Our society expects us all to get married. And with only rare exceptions, we all do just that. However, getting married is a rather complicated business especially at the rate this life keeps evolving. Marriage involves mastering certain complex hustling and courtship games, the rituals and the ceremonies that celebrate the act of marriage, and finally the difficult requirements of domestic life with a husband or wife. It is an enormously elaborate round of activity, much more so than finding a job, and yet while many resolutely remain unemployed, few remain unmarried.
In Africa, our forefathers believed in arranged marriages. They believed the institution was too sacred and valuable to live it to a chance meeting between two strangers. One billion people worldwide live in countries where marriage among relatives is common. Of this billion, one in three is married to a second cousin or closer relative or is the progeny of such a marriage. In some South Asian, Middle Eastern, and north African countries, as many as half of marriages are consanguineous. For example, in Pakistan, half of the population marry a first or second cousin, more than in any other country.
There are many reasons for arranged marriages. For one, marrying within the immediate family guarantees that wealth stays in the family. Partners will have similar socioeconomic status and similar family customs. Also, a good relationship may already exist among parents in law. Furthermore, divorce rates are believed to be lower.
However, there are also downsides, chief of which are health consequences. The frequency of genetic disorders among such children is around twice than in children of non-related parents. It is thought that about 20% of the reproductive problems are genetic or chromosomal. That is why the genetic testing is now an essential part of the fertility study carried out on couples who come for help to have children. Karyotype, chromosome Y microdeletions or the fragile X chromosome syndrome are tests usually recommended in the fertility consultation. A positive result in any of them will allow us to establish a genetic base for the patients’ reproduction problems.
Maryam’s* story started as a very common one. A young beautiful girl catches the interest of a young man who also happens to be her cousin. They are both well behaved, educated individuals, and their parents could not be happier. A love match between family members?
Manna from heaven!
Wedding preparations were well on the way when one uncle suggested that they go for the required pre-marital screening tests. The elderly man was concerned because one of his daughters, who was also Maryam’s cousin, had a child who was recently diagnosed with thalassemia. Thalassemia is a type of inherited blood disorder passed from a parent’s genes to their children. Since it is a genetic problem, there is no known prevention for thalassemia. Thalassemia causes the body to make fewer healthy red blood cells and less haemoglobin than it needs for regular functions. The symptoms range from mild to severe cases of anaemia, weakness, poor appetite, dark urine, delayed development, jaundice and bone problems, especially the bones in the face.
The couple were enlightened enough to take their uncle’s advice. Fortunately, all the tests turned out negative except for a little glitch; both carried the gene for thalassemia. The doctor at the hospital in Cairo explained the results to them.
Similar to Sickle cell anaemia, Thalassemia is one of the hereditary recessive disorders. If one copy of the mutated gene is present in the father and the mother, there is a chance that the disorder will be passed on to their children. If only one of the copies is mutated, the person will be a carrier and won’t have any of the clinical symptoms of the disease. If by chance both the man and the woman are carriers of the same autosomal recessive disorder, there is a 25 per cent chance that they will pass it on to their children.
The families arranged a meeting. Voices were raised, emotions were clouded, and veins became distended. The women wanted to give the marriage a chance; Afterall it was only a 25% chance. 1in 4. It was a risk they could take. The bride to be cried until her eyes were swollen red. The uncle who had insisted on the tests was adamant that the marriage be cancelled. He did not care to see another child suffer. The bridegroom sat silently, watching as his parents decided on his fate. He begged and begged when the wedding was cancelled, citing his great love for his fiancé.
Later, his father called him to his room and soothed him. “What has love got to do with marriage? In our culture, marrying for love is still not highly looked upon, even if you younger, educated people increasingly choose that path.”
When weddings are annulled for medical reasons, people tend to get emotional. They try to rationalise their reasons based on religion, the sanctity of marriage and the fickleness of science. Questions arise: Did our forefathers undergo these tests? Why go looking for trouble? What is all the fuss about? Why make a mountain out a molehill?
They forget that a child born with a disease that cannot be cured is not a mere ‘molehill’. The birth of a child with thalassemia major is associated with many problems for the immediate family, and society. Taking care of people with the disease imposes high costs on the health care system in addition to negatively affecting the family and the society. About 25% of a country’s blood reserves are spent on the treatment of sickle cell anaemia and thalassemia patients annually. Due to the high costs of treatment and the economic, social, and psychological problems imposed on the families and the society, it is important to prevent the birth of children with thalassemia, increase awareness about this disease, and discourage carrier couples from marrying each other.
Studies have shown that some of the reasons people give for getting married against medical advice include giving inadequate or inaccurate information to people, strong emotional attachment between couples, taking the test at the wrong time, family interference and their insistence on marriage, having seen cases with no problems, and specific thoughts and beliefs.
According to the genetic pattern of the disease, if marriages take place between beta thalassemia carriers, the incidence of thalassemia major in the community will increase significantly. The results of many studies have indicated that thalassemia can be controlled and prevented by screening, genetic counselling, and prenatal diagnosis. In many countries such as Italy (since 1943) and England (since 1973), screening and abortion programmes have been implemented successfully. In Iran, more than a decade has passed since the implementation of the thalassemia screening and control programme. However, many cases of major thalassemia are still being reported globally.
The decision to break free of a loving relationship between a couple is not an easy one. And yet, it has to be done. When a person sacrifices his happiness of marrying the love of his life to spare his unborn children any disease condition, that is the definition of true love.
Maryam and her fiancé eventually parted ways, married other people, and had healthy children. Theirs may not have been a ‘happily ever after’, but at least they did their part in making the society a better place.
