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THALASSEMIA (Mediterranean Anemia; Hereditary Leptocytosis)

THALASSEMIA
(Mediterranean Anemia; Hereditary Leptocytosis)

DESCRIPTION

Thalassemia is an inherited form of anemia in which red blood cells contain less hemoglobin than normal.
Appropriate health care includes:
  • Self-care.
  • Physician's monitoring of general condition and medications.
  • Hospitalization for repeated transfusions as needed.

    SIGNS & SYMPTOMS

  • Fatigue.
  • Paleness.
  • Breathlessness.
  • Irregular heartbeat, especially with exertion.
  • Bloody or dark urine.
  • Jaundice (yellow skin and eyes).
  • Leg ulcers.
  • Enlarged spleen.

    CAUSES

  • Destruction of abnormal blood cells in your child's spleen and other sites.
  • Inadequate manufacture of normal amounts of hemoglobin-A.

    RISK FACTORS

  • Poor nutrition, especially a diet likely to produce other anemias.
  • Obesity.
  • Family history of thalassemia.
  • Genetic factors, including absence of the gene necessary to manufacture hemoglobin-A. The disorder first appeared in persons of Mediterranean heritage; it also affects people from the Middle East and Far East.

    PREVENTING COMPLICATIONS OR RECURRENCE

    Cannot be prevented at present, especially if the mother and father have thalassemia or the thalassemia genetic trait. If you have a family history of thalassemia, obtain genetic counseling before having children.

    BASIC INFORMATION

    MEDICAL TESTS

  • Your own observation of symptoms.
  • Medical history and physical exam by a doctor.
  • Laboratory blood tests and bone-marrow examinations.

    POSSIBLE COMPLICATIONS

  • Many years of thalassemia produce gallstones.
  • Repeated transfusions increase your child's risk of transfusion reaction or kidney damage.

    PROBABLE OUTCOME
    This condition is currently considered incurable. However, your child's symptoms can be relieved or controlled. It usually causes death by early adulthood or middle age, depending on the severity of the symptoms. Scientific research into causes and treatment continues, so there is hope for increasingly effective treatment and cure.

    TREATMENT

    HOME CARE

    The only treatment for thalassemia is periodic hospitalization for blood transfusions when the child's symptoms become disabling.

    MEDICATION

  • Medicine usually is not necessary for this disorder. For minor pain, use non-prescription drugs such as acetaminophen.
  • See Medications section for information regarding medicines your doctor may prescribe.

    ACTIVITY
    After treatment, your child can resume normal activity as soon as possible.

    DIET & FLUIDS
    No special diet. Don't give the child iron supplements; they make symptoms worse.

    OK TO GO TO SCHOOL?

    Yes.

    CALL YOUR DOCTOR IF

    Your child has symptoms of anemia (fatigue, paleness, irregular heartbeat, breathlessness).

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