May 1999

Dear Friends & Family:

Greetings from Malawi and Mulanje Mission Hospital. I have never seen as much rainfall in my life as the amount that has fallen around the great Mulanje Massive here in southern Malawi during March and early April. It may not be for forty days and forty nights, but it rained nearly every day and every night for at least 28 days. We are appreciating the warm sunshine we have today, and the chance to dry our clothes outside.

The practice of medicine is a fascinating, lifelong adventure. Those four long years of studying and memorizing obscure facts and diseases in medical school can sometimes pay off decades later. Here is an example.

I must first confess that I am not exactly thrilled when people under the age of puberty come to my office for medical attention. After medical school, I studied obstetrics and gynecology for four years, and we hardly ever had to take care of children. So I guess I have gotten into the habit of taking care of grown-up women, who usually answer questions easily and don't scream when you try to examine them. Well, there is actually a little bit of screaming, but that is only occasionally, when they are giving birth.

Yesterday, a mother brought her seven-year-old son, whom we will call Jack, to me because of a swollen, painful knee. Jack looked healthy and intelligent except for his knee, which was about triple its normal size. Looking back over my vast experience of this problem, which includes one other patient, I thought maybe Jack might have juvenile arthritis. So I put him in the hospital on aspirin.

This morning I saw Jack again and pushed on his knee. It felt like it might have fluid in it, but it was not hot or red, and Jack had no fever. I decided to stick a needle in the knee and was surprised to get back old-looking blood, blood which was a little dark. There was no pus, which would mean an infection. I was looking at Jack, and wishing I were a pediatric orthopedic doctor, or at least an orthopedic doctor, when suddenly it hit me, the little Tsar Alexei. He was the son of Nicholas II, Tsar of all the Russians, and he had swollen knees and another disease—not arthritis, but hemophilia.

Hemophilia is a hereditary disease carried only by females and affecting only males. The sons of women who carry the hemophilia gene on their X chromosome can inherit it from their mothers. The daughters cannot inherit the disease, but can become carriers of the gene. Hemophilia is caused by the lack of one of the clotting factors in the blood, and can manifest itself early in life with bleeding after even minor trauma. People who suffer from hemophilia can have bleeding into weight-bearing joints, like hips and knees, and even internal bleeding.

They taught us in medical school to always take a history, talk to the patient, or in this case his mother. It turns out that Jack is the seventh and last child. There are four sisters and one older brother, who is living and healthy. All of the sisters are healthy. Jack had an older brother who died at the age of five. I asked the mother what this brother had died of? "The same thing," she replied. "What same thing?" "He had a swollen knee."

One in ten thousand males is affected by this disease. In medical school, we used to say, one in ten thousand is very rare, but when it is you or your loved one, that is one hundred per cent. Some people consider it a disease of royalty because Queen Victoria carried the gene and passed it on to her daughters, who married into the royal families of Spain and Russia. So that was how poor Alexei came to be affected.

We will not be able to offer much diagnostic or therapeutic support to this boy if he really does have hemophilia. We do not have even a very basic laboratory test to start to make the diagnosis. You may say, well, why don't you have it? It is because we do not need this test very often, and it is too expensive to have things available that are not used very often. Malawi (and this could be said about the rest of Africa) is a land of limited resources, and what little is available must be used wisely and sparingly.

I asked Jack's mother if she could take him to Blantyre, which is 35 miles away, where there is a large regional hospital, where they could do some tests. She replied that no one in her family had ever been to Blantyre. I think it would be taking food out of their mouths for them to buy the bus tickets to get to Blantyre. Even if Jack and his mother could make it to Blantyre, the hospital there does not have the clotting factors necessary to treat his disease.

So what is there left to do? I learned something else in medical school. Dr. Smiley Hill told us that being a doctor is not really so difficult. It boils down to three basic things, "Work hard, care, and be there."You have to work hard for the patient (and this includes using your brain), care for and about the patient, and be there when you are needed. So I have looked up hemophilia in a big medical book, where it says you should never give aspirin to someone with this disease because it may interfere with the platelets, factors that help the blood to clot. So I can stop the aspirin that I ordered. I can continue to be supportive of Jack and his family, and be there when they need me.

Not to be too philosophic, but I feel I must mention Alexei once again. Even though he was the son of one of the most rich and powerful men in the world, Tsar Nicholas II of Russia, he still suffered from this disease, and had an unhappy end with all his family, when all of them were executed by the Bolsheviks in 1918.

Sue Makin