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HEMOPHILIA

Definition:

A deficiency of certain factors responsible for clotting of blood that results in episodes of bleeding, which could at times be dangerous. Hemophilia is an inherited condition.

Sex or Age most Affected

Hemophilia affects 1 in 10,000 male children and appears early in childhood.

Signs & Symptons

  • 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 particular clotting factor. The mother passes this deficiency to male children where she herself does not suffer but is only a carrier.

Prevention

Cannot be prevented at present. If your family has a history of hemophilia, obtain genetic counseling before having children.

Diagnostic Measures

  • Observations of symptoms i.e. excessive bleeding from minor cuts or minor blows leading to swollen joints.
  • History and exam by a doctor.
  • Laboratory blood studies.

Possible Complications

  • Dangerous bleeding episodes requiring emergency treatment.
  • Permanent joint disability caused by persistent bleeding.
  • Since these patients require frequent blood transfusions, Hepatitis or HIV from repeated blood transfusions.

Probable Outcomes

This condition is currently considered incurable, but not fatal. If bleeding can be controlled, patients can expect a near normal life span.

TREATMENT

General Measures

  • For bleeding at any accessible site, apply direct pressure by hand or elastic bandage or apply ice and elevate the limb. Call your doctor immediately.
  • A child, who has hemophilia, MUST always carry an identification card, with information about his disease, steps to be taken in case of injury/bleeding and family physician to be contacted.

Medication

Your doctor may prescribe:

  • Medication to reduce joint pain.
  • Transfusions of plasma or clotting factors, depending on severity.
  • Don’t give aspirin or any other painkiller without doctor’s advice. It may increase bleeding.

Activity

Avoid activities that can cause injury, such as contact sports. Child should be allowed to swim, bicycle or walk instead. Otherwise, no restrictions.

Contact your Doctor

  • You notice any of the symptoms of hemophilia in your child.

The following occur after diagnosis:

  • Injury with swelling. This may indicate bleeding under the skin.
  • Bleeding that isn’t quickly controlled.
  • Tender, painful, swollen joints
  • Head injury with vomiting.