PHOENIX – Health care experts are calling on federal and state agencies to take action to provide better care for the estimated 100,000 Americans who suffer from sickle cell disease.
Sickle cell is the most common inherited blood disorder in the U.S. and disproportionately affects African Americans, occurring in 1 out of every 365 births of Black children, according to the U.S. Centers for Disease Control and Prevention. Sickle cell patients may experience frequent infections, fatigue, pain, eye problems and a higher risk of stroke.
In February 2019, at the behest of the Department of Health and Human Services’ Office of Minority Health, a committee of experts convened to develop recommendations for better managing sickle cell disease and improving care for patients throughout their lives.
That work concluded in fall 2020 with a 500-page “blueprint for action,” published by the National Academies of Sciences, Engineering and Medicine, that recommends 26 steps over the next five years. Those steps include:
- Establish a national system to collect and link data related to the disease.
- Work to increase the number of qualified professionals providing this care.
- Remove financial barriers related to the costs of some treatment therapies.
- Expand rehabilitation programs for adults living with sickle cell.
The disease occurs when red blood cells contort into a crescent-shaped “sickle.” Those cells can’t move easily throughout the body and can block blood flow. Sickle cells also die early, leaving fewer healthy cells to carry oxygen throughout the body.
The academies’ report noted that although the disease is rarely fatal to children, sickle cell has a higher mortality rate in adults, in large part because of progressive organ damage. Committee members called for more help for adult patients.
“The first patient living with sickle cell disease was described in the U.S. in 1910. That individual probably received the same supportive care for his pain that we are providing in 2020, so we really need to do this work,” Dr. Kim Smith-Whitley, a committee member and director of the Comprehensive Sickle Cell Center at Children’s Hospital of Philadelphia, said in a video conference announcing the report’s release.
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Brett Giroir, U.S. assistant secretary of health in the Trump administration, said during an October webinar that additional curative therapies “are in the pipeline, but they’re highly expensive.”
“We need to understand how to pay for them so we don’t get trapped in a situation which is worse than not having a cure – and that is having a cure that no one can get,” he said.
Another focus laid out in the report is improving the availability of health care professionals, such as hematologists, particularly in rural areas and communities of color.
Martha Liggett, executive director of the American Society of Hematology, said grant money could help add more sickle cell research and treatment centers across the country.
“We’re working on a more general distribution for hematologists and for other health care providers, nurses” and physician assistants, Liggett said. “We need a whole team of people that are educated about how to deliver care properly.”
Experts in the field note the lack of research and data has left patients feeling neglected, but Giroir said part of the solution is pushing harder for additional funding.
“Despite the fact that the CDC has a lot of money, it’s really in buckets that it needs to be spent for,” Giroir said. “You can’t take funding out of measles and put it into sickle cell disease. It’s very important for all of us as we push forward to make sure there is advocacy for funding.”
Brooke Cook of the Sickle Cell Foundation of Arizona said merely increasing awareness of the disease is one of the biggest challenges. The group hosts virtual sessions to keep patients and their families informed.
“We are kind of in a growing period right now for sickle cell where people are starting to talk about it and hear about it more,” Cook said. “But in Arizona, we are definitely a couple steps behind where we’d like to be compared to others.”
To get a better handle on sickle cell disease data, the CDC began a data collection program in 2015. So far, only nine states are enrolled – Arizona isn’t one of them. A 2010 report in the American Journal of Preventive Medicine estimated more than 800 Arizonans suffer from the disease.
