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Other questions you may be asked

Q. Why does your right knee always give you trouble?

My right knee is a target joint. I had a lot of bleeds in it when I was growing up. Each time I had a bleed, blood would accumulate in my right knee joint cavity. Over time, blood can act like acid on the smooth, protective cartilage that everyone has on the ends of their bones. The blood damaged the cartilage on the ends of my upper and lower right leg bones, where they meet at the knee. The cartilage is no longer smooth, and it makes my knee stiff and causes it to bleed often.

Q Why do you give yourself an infusion when you don't even have a bleed?

I'm on prophylaxis. Prophylaxis means prevention. Boys and girls on prophylaxis are infused with factor up to three times a week, whether they need it or not. This keeps the factor levels high in their blood so they do not even get bleeds. There are children with hemophilia today who never experience bleeds because they are on prophylaxis! New information shows that long-term prophylaxis will help protect my joints. So when I am an adult, my joints will be in great shape.

Q Then boys with hemophilia really have nothing to worry about, right?

Wrong! Prophylaxis is used only by some people with hemophilia. It is a very expensive medical treatment because it uses a lot of factor and factor is expensive. Some people have medical insurance that will pay for prophylaxis. Many people do not, however.

Q If you replaced all the blood from someone with hemophilia with the blood of someone without hemophilia, would this cure his hemophilia? The new blood would have factor in it, right?

A blood transfusion would stop bleeds, but only for about 12 to 24 hours. Just as we eat and use up our food, and need to eat more, so the body also uses up factor daily and must make more. In people with hemophilia, the chemical directions for blood clotting are faulty and scrambled. The body does not know by itself how to make enough factor that works to clot blood. A one-time transfusion (just like a great feast)  would not cure hemophilia (nor leave your stomach full forever) because the body uses up its factor (or food). As the factor is used up, the genes need to send directions to the body to make more factor, to replenish the supply. People with hemophilia cannot do this. Their only hope of a cure lies in finding a way to make their bodies capable of making their own factor. Until then, they continue to depend on infusions of factor.

Q I heard that the medicine may not work for everyone. Why?

They may have an inhibitor. An inhibitor is a substance that develops in the bloodstream and attacks the factor when it is infused. An inhibitor destroys factor before it has time to stop a bleed. This requires careful medical attention.

Q But why does the body attack the factor?

People with severe hemophilia have so little factor in their bloodstream that when factor is infused to stop a bleed, their bodies do not "recognize" the medicine as a normal substance that belongs there. The body treats the factor as an invading substance that does not belong - and tries to destroy it. Several different treatments can be tried to destroy the inhibitor. Luckily, most boys with hemophilia do not develop inhibitors.

Q When you marry a woman and have children, will any of them have hemophilia?

No. Neither my sons nor my daughters will have hemophilia. But all my daughters will be carriers of hemophilia. That's a fancy way of saying that they can pass hemophilia on to their sons, my grandsons. If I have two daughters, they will each be carriers. But they won't have hemophilia! When they have children, each of their sons - my grandsons - will have a 50% chance of having hemophilia.

Q. Do girls ever get hemophilia?

It is extremely rare, but yes! If a man with hemophilia married a woman who was a carrier then they might have a girl with hemophilia. The chances of having a girl with hemophilia would be 50%.

There are girls with hemophilia, and boys with hemophilia, whose mothers are not even carriers! Occasionally something goes wrong inside the cells of a developing baby, even when the parents have nothing wrong with them. In one-third of all cases of hemophilia, the mother is not a carrier of hemophilia! Something just went a little wrong inside the baby.

Q. Will you always have hemophilia ?

Yes. Unless they find a cure, I will always have it. Some people think of hemophilia like other diseases that get better or worse, but hemophilia is more like having a finger or toe that never worked correctly from birth. The body can't fight hemophilia like other diseases, because there is nothing to fight! Hemophilia is not a germ or infection. A protein in my blood does not work properly. Everything else works successfully in my blood and body.