HEMOPHILIA |
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DESCRIPTIONHemophilia is an inherited deficiency of a blood-clotting factor that results in dangerous bleeding. Primarily blood and bone marrow throughout the body are involved. Hemophilia affects 1 in 10,000 males, and appears early in childhood. Females carry the disease but do not exhibit symptoms.
Appropriate health care includes:
Physician's monitoring of general condition and medications. Doctor should be a qualified hematologist (blood specialist).
Hospitalization or care in an outpatient facility for transfusions of plasma and various blood factors when needed.
Self-care.
SIGNS & SYMPTOMS
Painful, swollen joints or swelling in the leg or arm (especially the knee or elbow) when bleeding occurs.
Frequent bruises.
Excessive bleeding from minor cuts.
Spontaneous nosebleeds.
Blood in the urine.
CAUSES
The deficiency of a coagulation factor passed by a female carrier to male children in an X-linked recessive gene.
RISK FACTORS
No known risk factor.
PREVENTING COMPLICATIONS OR RECURRENCECannot be prevented at present. If your family has a history of hemophilia, 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 studies.
POSSIBLE COMPLICATIONS
Dangerous bleeding episodes requiring emergency treatment.
Permanent joint disability caused by persistent bleeding.
Hepatitis or AIDS from blood transfusions.
PROBABLE OUTCOME
This condition is currently considered incurable but not fatal. If bleeding can be controlled, patients can expect a nearly normal life span. Scientific research into causes and treatment continues, so there is hope for increasingly effective treatment and cure.
TREATMENT
HOME CARE
For bleeding at any accessible site, apply direct pressure by hand or elastic bandage or apply ice and elevate the child's limb. Call your doctor immediately.
In case of emergency, your child should wear a bracelet or pendant mentioning hemophilia.
MEDICATION
Your doctor may prescribe:
-- Medication to reduce joint pain.
-- Transfusions of plasma or clotting factors.
Your child should not take aspirin. It may increase bleeding.
See Medications section for information regarding medicines your doctor may prescribe.
ACTIVITY
Your child should avoid activities that can cause injury, such as contact sports. Urge the child to swim, bicycle, or walk instead. Otherwise, no restrictions.
DIET & FLUIDS
No special diet.
OK TO GO TO SCHOOL?When appetite has returned and alertness, strength, and feeling of well-being will allow.
CALL YOUR DOCTOR IF
Your child has symptoms of hemophilia.
The following occurs after diagnosis:
-- Injury with swelling. This may indicate bleeding under the skin.
-- Bleeding that isn't quickly controlled.
-- Tender, painful, swollen joint.
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