The New Nation - Internet Edition

Syed Deedar Bakht

New modern technology have made inroad to help solve many complicated problems including health problems in the word. Intensive research for invention of appropriate medicine for complicated disease is a continuous process. No doubt researcher have achieved a lot of success for curing and preventing complicated diseases. Efforts are also being carried out for curing complicated fetal diseases like cancer and diabetic etc. But unfortunately not much concentration has given for curing and preventing very complicated and fetal disease "Thalassaemia."

Thalassaemia is a blood related genetic disorder inherited from parents. The main source of the disease generates from detective gene of the body. The disease cannot be caught from any other sources, only the parents in "Thalassaemia trait" put their beloved children life through a futile exercise for a normal life and half defeated a battle. Till now the disease cannot be prevented and cure is beyond the reach at almost all people in Developing countries like Bangladesh.

Thalassaemia patients need life long regular blood transfusion along with other medication to lead a normal life. Regular transfusion of only RBC component of blood help keep the patients average haemoglobin level normal. The patients should be transfused when haemoglobin level comes down below normal level. This is such a fetal disease that kills the RBC so quickly for a requirement of blood transfusion within about 4 weeks to maintain haemoglobin level normal.

Till now there is no other means for permanent cure of the disease, inadequate blood transfusion and absence of essential medication leads to obvious inevitable death of the patients within 5-15 years of age. With proper and timely blood transfusion and medication patients can lead near a normal life for about 50 years.

Thalassaemia can be classified as thalassaemia minor and thalassaemia major.

Thalassaemia minor means the subject is a genetic carrier of the disease but does not show any symptom of the disease. The patients may occasionally experience mild anaemia which can be cured with normal treatment for anaemia. Only special blood screening can detect a person as a carrier of Thalassaemia.

Thalassaemia major means, the patients has inherited the disease. In such case both the father and the mother must be carrier of Thalassaemia. If only one of the couple is a carrier, offspring may at best became a carrier but cannot be a diseased one. When a child is born with Thalassaemia major, it is obvious the parent of the patient are carriers and as such the offspring has inherited the genetic disorder contributed by both father and mother where by his bone marrow produces RBC with defected haemoglobin. This haemoglobin are too short lived and defective, and incapable of generating sufficient oxygen carrying capacity. As a result the subject becomes acute anaemia and in absence of proper diagnosis and treatment the patient is destined to die within 3 to 5 years of birth.

It is extremely interesting to note that till today thalassaemia is not found all over the world for the reasons not clearly understood. The main concentration of the disease is in the countries around Mediterranean ie, Greece, Italy, Saudi Arabia, Egypt, Afghanistan, Pakistan, India, Bangladesh Myanmar, Thailand, Malaysia, Indonesia, Maldives, Srilanka, etc and some other countries in the region.

In Bangladesh, till recently people were not at all aware of this fetal killer disease.

With the advancement of technology, Bangladesh Doctors also felt the necessity for introduction of modern process of diagnosis of the disease including application of modern technology. Very recently Bangladesh Thalassaemia Society (BTS) carried out "Thalassaemia carrier detection survey" among the marriageable boys and girls. The sample survey reveals that near about 10% of the population is Thalassaemia carrier. Considering above percentage of carrier and current population of Bangladesh, about leone) crore 40 lac people are carrier and 8 to 10 thousand children are estimated to born every year and all most all of them slowly pass away within 3-5 years without any diagnosis and proper treatment or maltreatment particularly in the rural areas where treatment facilities are not adequately available to combat this fetal disease. The diseased children also fell victim in the rural areas due to ignorance particularly in the poorer section of the population, who hardly any means to treat their wards. It is yet be estimated how many diseased children are dying every year due to this incurable fetal disease. It is also estimated that about 20000 children are counting their last days every year amidst anguish and horror.

This fetal disease was almost unknown in our country. Very recently Bangladesh Thalassaemia Society (BTS) and some other organisation have made mass awareness campaign through symposium and seminar to help people know about this killer disease and its treatment regimen. Imbued with social responsibility many philanthropist and volu it is very scanty and meagre.

Without regular blood transfusion, the Thalassaemia patients cannot lead normal life. The inevitable consequence of repeated blood transfusion, Iron contents of the blood forms deposition in the heart, lunge and kidney and due to iron overload, the patients suffers from many other complicated diseases and develop hepatocellular damage, skin pigmentation and growth retardation followed by pubertal failure, insulin dependent diabetes mellitus, hypothyroidism, cardiac failure, and infections in some other organs in the body. Iron chelation therapy throughout lifelime is only way out to lead normal life. But it is too costly to bear by the low income group patients. Till now the disease cannot be prevented and cure is beyond the reach of almost all people in developing countries like Bangladesh. It deserves mention this hereditary disorder can only be cured by having very very costly and risky bone marrow transplantation which is yet to be available in Bangladesh .

In absence of regular treatment in the state run hospitals and private clinics, the BTS which is formed with the parents of the Thalassaemia Patients they through it the obligatory to shoulder the responsibility to provide treatment to patients suffering from Thalassaemia, particularly to those who belong to poorer section of the people. The BTS also felt the need to run the hospital with an objective to provide facilities like, Blood transfusion service, iron chelation therapy service, Blood bank for Carrier and disease detection services, Vaccination services, Educate people to be aware of high incidence of the disease, Motivate people to donate blood voluntarily.

Thalassaemia is such a complicated and fetal disease, that it needs to be explained and outlined before the people who are yet to know much about the massive disastrous effect of the disease. For that matter massive mass awareness campaign is needed to be launched throughout the country.

People are to be educated about the disease and its effects on a family, and crippled. The family virtually becomes economically/financially ruined for the treatment of their disease d child. Following steps can help contain the disease and further spread of the Thalassaemia.

(a) Message to reach to all the marriageable boys and girls to screen their blood before marriage so that their future offspring is out of possible allack of the disease.

(b) Guardians of the marriageable boys and girls are to be motivated to screen the bloods of their words before settlement of marriage.

(c) Government to instruct the marriage registrar to see blood screening report from doctors to ensure that either at the boy or the girl is free from Thalassaemia trait.

(d) Campaigning to reach at all Upazila level.

(e) Blood screening facilities are to be available at all Upazila level.

(f) Blood transfusion facilities are to be provide at all Upazila level Hospitals.

Massive mass awareness campaign is only way out to educate the people about disastrous effect of the disease. Such campaign will also educate the people to create social movement to help prevent the disease and make Bangladesh free from Thalassaemia.

(The writer is not a doctor. Inaverdent error is regretted)