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:

• Acute chest syndrome
• Anemia
• Blindness/vision impairment
• Brain and nervous system (neurologic) symptoms and stroke
• Death
• Disease of many body systems (kidney, liver, lung)
• Drug (narcotic) abuse
• Erectile dysfunction (as a result of priapism)
• Gallstones
• Hemolytic crisis
• Infection, including pneumonia, gallbladder inflammation (cholecystitis), bone infection (osteomyelitis), and urinary tract infection
• Joint destruction
• Leg sores (ulcers)
• Loss of function in the spleen
• Parvovirus B19 infection, leading to low red blood cell production (aplastic crisis)
• Splenic sequestration syndrome
• Tissue death in the kidney

Calling your health care provider:

Call your health care provider if you have:

• Painful crises
• Any symptoms of infection (fever, body aches, headache, fatigue)