Department of Health: Testing for Sickle Cell Disease Is Important and Treatment Is Available

HARRISBURG, PA — Secretary of Health Dr. Rachel Levine is reminding Pennsylvanians of the seriousness of sickle cell disease and the importance of getting tested for it. Sickle cell disease is the most common inherited blood disease.

“We want people to get tested for sickle cell disease if they believe they could be a carrier of it,” Secretary of Health Dr. Rachel Levine said. “We inherit traits from our parents like eye and hair color, but they also pass along internal traits like blood type and sickle cell conditions. It is important to be tested to confirm if you have sickle cell disease, so that treatment for the disease can be started right away to further protect yourself and your family.”

Sickle cell disease is an inherited blood disease where an individual’s red blood cells take a crescent or sickle shape. This change in shape can create blockages that prevent blood from reaching parts of the body. As a result, people with sickle cell complications can experience anemia, gallstones, stroke, chronic pain, organ damage and even premature death.

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According to the Centers for Disease Control and Prevention (CDC), sickle cell disease affects approximately 100,000 Americans. This disease has a greater influence on African American and Hispanic populations but is also found among many other races and ethnicities.

Sickle cell disease is one of the 10 mandatory diseases screened for newborns. These screenings are conducted with the goal of eliminating or reducing death, disease and disability in newborn children. In addition, sickle cell disease can be diagnosed before birth to provide an early diagnosis and find treatment.

Treatment can help those with sickle cell disease live well and be healthy, but there is ultimately no cure for sickle cell disease. Treatment requires:

  • Finding good medical care and getting regular checkups;
  • Staying up to date on vaccinations and washing hands frequently to prevent infections;
  • Learning healthy habits;
  • Looking into clinical studies; and
  • Finding support and assistance.
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Studies have shown that donated bone marrow or stem cell transplants have helped cure sickle cell disease in children with severe cases of the disease. This means that the healthy donated bone marrow or stem cell transplant replaces an individual’s bone marrow that is not working properly.

Bone marrow or stem cell transplants can be risky and for the donation to work the individual would need to be a close match like a brother or sister.

The Wolf administration has developed a prescribing guideline for the treatment of acute and chronic pain in patients with sickle cell disease to assist physicians treating patients with the disease. The guideline provides best practices to treat acute painful crises that occur with sickle cell disease patients as well as best practices for chronic pain care.

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The sickle cell disease guideline addresses the specific needs of that patient population.

This can help prevent the misapplication of recommendations to populations that are outside the scope of other prescribing guidelines, including patients experiencing acute sickle cell crises. It is especially important to have resources specifically for the treatment of sickle cell disease patients as this patient population often experiences racial disparities and stigma.

More information on sickle cell disease can be found on the Department of Health’s website at www.health.pa.gov.

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