Autumn 1999 (7.3)
Pages 68-70

Hemophilia in Azerbaijan
On Our Own Now

by Gulnara Huseinova

The drama that influences one's life is not always shaped by political conflicts. Sometimes medical conditions can do it as well. One of the most poignant cases in history is Russia's Czar Nicholas II (1868-1918) and his son Alexander, who was born with hemophilia. The condition was so little understood that the Czar nearly went out of his mind with grief over his son's situation. Eventually, he discovered the mystic Rasputin, who seemed to bring some sort of relief to his son. Over time, Rasputin gained more and more political authority, which essentially led to the collapse of the Czar's power and the demise of the Russian Empire (1917).

Gulnara Huseinova's family has had to deal with the tragedy of hemophilia several times-first with her younger brother and now with one of her sons. Here she describes how inadequate the treatment has been for those living in Azerbaijan during the past 30 years.

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The day my son was born was such a glorious day for me and my husband. I was 22 at the time. It was a long-awaited dream. We called him Ayaz which means "breeze".

Hemophilia causes blood to accumulate in the joints - an excruciatingly painful condition. There is no Factor VIII in Azerbaijan to facilitate blood clotting. An estimated 2,000 victims suffer from this crippling disorder.

But two days later, the thing that I feared most in life for my child became evident. Ayaz developed a large hematoma [bruise] on his head. Despite doctors' efforts, his condition grew worse and worse until they weren't sure that he would even survive. I cried night and day, praying that this was all a bad dream that would suddenly vanish.

I didn't want to accept that Ayaz had hemophilia. I would have preferred that he had been born a cripple rather than have this dreadful disease. I didn't want to believe it. And then the doctors "added salt to our wounds" by telling us that if the baby managed to survive, he would be mentally handicapped.

My mother's reaction was even worse than mine. She came to the hospital and when she realized the situation, she confided in me. "You know how much I've suffered with your brother.

Don't suffer like I did. Don't bring the baby home! Leave him at the hospital. Even if they manage to save his life, don't bring him home." But I couldn't do that. He was my baby-no matter what. I had conceived, carried and bore him. I couldn't abandon him. But my mother had good reason for her advice. My younger brother Ali had been born with hemophilia in 1973. I was eight or nine years old at the time. Nine months later, the nightmare began and all normalcy in our household ended. Bruises began appearing all over his body. At first, we thought it was normal because he had very tender skin.

Above: Gulnara's son Ayaz, riding a horse, an experience that would have been unthinkable for a hemophiliac only a decade ago.

But then he caught a cold and my mom tried to treat him with a few nose drops. The sensation, of course, was uncomfortable, and my brother started rubbing his nose and that's when it started to bleed and bleed and bleed. We couldn't stop the bleeding. Fifteen days passed. Poor child! He had lost so much blood and gone through so much trauma.

Everyone panicked. We took him to a number of doctors, but no one could diagnose him correctly. Finally, we took him to the Mother and Child Care Hospital in Baku where they told us that Ali had hemophilia.

Hemophilia? What was that? In those days, even in America, they were just beginning to understand how to use Factor VIII to make the blood clot. In the USSR, there was no such thing as a clotting factor and so they transfused my brother with blood and plasma. But by that time, he had lost so much blood. They told my mother how serious his condition was and that we had to be very careful. With hemophilia, the slightest bump or fall could cause internal bleeding. Sometimes, the internal hemorrhaging can occur spontaneously for no apparent cause at all.

Unfortunately, the blood accumulates in the joints, particularly in the knees and ankles, which swell enormously. The pain is excruciating.

After that, my parents were not able to live a normal life. To tell you the truth, I can't remember ever spending a normal holiday together with our family. My brother was always in the hospital. Even though my mother had graduated with excellent grades from the Institute of Economy, she could not pursue a career. Instead, she stayed at home to take care of her son.

They were forever in and out of hospitals both in Baku and Moscow. My father had received the first Master's Degree and first Doctorate in Law in Azerbaijan, but his career, too, was affected. When my mother was with our brother, Dad took care of us four girls-Tarana, Matanat, me-Gulnara and Sevinj.

When Mom herself got ill, my older sister would carry my little brother to school in her arms. He was seven and she was 15. His leg had fully atrophied and he walked with such difficulty and refused a wheelchair. Even later at 12 when he was offered a cane in Moscow, he would have nothing to do with it.

As my brother grew up, naturally he wanted to play and be as active as any normal child, but he always ended up with painful bruises every time. How can you tell an active child: "Don't run! Don't jump! Don't ride a bicycle! Don't play soccer! Don't climb trees!" That's like saying, "Don't be a boy!"

When Ali was six, he fell and injured his knee while playing in the street with friends. The doctors here in Azerbaijan said that he should lie down and elevate his leg.

Everyone from the Republics went to Moscow for treatment but even there, they knew so little about treating this genetic bleeding disorder. In the Republics, all they could do was transfuse plasma and cryoprecipitate, the substance in blood that facilitates clotting.

Ali's leg became immensely swollen and bent at the knee, which interfered with normal walking. Doctors thought they could straighten it out by infusing cryoprecipitate. But they didn't succeed and, today, he suffers from one leg being seven centimeters shorter than the other. He tries to conceal the situation with a shoe that has a thicker sole.

