What is hemophilia? 

The word hemophilia derives from two Greek words: haima, meaning blood, and philia, meaning affection. 

Hemophilia is an hereditary condition. This means that it is passed on from mother to child at  the time of birth. 

The  blood of a person with hemophilia  does not clot normally. He does not  bleed more profusely or more quickly than other people; however, he bleeds  for a longer time. 

Many people believe that hemophiliacs  bleed a lot from minor cuts. This  is a myth. External wounds  are usually not serious. Far more important is  internal bleeding (hemorrhaging). These hemorrhages are  in joints, especially  knees, ankles  and elbows; and into tissues  and muscles. When bleeding occurs in a vital organ,  especially the brain,  a hemophiliac's life is in danger. 

What are other names for hemophilia A? 

Hemophilia A is called by two other names: 

• Classical hemophilia,  because it is the most common  of the factor deficiencies  and
• Factor VIII deficiency hemophilia, because it is the lack  of the factor 8 (written factor VIII)  proteins in the blood that causes the clotting problem. 

What are other names for hemophilia B? 

Hemophilia B also goes by two other names: 

• Christmas Disease, named after Steven Christmas,  a Canadian who in 1952 was  the first person  to be diagnosed with this distinct  form of hemophilia and 
• Factor IX  deficiency hemophilia, because factor 9 (written  factor IX) is the blood protein which  is lacking and whose absence slows down the normal  clotting process. 

How  common is hemophilia? 

Both hemophilia  A and B are very rare disorders.  Hemophilia A affects fewer than 1  in 10,000 people,  or about 2500 Canadians. Hemophilia  B is even less common, affecting approximately 1 in 50,000 people,  or about 600 Canadians. 

Who is affected by hemophilia? 

Hemophilia affects people of all races, colours and ethnic origins. 

The most severe forms of hemophilia affect almost only males. Females  can be seriously affected only if the father is  a hemophiliac and the mother is  a carrier, or in the case  of X-inactivation when  a woman's normal X-chromosome is inactive in the production of factor VIII  or IX. These cases are extremely rare. 

However, many women  who are carriers have symptoms  of mild hemophilia. We  are only now fully recognizing  the importance of  bleeding in carriers and the degree  to which these symptoms affect a woman's quality  of life. 

As hemophilia is  a hereditary disorder,  people are  affected at birth. This means that children  can have hemophilia. In fact,  hemophilia is often  diagnosed in the first year of life. 

How serious is hemophilia? 

Without proper treatment, hemophilia is crippling and often fatal. With modern treatment, most people with hemophilia can lead full, active lives. 

Hemophilia is classified as severe, moderate  or mild. 

CLASSIFICATION:             LEVEL OF FACTOR VIII OR IX IN THE BLOOD 

Severe                                    Less than 1% of normal
Moderate                                1 to 5%  of normal 
Mild                                         5  to 30%  of normal

Severe hemophiliacs  with less than 1%  of the normal level of factor VIII or IX in the  blood have hemorrhages several times a month. The bleeding is often  the result of a minor bump or twist. Sometimes, there is often no apparent cause  for the bleeding. 

Moderate hemophiliacs bleed less often. Their hemorrhages  are often  the result of minor trauma, such as a sports injury. 

Mild hemophiliacs have even fewer hemorrhages. They may be aware  of their bleeding problem only in  the case of surgery, a tooth extraction or a serious injury. Women  with mild hemophilia may bleed more during menstruation (periods).