World Hemophilia Day 2015

Date: April 18, 2015

Bleeding Disorders Prophylaxis Hemophilia von Willebrand disease (VWD)

World Hemophilia Day 2015 : Building a family of support

 

 The World Federation of Hemophilia (WFH) is providing global leadership to improve and sustain care for people with inherited bleeding disorders, including hemophilia, von Willebrand disease, rare factor deficiencies, and inherited platelet disorders.

 World Hemophilia Day provides an opportunity to talk to your extended family and friends, colleagues, and caregivers to raise awareness and increase support for those living with an inherited bleeding disorder.

Hemophilia day was established in 1989 with the support of the World Federation of Hemophilia (WFH). The date of 17 April was chosen to honor Frank Schnabel the founder of WFH, whose birthday falls on the same date.

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Bleeding Disorders:- 

In people with bleeding disorders, the clotting process doesn’t work properly. As a result, people with bleeding disorders can bleed for longer than normal, and some may experience spontaneous bleeding into joints, muscles, or other parts of their bodies.

i- von Willebrand disease (VWD)- The most common bleeding disorder is von Willebrand disease (VWD). It is generally less severe than other bleeding disorders. Many people with VWD may not know that they have the disorder because their bleeding symptoms are very mild.

ii- Rare clotting factor deficiencies- These are disorders in which one of several clotting factors is missing or not working properly. Less is known about these disorders because they are diagnosed so rarely. In fact, many have only been discovered in the last 40 years.

iii- Inherited Platelet Disorders- These are conditions in which platelets don’t work the way they should, resulting in a tendency to bleed or bruise.

iv- Hemophilia - Hemophilia is a bleeding disorder that affects approximately 1 in 10,000 people. People with hemophilia do not have enough clotting factor VIII or IX in their blood. As a result, they can bleed for longer than normal. It is         the oldest known genetic bleeding disorder and is caused by a deficiency in the genes and impairs the body’s ability to control blood clotting, which is used to stop bleeding when a blood vessel is broken.

Types of hemophilia

There are two types of hemophilia and each type is linked to a low level of a certain clotting factor.

  • Hemophilia A: It is the more common type and is linked to low levels of clotting factor VIII (8). The disease is present in about 1 in 5000 to 10000 male births.
  • Hemophilia B: It is rarer and is associated with low levels of clotting factor IX (9). The disease occurs in around 1 in about 20000–34000 male births.

Causes of Hemophilia 

People are born with hemophilia. However sometimes hemophilia can occur when there is no family history of it. This is called sporadic hemophilia. About 30% of people with hemophilia did not get it through their parent’s genes. It was caused by a change in the person’s own genes.

The hemophilia gene is passed down from parent to a child. The genes for hemophilia A and B are on the X chromosome. For this reason, hemophilia is called an X-linked (or sex-linked) disorder. The figure below explains how the hemophilia gene is inherited.

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Prophylaxis
Prophylaxis is the regular infusion of clotting factor concentrates in order to prevent bleeding. The idea of prophylaxis came from the observation that people with moderate or mild hemophilia (who have clotting factor levels of 1% or more) rarely experience spontaneous bleeding. They also have less joint damage than people who have severe hemophilia.