"BLOODY" DISEASE

In our cruel times, there is almost no room for compassion and mercy for those surrounding us. Everyone is trying to explain this indifference by overwhelming workload and numerous problems. But nothing can really justify such an attitude, especially in cases when ill children are involved. Even modern medicine is not capable of curing them all.

About 200 children are born in Azerbaijan every year suffering with a virulent form of thalassemia. For this indicator our country is one of the front-runners among post-Soviet republics.

Only several decades ago information about thalassemia was largely hushed up and very few people knew that the disease is so widely spread. Children born with this dreadful diagnosis did not receive necessary treatment and virtually died in the hands of their parents. Those days are gone now and it has already been established that with proper and timely treatment, people suffering from thalassemia can live and be fully-fledged members of society.

World Health Organization specialists have recently calculated that if treatment of thalassemia is not duly organized in the coming years, the number of people suffering from the disease will reach 16,000 over 45 years.

Mediterranean anemia 

Thalassemia is the disease spread in southern, mainly coastal countries. Its name is derived from Greek thalassa, which means sea. This is a hereditary blood disease which appears as a result of a genetically preconditioned low level or a complete absence of the oxygen-conducting component of albumen - hemoglobin. Thalassemia is inherited from parents. Parents, however, do not have to be infected. They can only be carriers of the thalassemia gene and do not have to have serious health disorders. For some genetic reasons, the thalassemia gene in a child can manifest itself in a much stronger way than in parents, which can lead to a disease requiring immediate and continuous treatment. Thalassemia is manifested in the form of anemia (deficiency of blood) of varying degree of severity, high content of iron in blood, enlarged liver and spleen. b-thalassemia is particularly widespread and can be of three forms: mild, intermediary and virulent or major.

The Coolie Disease, better known as the Mediterranean anemia or major thalassemia, runs very seriously. The point is that the hematopoietic system of a patient cannot maintain the level of oxygen-conducting component of albumen, hemoglobin, sufficient for normal quality of life. Therefore, regular blood transfusions are required throughout a person's life. Intermediate and mild forms of thalassemia are less serious and usually do not require such transfusions.

Specialists have calculated that there are approximately 100,000 people suffering from b-thalassemia in the world. Due to the constant migration of the population from one country to another, there is not a single country where population would be unaffected by the disease. It is worth mentioning that due to the lack of diagnosing and treatment, most patients in developing countries suffering from a grave form of thalassemia die at the age of 5.

In a number of Azerbaijani districts, the frequency rate of this disease reaches record levels. The average proportion of thalassemia carriers is 8 per cent of the total Azerbaijan population, which means that a twelfth of the population are carriers of this pathological gene.

According to the chief hematologist of the country, Professor Cingiz Asadov, the number of people suffering from major thalassemia in Azerbaijan exceeds 1,000. Asadov says that official figures do not often overlap with unofficial statistics. For instance, such people also live in high-land regions, but many of them are not officially registered, do not seek medical advice or do not know what they are suffering from. In order to preserve the life of such people, it is necessary to conduct blood transfusion at least once a month. Since inheritance of the disease has nothing to do with sex, boys and girls are equally susceptible. 

Between life and death 

Murad was born in 1992. After examination at a maternity hospital, doctors did not discover any disorders and told Murad's mother that the child was perfectly healthy. Parents were extremely delighted to have the long-awaited child. As other kids, the boy was agile and inquisitive. Murad would catch a cold very rarely and the only thing that worried his mother was that the child got tired too soon. The anxious parents asked for specialist advice, but doctors quickly calmed them down, saying that the child was growing up, that this wasn't anything extraordinary and would not last long. After such explanations the child's parents tried not to pay too much attention to the problem, but that did not last long.

When the child turned six months, he, unlike normal kids of this age, could not sit. At the age of nine months Murad's mother started noticing paleness on his face and when the child turned 14 months, his stomach started growing out of proportion, while the bones of arms and legs remained unusually skinny. Constant visits to doctors did not help a lot because no-one could establish the exact diagnosis. When Murad turned three years, his health started deteriorating. Then friends recommended that they see a hematologist. After lab analyses the doctor handed down a terrifying verdict - thalassemia.

