), human immunodeficiency virus (HIV; 18.8%), and liver disease (9.8%). Approximately 76.1% of deaths occurred in non-Hispanic (NH) white males, 13.3% in Black males, 7.4% in Hispanic males, 2.2% in Asian males, and 0.9% in American Indian/Alaska Native males, respectively.

Between 1999 and 2020, the age-adjusted rHD rate declined by nearly half from 1.37 per 1 million males in 1999-2004 to 0.76 per 1 million males in 2015-2020 for all race and ethnicities combined. rHD rates declined by 46% in NH white, 44% in NH Black, and 42% in Hispanic males, respectively. Despite this, rHD rates remained higher and were on average 30% greater in NH Black versus NH white males.

The investigators found that, among males (white, Black, and Hispanic) with hemophilia listed as a cause of death, the median age at death rose from 54.5 years in 1999-2009 to 65.5 years in 2010-2020. In the case of NH Black males, however, rates of death were higher, and deaths occurred earlier in life (56 years) when compared with NH white males (68 years). Thus, the average age of death among Black males with rHD in 2010-2020 approximated what was observed in white males a decade earlier.

Women have milder forms of hemophilia than men, so Dr. Fedewa was not surprised to see that recorded hemophilia death rates for females (n=930, with an age-adjusted rate of 0.22 per 1 million females) were far lower than those for males, and the median age of death was like that of the U.S. general population. Moreover, the age-adjusted hemophilia death rates in females remained steady during the study period. However, researchers stated that it was unknown whether the health care providers who completed the death certificates were aware of hemophilia carriership or the factor activity level of each female decedent, meaning that accurate data may be limited.

The authors stated that death rates are a function of both disease occurrence and survival, and because hemophilia occurrence is likely similar across race and ethnicities, the findings suggest lower hemophilia-specific survival among Black males compared to white males. Other previously established characteristics associated with hemophilia-specific survival include age, severity, and the development of inhibitors. Previous research has also established that, on a broader scale, disparities in economic mobility, lower educational attainment, barriers in access to health care, quality of care, and greater burden of comorbidity have meant that Black males in the general U.S. population have higher death rates than white males.

Dr. Fedewa noted that hemophilia is a rare chronic disease that requires longstanding management, with 80% of patients receiving treatment at one of approximately 140 hemophilia treatment centers across the country. “The persistent gap is a little disappointing” said Dr. Fedewa. She explained that when mortality improves overall, one would hope that the racial disparity gap would too, but this wasn’t the case for hemophilia.

“This study is really detecting the problem,” said Dr. Fedewa, adding that, “next we need to better understand the problem.” She suggests that until that work has been completed, hematologists draw upon their personal knowledge to determine if there are actions they can take to close potential gaps in hemophilia survival.

The authors noted that the study was limited by its use of death certificate data, which included a person’s cause of death but did not include whether individuals had been diagnosed with hemophilia or whether they were receiving care at a hemophilia treatment center.

Any conflicts of interest declared by the authors can be found in the original article.

Reference

Fedewa SA, Payne AB, Tran D, et al. Racial and ethnic differences in reported hemophilia death rates in the United States. Haemophilia. 2023;29(6):1410-1418.