What about other bleeding disorders?
Most other bleeding disorders affect boys and girls equally and are one of two types:
Clotting factor deficiencies
Platelet disorders
Clotting factor deficiencies occur when there is a lack of one of the clotting factors in the blood that work to stop bleeding. When one of these factors is missing it is like having a line of 13 dominos with one of them taken away. When you try to make them all fall down by knocking over the first one, the process stops where there is a gap for the missing domino. The clotting factors are each given a number which is a Roman numeral. The important ones to know are:
I -- one
II -- two
V -- five
VII -- seven
X -- ten
XI -- eleven
XIII -- thirteen
With haemophilia A there is a lack of factor VIII (eight), with haemophilia B there is a lack of factor IX (nine) and with von Willebrand's disease there is a von Willebrand's factor.
There are also a number of related bleeding disorders where there are other missing clotting factors. These are much rarer than haemophilia. The effects will depend, like haemophilia, on how much clotting factor is missing, and so range from 'mild' (where there is still a fair amount of clotting factor) through to 'severe' (where there is hardly any or none). Even if the bleeding disorder is mild, after surgery, giving birth to a baby, or a major injury someone may bleed for longer than someone without a bleeding disorder. If the bleeding disorder is severe, people can also get bleeding into their joints, muscles and soft tissues, which can be very painful.
Factor I (fibrinogen) deficiency: Joint bleeds are uncommon, but there is more likelihood of oozing from small wounds than in haemophilia. Easy bruising will occur.
Factor II (prothrombin) deficiency: This is the rarest of this list. Nosebleeds, heavy periods, and easy bruising are features of this condition. Joint bleeds are rare.
Factor V deficiency: Easy bruising, nosebleeds, heavy periods, and joint and muscle bleeds are experienced by people with severe factor V deficiency.
Factor VII deficiency: Effects are usually mild but include nosebleeds, heavy periods, and bruising. Joint bleeds are rare.
Factor X deficiency: Bruising, joint bleeds, and heavy periods occur in people with this deficiency.
Factor XI deficiency: Sometime known as haemophilia C and is actually slightly more common than haemophilia B, especially in certain racial groups, although it usually not as severe as haemophilia B. Nosebleeds and heavy periods can occur. Over 1500 people are affected by this, but only around 50 need treatment for it in any year.
Factor XIII deficiency: In this very rare condition clots can form in the normal time but they tend to break down later. This causes a delay in healing after injury or surgery. Without treatment it is hard for a baby with factor XIII deficiency to survive in the womb.
Platelet disorders
Platelets are small disc-shaped fragments of cells which circulate in your blood. Whenever a blood vessel is punctured, they clump together to form a clot. When there isn't enough of them or they don't stick together properly, there is a platelet disorder.
Over 1000 people in the UK have a platelet disorder. LIke most of the other bleeding disorders, they are inherited from either or both of your parents. Three of the more common ones are:
Glanzmann's Thrombasthenia is where the platelets don't stick together properly. It can be mild, moderate or severe. Bruising, nosebleeds and mouth bleeds can occur and can be severe. Women and girls can have heavy periods.
Bernard-Soulier Syndrome is where platelets cannot stick very well to the walls of injured blood vessels. Nose and mouth bleeds, bruising, bleeding after trauma and heavy periods in girls and women can occur.
Platelet storage pool deficiency is where the platelets aren't very good at releasing chemicals to round up other platelets to form a "platelet plug" which is how a clot is formed.
For more information on these and other bleeding disorders, there are some detailed fact sheets available on the Canadian Hemophilia Society's web-site.
Adapted from ‘Living with Haemophilia’ by Dr Peter Jones. Published by Oxford University Press. Re-produced with kind permission of the author and publisher.
