Hemophilia in Malaysia

The Hemophilia Society of Malaysia organizes training workshops for patients with support from the World Federation of Hemophilia (WFH) and Baxter Healthcare.
IMAGINE having a condition where you could end up bleeding to the point where you suffer from joint deformity, all because of a simple knock or bump. This is the reality of a person living with hemophilia.
Hemophilia is not as rare as you might think, but very few people know much about it. There are so many myths about this condition, and people who live with it not only have to deal with daily adjustments, they also need to educate the people around them.
Hemophilia is a life-long bleeding disorder that prevents blood from clotting properly due to a lack of clotting factor. This results in prolonged bleeding; hence simple cuts and wounds can have severe and debilitating consequences for individuals with hemophilia, especially when bleeding occurs internally.
Hemophilia is a genetic condition carried on the X chromosome and therefore passed on by women. With each pregnancy, a woman who is a carrier of hemophilia has a 25% chance of giving birth to a son with hemophilia.
Since the genetic make-up of the father’s sperm determines the sex of the baby, all the daughters of a man with hemophilia will be carriers of the condition, carried on the X chromosome, but none of his sons.
In about 30% of cases, there is no family history of the disorder and the condition is the result of a spontaneous genetic mutation.
The most severe forms of hemophilia affect almost only males. Although extremely rare, a female can be affected if her father has hemophilia and her mother is a carrier. Many women who are carriers can also show symptoms of mild hemophilia.Two of the most common forms of hemophilia are A and B, with hemophilia A being the more typical form. More than 400,000 people in the world have hemophilia. In Malaysia, there are approximately 900 people affected by hemophilia A and another 200 affected by hemophilia B.
People living with hemophilia do not have enough of, or are missing, one of the blood clotting proteins naturally found in blood.
Clotting factors, designated by Roman numerals I through XIII, are proteins found in blood that work together to make blood clot. When the body detects bleeding, clotting factors are switched on in a specific order, each activating the next factor in the "blood clotting cascade" to ultimately form a fibrin clot. The fibrin clot acts like a net, holding the platelets together to create a firm blood clot.
In people with hemophilia A, clotting factor VIII is not present in sufficient amounts or is absent. Factor VIII is one of the clotting factor proteins that help produce the fibrin clot. In such people, firm blood clots do not form in the wound and bleeding is not easily stopped. Without enough factor VIII, patients can experience spontaneous, uncontrolled internal bleeding that is painful, debilitating and damaging to joints.
If untreated, patients with severe hemophilia A have a greatly reduced life expectancy.
"The training workshops that are being held are a crucial form of on-going education in managing and dealing with hemophilia, because it is possible for individuals with the condition to live relatively normal and healthy lives when proper treatment and care is administered," explained Mohamed Aris Hashim, President of the Hemophilia Society of Malaysia.
Hemophilia is treated by replacing the missing clotting factor in the blood via an injection containing the needed factor. Bleeding stops when enough clotting factor reaches the bleeding site.
Treatment for hemophilia has evolved tremendously over the past few decades. The development of plasma concentrates was a breakthrough that allowed patients to stop their bleeding via a simple injection that could be self-administered. However, concerns with contracting blood-borne infections such as hepatitis B, hepatitis C and HIV continued to be a problem.
It was not until the 1980s that genetically engineered factor concentrates were developed, leading to the very recent and revolutionary innovation of a recombinant factor VIII treatment that is free from human plasma and animal-derived albumin which eliminates the risk of pathogen transmission.
"The greatest challenge for people with bleeding disorders in many countries is access to safe and affordable treatment. WFH is working to achieve our vision of treatment for all, which includes improved access to clotting factor concentrates. Besides that, it also means proper diagnosis, management, and care provided by an interdisciplinary team of trained specialists," said Mark Skinner, President of the World Federation of Hemophilia and a hemophilia patient.
The full day workshop, which was divided into separate sessions for men, women and children to address the different challenges each are faced with, was held at Hospital Ampang and attended by 150 participants. Topics included Raising a Child with Hemophilia, fitness for women and men as well as group discussions on the emotional adjustments that are necessary in a family with hemophilia.
"Living with hemophilia in the family is a constant learning process, hence family and social support is one of the main reasons why the society exists," explained Aris. "We’ve found that having like-minded individuals sharing their stories and experiences is like having a very large extended family, and all our activities, such as this workshop, are aimed at helping each other live our best lives possible.”
The Hemophilia Society of Malaysia was established in 1980 specifically to promote the welfare and to help patients and their families in overcoming the myriad of problems associated with this potentially crippling condition.
It has been regularly bringing together families from various parts of Malaysia to participate at educational workshops and camps, producing educational materials and promoting blood donation campaigns.
The Ministry of Health Malaysia has accredited the society as the official representative of the hemophilia and other bleeding disorders community in the country. The society is also affiliated with the World Federation of Hemophilia that has 115 member countries worldwide.