Daffodil International University
Educational => You need to know => Topic started by: ishaquemijee on November 21, 2011, 03:45:41 PM
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Master Elias uddin, 3 years old boy came with the complaint of rough, dark, ugly looking patch over lower limbs, buttocks and trunk. It was a term baby. At delivery he was encased in a balloon like membrane and he had difficulty in breathing. He was transferred to Dhaka Shishu Hospital. He then survived but began to develop skin patch weeks later. Finally it was diagnosed as Lamellar Ichthyosis. A genetic disease (Commonly seen in consanguineous marriage) clinically characterized by rough, dry, ugly looking skin (Pig skin). He is a very beautiful boy and very attractive when he keeps himself in dress. But when undressed his lower part of the body is so ugly looking that even his parents do not like the boy. His aunt and uncle have been taking care of him since four month of age. He knows them as his parents and his own parents as uncle/ aunt! Happiness of the whole family has gone away. They have spent lacs of taka and now ready to spend millions more just to get the cure. Few years ago he went to UK but did not get any cure. There is no cure of this boy at present. What a hell they are living in! His parents have consanguineous marriage.
Master Raihanul karim, 5 years old boy came with the complaint of multiple blisters and ulcers over distal limbs, face particularly around mouth and trunk. They developed spontaneously or on trivial trauma. They are frequently complicated by bacterial infections and bound him suffer a lot. Finally heals with leaving behind progressing disfiguring scars. The cycle goes on and on all through the year for last four years. His condition is gradually deteriorating day by day. He was preceded by his brother who had similar disease and suffered up to the age of seven years and died. He is also following the same course for the same destiny. His parents are damn sure and waiting helplessly to see the same fate of their beloved boy. He is suffering from a genetic disease called Epidermolysis Bullosa. The disease is clinically characterized by development of blisters, ulcers and disfigurement of distal parts of body. There is no treatment for it at present. His parents are cousins e.g. have consanguineous marriage. It was a love marriage and they led happy life only for one (first) year. While they decided to have a baby since then their happy conjugal life turned into a hell. The boy came to me many times but not coming for last two years, perhaps he has left us leaving behind some terrible and sweet memories with me. I could still feel his touchy handshake of his soft tender little hand. I could easily remember his helpless appeal to live in this beautiful world with love and affection of his parents and nearest and dearest ones. But sorry to say that I could not do anything for him even with the help from specialist centers abroad.
Didarul Alam, 17 years old attractive boy. Very intelligent, never ever attended in a school for study but he knows both Bangla and English alphabets and can make word and language correctly. He spent his time with studying both Bangla and English daily papers. He nurtures nice dream of life as other boys do at his age. But he has a disease that clinically characterized by anesthetic limbs progressing to involve trunk gradually. He was fine at birth but has recurrent attacks of bad ulcers on foot, knees, elbows and hands. It is so bad that he could not do his normal daily work. His bones of distal limbs are being gradually destroyed. Surgeons have to undertake several amputations fist fingers / toes, then hands/ feet and finally legs. Now he is crippled by left below knee and right ankle amputations. He is going to die in that way. He is suffering from Dejerine Sottas Disease, a neurological disease characterized by progressive anesthesia, traumatic ulcers, destruction and deformity and finally death from there of. Similar disease had been seen in one of his elder brothers. His parents had consanguineous marriage. He will die even before his understanding the meaning and realizing the beauty of life. He knows he is going to die. What a terrible life he is leading!
Rezaul karim, is a seven years old boy of a twin. At birth both of them had brown hairs and bluish black eyes instead of dark hairs and eyes. Parents were happy then. At four years of age, both of them have had eye consultation for visual disturbances. Parents accepted it as usual. Recently past mother had come to me with the complaint of the boy's distal limbs, particularly his fingers and toes turning white. I know the disease is called Oculo-Cutaneous Albinism, a genetic disease. In this disease patient's whole skin will be white together with his hairs. He will face visual problems in daylight. In absence of melanin pigment his skin will burn in sun. He can't tolerate sunlight (You might have seen some of them by this time. They are white people with white hairs blinking in sun on the road). He may develop other autoimmune diseases later on. Patient will suffer life long. Finally there will be premature aging of skin and multiple skin cancers. Here also his parents are consanguineous.
These are the few examples of dreaded diseases commonly seen in consanguineous marriage. There are many respiratory, cardio-vascular, gastro-intestinal, genito-urinary, musculo-skeletal, cutaneous, neurological and endocrine diseases that result from chromosomal or genetic disorders frequently seen in consanguineous marriage. In these diseases parents are apparently healthy and 33% of their offspring expresses the disease. They can cause abortion, still birth, premature death, life long suffering to patients, their parents and family as a whole. There is no treatment for these diseases to date. They have to suffer mercilessly and helplessly. They are easily avoidable if we avoid marriage among cousins. Consanguineous marriage is well accepted in Muslim family. It is also endorsed by the all mighty Allah. It played an important role in generation of a new nation in the early Islamic era. It is associated with some dreadful diseases which are less frequently seen in non-consanguineous marriage.
Now, since 20% of the world population is Muslim there should not be any scarcity of brides and grooms in Muslim society. Considering dreadful hazards and suffering from there of, altogether it could be avoided. Those who have consanguineous marriage and at productive age they should have screening test for diseases at pregnancy and right step should be taken accordingly. In fact there are limited options in our hand and these are either abortion or killing baby at womb which is not permitted by the all mighty Allah or having diseased baby which will cause suffering, again this is not desirable and acceptable to us. In many cases of consanguineous marriage suffering is more than joys of life, it is better to avoid it. If not possible then genetic counseling at pregnancy must be done to avoid such terrible consequences.
(Dr. Delwar Hossain is Professor and Head Department of dermatology and Venereology USTC, Foy's Lake, Pahartoli, Chittagong. Email: delwar_ustc@yahoo.co.uk).
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Probably I will not marry my cousin!