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Sickle Cell Program provides comprehensive care
Working in partnership with primary care providers

Advances in preventative care and new medications have reduced the life-threatening complications of sickle cell disease, and many individuals can now live into their mid-40s and beyond. However, sickle cell disease is still severe, chronic and sometimes fatal.

In October, the Children’s Hospital Boston division of Hematology recruited Matthew Heeney, MD, to direct the hospital’s Sickle Cell Program. The addition will allow the program to see more patients not only under urgent circumstances, but also for routine care, a goal that Dr. Heeney says is crucial to meeting the needs of this underserved patient population.

“Sickle cell disease can be deceptive in that patients can be relatively healthy for years before there are serious complications,” says Dr. Heeney. “For that reason, many primary care practitioners don’t refer patients to our program soon enough. My goal is to encourage pediatricians and other care providers to refer patients for consultation and expert patient education at least once a year, regardless of their condition.”

Dr. Heeney says he was drawn to the Children’s program—which currently serves about 185 patients from infancy through young adulthood—because it offers cutting-edge screening, therapy and treatment in a field that is constantly changing. The program offers genetic counseling to families and helps them to manage the complications of sickle cell disease, which include pain, stroke risk, acute chest syndrome and life-threatening infection.

The program hopes to partner with primary care providers to manage the disease, not to take over the community physician’s role. Ellis Neufeld, MD, director of Clinical Hematology and the Boston Center for Genetic Blood Diseases, is a national advocate of the “medical home” concept, and says the physician-specialist partnership is particularly important because most doctors see very few sickle cell patients. “When you have only one or two sickle cell patients, it’s difficult to have a full view of what this disease can be like and what patients need to know,” says Dr. Neufeld. “Early referral for comprehensive assessment and expert education is the best way to prevent long-term complications.”

Using advanced assessment tools, Children’s hematologists can gauge each sickle cell patient’s risk of stroke and pursue the appropriate level of treatment. Treatment options range from health maintenance through pain medication, blood transfusions and penicillin, to the use of new drugs such as hydroxyurea (which may help reduce the frequency of pain crises and acute chest syndrome, as well as the need for frequent blood transfusions). Bone marrow transplantation is an option for the most advanced cases.

 

	Newborn screening: key to successful treatment
	In collaboration with Dr. Neufeld and other pediatric hematologists, Suzette Oyeku, MD, fellow in Health Services Research, is studying the needs of care providers with respect to sickle cell disease. Dr. Oyeku is working to assess provider knowledge of and comfort with the newborn screening process for the disease. She hopes her work will lead to better tools for pediatricians, nurse practitioners and family practitioners to use when interpreting newborn screening results and providing genetic counseling. “Studies have shown that if you identify affected Children early, you can decrease the morbidity and mortality associated with the disease,” says Dr. Oyeku.