Sickle Cell Disease - References

Prevention and Lifestyle Changes

General Precautions

To prevent or reduce the severity of long-term complications of sickle cell disease, several precautions may be helpful:

Have regular physical examinations every 3 - 6 months.

Have periodic and careful eye examinations.

Have sufficient rest, warmth, and increased fluid intake. (These are critical precautions for reducing oxygen loss and the risk for dehydration.)

Avoid situations, such as crowds, that increase risk for infections.

Avoid excessive demands on the body that increase oxygen needs (physical overexertion, stress). Low impact exercise (leg lifts, light weights) may be useful and safe for maintaining strength, particularly in the legs and hips, but patients should consult their doctor before starting any exercise program.

Avoid high altitudes if possible. If flying is necessary, be sure that the airline can provide oxygen.

Do not smoke, and avoid exposure to second-hand smoke. Both active and passive smoking may promote acute chest syndrome in patients with sickle cell disease.

Preventing Infections

Vaccinations. Everyone with sickle cell disease should have complete regular immunizations against all common infections. Children should have all routine childhood vaccinations. The following are important vaccinations for everyone with sickle cell disease:

Pneumococcal vaccines. All sickle cell patients should be vaccinated with the pneumococcal vaccine. There are two types of pneumococcal vaccines; the choice between them depends on the age of the patient. Infants and children less than 2 years of age should receive 4 doses of the pneumococcal conjugated vaccine (Prevnar) between 2 - 15 months of age. The pneumococcal polysaccharide vaccine should be administered at age 2 years or older, repeated after 3 - 5 years for patients younger than age 10, or in 5 years for patients older than age 10.

Vaccination against Haemophilus influenza, the major cause of meningitis in unimmunized children, starting at age 2 months.

Influenza vaccines ("flu shots") should be given every winter, starting at age 6 months.

Meningococcal vaccination for patients age 5 and older.

Hepatitis B vaccine. All children should receive this vaccine.