Treatment:

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 red blood cells, is required because of the rapid red blood cell turnover.

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

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

Hydroxyurea (Hydrea) is a drug used fro some patients to reduce the number of episodes of pain (including chest pain and difficulty breathing). It does not work for everyone.

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

Additional treatments for complications may include:

• Dialysis or kidney transplant for kidney disease
• Drug rehabilitation and counseling for the psychological complications
• Gallbladder removal (if there is significant gallstone disease)
• Hip replacement for avascular necrosis of the hip (death of the joint)
• Irrigation or surgery for priapism (persistent, painful erections)
• Partial exchange transfusion for acute chest syndrome
• Surgery for eye problems
• Transfusions or surgery for brain problems, such as strokes
• Wound care, zinc oxide, or surgery for leg ulcers

Bone marrow transplants can cure sickle cell anemia. However, very few people with this disease are able to find a suitable donor. Bone marrow transplantation may also include risks, including infection and rejection.

Support Groups:

Sickle cell anemia, like other chronic, life-threatening diseases, can cause great stress to the patient and family members. Joining a support group, where members share common experiences and problems, can relieve this stress.

See sickle cell anemia - support group.

Expectations (prognosis):

In the past, death from organ failure often occurred between the ages of 20 and 40 in most sickle-cell patients. More recently, because of better understanding and management of the disease, patients live into their forties and fifties.

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

Complications:

• Acute chest syndrome
• Blindness/visual impairment
• Death
• Erectile dysfunction (as a result of priapism)
• Gallstones
• Infection, including pneumonia, cholecystitis (gallbladder), osteomyelitis (bone), and urinary tract infection
• Joint destruction
• Leg ulcers
• Loss of function of the spleen
• Multisystem disease (kidney, liver, lung)
• Narcotic abuse
• Neurologic (brain and nervous system) symptoms and stroke
• Parvovirus B19 infection resulting in aplastic crisis
• Recurrent aplastic and hemolytic crises resulting in anemia and gallstones
• Splenic sequestration syndrome
• Tissue death of the kidney

Calling your health care provider:

Call your health care provider if acute painful crises occur or at the first sign of any infection.