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World Haemophilia Day

World Hemophilia Day is an international observance held annually on April 17 by the WFH. It is an awareness day for haemophilia and other bleeding disorders, It was started in 1989 April 17 was chosen in honour of birthday of Frank Schnabel, businessman from Montreal, Canada suffering from severe Haemophilia A.

What is Haemophilia? Haemophilia is a congenital bleeding disorder mainly affecting men only. Very rarely women are affected. It is of mainly two types: Haemophilia A and Haemophilia B

What causes Haemophilia ? Haemophilia is a congenital bleeding disorder of deficiency or absence of  proteins ,clotting factors FVIII and FIX in type A and B respectively. This disease is a genetic defect transmitted from mother to male baby. Females are the carriers but do not suffer.

What are the symptoms of Haemophilia?  Excessive Bleeding in newborn is from umbilical cord, after circumcision. In childhood and adults its mainly in the joints- ankle, knee, hip elbow etc. It can be an internal bleeding in brain, intestines, kidney, Most of the time bleeding is with minor trauma or spontaneous! Excessive bleeding after minor surgery like tooth extraction is common.

How is it diagnosed? If history of bleeding in individual and in his family, the patient should approach to the doctor who will advice  blood tests like finding levels of Factor VIII and Factor IX in the blood. These tests are done only in specialised laboratory.

What are the complications? Acute Internal excessive bleeding if not treated adequately in time

can cause death. Chronic recurrent bleeding in target joint causes joint damage –Haemophilic Arthropathy, making  life crippling ! Antibodies formed to clotting factors FVIII and FIX complicates stopping of bleeding.

What is the treatment? Treatment is replacing the deficient factor adequately and timely. In case of Haemophilia A Factor VIII and in case of Haemophilia B , Factor IX is given by injection in to the vein. There is no cure for this disease. The treatment involves multi-specialty doctors.

How the disease can be avoided? This disease is transmitted through the gens. It is possible to detect Haemophilia in the foetus during pregnancy.If the foetus is affected choice is given to couple if they wish to continue pregnancy.

Are there any other bleeding disorders? Yes, there are other disease like von Willibrand  disease or due to deficiency of other clotting factors or deficient platelets.( cells responsible for blood clotting)

How can I get more information about this disease? You can approach your doctor  or blood disease specialist – Haematologist or  local Haemophilia Society. International organisations like World Federation of Haemophilia are always giving  helping hand.

*Dr. Datta Dharmadhikari is a Consultant Haematologist at Bokamoso Private Hospital.

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