World Haemophilia Day: 19,000 People In India Suffer From Serious Blood Disorder
On April 17th, World Haemophilia Day, we explain the medical condition and the inheritance factor—and also look at data associated with it.
Haemophilia is an inherited bleeding disorder in which the ability of the blood to clot is severely reduced, causing the sufferer to bleed severely from even a slight injury.
Blood contains many proteins called clotting factors that control bleeding. People with haemophilia have low levels of these clotting factors—either factor VIII or factor IX.
The inheritance factor
Haemophilia is an inherited condition and occurs in families; however, in one-third of the cases it appears in families with no previous history of the disorder.
The sex of an individual is determined by a pair of ‘sex chromosomes’. Males are identified with one X and one Y chromosome (XY) and females with two X chromosomes (XX). Males inherit the X chromosome from their mothers and the Y chromosome from their fathers. Females inherit one X chromosome from each parent.
Haemophilia is caused by a defect in the X chromosome.
Men with haemophilia will pass the gene on to their daughters but not their sons. Women who carry the haemophilia gene can pass it on to their sons and daughters.
A female with one affected X chromosome is a “carrier” of haemophilia and won’t suffer from the condition herself. But she’ll suffer from haemophilia only if she has both X chromosomes. However, a boy born with a defective X chromosome will suffer from haemophilia.
According to the Annual Global Survey 2017, released in October 2018, there were 1.96 lakh people living with haemophilia across the world in 2017. There were 18,996 registered cases of haemophilia in India alone.
Haemophilia A and B are the two most common types of haemophilia and a total of 1,58,225 and 31,247 people suffer from it respectively. A total of 7,234 people reported bleeding , which could be other unknown types of haemophilia.
A rare worldwide disorder, Haemophilia A occurs in about 1 in 1,500 births, while Haemophilia B—which is even rarer—in about 1 in 20,000 births. A vast number of cases, however, are believed to be unreported, particularly in India.
Experts quoted by PTI estimated that nearly 80 per cent of Indians with the serious blood disorder are not diagnosed due to the absence of proper diagnostic facilities in the remote areas.
According to Haemophilia Foundation of India, the umbrella body for registration of the patient with the disorder, the cause of haemophilia is the inability of the body to produce the anti-haemophilic factor (AHF) in the required quantity.
There is no known cure for this disorder.
If not diagnosed early, the repeated bleeding into joints, bones muscles may lead to synovitis, arthritis and permanent joint deformities.
The bleeding itself can lead to wasting and the atrophy of muscles.
(Additional inputs by agency)