Trinicenter.com - Haemophilia a rare, expensive disease

By Tim Chigodo, www.herald.co.zw

Haemophilia is a rare, yet expensive and painful disease. The pain and agony of living with this debilitating disease is a nasty experience.

Very few Zimbabweans understand what it takes being haemophiliac.

Chido, not his real name shouts and jumps with joy each time his team-mates score.

He heaves and sighs, laughs and giggles at every miss.

At 15, Chido has had to painfully watch from the touch-line his age-mates run, kick, throw and punch the football. He cannot join them.

He used to participate in sporting activities, until a small wound almost killed him.

Chido suffers from haemophilia — a hereditary disorder with a tendency to bleed severely.

It is caused by the failure of the blood to clot normally.

For Chido and many other haemophiliacs, simple cultural practices such as circumcision or the removal of teeth could prove fatal if not extremely expensive.

Haemophiliacs must avoid violent and vigorous sports. They must avoid all forms of accidents which may lead to fractures and heavy bleeding, a tall order in a country where road accidents have become as normal as breathing.

"Haemophilia, a blood disorder, affects mostly males world-wide," says head of the Haematology Department at the University of Zimbabwe School of Medicine, Dr Andrew Zolani Cakana.

There is one in every 20 000 people in Zimbabwe with haemopholia A and one in every 40 000 with haemopholia B. "The condition is worse in Third World countries because of ignorance of the disease by the victims," Dr Cakana said.

Haemophilia Day has been set aside for April 17 every year to highlight the plight of haemophiliacs and promote awareness of the disease among the people.

"We are doing everything to make Zimbabweans aware of this condition through the celebration of Haemophilia Day," the haemophilia expert said.

People suffering from the disease are encouraged to wear bracelets so that they can quickly be identified when they are involved in an accident in order to get urgent medical attention.

Unlike common cold or malaria, haemophilia is a rare disease. Most people go through life without knowing anyone with haemophilia.

Since the disease is a blood-clotting disorder which prevents blood from forming any effective clot, it is characterised by a life long tendency of excessive haemorrhage and a greatly prolonged coagulation time.

Haemophiliacs commonly suffer from haemorrhages into joints which causes severe pain, swelling, warmth and muscle spasm.

With appropriate treatment, however, the lesion settles within a few days as the blood is reabsorbed, but with recurrence, the joints are damaged, wasted, leading to deformity and crippling.

"Most haemophiliacs in Zimbabwe have severe haemophilia. Most with mild haemophilia do not attend hospital and some do not even know that they have it unless they have an operation or an accident," says a spokesman for the Haemophilia Association.

Medical experts agree that Zimbabweans are ignorant, about ailments that afflict their lives, among them haemophilia. Most of them are not informed about haemophilia.

The low index of suspicion and ignorance has led to early deaths of haemophiliacs.

Nevertheless, ignorance is not the only factor leading to early deaths of haemophiliacs.

The majority of haemophiliacs die before they attain the age of 15 because of late diagnosis and inadequate treatment, say the medical experts.

A consultant physician and haematologist who declined to be named, says that the disease is extremely expensive, not just in monetary terms, but also in the time spent on hospitalisation and treatment.

Parirenyatwa Hospital is the only Zimbabwean institution equipped to handle haemophiliacs forcing many victims of the disease to travel long distances for the treatment and management of this rare condition.

Many give up due to the financial and travel logistics involved. This could only mean certain death.

Mere circumcision which normally costs very little money, would cost haemophiliacs more because of the procedures involved and required for their treatment.

Fresh frozen plasma, which is an alternative treatment of factor VIII, is not cheap either and costs poor patients more than they can afford per unit, with four such units required daily for ten days.

The factor is never readily available and has to be ordered well in advance of the treatment since most hospitals rarely stock it.

Fortunately for most haemophiliacs, fresh frozen plasma is free and available at the hospital.

A factor VIII substitute — cryoprecipitate costs more for the patients.

Through the Zimbabwe Haemophilia Association, patients are able to get donations of some of the required factors.

Money and pain are not the only price one has to pay for being a haemophiliac. Family conflicts might arise especially where the woman is thought to be the main carrier and cause of the disease in the family.

Children may miss school for long periods while adults may be absent from duty due to haemophilia.

The disease even denies children a chance to play with others. They cannot indulge in sports and normal games with other children especially if this involves physical body contact.

Since the treatment of haemophiliacs, repeatedly exposes them to blood products, patients are in constant danger of contracting HIV/Aids, Hepatitis B and other forms of liver disease.

There have been cases, especially in Europe where haemophiliacs contracted HIV/Aids during blood transfusion.

There are few international haemophilia training centres in the developing world. Technological advances in the developed world give children with haemophilia a chance to live normally.

In the developed world, once diagnosed early, children are kept on preventive medicine, which is administered three times a week for the rest of their life. However, this is expensive for many sufferers especially in the developing world.

Most Zimbabwean haemophiliacs can seek support and assistance from the Zimbabwe Haemophilia Association based at the Blood Transfusion Services.

Reading literature and speaking with professionals, combined with experiences of the patients, will make them feel better.

Though the genetic affiliation of haemophilia makes experts powerless in stopping its recurrence, they can only ensure that haemophiliacs lead a normal life by creating awareness and fundraising for haemophiliacs.

They can only offer psychological and physical support to enable the Chidos of this world play with their age mates.