Our clotting ability is heavily dependent on the X chromosome. Men have one X chromosome while women have two X chromosomes. This means that if a man has an abnormality in one of the clotting factors, that is factor VIII for Haemophilia A factor IX for hemophilia B or factor XI for hemophilia C, they will be born with this condition. For Women, it’s rare for them to have hemophilia as they have a backup X chromosome. This however makes them carriers of the disease and increases the chances of them passing it down to their sons.
1 in 10,000 people in Kenya live with this condition and only about 15% of those are properly diagnosed and on a treatment plan. This clearly shows the gap that is there in our healthcare system in terms of Haemophilia. Most clinicians miss the diagnosis due to incomplete history taking, with a little more probing, the bleeding tendencies are evident. Replacement therapy is used in acute bleeds where the missing factor is introduced to about 50% of normal levels.
The most common signs of hemophilia include:
- Bleeding into Joints
- Post circumcision hemorrhage
- Bleeding into soft tissue and easy bruising
- Hemophilic pseudotumors from repeated hemorrhages
- Spontaneous intracerebral hemorrhages
There are also a few misconceptions surrounding the condition which are as follows:
“Women who give birth to hemophiliac sons are bad omens” Lack of understanding of how the condition is inherited has seen so many families break up and children suffer from no medical attention.
“Men who take long to heal post circumcision are weak” Some men do not survive circumcision due to the risk of hemorrhage and some even bleed to death. It is recommended to circumcise hemophiliacs when they are young as healing will be faster.
“Only boys get hemophilia” Sometimes girls who are silent carriers also manifest the signs of hemophilia.
“All forms of hemophilia are due to deficiency in factor VIII” Wrong! There are three categories, Haemophilia A is due to low levels of factor VIII, Haemophilia B, Factor IX, and hemophilia C due to factor XI.
This April 17th in line with last year’s theme, let’s get involved in creating awareness of the facts of this condition.