What is Hemophilia?

Hemophilia is usually an inherited bleeding disorder in which the blood does not clot properly. This can lead to spontaneous bleeding as well as bleeding following injuries or surgery. Blood contains many proteins called clotting factors that help to stop bleeding, these patients lack one of these factor which results in bleeding.

People with hemophilia have low levels of either factor VIII (8) or factor IX (9). The sverity of hemophilia that a person has it determined by the amount of factor in the blood. The lower the amount of the factor, the more likely that bleeding will occur, which can lead to serious health problems and disability.

Transmission

Most people who have hemophilia are born with it. It almost always is inherited (passed down) from a parent to a child. Both hemophilia A and B are inherited in the same way because both the genes for factor VIII and factor IX are located on the X chromosome.

Common Signs of hemophilia Include

  • Bleeding into the joints. This can cause swelling and pain or tightness in the joints; it often affects the knees, elbows and ankles.

  • Bleeding into the skin (which is bruising).

  • Muscle and soft tissue causing a build-up of blood in the area (called a hematoma).

  • Bleeding of the mouth and gums and bleeding that is hard to stop after losing a tooth.

  • Bleeding after surgery (circumcision performed on male babies to remove the hood of skin, called the foreskin, covering the head of the penis).

  • Bleeding after having shots, such as vaccinations.

  • Bleeding in the head of an infant after a difficult delivery.

  • Blood in the urine or stool.

  • Frequent and hard-to-stop nosebleeds.

  • Life threatening bleed - brain bleed, GI bleed, throat bleed

Who is affected?

  • Hemophilia occurs in about 1 of every 5,000 male births.

  • Hemophilia A is about four times as common as hemophilia B, and about half of those affected have the severe form.

  • Hemophilia affects people from all racial and ethnic groups.

Diagnosis

  • Many people who have or have had family members with hemophilia will ask that their baby boys get tested soon after birth.

  • About one-third of babies who are diagnosed with hemophilia have a new mutation not present in other family members.

Whom To be tested?

  • History of Haemophilia in Maternal Family

  • Sibling with Hemophilia

  • Child of Carrier Mother

  • History and symptoms of prolong Bleeding

  • Female children of Father with Hemophilia for levels of factor

Complications

  • Inhibitors

  • Blood and Blood product Transfusion Related infections

  • Transfusion reactions

  • Disability - Locomotor

  • Psychosocial impact

How is Hemophilia Treated?

This is a Lifelong disorder which can be treated by various Medicines- Most effective of them is Factor Injections Factor VIII and Factor IX. This injection controls the bleeding immediately. Prophylaxis Physiotherapy.