Moses Murekate’s health deteriorates whenever he gets a simple infection or accident. Playing without the watchful eyes of his parents is nearly impossible to the 3-year-old.

His health situation is different and causes him to bleed profusely after wounding because of a rare blood disorder, an anomaly his parents realised two years ago when he slipped off the veranda, only to injure his head but bled from his nose.

"The bleeding was intense and I thought he had a concussion to his head,” recalls Florence Ngirimana, Murekate’s mother.

After the fall, Murekate’s shirt was soaked in blood, so unlike the seemingly minor slip as the repercussion looked grave.

Since then, his parents had to accept the fact that their boy was different from the rest, calling for different approaches.

"We take regular checkups and we don’t take chances with any sort of infection, be it flu, cough or fever,” she adds

Haemophilia is one of the most complicated diseases to manage.

What is haemophilia?

Congenital tendency to uncontrolled bleeding; usually affects males and is transmitted from mother to son.

People born with haemophilia have little or no clotting factor, a protein needed for normal blood clotting. Clotting factors work with platelets to achieve blood clotting.

Platelets are small blood cell fragments that form in the bone marrow—a sponge-like tissue in the bones. When blood vessels are injured, clotting factors help platelets stick together to plug cuts and breaks on the vessels and stop bleeding.

"If you have haemophilia, you may bleed for a longer time than others after an injury. You may also bleed inside your body (internally), especially in your knees, ankles, and elbows. This bleeding can damage your organs and tissues and may be life-threatening,” says Dr Osee Robert Karangwa, head of paediatrics department at Rwanda Military Hospital, Kanombe.

Rarely, haemophilia can be acquired. "Acquired” means you are not born with the disorder, but you develop it during your lifetime. This can happen if your body forms antibodies (proteins) that attack the clotting factors in your bloodstream. The antibodies can prevent the clotting factors from working.

Dr Karangwa explains that understanding the disease and early detection are key issues in handling haemophiliacs to prevent excessive bleeding.

Types of haemophilia

Depending on the level of the clotting factor, there are two main types of haemophilia A and B.

If you have haemophilia A, you are missing or have low levels of clotting factor VIII (8). About eight out of 10 people who have haemophilia have type A. If you have haemophilia B, you’re missing or have low levels of clotting factor IX (9).

"A person who is hemophilic when bruised can bleed until blood gets done from his body unless handled by a doctor as soon as possible, bodies tend to swell, nose bleeding is also another factor as well as joint swelling,” Dr Karangwa says.

But everyone can cope up with haemophilia especially when you are on treatment and taking the medication as prescribed.

"Because patients bleed endlessly due to blood clotting failure, even the simplest cut can cause jeopardy and haemophilic bleeding does not have age limit, it is in therefore imperative to treat it during early detection,” Dr Karangwa says.

"Haemophilia is not necessarily disability and the patient can live like other children when the disease is medically managed but the costs are high.”

Normally, people have proteins in their blood called clotting factors that act fast to plug wounds, haemophiliacs lack these proteins, making even minor bleeds difficult to stop and they have to survive on habitual treatment.

Treatment and complications

The main treatment option for people with severe haemophilia is to receive regular infusions of clotting factor. But 20 per cent to 30 per cent of people who get these infusions develop antibodies, called inhibitors, against the clotting factor. Once these inhibitors develop, it can be very difficult to treat or prevent future bleeding episodes.

Studies focused on haemophilia A, in which the clotting factor VIII is mutated, causing a defect in clotting, revealed that worldwide, one in 7,500 males is born with this disease.

However, after receiving infusions of FVIII, some patients develop antibodies against it. Their immune system responds to this foreign protein as an invader that must be attacked and eliminated.

But there is another option to prevent antibodies from forming and hence one of the most serious complications of haemophilia treatment but still the cost is limiting factor present only in developed countries.

Cost remains a big issue in the management of haemophilia, although there is a new technique, which uses plant-based capsules, with a potential to be a safe and cost-effective alternative.

health@newtimes.co.rw