A cut, scrape or bruise may be a routine childhood rite of passage for most of us but for student Poh Zi How, such minor injuries could land him in hospital.
The 14-year-old suffers from haemophilia.
Haemophilia is a genetically inherited disorder where there is a deficiency of a clotting factor in the blood. This causes the patient to bleed more easily and suffer from bruising and bleeding in muscles and joints.
As the disorder is a recessive condition linked to the X chromosome and males have only one X chromosome, the disorder affects males only.
Dr Tan Hooi Hwa, president of the Haemophilia Society of Singapore, said: 'People are not very understanding of what haemophilia is and patients are stigmatised.
'Mothers feel very guilty. In traditional families, the mothers are blamed for their sons' deficiencies.'
There are just over 300 haemophiliacs in Singapore, with more than 80 per cent diagnosed with Haemophilia A which means they lack a clotting factor called factor 8. The rest suffer from Haemophilia B and are deficient in factor 9.
Haemophilia is classified into three categories, namely mild, moderate and severe, and roughly a third of haemophiliacs here have severe haemophilia.
Patients with severe haemophilia can bleed spontaneously and need more care.
Parent's nightmare
Six months after her second son was born, financial planner Shirley Poh plunged into a 'nightmare which didn't stop until he was six'.
Her son Poh Zi How, now 14, was diagnosed with haemophilia and was constantly in and out of hospital. His condition stabilised only when he was in Primary 1.
Mrs Poh said: 'He kept crying and my husband and I didn't know why. We took him to a private hospital and ended up spending $8,000 on his hospital bill. I didn't know what haemophilia was at first.'
After Zi How was diagnosed with haemophilia, he was given prophylaxis or a regular regime of injections to prevent him from bleeding.
The Chung Cheng High School student said that he now injects a clotting factor through the back of his palm once a week and his monthly bills usually come up to at least $800.
When he bleeds, he has to keep away from doing sports or anything that may aggravate his bleeding.
A mild injury requires a recovery time of three weeks. And he has to inject himself with four to six vials of factor concentrate whenever he is injured.
The average lifespan of a haemophiliac is about 60 years, said Dr Gan Kim Loon, vice-president of the Haemophilia Society of Singapore.
There are three treatment centres for haemophilia in Singapore and they are at National University Hospital, Singapore General Hospital and KK Women's and Children's Hospital.
When a haemophiliac experiences bleeding, which is usually internal and signalled by pain or swelling, he has to treat himself by injecting the missing clotting factor into his bloodstream. These clotting factors are found in solutions known as factor concentrate and are derived from blood plasma. They can also be found in bio-engineered products called recombinants.
Although recombinants eliminate the risk of viral transmission when the clotting factor is injected into the haemophiliac, they are double the price of plasma-derived factor concentrate in Singapore.
Haemophiliacs risk infection of blood-borne diseases like HIV and Hepatitis C when they use clotting factor obtained from donated blood.
A vial of factor 8 concentrate usually costs $125 and heavy bleeding may require patients to inject four vials at a time. The Haemophilia Society of Singapore, which was set up in 1985, subsidises a quarter of the treatment costs while hospitals subsidise another half. The patient bears 25 per cent of the bill. But that amount is often still a hefty burden for most families.
Zi How said: 'I just take the injections when bleeding occurs. The problem is that it's a bit expensive for my family.'
Although haemophiliacs should avoid contact sports at all costs, DrTan encourages those he treats to stay active.
He explained: 'The more muscles they build, the less chance they will have of falling and injuring themselves.'
He recommends swimming, gym workouts and non-contact sports.
But he wishes that there was more money for patient care and subsidies here.
'Research has found that when you treat haemophiliacs early, they can lead a normal life,' he said.
?In Singapore, we're still doing on-demand treatment. The cost of factor concentrates is so high that many haemophiliacs still delay their treament. Dr Tan Hooi Hwa, President of the Haemophilia Society of Singapore
- Those interested in making a donation of finding more about haemophilia, contact the Haemophilia Society of Singapore on 6256-4636 or 6256-8166.
This story was first published in Mind Your Body, The Straits Times, on May 28, 2008.
