Sickle cell disease is a blanket term for several related genetic blood disorders, including sickle cell anemia. About 70,000 people in the United States have sickle cell disease — about 4,000 of them in Arkansas — and most are of African descent. In the United States, about one of every 500 African-American babies is born with sickle cell disease, and an estimated one in 12 black people carries the sickle cell gene. Sickle cell disease is also more common in people of South American and Central American ancestry (in the U.S., one in 900 Hispanic babies is born with the disease, and one in 16 Hispanic adults carries the gene) as well as people of Indian, Saudi Arabian, Italian, Turkish and Greek heritage. The most common types of sickle cell disease in the U.S. are Hb-SS, Hb-SC and two types of sickle thalassemia.
Among Arkansas Medicaid patients, sickle cell syndrome is often misdiagnosed or undertreated. The Arkansas Foundation for Medical Care is working with the Arkansas Department of Human Services and Arkansas Medicaid to improve diagnosis and management of this difficult disease, and to increase the use of hydroxyurea therapy among patients who fit the treatment protocols.
Suggestions for improvement are:
- All patients with sickle cell syndromes should have documentation of hemoglobin electrophoretic patterns in the chart at their primary care provider's office.
- Patients with hemoglobin SS need focused attention on preventive therapy, including early use of antibiotics, to prevent life-threatening sepsis.
- Reminder stickers for essential preventive services could help improve care for this population.
- Careful coding of sickle cell syndromes can help target appropriate services and enable state policy-makers to better understand the provision of care to sickle cell patients.
The project's quality indicators are:
- Provide regularly scheduled comprehensive medical evaluations every two to four months for special health care needs and disease-related problems.
- Consider referral to a hematologist in the geographic area to ensure continued hematology follow-up and multi-disciplinary management.
- Maintain familiarity with developmental, educational and community resources for sickle cell disease.
- Ensure that patients with sickle cell disease are regularly reviewed for the possibility of hydroxyurea treatment.
- Provide a comprehensive medical home for patients and families.