Sickle cell anemia Health Article

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.

Prevention

Sickle cell anemia can only result when two carriers with sickle cell trait have a child together. Therefore, genetic counseling is recommended for all carriers of sickle cell trait. About 1 in 12 African Americans has sickle cell trait. Diagnosis of sickle cell anemia during pregnancy is also available.

Prompt treatment of infections, adequate oxygenation, and preventing dehydration may prevent sickling of red blood cells. Antibiotics and vaccinations may prevent infections.

General health visits with a physician are recommended to ensure the patient is getting adequate nutrition, maintains proper activity levels, and receives proper vaccinations.

PREVENTING CRISES

Parents whose children have sickle cell disease should encourage their children to lead normal lives. However, in order to decrease the occurrence of sickle cell crises, consider the following precautions:

To prevent loss of oxygen from tissues, avoid the following:

1. Demanding physical activity, especially if the spleen is enlarged
2. Emotional stress
3. Environments with low oxygen content (high altitudes, non-pressurized airplane flights)
4. Known sources of infection

To promote proper hydration:

1. Recognize signs of dehydration
2. Avoid too much exposure to the sun
3. Provide access to fluids, both at home and away

To avoid sources of infection:

1. Keep child properly immunized as recommended by the health care provider
2. Consider having the child wear a Medic Alert Bracelet
3. Share above information with teachers and other caretakers as appropriate

Be aware of the effects that chronic, life-threatening illnesses can have on siblings, marital relationships, parents, and the child.

References

Goldman L, Ausiello D. Cecil Textbook of Medicine. 22nd ed. Philadelphia, Pa: WB Saunders; 2004:1030-1039

Hoffman R, Benz Jr. EJ, Shattil SJ, et al., eds. Hematology: Basic Principles and Practice. 4th ed. Philladelphia, Pa: Churchill Livingston; 2005:591-634.