Sickle cell anemia

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Blood cells	Formed elements of blood	Red blood cells, multiple sickle cells	Red blood cells, normal
Red blood cells, sickle and pappenheimer	Red blood cells, sickle cell	Red blood cells, sickle cells

Anemia - sickle cell; Hemoglobin SS disease (Hb SS); Sickle cell disease

Patients with sickle cell disease need continuous treatment, even when they are not having a painful crisis. Supplementation with folic acid, an essential element in producing cells, is required because of the rapid red blood cell turnover.

The purpose of therapy is to manage and control symptoms and to try to limit the frequency of crises.

During a sickle crisis, certain therapies may be necessary. Painful episodes are treated with analgesics and adequate liquid intake. Treatment of pain is critical. Non-narcotic medications may be effective, but some patients will require narcotics.

Hydroxyurea (Hydrea) was found to help some patients by reducing the frequency of painful crises and episodes of acute chest syndrome and decreasing the need for blood transfusions. There has been some concern about the possibility of this drug causing leukemia, but as yet there are no definitive data that Hydrea causes leukemia in sickle cell patients.

Newer drugs are being developed to manage sickle cell anemia. Some of these agents work by trying to induce the body to produce more fetal hemoglobin (therefore decreasing the amount of sickling) or by increasing the binding of oxygen to sickle cells. But as yet, there are no other widely used drugs that are available for treatment.

Bone marrow transplants can be curative, this therapy is indicated in only a minority of patients, predominantly because of the high risk of the procedure (the drugs needed to make the transplant possible are highly toxic) and difficulty in finding suitable donors. Also, bone marrow transplants are much more expensive than other treatments.

Antibiotics and vaccines are given to prevent bacteria infections, which are common in children with sickle cell disease.

Additional treatments may include:

• partial exchange transfusion for acute chest syndrome
• transfusions or surgery for neurological events, such as strokes
• dialysis or kidney transplant for kidney disease
• irrigation or surgery for priapism
• surgery for eye problems
• hip replacement for avascular necrosis of the hip (death of the joint)
• gallbladder removal (if there is significant gallstone disease)
• wound care, zinc oxide, or surgery for leg ulcers
• drug rehabilitation and counseling for the psychosocial complications