VANCOUVER -- Two hundred hemophiliacs from around the world visited Vancouver to attend a major conference on this the very rare bleeding disorder. Hemophilia affects one in about 10-thousand births. It stems from a congenital lack of clotting factors. But with the right drug treatment, this disorder can now be much better managed.
Marc LaPrise doesn't go anywhere without his medical kit because he never knows when he may have a bleeding episode. A long walk or bump on the knee could set things off.
LaPrise caught it right on time. What happens is, it starts an internal bleed. So, he can feel his knee cap floating up and blood entering the joint, which is intensely painful for him.
To stop a bleed, Marc uses a synthetic clotting factor made using recombinant DNA technology.
"This is where I usually inject. You just put in right into your arm and ten minutes later the bleeding stops."
In Canada, Hemophiliacs do have access to these very expensive drugs, but in developing countries, it's a different story.
Siddhartha Ojha, Hempphiliac From India, says, "due to regular joint bleeding, bleeding in both ankles, my left knee, both elbows and shoulders, it resulted in deformity. Now, I'm almost 70% disabled."
Dr. John Wu, Director of Hematology Program, BC Children's Hospital, says, "what we're doing now is actually to try to prevent the bleeds from happening, of course if a bleed happens, we treat right away to minimize damage."
Dr. Wu says preventative treatment once or twice a week with good support at an early age can protect the joints so youngsters can lead active lives.
Dr. Wu says, "I have lots of my patients, playing different sports, sometimes even hockey in some of them, but they have to be very careful."
Marc is happy youngsters are now treated with recombinant clotting factors not made from human plasma, because it means they won't have to go through what he did as a teen. At 14, he received tainted blood infected with HIV, Hepititus B and C.
Laprise says, "when I was diagnosed, I was told maybe ten years. That was 18 years ago. So far, so good. The medication is
Hemophilia is passed down to sons from mothers who are carriers. About a third of Hemophilia A cases are new with no family history, the condition arising from mutations. By the way, Siddhartha, and other hemophiliac delegates did receive free treatments this week during the World Congress, courtesy of the pharmaceutical companies at the conference.
Monday May 29, 2006