Doctors treating the child were extremely downbeat. When asked by the mother who had little knowledge of thalassemia at the time, they only shrugged their shoulders making it clear that there was no hope that her only child would survive. Murad spent about a month at the hospital, but there was no progress in his condition. He was slowly dying.

Since there was no improvement, the mother decided to take him home: she thought if things were so bad at hospital, they wouldn't be much worse at home. The mother didn't leave the child's bedside for a minute and was at the same time studying medical literature to find out more about the disease. It was thanks to this self-education that she learned that while thalassemia could not be cured completely, it was still quite possible to lead a normal life with the disease. It wasn't long before she started inquiring which foreign clinic she could apply to. It turned out that there are quite a few specialists in Turkey dealing with thalassemia. Then another problem emerged - lack of financial resources required for treatment. But thanks to well-to-do relatives, the mother and the child soon left for Turkey where the child was actually saved in a short period of time.

Today Murad is 12 years old. He goes to an ordinary secondary school and is friends with ordinary children. If you look at the boy, it is impossible to guess that this cheerful and blithesome kid was once on the verge of dying. He already knows everything about his disease and does not conceal that he has thalassemia. His dream is to become a doctor. He wants to become a doctor and help people with the same diagnosis…

The state is no longer uninvolved 

The life of children suffering from thalassemia and their parents can hardly be described as cloudless. We have to face many difficulties on a daily basis. There is always a lack of donor blood. There is still a shortage of required medicines. The state has recently started providing support to the small category of population. On 29 December 2005, President Ilham Aliyev signed Resolution No 242 on providing state support to children suffering from thalassemia, while the State program of assistance to people suffering from the disease commenced on 15 May 2006.

According to Naila Quliyeva, president of the Savab Dunyasi Association of children suffering from thalassemia, it is difficult to name the exact number of children with thalassemia in Azerbaijan. She indicated that 657 kids are registered with the Association, while according to official statistics there are over 1,000 people suffering from thalassemia.

"No-one can cite the exact number of people suffering from thalassemia in Azerbaijan. This requires serious research which must cover all regions of Azerbaijan, including its most remote areas. Unfortunately, the rural population is not informed of the issue at all. This is why, it happens that a child suffering from thalassemia dies and parents, who failed to seek medical assistance, don't even know the reason. 

"At present, we have great hopes for the Thalassemia Center being built in the Narimanov district of Baku. We hope it will be opened within one to two years. The center will engage not only in treatment - there will also be a genetic laboratory in it unique for Azerbaijan.

"On behalf of all people suffering from thalassemia of Azerbaijan, we would like to express our gratitude to the Heydar Aliyev Foundation and its President Mehriban Aliyeva who, in fact, initiated the construction of the Center.

"Fortunately, the state has also stepped up its effort to resolve our problems. This is why people suffering from thalassemia can now receive medicines free of charge and take special tests. But the shortage of donor blood is still one of the key problems. On 8 February 2006, on Mehriban Aliyeva's initiative, a round-table meeting, "Life without thalassemia", was held. The meeting prompted the Caucasus Clerical Office, the Institute of Hematology and other institutions to join the campaign to resolve the problem of donor blood shortage. These institutions are helping us engage new blood donors," she said.

N. Quliyeva said that children suffering from thalassemia are facing another major problem - more than 90 per cent of them are also carriers of common-source hepatitis and serum hepatitis. All the children were infected with hepatitis at the time of transfusion and now, in addition to the treatment for thalassemia proper, they have to fight hepatitis which, as is known, can lead to hepatic cirrhosis. This treatment is also very costly and not all families have the resources to buy the required medicines on a monthly basis. She expressed hope that the opening of the Center would help children receive free medication for hepatitis as well.

"While speaking about thalassemia, it must be stressed that the disease can be prevented. The point is that a child may and may not be infected if both parents are carriers of the notorious gene. The likelihood of infection in this case is 25 per cent.

"In some countries married couples have the opportunity to conduct a genetic analysis if both fall under the risk group. Future mothers can undergo prenatal diagnosing as early as in the second month of pregnancy. If it is established that the child will suffer from a severe form of thalassemia, the mother can decide whether or not to keep the child. Such practice exists even in Iran where abortions are banned.

"Our association has already made the suggestion to enable married couples to perform the same analysis in Azerbaijan. We hope this issue will be considered by appropriate institutions," N. Quliyeva said.