Patients with sickle cell disease need ongoing treatment, even when they are not having a painful crisis. They should take supplements of folic acid (essential for producing red blood cells) because red blood cells are turned over so quickly.

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

During a sickle cell crisis, you may need certain treatments. Painful episodes are treated with pain medicines and by drinking plenty of fluids. It is important to treat the pain. Non-narcotic medications may be effective, but some patients will need large doses of narcotics.

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

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

Blood transfusions are used to treat a sickle cell crisis. They may also be used on a regular basis to help prevent strokes.

Other treatments for complications may include:

• Dialysis or kidney transplant for kidney disease
• Drug rehabilitation and counseling for psychological complications
• Gallbladder removal (if you have gallstone disease)
• Hip replacement for avascular necrosis of the hip
• Irrigation or 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, transplants have many risks, including infection, rejection, and graft-vs-host disease. Therefore, they are currently not an option for most patients. Also, sickle cell anemia patients are often unable to find well-matched donors.

Sickle cell anemia 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

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.

• 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

Call your health care provider if you have:

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