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HEMOPHILIA IN CHILDREN
Blood tests help in diagnosing the disease. By determining the factor levels in the blood the diagnosis can be confirmed.
How is hemophilia treated ?
Replacement of the deficient factor in the blood is the mainstay of the therapy. There are various factor concentrates available. These factors can be given according to various treatment regimes:
1. Demand therapy :- Infusion of a factor at the time of a bleed.
2. Preventive therapy :- Instead of receiving the factor on demand, the patient receives infusions at regular intervals to maintain a functional level of factor in the blood at all times.
3. Continuous infusion :- It is usually given before or after elective surgery or after major trauma.
What are other general measures to be taken care of in hemophilia ?
A: Newborns with hemophilia rarely bleeding. Delivery by vacuum extraction is contra-indicated. If fetus is large or if labor is difficult, caesarian section should be considered. Newborns with hemophilia should not be circumcised. Routine intramuscular injections may be given. Immunization against Hepatitis A and B is also advisable.
For babies with hemophilia, the home environment should protected. The floor should be carpeted. Trouser and long sleeved shirts should be padded internally at the levels of the knees and elbows to protect from falls and bumps.
For children, contact sports should be avoided. Good dental care should be advised. Physical exercise is advised as strong muscles support joints and may prevent profuse bleeding.
How are the joints protected in hemophiliacs?
A: In hemophiliacs, joint bleeding and re-bleeding is common. With repeated hemorrhages, chronic joint damage may occur causing rebleeding. Thus, a vicious cycle is created. Joint cartilage is gradually destroyed and bone is reabsorbed causing degenerative arthritis. Joint bleeding during growing years may cause different lengths of the two limbs. Hence, prevention of joint bleeding and early treatment of joint bleeds is advocated to maintain the full mobility of the joint.
What are other therapies available for hemophilia?
A: Gene therapy for hemophilia A and B is being tried in animal studies.
How can Hemophilia be detected in the unborn child within the womb?
A: Invasive tests done at 8 weeks or 12-14 weeks can be used for prenatal diagnosis by DNA analysis if the mother's carrier state is known. These tests remove a part of the amniotic fluid (little water from the water sac in which the baby is growing in the womb) or other cells from the placenta. If DNA analysis is not available, fetal blood (blood from the unborn baby's vein) can be aspirated at 18-20 weeks gestation to determine the factor levels in the fetus.
What is the prognosis of a child with hemophilia?
A: Children with mild hemophilia can lead a normal life. With the advent of factor therapy, children with moderate and severe hemophilia, if treated early can lead a near normal life.
Last updated on 24-06-2002
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