Hemophilia

Hemophilia is a group of hereditary blood ailments where blood does not properly clot. The bleeding disorders are due to defects in the blood vessels, coagulation mechanism or the blood platelets. An individual is likely to bleed spontaneously or for a longer period than a healthy individual after surgery or injury.

What are the indications of hemophilia?

The symptoms of hemophilia vary depending on the degree of blood clotting factor deficiency and nature of any injury.

The 3 levels of hemophilia are based on the level of clotting factors present in the blood such as:

  • Mild hemophilia – above 5%
  • Moderate hemophilia – 1-5%
  • Severe hemophilia – less than 1%
    Hemophilia

    The symptoms of hemophilia vary depending on the degree of blood clotting factor deficiency and nature of any injury.

Mild

There are no symptoms until an occasion occurs that damages the skin or underlying tissue such as surgery or a dental process and results to prolonged bleeding.

Moderate

A child easily bruises and might also have internal bleeding symptoms especially around the joints and after a fall or sustaining a blow. The bleeding that occurs within a joint is referred to as joint bleed.

Severe

The symptoms strikingly resemble the moderate form but occur frequently and more severe. A child often bleeds for no evident reason which is called as spontaneous bleeding.

In most cases, children around 18 months of age end up with bleeding in the nose or mouth or have apparent bruising especially on the legs. The symptoms that might arise include:

  • Several deep or large-sized bruises
  • Blood in the urine or stools
  • Joint pain or swelling
  • Unexplained bruising or bleeding
  • Unexplained nosebleeds and gum bleeding
  • Joint tightness

Management

In the past, many patients with hemophilia died prematurely. Unfortunately, most of the deaths were due to childhood surgery or injury. At the present, most cases are expected to live long and active lives.

A big breakthrough in treatment is when the coagulation factor deficiencies associated to hemophilia could be determined and replaced by products derived from human blood.

In the previous years, plasma or whole blood infusions were started to regulate the episodes of bleeding. With this approach, bleeding was only partly treated. Cryoprecipitate which is created via cold precipitation of the frozen plasm was the initial effective treatment. The freeze-dried concentrates are produced from human plasma that contain the right amounts of Factors VIII and IX become available.

More Information / Disclaimer

The information posted on this page on hemophilia is for learning purposes only. Learn to recognize the indications of blood disorders by taking a standard first aid course with Kelowna First Aid.

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