HaemophiliaDefenition: It is a group of hereditary geneticdisorders that impair the bodys ability tocontrol blood clotting or coagulation, which is used to stopbleeding when a blood vessel is broken. So if you havehemophilia, you may bleed for a longer time than othersafter an injury.
What Causes Hemophilia? Its caused by a defect in one of the genes thatdetermine how the body makes blood clotting factorVIII or IX. These genes are located on the Xchromosomes. Chromosomes come in pairs.Females have two X chromosomes, while males haveone X and one Y chromosome. Only the Xchromosome carries the genes related to clottingfactors. A male who has a faulty hemophilia gene onhis X chromosome will have hemophilia.
A female must have the faulty gene onboth of her X chromosomes to havehemophilia, which is very rare. If a femalehas the faulty gene on only one of her Xchromosomes, she is a "hemophiliacarrier.” Carriers dont have hemophilia,but they can pass the faulty gene to theirchildren. Below are two examples of howthe hemophilia gene is inherited.
TreatmentThe main treatment for hemophilia is calledreplacement therapy. Concentrates of clotting factorVIII (for hemophilia A) or clotting factor IX (forhemophilia B) are slowly dripped or injected into avein. These infusions help replace the clotting factorthats missing or low.Clotting factor concentrates can be made fromhuman blood. The blood is treated to prevent thespread of diseases, such as hepatitis. With thecurrent methods of screening and treating donatedblood, the risk of getting an infectious disease fromhuman clotting factors is very small.
Other Types of TreatmentsA-Antifibrinolytic MedicinesAntifibrinolytic medicines (including tranexamic acidand aminocaproic acid) may be used with replacementtherapy. Theyre usually given as a pill, and they helpkeep blood clots from breaking down.These medicines most often are used before dentalwork or to treat bleeding from the mouth or noseB-DesmopressinDesmopressin (DDAVP) is a man-made hormone usedto treat people who have Haemophilia. DDAVPstimulates the release of stored factor VIII. DDAVPusually is given by injection or as nasal spray.
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