Thalassaemia is a blood disorder also known as Cooley’s Anaemia or Mediterranean Anaemia. It is important to understand the structure and function of the blood in our body before Thalassaemia can be understood.
Blood
Blood is pumped round by the heart. It is made up by a yellow liquid called plasma and 3 types of cells - white cells, platelets and red cells.
The white cells defend the body against infections. The platelets stop blood loss when the body is cut. The red cells carry oxygen from the lungs to the body tissues. They are full of red pigment called haemoglobin. There are many more red cells than white cells in our blood.
The commonest from of anaemia is Iron deficiency anaemia which is due to lack of Iron in one's diet. It can be cured by taking Iron medication.
‘Normal’ people have two normal genes for haemoglobin from each parent. Those who have one normal gene from one parent and one altered or abnormal gene from the other parent are healthy because one gene is working well. They are said to be healthy carriers of Beta-Thalassaemia Trait. Since genes are inherited from each parent, at least one parent must be a carrier. People with Beta-Thalassaemia Major have two altered genes from haemoglobin, one inherited from each parent, so both their parents must be carriers.
When both parents are normal - they cannot possibly pass on Thalassaemia Trait or Thalassaemia Major to their children. When one parent has Thalassaemia Trait and one is ‘normal’-50% of children will be Thalassaemia carriers.
When both parents are Beta Thalassaemia carriers - they are a ‘couple at risk’ - 25% of children will be normal, 50% will be Beta-Thalassaemia carriers and 25% will have Thalassaemia Major. It is possible to test the foetus during pregnancy to see if it has Thalassaemia Major.
If Thalassaemia Major is not treated, the child stops growing and becomes weaker and weaker. The spleen gets larger and larger as a result of which the stomach gets big. The cheek bones and bones of the forehead also begin to bulge and death occurs if no treatment is given.
The ultimate cure for Thalassaemia Major is a bone marrow transplant. It involves a brother or sister donating his or her bone marrow. The whole procedure is extremely painful, dangerous and expensive to the sum of over £50,000.
These results suggest that over 7 out of 100 people in our community are Thalassaemia trait carriers. If 2 carriers marry, heir children will have a 25% chance of producing children with Thalassaemia Major. This would be a major disaster for them as well as for the affected child.
Recently the Medical Advisory Board has received several children (patients) from overseas for treatment of Thalassaemia Major. Many have died. It is therefore very important to avoid marriage between two Thalassaemia carriers.
Thalassaemia status can only be ascertained by a blood test. It is therefore recommended that all intending brides and grooms should undergo a Thalassaemia blood test before getting married. It is also recommend that Thalassaemia screening be carried out throughout our community.
Since the increase of HIV and AIDs is also increasing in our community, they might as well have an Aids test too after full counselling.
Since the likelihood of Thalassaemia occurring in an extended family is higher than average, cousin marriages should also be avoided.
For more information please contact the Medical Advisory Board, World Federation of KSIMC, 106/108 Anderton Park Road, Moseley, Birmingham B13 9DS. United Kingdom.
In wake of a world-wide decline in health we need to restrict smoking in Community public areas
One would tend to think that with all the medical advances that we have seen and with all the research that is being done by medical scientists to analyse diseases and seek cures, we would soon be seeing less cancer, less heart diseases and less diabetes world-wide. But this is not the case now and neither will it be in the years to come.
A new report by the World Health Organization predicts the number of cancer cases will double in most countries over the next 25 years. The same has also been forecasted for diabetes. According to WHO, the solution to this global problem is actually quite low-tech. Their group report doctor tells you, "Don’t smoke. Take a healthy diet rich in vegetables and fruits. Don’t become obese. Carry out physical exercise,"
Smoking is mentioned first because the smoking habit is spreading world-wide despite the many warnings that have been issued and despite the statutory warnings on packets indicating that smoking is hazardous to health. Smoking rates are reported to have gone down in the United States but that’s not true for the rest of the world where American tobacco companies have exported the problem. According to WHO statistics, about 60 percent of 18-year-olds in France smoke and the situation is not very different in Frankfurt and many other places.
The situation in Eastern Europe is reported as being even worse and the result is that they have reached levels of lung cancer rates that are the highest ever reported in the history of man. In China as in Canada, the government has recognized and identified smoking as being a big problem and legislation is gradually being passed to ban smoking in public places. WHO hopes this and similar steps will lead to less cancer and heart diseases and, eventually, better global health.
