Warriors and Survivors

Children Cancer Stories by Rukh Yusuf - Blog # 16

I am Rukh Yusuf, Clinical Pharmacist, also specialized in Total Parenteral Nutrition and Bone Marrow Transplant. I have been working in Pediatric Oncology unit of a public hospital for several years. The mission of this blog is to bring to you the real-life stories of child patients suffering from cancer. Cancer is still a difficult disease to handle and treat. However, when it strikes the children, some so young that they cannot even speak, their agony is beyond expression and words. Let us pray especially for children suffering from cancer for early and complete remission. May Allah shower His Merciful Blessings upon them. Aameen.

Possible Effects of Cousin Marriages – 3

Last blog’s warrior was six years old Sidra from Sheikhupura suffering with Beta Thalassemia Major. She is a child of consanguineous marriage. The siblings of her parents were also married to their first, or second cousins. There is a trend of cousin marriage in her family. With repeated transfusions, iron keeps accumulating which is a common complication of thalassemia syndromes, and which could lead to the development of organ damage. Usually In these patients, iron deposition in parenchymal tissues begins within 1 year of starting the regular transfusions.

Today, we shall talk about the role cousin marriage as a reason for thalassemia. Thalassemia is an autosomal inherited disorder of hemoglobin which is an important oxygen carrier of our body. Without hemoglobin our cells would die.

A person inherits Hemoglobin gene from his/her parents. If only one of the genes is faulty, the child gets only half of the corrupt hemoglobin. This decreases the total oxygen carrying capacity of blood, but still the other half of the hemoglobin is intact and that can transport oxygen. This is called Thalassemia minor. People who have a Thalassemia trait can have it silently for life without knowing it, but trouble starts when both copies of the gene are corrupt. This occurs when two people with thalassemia trait marry and have a child. That child has 25 percent chance of developing full blown thalassemia major and fifty percent chance of developing thalassemia minor.

This is also a fact that the risk of inheriting thalassemia in Pakistan is almost the same between cousin marriages as between marriages outside family. It is so because even if there is no cousin marriage in the immediate generation, there would be history of it in the previous generations. And, although being widely reported, inbreeding is not the only cause of increase in thalassemia, we are observing in Pakistan.

Cousin marriages may come with many genetic diseases like cystic fibrosis, Gaucher’s Disease, Hemochromatosis, Sickle cell disease, Phenylketonuria and Thalassemia. The list is longer than above. As per study by Aideen Maguire et al. “A child of consanguineous parents is at increased risk of common mood disorders and psychoses”.

These diseases are a long-term burden in terms of healthcare cost and lifelong misery not only for patient, but for the whole family. There is urgent need for prevention and cure of such diseases. These diseases can be prevented by two ways:  One: Cousin marriage must not be a custom within family. Marriages should be planned within and outside of the family equally.  In this way genetic defects may not get strengthened in generations.

Two: Taking a blood test before marriage. “In Indonesia and Iran, it is a common practice that if you’re opting for a cousin marriage the couple-to-be have to take a blood test for these diseases,” By doing so, these countries have almost gotten rid of thalassemia.

In Pakistan, the annual number of infants born with genetic and other disorders related to consanguineous marriage is the highest as compared to other countries from Eastern Mediterranean region.

Although the management and control of genetic disorders including thalassemia and mental health issues, is a difficult task, it can be achieved via the assistance of prenatal diagnosis and prevention programs. The only way to prevent genetic disorders in Pakistan is to make and implement a law for widespread screening before marriage. Moreover, cousin marriage was never forbidden but making consanguineous marriages as a custom from generation to generation should be avoided to limit the future burden of complex genetic and other complications including thalassemia.

 

We all pray for ease for Sidra and her family. Aameen

 

Note: Names have been changed to protect identity.