SICKLE-CELL ANEMIA |
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DESCRIPTIONSickle-cell anemia is an inherited blood disorder that causes anemia, episodes of severe pain, low resistance to infection and chronic poor health. It is not cancerous. The bone marrow, lymph glands, spleen, liver, and thymus are involved. Symptoms of sickle-cell anemia usually begin around 6 months of age and last a lifetime.
Appropriate health care includes:
Physician's monitoring of general condition and medications. If your child has the condition, seek special treatment from a pediatrician with special knowledge of this condition.
Psychotherapy or counseling may be helpful in adapting to this condition, especially for children.
Hospitalization at times of severe attacks.
SIGNS & SYMPTOMS
Anemia with shortness of breath, rapid heartbeat, fatigue, and jaundice.
Episodes of pain in joints, chest, abdomen, and back.
Frequent infections, especially pneumonia.
Nerve impairment.
Delayed growth and development.
Skin ulcers, especially on the legs.
CAUSES
This disease is hereditary. Persons with the gene may pass it on to their children. Red blood cells change from round to sickle shapes; this causes blockage in the capillaries. Low oxygen in the tissues is partly responsible for the changed shape. The change occurs in attacks that cause pain and disability. The disease occurs mostly in black people.
RISK FACTORS
Family history of sickle-cell anemia. The following may aggravate symptoms:
Ascending to high altitude, as in driving up a mountain or flying.
Pregnancy.
Surgery.
Injury.
PREVENTING COMPLICATIONS OR RECURRENCEIf you have a family history of sickle-cell anemia, ask your doctor to test you. If the condition is present, obtain genetic counseling before starting a family. A less serious condition, sickle-cell trait, may be present. It will not cause the disease, but genetic counseling is still desirable.
BASIC INFORMATION
MEDICAL TESTS
Your own observation of symptoms.
Medical history and physical exam by a doctor.
Laboratory blood studies. Simple screening tests are also available. They may be done at birth if there is a family history of sickle-cell anemia.
X-rays of bones and lungs.
POSSIBLE COMPLICATIONS
Infections of the lungs and bones.
Kidney failure.
Eye disease.
Stroke.
PROBABLE OUTCOME
Sickle-cell anemia is incurable and your child's life expectancy is reduced. A few patients reach adulthood. Most patients die prematurely of infection or stroke.
TREATMENT
HOME CARE
During an attack, keep your child warm. Apply warm compresses to painful areas.
Maintain your child's immunization schedule, including a pneumonia vaccine.
Don't let your child fly, even in pressurized planes, without oxygen. Check with your airline.
Urge your child to wear a Medic-Alert bracelet or pendant (See Glossary).
MEDICATION
No medications are yet available to control this condition. For severe attacks, intravenous fluids, blood transfusions, and pain relievers are helpful.
ACTIVITY
Your child should avoid strenuous exercise and exposure to cold temperatures. Bedrest is necessary during acute attacks.
DIET & FLUIDS
Your child should drink at least 8 glasses of water a day -- more if there is a fever. This helps keep blood cells from collecting and blocking capillaries.
OK TO GO TO SCHOOL?Yes. OK between attacks or injury.
CALL YOUR DOCTOR IF
Your child has signs and symptoms of sickle-cell anemia.
You want to know if you have the sickle-cell gene.
Your child has the disease, and symptoms recur after a period of remission or your child develops fever or other signs of infection.
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