At Community level we ought to also think of restricting smoking in public areas like mosques, madressas, schools, libraries and sports complexes. Well don't we all know that secondary smoke can also kill and what about the many smokers who will unduly grumble? Well, all we have to tell them is that we care about them and hence the smoke zone restrictions!
........you can stop smoking by using the new nicotrol inhaler
PEOPLE trying to quit smoking, who want an alternative to nicotine patches and chewing gum, could use an inhalable alternative that uses typical smoker’s behaviour.
The U.S. Food and Drug Administration (FDA) recently approved the nicotrol inhaler, a nicotine inhalation system for prescription use. The person trying to quit smoking, gets the nicotine replacement therapy by inhaling through a cartridge placed in a mouthpiece.
Advanced Therapeutic Products Inc., which developed the device, claims that it is the first form of nicotine replacement therapy to help control a smoker’s craving for cigarettes, but also provide one of the behavioural patterns that goes with smoking, the hand-to-mouth ritual.
Discouraging cousin marriages
Our Holy Prophet Muhammad Mustafa (S.A.W.W) has said, "........don’t get married to a very close relative because the offspring of such marriage will be weak. Defects out of such marriages will not be rectified until three generations of marriages of non-relatives".(Nafhaatun Muhammadia by Muhammad Jawad Maghniya - Pg. 156).
One of our Mujtahedeen, Ayatollah Syed Naasir Makarim Shirazi has studied this subject in detail and has recommended that cousin marriages should indeed be avoided (Falsafa-e-Ehkaam by Ayatollah Nasir Makarim Shirazi: Pg. 119-122). He says that according to various narrations of our Aimmah, cousin marriages should be avoided. When we asked Agha directly, he wrote to us saying "........such marriages have been discouraged in some of the traditions (ahaadiths)".
Cousin marriages are also known as consanguineous marriages. The practice of such marriages is very old indeed and has cultural and religious ties too. In some religions like Islam it is allowable whereas in Hinduism, it is forbidden.
Some of the reasons of such marriages given above may be plausible like the boys’ and girls’ knowledge of each other’s cultures but surely this can be achieved without marrying within a family. Other reasons given are rather selfish. It is indeed immoral to pursue such marriages at a risk to the lives of offsprings simply to preserve one’s wealth or vanity. Our Aimma have married within the family (only two of them) but have discouraged them. They were pure (Aayat-e-tatheer). Are we?
Medical research has shown that such marriages should be avoided because children born from such marriages are adversely affected. They have an increased chance of being physically or mentally retarded or to be afflicted by defects like blindness, deafness etc.
2) The MAB has abstracts of 19 published research papers demonstrating increased incidence of birth defects in children born out of cousin marriages.
3) A study about a decade ago of British Pakistanis has shown an increasing trend of cousin marriages i.e. 33% in Pakistan but 55% in Yorkshire! Journal of Medical Genetics, 1988.
4) Incidence of Cancer and birth defects in children born in cousin marriages have been found significantly higher. (British Journal of Cancer -1995). Archives of Diseases in Childhood 1994; Ciba Foundation Bulletin No. 29 Pg. 11.
5) In Lebanon, such marriages are discouraged. It is now a legal requirement for couples to be tested for hereditary disorders like thalassaemia, sickle cell disease, asthma, eczema haemophilia, epilepsy, some specific cancers etc. before they can marry. (The Lancet, Vol. 344, November 19, 1994)
6) Consanguineous marriages can increase the risk of inherited diseases in offspring. Prenatal mortality is high among the British Pakistani population largely due to an increased incidence of congenital malformation. Much of this is associated with parental consanguinity. There is also a considerable increase in congenital disorders and handicapping diseases resulting from parental consanguinity. (Social and genetic implications of customary consanguineous marriages among British Pakistanis. Galton Institute. Occasional papers. Second Series. March 1992).
It is therefore very important to avoid cousin marriages. However, it is not haram and such marriages cannot be stopped by force but it is vital that our community is made aware of dangers of such marriages.
The incidence of Thalassaemia is very high in our community (see article on Thalassaemia). The likelihood of the couple (both - the girl and boy) to be Thalassaemia carriers is high if they are cousins and such marriages can lead to the offsprings suffering from Thalassaemia Major which would be a disaster for the child and the parents.
We would therefore recommend to avoid Cousin Marriages. If this cannot be avoided, ensure that (a) there is no family history of inherited diseases like thalassaemia, sickle cell disease, asthma, eczema haemophilia, epilepsy, some specificcancers etc. in either the boy or the girl. (b) the couple undergo blood tests and counseling.
If there is a family history of inheritable disease in both of them or if the blood tests are positive, such marriages must be stopped.