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Living with haemophilia

Danuska Da Gama

Haemophilia is usually an inherited genetic disorder that impairs the body’s ability to make blood clots, a process needed to stop bleeding. This results in people bleeding longer after an injury, easy bruising, and an increased risk of bleeding inside joints or the brain. In Goa itself, there are close to 300 cases of haemophilia according to the Goa Haemophilia Society, most of which are mild cases.

People with haemophilia do not have enough clotting factor VIII or IX in their blood. As a result, people with bleeding disorders can bleed for longer than normal, and some may experience spontaneous bleeding into joints, muscles, or other parts of their bodies. While it is a genetic disorder, it can occur even when there is no family history of haemophilia.

Most bleeding in haemophilia occurs internally, into the muscles or joints. The most common muscle bleeds occur in the muscles of the upper arm and forearm, the iliopsoas muscle (the front of the groin area), the thigh, and the calf. The joints that are most often affected are the knee, ankle, and elbow. If bleeding occurs many times into the same joint, the joint can become damaged and painful.

Repeated bleeding can also cause other health problems like arthritis. This can make it difficult to walk or do simple activities. However, the joints of the hands are not usually affected in hemophilia (unlike some kinds of arthritis).

Diagnosis is by testing the blood for its ability to clot and its levels of clotting factors. However, the symptoms can be watched out for. Paediatrician and paediatric intensivist at Healthway Hospital, Sumant Prabhudesai says that repeated episodes of bleeding due to cuts and in the oral cavity can be a sign of haemophilia.

“In severe cases, when bleeding occurs in the joints and muscles it can lead to restricted movement of joints and muscle bruising. These can be painful and restrictive. In children, such a scenario can limit their range of activity,” he explains.

Goa Haemophilia Society is a support group for patients of haemophilia in Goa which meets regularly with patients and their families. Started a decade ago, the society presently has 56 registered members. The support group advises and offers information to patients and their families.

Paediatrician and founder member of Goa Haemophilia Society, Dhanesh Volvoikar states that haemophilia usually affects males. But, affected females are carriers of the genes and can transmit this condition to next generation.

“The ailment is mostly identified in a child who bruises easily while playing, or if a child is inflicted with a cut, bleeding doesn’t stop easily,” he says. He goes on to explain that an affected person often only becomes aware of the condition only at an older age during dental procedures or any other minor surgery when the bleeding refuses to subside.

“In severe cases, internal bleeding around joints and in muscle tissue can lead to immobility of the joints which may even render the patient wheelchair bound,” says Volvoikar, referring to a few such cases in Goa.

Haemophilia is a disease like any other disease and can happen to anybody. It is not the end. The good news is that haemophilia can be managed effectively with care and precaution. Treatment is now easily available at Goa Medical College as well as private hospitals. It is also beneficial that those diagnosed with haemophilia register with Goa Haemophilia Society as support to patients and their families is offered. The ailment can thus be managed well and individuals can lead near-normal lives.

“In the immediate future, we are looking at a preventive kind of treatment where patients will be administered regular doses of prophylactic replacement of defective factor which is required for clotting of blood every month so that haemophiliacs can lead a perfectly normal life with unrestricted activity,” says Volvoikar.

 

Symptoms and precautions

l Frequent swelling and bruising of the skin, and bleeding in children needs to be clinically evaluated and checked by a doctor. It’s best not to ignore it

l It is necessary for patients of haemophilia to avoid injuries which can lead to cuts and bruises.

l Prior to a dental treatment, individuals with haemophilia need to take appropriate clinical treatment and precaution to ensure that the dental procedures are carried out smoothly.

l Unexplained swelling and bruising of the skin without any kind of trauma needs to be clinically investigated by a doctor.

l For children with haemophilia its best to avoid contact sports of any kind. Instead, swimming and cycling, with appropriate gear, are good forms of activity for children and adults with the ailment.

l Regular physiotherapy is crucial to ensure good joint health and keep the joints moving. Light exercises and yoga are beneficial.

l Obesity in children and adults is a concern as it puts pressure on the joints. Hence it’s important to keep the weight in check.

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