The Next Generation
Hemophilia does not manifest itself in women but they may be carriers, meaning they have a 50 percent chance of transmitting the disease to their sons. When I got married, I suspected that I might be a carrier but was never checked. Both of my sisters had healthy boys.

At first my son Ayaz was quite active and healthy-looking. I found him so cute and funny that whenever I looked at him, I could nearly forget his ravaging disease. My sisters warned me not to let him run or jump, but I wouldn't listen. If anyone made the slightest mention of his illness, I became upset.

But serious problems began when he became three. He started bleeding internally at his joints at the slightest injury or bruise. The pain was indescribably intense. I can't imagine grownups being able to handle it better than he did.

During the Soviet period, the workforce was obliged to give blood. Hemophiliacs like my brother were provided with plasm and "cryo". There was no such thing as Factor VIII that you could administer yourself when the child screamed in pain in the middle of the night.

But when the Soviet Union collapsed, we lost the infrastructure in our hemotology centers. No longer were blood donations mandatory. Thus, if your child needed blood, you had to go and find someone personally who would be willing to go to the clinic and transfuse blood directly to the patient. Hopefully, the donor's blood was disease-free-no syphilis, hepatitis or AIDS. There were no reagents to test for such diseases. One could only hope.

Nor was there a secure way to store blood. You had to transfuse it right away-from donor directly to patient. Often there weren't even blood bags, so the blood would be collected in glass jars. Nor was there dependable refrigeration or back-up generators when the electricity went off-which it did so often.

It was especially difficult for anyone in need of blood between the years of 1992 and 1996. What can you do when you see your own child suffering in front of your eyes, and you're helpless to alleviate the child's pain especially when you feel so guilty as a mother for being the genetic link that give him this disease in the first place? Sometimes you feel it's impossible for your heart to break any more than it already has.

The situation got so desperate with Ayaz that all I could think of was to try to find a way to take him out of the country to a place where he could receive treatment and live a normal life. For two years I made the rounds, knocking on every door of every organization and company I could think of, but to no avail. Nobody wanted to sponsor us.

But then a friend advised me to stop thinking about going abroad and focus my energy on setting up an organization inside Azerbaijan which could serve to help my child as well as others.

I had no options. There was no way out. I had to do something from within the country. That's when I started organizing parents of hemophiliacs. In May 1996, we created the Azerbaijan Republic Association of Hemophiliacs. Today, more than 700 hemophiliacs are registered, from all over Azerbaijan.

In general, not many people know about hemophilia in Azerbaijan. The public has not been properly informed. Both hemophiliacs and their families try to hide the disease. They feel ashamed and guilty that they have such a condition in their family.

Often, when the disease is first discovered, they don't know how to handle it and don't know to pay attention during the early stages of the disease. When their child screams out in pain in the middle of the night, they don't know what to do. Parents need to be educated so that the child doesn't reach such a critical stage. As a parent who provides most of the treatment for my child, I have always emphasized that a lot depends on how much the caretaker knows about the disease.

My son, 10, now understands the nature of his disease and how to take care of it. If I'm not around when he "gets a bleed", he knows to apply a piece of ice or a bandage, depending upon the situation. He knows that he should be careful when he is playing, running or even walking. He is not confined to bed, although my brother had already become an invalid by the time he was six.

Not only has Ayaz survived hemophilia, he's been able to lead a fairly normal life. On an excursion to the country a few months ago, he was even able to ride a horse! This kind of thing would have been unthinkable a decade ago for hemophiliacs in Azerbaijan. There's no way that my brother Ali could ever have had such an experience.

We're really just getting started with our Hemophiliac Association, which is primarily made up of parents whose children have the disease. We know there are at least 1,000 hemophiliacs who have been deprived of adequate treatment; 55 percent of them are school-aged. Already we have compiled a database of 700 patients describing the extent of their disease and their blood types.

But there is still an immense amount of work for us to do. We need access to the Internet, to other hemophiliac associations throughout the world. We need to exchange ideas and find organizations and materials that can help us educate our parents and our public. We need to find solutions for treating this disease like they do in other countries that don't have clotting factor either. At present, a single treatment of Factor VIII can cost upwards of $500, which is prohibitively expensive for us. There is no state health insurance in Azerbaijan. How can a person who is living on a monthly salary of less than $100 handle these expenses? It's impossible.

Independence has shown us that we can't sit by idly. We who have suffered the most have begun the journey to search for ways to ease our children's physical trauma. When our children are safe and feeling healthy, this will assuage our own psychological pain.

The Azerbaijan Republic Association of Hemophiliacs has received assistance from numerous individuals and organizations including British Petroleum, Ernst & Young, ISAR and Rafiga Sadighova, the wife of Azerbaijan's Ambassador to Germany. Grants have helped pay for the translation of brochures, the registration of hemophiliacs, the creation of six regional divisions and medicine and operations for individuals. Texaco has made a tremendous contribution by undertaking the entire refurbishing and equipping of the National Blood Bank. Gulnara Huseinova may be contacted at the Hemophiliac Asso-ciation in Baku at Tel: (99-412) 40-41-17, 40-01-89 or 68-34-79. Jala Garibova also contributed to this article.

From Azerbaijan International (7.3) Autumn 1999.
© Azerbaijan International 1998. All rights reserved.

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