Call for awareness of hemophilia

As many as 10,000 children are suffering from hemophilia, including 3,000 in Karachi alone, but their number could be much higher as no data or national registry for such patients exists in Pakistan. Hemophilic patients can live a normal life if the government, like many other countries of the world, provides factors or medicines needed for the treatment of these patients.

“Hemophilia is a chronic blood disorder, which not only puts lives of children at risk but also causes severe psychiatric illnesses, including anxiety, depression and inferiority complex in them and increase their dependence on drugs. There is an urgent need to make people aware of this disease so that children suffering from it could be properly treated and could live a normal life,” eminent pediatric hematologist Dr Saqib Ansari said while speaking at an awareness seminar on Tuesday.

Hemophilia is a rare genetic disorder in which the blood of a patient does not clot normally. Several organisations, including Omair Sana Foundation and Fatimid Foundation, held awareness seminars on the occasion of World Hemophilia Day, which is observed globally on April 17 to raise awareness about the dreaded disease. Dr Saqib Ansari, who is also the general secretary of Omari Sana Foundation, said that in normal human beings, bleeding stops naturally in case of a minor injury as the blood starts clotting due to the presence of a specific protein, but in some children, due to the absence of that protein, bleeding does not stop and the patient can even die due to severe blood loss.

“Hemophilia can be divided into three types: type A is due to the deficiency of factor VIII, type B also known as Christmas disease is caused by the deficiency of factor IX, and the third kind ‘C’ is caused due to the deficiency of factor XI. One more related disease called Von Willie Brand’s is caused due to the deficiency of von Willebrand factor deficiency.”

According to Dr Ansari, children suffering from hemophilia requires injections of factors whose absence causes the disease, but as these injections are very costly and often are not available in the country, such patients are given FFP or frozen plasma but this transfusion often puts these children at the risk of getting viral hepatitis and even HIV/AIDS.

“Unfortunately, in many cases unscreened or poorly screened blood products are transfused to children, and due to that, many of children who are dependent on others’ blood or blood products contract viral hepatitis or HIV/Aids,” he deplored and urged the government to ensure the availability of the factors for the treatment of hemophilia patients.

Other hematologists and experts at an awareness seminar organised by Fatimid Foundation said patients with hemophilia faced internal or external bleeding, both spontaneously or due to trauma and they experienced gums bleeding, bleeding from nose, bleeding in large joints of the elbows, knees ankles and shoulder joints, bleeding in urine, bleeding in stool or even they may get bleeding in the brain.

The bleeding mostly occurs in the large joints, these internal bleeding can vary in frequency from three times a week to three or more times a year, they said and added that if such patients remained untreated, prolonged excessive bleeding may prove fatal or cause severe anaemia.

According to the experts, bleeding in joints causes severe deformities of joints, flexion, extension deformities, weakness of legs, shortening of leg, which makes a patient crippled. Bleeding in the brain and abdomen are very dangerous and may cause death of such hemophilic patients, they added.