NeDina Brocks-Capla doesn't like to go in her bathroom these days. It's where her son Kareem Jones died five years ago, at age 36, from sickle cell disease. Brocks-Capla thinks her son might still be with her if he'd received proper medical care, Jenny Gold reports for Kaiser Health News (KHN).
Background on sickle cell care
Sickle cell disease is a genetic disorder that affects about 100,000 U.S. patients. Most patients are African-American. The disease makes it difficult for red blood cells to deliver oxygen to cells throughout the body. It can trigger an acute sickle cell episode, which causes excruciating pain by restricting a patient's blood flow, and if left untreated can damage vital organs and cause strokes.
Proper treatment typically involves fluids, pain medication, and oxygen—but patients often struggle to receive adequate care. Data from the American Society of Hematology show the mortality rate for adults with sickle cell disease has increased 1% annually since 1979, and half of adults with sickle cell die by their early 40s. What's more: Life expectancy for patients has peaked and fallen. Life expectancy in 1994 was age 42 and age 48 for men and women, respectively, but in 2005 it fell to age 38 for women and age 42 for men, according to KHN.
Barriers to care
Elliott Vichinsky, who opened a sickle cell center at UCSF Benioff Children's Hospital Oakland in 1978, said, "One of the national crises in health care is the care for adult sickle cell. 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."
For example, research has shown sickle cell patients have to wait up to 50% longer to receive help in the ED compared with other patients experiencing pain.
There are several reasons why care for sickle cell patients is limited, including low provider payment rates and low funding levels for research into the disease.
For patients who are able to access care, experts say there are a limited number of FDA-approved treatments for sickle cell. Until last year, the only FDA-approved treatment for sickle cell diseases was hydroxyurea, which was approved first as a cancer drug in 1967. The FDA in July 2017 approved Endari, a drug to treat sickle cell disease specifically.
Despite those limitations, Vichinsky said several advancements, including screenings for the early signs of organ failure and interventions to prevent premature deaths, have helped sickle cell patients at his clinic and other specialty clinics live two decades longer than the average patient with sickle cell.
How discrimination plays a role in sickle cell care barriers
But for many sickle cell patients such care is not available, and some experts say discrimination plays a role—and reflects broader discrimination African-Americans experience in the medical system. Keith Wailoo, a professor at Princeton University who wrote about the history of the disease, said sickle cell is "a microcosm of how issues of race, ethnicity and identity come into conflict with issues of health care."
When Brocks-Capla took her son to the ED, doctors stabilized him but did not diagnose the underlying cause of his distress. He went home in "incredible pain," KHN reports and later died. He had a seizure, his mother said, and had suffered several small strokes—which the hospital didn't detect, according to Brocks-Capla.
Vichinsky said, "I would say 40% or more of the deaths I've had recently have been preventable—I mean totally preventable. It makes me so angry. I've spent my life trying to help these people, and the harder part is you can change this—this isn't a knowledge issue. It's an access issue" (Gold, Kaiser Health News, 11/6/17).
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