An introduction to hemophilia
website by Helen for SNC 2DW - 2016
Paper cuts may be annoying for the average person, but for someone with hemophilia, they can be life-threatening.
Hemophilia, sometimes written as haemophilia-- the two names describe the same disorder--, is a rare blood disorder that affects approximately 1 in 5 000 males (more on why it doesn’t affect nearly as many females in causes and symptoms). This is caused by insufficient clotting factors in the blood; without enough clotting factors, cuts and bruises will not stop bleeding and may cause people to bleed out.
Even if small cuts can be life-threatening for hemophiliacs, the real problem is internal bleeding within deeper tissues. Pressure can be applied to smaller cuts, so that the platelets can stop the bleeding in time; when blood vessels burst inside the body, treatment is a bit more difficult.
Hemophilia, sometimes written as haemophilia-- the two names describe the same disorder--, is a rare blood disorder that affects approximately 1 in 5 000 males (more on why it doesn’t affect nearly as many females in causes and symptoms). This is caused by insufficient clotting factors in the blood; without enough clotting factors, cuts and bruises will not stop bleeding and may cause people to bleed out.
Even if small cuts can be life-threatening for hemophiliacs, the real problem is internal bleeding within deeper tissues. Pressure can be applied to smaller cuts, so that the platelets can stop the bleeding in time; when blood vessels burst inside the body, treatment is a bit more difficult.
An image of a blood clot: fibrin sticks to the red blood cells and creates clumps of blood
Types of Hemophilia
There are 2 main types of hemophilia: Type A→ lack/deficiency of clotting factor VIII Type B→ lack/deficiency of clotting factor IX If someone has less than 30% of clotting factors VIII or IX, they are considered to have mild hemophilia As well, there are less common types: Hemophilia C→ lack/deficiency of clotting factor XI (this form of hemophilia occurs equally in both genders and is most common in Ashkenazi Jews) Parahemophilia→ a rare, mild form of hemophilia resulting from a deficiency of clotting factor V Acquired Hemophilia→ this is an autoimmune disease in which the body mistakenly produces antibodies that target and destroy clotting factors, usually factor VIIII |
the bloodstream of an average person contains enough clotting factors to clot blood when needed
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Von Willebrand Disease is a more common bleeding disorder that is not to be mistaken for hemophilia; although both ailments result in slow/no clotting, their causes are different. VWD is caused by a lack of the Von Willebrand factor, a clotting factor responsible for preventing bleeding in skin and mucous membranes; bleeding in epithelial tissues --such as the nose and intestine-- is more of a problem for those with VWD than bleeding in deep tissues (a problem for those with hemophilia). VWD is more common than hemophilia; about 1% of the US population are believed to have it, the most common being a mild subtype of VWD (type 1). This bleeding disorder occurs equally between genders, though women have additional complications due to heavier blood loss during menses and childbirth.
Other types of clotting factors exist, and a deficiency in any will cause similar bleeding disorders, such as Congenital factor X deficiency. The prefix hemo means “blood” and philia means “love”; interestingly enough, hemophilia does not mean “bloodlust”. |
Questions about hemophilia?
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