2. Haemophilia
Defenition: It is a group of hereditary genetic
disorders that impair the body's ability to
control blood clotting or coagulation, which is used to stop
bleeding when a blood vessel is broken. So if you have
hemophilia, you may bleed for a longer time than others
after an injury.
5. What Causes Hemophilia?
It's caused by a defect in one of the genes that
determine how the body makes blood clotting factor
VIII or IX. These genes are located on the X
chromosomes. Chromosomes come in pairs.
Females have two X chromosomes, while males have
one X and one Y chromosome. Only the X
chromosome carries the genes related to clotting
factors. A male who has a faulty hemophilia gene on
his X chromosome will have hemophilia.
6. A female must have the faulty gene on
both of her X chromosomes to have
hemophilia, which is very rare. If a female
has the faulty gene on only one of her X
chromosomes, she is a "hemophilia
carrier.” Carriers don't have hemophilia,
but they can pass the faulty gene to their
children. Below are two examples of how
the hemophilia gene is inherited.
7.
8.
9. Treatment
The main treatment for hemophilia is called
replacement therapy. Concentrates of clotting factor
VIII (for hemophilia A) or clotting factor IX (for
hemophilia B) are slowly dripped or injected into a
vein. These infusions help replace the clotting factor
that's missing or low.
Clotting factor concentrates can be made from
human blood. The blood is treated to prevent the
spread of diseases, such as hepatitis. With the
current methods of screening and treating donated
blood, the risk of getting an infectious disease from
human clotting factors is very small.
10. Other Types of Treatments
A-Antifibrinolytic Medicines
Antifibrinolytic medicines (including tranexamic acid
and aminocaproic acid) may be used with replacement
therapy. They're usually given as a pill, and they help
keep blood clots from breaking down.
These medicines most often are used before dental
work or to treat bleeding from the mouth or nose
B-Desmopressin
Desmopressin (DDAVP) is a man-made hormone used
to treat people who have Haemophilia. DDAVP
stimulates the release of stored factor VIII. DDAVP
usually is given by injection or as nasal spray.