What is Christmas illness?

Hemophilia B is sometimes referred to as Christmas illness, because a young boy named Stephen Christmas was the first patient to be recognized that he had this form of haemophilia. There are several types of haemophilia and all are diseases that cause blood clotting problems. Christmas disease is characterized by insufficient amount of plasma protein from the blood, called the IX factor. It is a hereditary state caused by a recessive gene on X chromosome; Women who have another X chromosome, which are likely to prevent an expressive recessive gene gene, can therefore be carriers of the disease and show no symptoms. Hemophilia B is much more common in men who do not have the second X chromosome to protect them from the recessive gene that causes it.

women who are wearers of a recessive gene that causes Christmas diseases have a 50 percent chance of handing over the gene for both their sons anddaughters, because they contribute one chromosome X to their children. Boys who inherit the gene from their mothers will always have a disease because the Y chromosome does not prevent the gene in expression. The boy cannot inherit the illness from his father because he receives chromosome y only. The girls inherit chromosome X from their mothers and their fathers, so they only develop the disease if they inherit the gene from both parents, and are simply carriers if they inherit the gene from only one parent. Women with haemophilia B will always pass on the gene to their children, because both their x chromosomes have a gene and men with haemophilia B will always pass on the gene to their daughters because their only x chromosome carries gene.

The severity of Christmas disease usually depends on how much IX factor is present in the blood. If the IX factor levels are extremely low, patients may experience spontaneous bleeding of an apparent reason. Patients whose levels are slightly to medium low may bleed too much after injury or surgery. Symptoms of severe haemophiliaEqually easier to detect and usually include heavy bruises, swollen or painful joints, bloody urine or stools, noseblees and prolonged bleeding after trauma or injury. People with mild haemophilia do not have to show symptoms until dental procedure or surgery that causes rich bleeding.

Christmas disease is treated by replenishing the patient's blood concentrates IX. These concentrates can be administered at home on the basis of need whenever the patient begins to bleed or can prescribe them to a doctor and dentist before certain procedures to prevent severe bleeding. People who have severe haemophilia B may have to be treated with IX factor regularly as a preventive measure.

with treatment people can generally lead to relatively normal lives by Christmas disease. However, there is some risk of health complications caused by excessive bleeding, including joint damage and intracerebral bleeding. Other risks associated with Christmas diseases are withspoken hepatitis B due to frequent exposure to blood products, developing thrombosis after treatment and development of IX factor inhibitors that can cause inefficient treatment.