Hemophilia

April 17 is the World Hemophilia Day 

Hemophilia is a life-threatening disorder that affects the blood clotting mechanism of the body. It is a genetic condition carried by the X chromosome, which makes men more likely to suffer from it.

Living with hemophilia can be a challenge for people suffering from it. They need to be extra careful about getting hurt because even a minor injury can cause profuse bleeding and turn fatal. When a healthy individual suffers an injury or wound, a stream of platelets and blood clotting factors is directed to the site of injury which allows proper healing of the wound. But in people suffering from hemophilia the platelet levels or the blood clotting factors are extremely low. This causes the wound to bleed continuously resulting in complications.

But these complications can be well prevented with appropriate treatment at the right time. Our expert and renowned hematologist Dr Subhaprakash Sanyal discusses treatment options, advances in treatment of hemophilia and offers a few tips for hemophilia patients to prevent complications. 

What are the deciding factors for hemophilia treatment?

Dr Sanyal: Hemophilia is mainly of two types: Hemophilia A and B. Hemophilia A is caused by deficiency of blood clotting factor VIII and Hemophilia B is caused by deficiency of clotting factor IX. Hemophilia mainly causes muscle and joint bleeding and can be mild, moderate or severe. So, severity of bleeding is the main factor based on which the treatment is decided.

What treatment is required?

Dr Sanyal: Clotting factor replacement therapy or infusion of blood clotting factors is the main treatment option in both types of hemophilia. Concentrates of clotting factors VIII and IX are administered to the patient depending on the severity of bleeding. For example, if the patient suffers mild bleeding, say a nosebleed, then a 30% concentrate of clotting factor is given. For severe bleeding, say in the brain, about 80 -100% of concentrate may be given. When a hemophilia patient needs to undergo a surgery for some other medical condition, then a hematologist is consulted for the concentrate of clotting factors that needs to be transfused. But, the cost of a single dose of these clotting factors is quite high (about 3000-3500 rupees). Major bleeding episodes may also require drug treatment.

What are complications following the treatment?

Dr Sanyal: One of the major complications takes place when the body produces inhibitors to the clotting factor concentrates. These inhibitors are actually antibodies that cells of your immune system produce because they recognize the clotting factors as foreign and start destroying them by producing antibodies. The chances of complications are 30% in severe cases of hemophilia, 3% in moderate hemophilia and 0.3 % in mild hemophilia cases.

How are these complicated cases handled?

Dr Sanyal: We use factor VIII inhibitor bypass activity (FEIBA) and activated recombinant factor VII (rFVIIa) to manage complications of hemophilia treatment. FEIBA helps to control bleeding by inducing and facilitating the production of blood clotting protein called thrombin. As of now, these two coagulation factors are the only ways to manage of complications. Again, the cost involved is too high.

Are there any advanced treatment options for hemophilia?

Dr Sanyal: Not yet. But, there are a lot of studies examining the potential of gene therapy in the pipe line which look quite promising.

Can you give some tips for hemophiliacs to improve their quality of life?

Dr Sanyal: The first thing is that all patients should be well aware about the disease, including the severity and complications. Here are some suggestions that may help:

  • Hemophiliacs should not always consult a qualified hematologist rather than a general practictioner.
  • They avoid taking painkillers for minor problems unless prescribed
  • Do not take intramuscular injections because they can cause complication called hematoma
  • Patients should always consult a hematologist before undergoing even a minor surgery like tooth extraction
  • There’s a myth that hemophiliacs cannot live a proper life because performing any kind of surgery is difficult in them. The fact is, any kind of surgery can be done if proper correction factor is given at the right time.

Alternative treatment for hemophilia:

Dr Tapas Kundu, senior homoeopathic practitioner affiliated with the Other Song Homoeopathic Academy and Clinic Homoeopathic, discusses the role of homeopathic medicine as a supportive therapy in managing severe cases of hemophilia.

‘Unlike allopathy, homeopathic medicines can be administered in the non-bleeding phase because the medicine is not antagonistic to the modern management. It can also be used as an adjuvant therapy to the main mode of management and still shows significant results. We have treated a lot of patients using haemophilic mother tincture in cold water for external bleeding. In acute manifestation, we make the patient maintain a diary with a personal agenda, his interaction with family and the world, and provide treatment based on the output. This works the best, and in most cases, after one or two doses, the reduction is substantial. Sometimes, further medication is not even required.

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