SICKLE CELL ANEMIA

Sickle Cell anemia is an inherited disease that results in abnormal red blood cell production. The disease is frequently diagnosed during infancy when the baby has pain and swelling of the hands and feet. During periods of oxygen stress, (such as with exercise, with dehydration, or during an illness) the red blood cells change shape into something that looks like a "sickle". These cells block small blood vessels resulting in pain. Damage can also occur in the kidney, eyes, and brain. However, episodes of pain (frequently involving the legs or chest) are the most common complaint.

The treatment of a painful episode includes fluids, pain medications, and occasionally antibiotics, blood transfusions and/or oxygen. Hydroxyurea may prevent sickle cell painful episodes.

The only cure for sickle cell anemia is to replace the bone marrow with healthy bone marrow from a donor. The new bone marrow will then make healthy blood, free of sickle cell disease. This treatment is quite aggressive and has many risks. Transplantation therapy is relatively new and not yet considered standard treatment for sickle cell disease.

The Adult Stem Cell- Bone Marrow Transplant Program of Hackensack University Medical Center has an active transplant treatment protocol for Sickle Cell Anemia. For information call (201) 996-5849.

The transplant trial offers an allogeneic transplant from an HLA-matched brother/sister using high-dose chemotherapy. To be eligible, patients with sickle cell must have advanced disease with either a history of neurolgic complications, chest syndrome, kidney disease, recurrent pain, or chronic red cell transfusion requirements. For more information on this trial click here.