Even as the city observed World Haemophilia Day on Thursday, the Karunya pharmacy in the district remained short of clotting factor concentrates.
Factors 8 and 9 are blood-clotting proteins needed to save the lives of haemophiliacs in case of an injury. In the absence of these coagulation factors, the patient may even bleed to death.
A patient who came from a neighbouring district to Ernakulam could not receive factor 8.
The patients are not given the factors to stock and have to approach the pharmacy every time there is an injury. If patients get the factors through taluk hospital outlets, it could considerably reduce the strain of travelling to just one outlet in the entire district, said a volunteer of Haemophilia Society.
The State had recently enhanced support for haemophilia patients by raising the free treatment limit from Rs. 2 lakh to Rs. 3 lakh.
The Karunya pharmacy at the General Hospital has been providing the factors free of charge to haemophiliacs.
But the long holiday after the polling day upset the supply chain at the pharmacy. The pharmacist told The Hindu that it was only a temporary phase and the supplies would soon be restored.
In the open market, the factor costs are high. A unit costs Rs. 12. For minor bleeds, a patient may require about 10-20 units per kg per dose, every 12 hours for two to three days.
Genetic disorderHence, a patient weighing 50 kg would require a minimum of 500 units per dose. Major bleeds would increase the requirement of units by more than five times.
Haemophilia is a genetic disorder that impairs the body’s ability to control blood clotting or coagulation. The severity of the disorder depends on the amount of clotting factor that is missing. The main danger is uncontrolled internal bleeding that starts spontaneously or results from an injury. Bleeding in joints and muscles can cause stiffness, pain, severe joint damage, disability, and sometimes death.
The disease occurs only in males while females serve as carriers. The disease is inherited from infected fathers to sons.
In 30 per cent of the cases, it could also be caused by mutation in gene Haemophilia A. It is the most common form of the disorder, present in one in 5,000-10,000 male births. Haemophilia B occurs in one in 20,000-34,000 male births.
The first Haemophilia Treatment Centre in the State at the district hospital in Aluva is striving to be a centre for excellence. Though the centre has facilities for treatment and physiotherapy, the factor availability needs to be more spread out.
‘Speak Out. Create Change’ is the year’s slogan for World Haemophilia Day, encouraging patient communities across the world to go on with dialogue and drive change.
