Haemophilia

What is Haemophilia?

 

Haemophilia is an inherited haemorrhagic blood disorder. The defect lies in the clotting mechanism of the blood and is characterised by a life long tendency to excessive internal or external haemorrhage.

 

This blood disorder can be classified into three kinds, Haemophilia ‘A’ is due to the deficiency of factor VIII. Haemophilia ‘B’ also known as Christmas disease is due to the deficiency of factor IX. The third kind ‘C’ is due to the deficiency of factor XI. One more related disease called Von Willie Brand’s is due to the deficiency of vwantigen deficiency.



Symptoms:

 

Patients with Haemophilia very often have internal or external bleeding, both spontaneously or on trauma i.e. 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 bleedings can vary in frequency from three times a week to three or more times a year.



Consequences:

 

If Haemophilic patients remain untreated, the result would be prolonged excessive bleeding which may prove fatal or cause severe anaemia. Bleeding in joints causes severe deformities of joints, flexion/extension deformities, weakness of legs, shortening of leg and so on, which makes a patient crippled. Bleeding in the brain and abdomen are very dangerous and may cause death of such haemophilic.



Treatment:

 

  • i) Both Haemophilia "A" and Haemophilia "B" are treated by replacing the missing clotting factor, the commonest source is human blood plasma. Haemophiliacs are also treated with synthetic factor VIII, which has been produced by using a technique called Recombinant technology.
  • ii) Once an haemophiliac patient gets bleeding in joints and as soon as bleeding stops into the joints by factor replacement therapy, patients are recommended for Physical Therapy and Rehabilitation to make the joint less deformed. Physiotherapy plays an important part in patient’s treatment and every effort is made to enable him to return to his routine work.

 

 

Cost of treatment:

 

Cost of treatment varies with degree of deficiency from mild to severe.

 

In Pakistan the cost of one injection of Factor VIII in the open market is approximately the equivalent USD 80/-. If a severely affected patient needs 12-16 injections per month, the cost of treatment would be approximately USD 11,500 ~ USD 15,500/- per year.

 

All Centres of Fatimid Foundation are equipped to make various blood components itself, which includes cryopriopitate (factor I & VIII),  cryosupenatent (factor IX and other factors) , platelet concentrate and therefore are able to provide the above cost out of its own fund raising resources. Fatimid Foundation shoulders the responsibility of treating over 3100 registered underprivileged haemophiliac patients free of charge and therefore requires substantial government support to subsidise this cost.