Access to Care, Survival Are Enduring Hurdles for Patients With Sickle Cell Disease

As part of an ongoing series on discrimination in the United States, NPR reported that while life expectancy for almost every major illness is improving, patients with sickle cell disease (SCD) can expect to die younger than they did more than 20 years ago. In 1994, life expectancy for patients with SCD was 42 years for men and 48 years for women; in 2005, life expectancy dropped to 38 years for men and 42 years for women.

About 100,000 people in the United States have SCD, and most of them are African American. SCD is “a microcosm of how issues of race, ethnicity and identity come into conflict with issues of health care,” said Keith Wailoo, PhD, from Princeton University, who has written about the history of the disease. Elliot Vinchinsky, MD, who started the Comprehensive Center for Sickle Cell Disease at UCSF Benioff Children’s Hospital Oakland in California, said, “This group of people can live much longer with the management we have, and they’re dying because [they] don’t have access to care.” With proper care, patients with SCD can live well into their 60s.

Funding by the federal government and private foundations for the disease pales in comparison to those for other disorders. Cystic fibrosis research, for example, receives seven to 11 times more funding per patient than SCD, according to a 2013 study published in Blood. From 2010 to 2013 alone, the U.S. Food and Drug Administration (FDA) approved five new drugs for the treatment of cystic fibrosis. There are only two FDA-approved treatments for SCD: hydroxyurea (approved in 1998) and L-glutamine oral powder (approved nearly 20 years later in July 2017).

In the 1960s and 1970s, SCD became a touchstone for the civil rights movement. At the time, the average patient died before age 20. The Black Panther Party took up the cause and began testing people at their “survival conferences” across the country. The federal government took notice, and, in 1971, President Richard Nixon told Congress, “It is a sad and shameful fact that the causes of this disease have been largely neglected throughout our history. We cannot rewrite this record of neglect, but we can reverse it. To this end, this administration is increasing its budget for research and treatment of [SCD].”

For many years, funding increased, newborn screening took hold, and, by the 1990s, life expectancy doubled, with patients living into their 40s. But over time, funding waned, clinics closed, and life expectancy started dropping again.

Clinics like Dr. Vinchinsky’s continue to help; “I feel I owe my life to [the program]. If it wasn’t for [Dr. Vinchinsky] and the things that he did for me, my family wouldn’t have me.” said Derek Perkins, 45, who has received treatment at the sickle cell center in Oakland since childhood. In addition, the American Society of Hematology is prioritizing research and funding through its SCD Initiative.

Sources: NPR News, November 4, 2017; Lanzkron S, Carroll CP, Haywood C Jr. Mortality rates and age at death from sickle cell disease: U.S., 1979–2005. Public Health Rep. 2013;128:110-6.