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Haemophilia: Causes, Symptoms, Treatment and Prognosis

Usually a wound stops bleeding within 10 minutes unless it’s very serious. In haemophilia,
an inherited lack of blood clotting variables as a result of unusual, genes, even a little
cut may bleed for hours or days, and there perhaps episodes of spontaneous bleeding.
The considerably more infrequent Christmas disease has similar characteristics and is named after the man in whom the disorder was first diagnosed. Both conditions affect only males.

What Are The Causes of Haemophilia?

Haemophilia and Christmas disease are both because of lack of a protein involved in blood clotting. In haemophilia, the deficient protein is Factor VIII; in Christmas disease, the protein is Factor IX. In both these states, the lack is caused by a defective gene. The specific gene called for is distinct in both illnesses.

In both disorders, the abnormal gene is situated on an X chromosome (X- or sex linked inheritance). Girls don’t develop the disorder because they have two X chromosomes and the normal gene on the other X chromosome compensates for the unusual gene. Guys with the unusual gene do develop the disease because they’ve just one X chromosome (the other is a Y chromosome) and so don’t have a regular copy of the gene to compensate for the unusual one. Girls, nevertheless, may pass on the defective gene to their kids. Each kid, male or female, has a 1 in 2 chance of inheriting the defective gene.

In one third of all instances, the states are due to a spontaneous gene abnormality and there’s absolutely no family history of haemophilia or Christmas disease.

What Are The Symptoms of Haemophilia?

The symptoms are highly variable, and their severity depends upon how much Factor VIII or IX is really created. The symptoms generally develop in infancy and contain:
• easy bruising, even after mild harm
• abrupt, painful swelling of muscles and joints as a result of internal bleeding
• prolonged bleeding after an injury or a minor surgical operation
• blood in the urine.
Without treatment, prolonged episodes of bleeding into the joints may bring about long term damage and, eventually, may result in deformity of the joints.

What are the Measures of Haemophilia?

The physician will arrange for evaluations to see the length of time your blood takes to clot and to quantify the amount of Factor VIII or IX. The objective of treatment will be to keep the clotting factors at a high enough level to prevent bleeding. If you’ve got a serious type of either illness, you’ll likely need routine intravenous injections of Factor VIII or IX to increase the amounts of these variables in the blood.

If you’ve got a moderate form of either illness, you may want shots just after an injury or before operation. You may also be prescribed desmopressin, which includes pituitary hormone, to foster levels of Factor VIII. Many people develop antibodies to Factor VIII nutritional supplements, making treatment difficult. These individuals may must take an immunosuppressant drug to ruin the antibodies.

What’s The Prognosis of Haemophilia?

If you’ve got haemophilia or Christmas disease, you can lead an active life but need to prevent sustaining any injuries. Actions like swimming, running and walking are valuable, but contact sports, for example wrestling and football, should be prevented. Routine dental care is essential to prevent the danger of bleeding from inflamed gums.
If you’ve got a family history of either illness, you should get medical advice when planning a pregnancy.

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