Ten Compelling Facts About Hemophilia

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  1. Hemophilia is a bleeding disorder.
  • Coagulation of blood is vital to maintain hemostasis (causes bleeding to stop after direct injury to blood vessels).
  • Platelet and blood clotting factors is essential in a series of complex mechanisms in the process of coagulation.
  • This disorder involves defective mechanism of blood coagulation which results in an uncontrollable bleeding tendency.

        2.Hemophilia is a group of disorders

  • It does not refer to only one kind of disease.
  • It comes in three different conditions. A (Classic Hemophilia) Hemophilia B (Christmas Disease) and Hemophilia C.
  • There are about thirteen coagulation factors (I through XIII) which cause different types of Hemophilia.

        3.Hemophilia primarily affects male.

  • Change in one of the genes that are greatly involved in blood clotting factor production.
  • The mutation of X chromosome.
  • Some cases of Hemophilia in males are diagnosed after circumcision followed by heavy bleeding at the circumcision site.
  • Hemophilia A is about four times more common than B.
  • Hemophilia C affects both sexes. it is the milder form of the disease.

        4. Hemophilia has been termed as the Royal disease.

  • Women are Hemophilia carriers.
  • Queen Victoria of England was a carrier, passing the genes (to her daughters) to the royal families and her grandsons manifesting the symptoms.

5. Hemophilia affects people from all ethnic groups.

  • Hemophilia is considered a rare disease but all racial and ethnic groups are equally affected.
  • There is no geographic proclivity in the occurrence of hemophilia.

       6.Hemophilia has a good prognosis.

  • Advances in medical treatment of hemophilia permitted a nearly normal life expectancy of affected individuals.

7. Hemophilia is treated through coagulation factor replacement therapy.

  • In order for the blood to clot properly, factor concentrates are administered to patient having this kind of disorder.
  • This is done through intravenous transfusion of a commercially prepared concentrates.
  • There are two commonly used for coagulation replacement therapy available for hemophilia treatment. Plasma-Derived factor concentrates and Recombinant factor concentrates.
  • Plasma (liquid part of the blood) is purified separating the clotting proteins and bring the finished product into freeze.

8. Hemophilia currently has no cure.

  • Liver transplant offers hope for cure of hemophilia. However, this imposes an ethical dilemma because liver transplant is only done to save one’s life in cases of totally damged liver but not to cure any other kind of diseases.
  • Clotting factors are made in the liver.
  • A new liver made normal sustainable amount for the patient’s blood coagulation but this is too risky as an option to cure hemophilia.

        9.Hemophilia may lead to serious complications.

  • Chronic joint disease is one of the complications of the disorder which results in decreased or loss of mobility.
  • This is due to repeated bleeding around joints.
  • Ankle and knee joints are often affected.
  • It is important to maintain a healthy weight to address this complication because the heavier an individual, the more pressure and strain are put on the joints.

10. Hemophilia management requires comprehensive understanding about the disease.

  • This is achieved through health education (information dissemination) amongst affected individual.
  • People with Hemophilia assume great responsibility in managing their own care by learning the basic concepts of the disease.
  • The management of Hemophilia is focused on the quick treatment of bleeding episode to avoid serious side effects.
  • Self administration of factor concentrates can be learned by families or the patient itself to do it at home.
  • Increasing awareness about the disease may help the affected individual improve their quality of life.

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