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Sickle cell anemia - Treatment

Alternative Names

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

Treatment:

The goal of treatment is to manage and control symptoms, and to limit the number of crises.

Patients with sickle cell disease need ongoing treatment, even when they are not having a painful crisis.

Folic acid supplements should be taken. Folic acid is needed to make red blood cells.

Treatment for a sickle cell crisis includes:

  • Blood transfusions (may also be given regularly to prevent stroke)
  • Pain medicines
  • Plenty of fluids

Other treatments for sickle cell anemia may include:

  • Hydroxyurea (Hydrea), a medicine that may help reduce the number of pain episodes (including chest pain and difficulty breathing) in some people
  • Antibiotics to prevent bacterial infections, which are common in children with sickle cell disease

Treatments for complications of sickle cell anemia may include:

  • Kidney dialysis or kidney transplant for kidney disease
  • Drug rehabilitation and counseling for psychological complications
  • Gallbladder removal in those with gallstone disease
  • Hip replacement for avascular necrosis of the hip
  • Treatments, including surgery, for persistent, painful erections (priapism)
  • Surgery for eye problems
  • Wound care, zinc oxide, or surgery for leg ulcers

Bone marrow or stem cell transplants can cure sickle cell anemia. However, they are current not an option for most patients. Sickle cell anemia patients are often unable to find well-matched donors.

Support Groups:

Joining a support group where members share common experiences can relieve the stress related to caring for someone with a chronic disease. See: Sickle cell anemia - support group

Expectations (prognosis):

In the past, sickle cell patients often died from organ failure between ages 20 and 40. Thanks to a better understanding and management of the disease, today, patients can live into their 50s or beyond.

Causes of death include organ failure and infection. Some people with the disease experience minor, brief, infrequent episodes. Others experience severe, long-term, frequent episodes with many complications.

Complications:

Calling your health care provider:

Call your health care provider if you have:

  • Painful crises
  • Any symptoms of infection (fever, body aches, headache, fatigue)
  • Reviewed last on: 2/28/2011
  • David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc., Yi-Bin Chen, MD, Leukemia/Bone Marrow Transplant Program, Massachusetts General Hospital.

References

Lee MT, Piomelli S, Granger S, et al. Stroke prevention trial in sickle cell anemia (STOP): extended follow-up and final results. Blood. 2006;108:847-852.

Brawley OW, Cornelius LJ, Edwards LR, Gamble VN, Green BL, Inturrisi C, et al. National Institutes of Health consensus development conference statement: hydroxyurea treatment for sickle cell disease. Ann Intern Med. 2008;148:932-938.

Geller AK, O'Connor MK. The sickle cell crisis: a dilemma in pain relief. Mayo Clin Proc. 2008;83:320-323.

Hebbel RP. Pathobiology of sickle cell disease. In: Hoffman R, Benz EJ, Shattil SS, et al., eds. Hematology: Basic Principles and Practice. 5th ed. Philadelphia, Pa: Elsevier Churchill Livingstone;2008:chap 42.

Saunthararajah Y, Vichinsky EP, Embury SH. Sickle cell disease. Clinical features and management. In: Hoffman R, Benz Jr. EJ, Shattil SS, eds. Hematology: Basic Principles and Practice. 5th ed. Philadelphia, Pa: Churchill Livingston; 2008:chap 43.

U.S. Preventive Services Task Force. Screening for Sickle Cell Disease in Newborns: U.S. Preventive Services Task Force Recommendation Statement. Agency for Healthcare Research and Quality, Rockville, MD. Sep 2007:AHRQ Publication No. 07-05104-EF-2.

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