For this edition of the EHC Newsletter we asked Gulnara Huysenova, President of the Azeri NMO, to talk to us about her experience of sharing her life with people with haemophilia (her brother and her son) in a country with poor access to treatment. She also gives us an overview of the origins of the association and its current activities. Origins of the association 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’ in Azeri. But two days later, the thing that I feared most in life for my child became evident. Ayaz developed a large haematoma on his head. Despite the 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 did not want to accept that Ayaz had haemophilia. I would have preferred that he had been born a cripple rather than have this dreadful disease. I did not 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 could not do that. He was my baby, no matter what! I had conceived, carried and bore him. I could not abandon him. But my mother had good reasons for her advice. My younger brother Ali had been born with haemophilia in 1973. I was eight or nine years old at the time. Nine months later, the nightmare began and all normality in our household ended. Bruises began appearing all over his body. At first, we thought it was normal because he had very tender skin. 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 is 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 had 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, the capital of Azerbaijan, where they told us that Ali had haemophilia. Haemophilia? 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 Union of Soviet Socialist Republics (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 18 mother how serious his condition was and that we had to be very careful. With haemophilia, the slightest bump or fall could cause internal bleeding. Sometimes, the internal haemorrhaging 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, Sevinj and me. 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; he walked with such difficulty and refused a wheelchair. Even later at twelve 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 USSR went to Moscow for treatment but even there, they knew so little about treating this genetic bleeding disorder. In the USSR, 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 centimetres shorter than the other. He tries to conceal the situation with a shoe that has a thicker sole. The Next Generation Haemophilia does not manifest itself in women but they may be carriers, meaning they have a 50 per cent chance of transmitting the disease to their sons. When I got married, I suspected that I might be a carrier but was never checked. All 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 would not listen. If anyone made the slightest mention of his illness, I became upset. But serious problems began when he turned three. He started bleeding internally in his joints at the slightest injury or bruise. The pain was indescribably intense. I cannot imagine grownups being able to handle it better than he did. 19 During the Soviet period, the workforce was obliged to give blood. Haemophiliacs like my brother were provided with plasma and cryoprecipitate. 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 haematology centres. 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 and was not affected by blood-borne diseases such as syphilis, hepatitis or HIV. There were no reagents to test for such diseases. One could only hope for the best. Additionally, there was no secure way to store blood. You had to transfuse it right away from the donor directly into the patient. Often there were not even blood bags, so the blood would be collected in glass jars. Furthermore, there was no 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 are helpless to alleviate the child's pain especially when you feel guilty as a mother for being the genetic link that gave him this disease in the first place? Sometimes you feel it is 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 organisation 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 is when I started organising parents of people with haemophilia. In May 1997, we created the ‘Azerbaijan Republic Association of Haemophiliacs.’ Today, almost two decades later, over 1,400 people with haemophilia and von Willebrand Disease are registered from all over Azerbaijan. In 1999, we became a member of the EHC and in 2000, we became a member of the World Federation of Hemophilia (WFH). Thanks to the attention and care of the head of state, President Ilham Aliyev, and First Lady Mehriban Aliyeva, the Ministry of Health of the Republic of Azerbaijan periodically take measures to solve the problems of patients with haemophilia. The Ministry of Health has successfully implemented and enforced decrees and decisions of the head of state to improve the quality of life of patients with haemophilia, ensuring that the government budget would cover the purchase of coagulation factor concentrates. Moreover, the National Haemophilia Centre is now equipped with modern technologies for the diagnosis of bleeding disorders. Additionally, medical staff is trained to deal with various surgical procedures relevant for people with haemophilia. The most recent haemophilia state programme (2011-2015) has implemented a series of actions; the most important were the provision of factor concentrates and the establishment of a national register for people with bleeding disorders to collect data on the distribution and use of coagulation factor concentrates and to evaluate the effectiveness of health care. 20 Thanks to these reforms, we have now been able to achieve 1.4 international units (IU) per capita of FVIII concentrates and to improve quality of life for people with haemophilia, many of who nowadays do not differ from healthy people. As for Ayaz, I am happy to report that he is now a healthy 26-year-old working as a dentist. He is also active with the Society and recently took part in the EHC Youth Leadership Workshop (see article pg. 8). About the Azeri NMO As for the Azeri haemophilia association, our current objectives are to promote the quality and safety of haemophilia care as well as social and medical rehabilitation in Azerbaijan. In this regard, we actively collaborate with the government to ensure that safe and effective coagulation factors concentrates are purchased. Our organisation also aims to improve the level of social protection for people with bleeding disorders. In this regard we try to provide our members with legal advice on their rights and what they are entitled to. Like other haemophilia organisations, our objective is also to bring together people affected by the same disorder and we do so by developing social programs such as health-oriented camps. Finally, we promote safe and non-remunerated blood donation. Our organisation is managed by a board of seven people, including myself. We also have a small office composed of an administrative assistant, a translator and an accountant. Between 2001 and 2004, we took part in a WFH Twinning Programme with the Turkish Haemophilia Society. Between 2004 and 2008, we completed the WFH Global Alliance for Progress (GAP) project, which allowed our medical professionals to gain additional medical training in the area of haemophilia and other bleeding disorders. Furthermore, our country was able to purchase laboratory and medical equipment and we received 600,000 IU of coagulation factor concentrates from the WFH Humanitarian Aid programme. During this project, representatives from our Ministry of Health attended the WFH Congress, workshops and seminars abroad on haemophilia and we even signed a Memorandum of Understanding. Furthermore, we organised several conferences and seminars in Azerbaijan to educate and raise awareness about haemophilia. More recently, we started a two-year project in 2013 on ‘Decentralisation of care of haemophilia patients through education and capacity-building centres for the diagnosis of haemophilia.’ This project, funded by industry, aims to provide training and establish laboratories for the diagnosis of bleeding disorders in the cities of Baku, Ganja and Shirvan.