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Sickle Cell Disease Program

 Sickle Cell Disease Program
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 Blood Disorders
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Flower Receiving Treatment at Children's
Sickle Cell Clinic
Children with sickle cell disease are treated on an outpatient basis through the Hematology Clinic, which is held on Wednesday mornings and Monday and Friday afternoon on the 6th floor of the Fegan building. Regular health maintenance visits are recommended at least annually. More frequent visits are scheduled as needed.

Specific treatment for sickle cell disease and its complications will be determined by your child's physician based on:

  • your child's age, overall health and medical history
  • severity of the disease
  • your child's tolerance for specific medications, procedures or therapies
  • expectations for the course of the disease
  • your opinion or preference
Treatment options may include, but are not limited to, the following:
  • Penicillin to prevent infections
  • Drinking plenty of water daily (8 to 10 glasses) or receiving fluid intravenously to prevent and treat pain crises
  • Blood transfusions for anemia, and to prevent stroke; transfusions are also used to dilute the sickled hemoglobin with normal hemoglobin to treat chronic pain, acute chest syndrome, splenic sequestration and other emergencies.
  • Pain medications for sickle cell crises
  • Folic acid to help prevent severe anemia
  • Hydroxyurea, a medication recently developed that may help reduce the frequency of pain crises and acute chest syndrome; it may also help decrease the need for frequent blood transfusions.
  • Stem cell transplantation, a treatment involving hematopoietic stem cells, the specific type of cell from which all blood cells develop. Transplantation of normal stem cells from a sibling to produce normal blood production.
In addition to administering physical care, the sickle cell clinic staff educates patients and families in the areas of:
  • the reduction of acute and chronic complications
  • strategies for a health approach to chronic illness
  • genetic counseling for both the patient and the family
Preventative Care
A child's ongoing health maintenance program requires the involvement of many specialists. Some preventative procedures, accessible through specialized departments at Children's Hospital Boston, including:
  • Preoperative assessment to prevent operative complications
  • Access to reference laboratory for complex sickle hemoglobinopathies
  • Transcranial Doppler (TCD) screening program for early detection of patients at risk for stroke
  • Chronic red blood cell transfusion program for those children identified as being at high risk for stroke
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