Friday, July 16, 2021

Warriors and Survivors

 

 Children Cancer Stories by Rukh Yusuf - Blog # 14

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 – 1

Eleven months old Abdul Ghani is today’s warrior. Abdu Ghani is only child of his parents who belong to Sialkot from a lower middle class family. His mother was worried when he had recurrent fever and abdominal swelling since his birth. She reached pediatric hospital in Lahore after many referrals. Abdul Ghani was diagnosed as case of Bilateral Wilms tumor. (I will discuss Wilms tumor in next blog). So far Abdul Ghani has received six pre op chemotherapy cycles and waiting for surgery. Let’s pray and hope his successful surgery and recovery.

Poor child is having this disease since birth, As per family history, Abdul Ghani’s mother is married to her first cousin, Grandparents of both of them were first cousins too. Most of siblings are married to their first, second or even fourth cousins. There are many diseases labeled as genetic disorders but there are many not labeled as genetic disorder but have relation with genetic mutation and disorder and cousin marriage is directly linked with genetic diseases.

Marriage between cousins is taboo in much of the Western world. In the United States, 31 of 50 states outlaw marriage between first cousins, or allow it only under certain circumstances.

Although cousin marriage is banned in much of the US, the practice is tolerated and even encouraged in other parts of the world including Pakistan. In South Asia and the Middle East, for example, 20-50% of marriages are between first cousins or even closer relatives. They're in good company. More than 10% of people worldwide are married to a second cousin or closer, or have parents who are cousins.

Genetically speaking that means they shared their DNA. And the more closer relative they marry  the more DNA they share, the greater the chance their offspring will have a genetic disease like cystic fibrosis, sickle-cell anemia and Thalassemia.

Most important is, Parents don’t have to be sick in order to give child a genetic disease.

For example: Sickle cell anemia is caused by a mutation in the gene, both mother and father must pass the defective/ mutated form of the gene for a child to be affected. So, if child only have one defective copy, kid is unaffected. Instead, he/she is called a “carrier”. If one carrier marries with a non-carrier, there’s no risk of the kids getting sick. But when both parents carry a defective copy of gene, then the kids have a 25% chance of inheriting two copies of the defective gene and having the disease.

Scientists have calculated the numbers and it shows the risk that the cousins would have a kid who inherits a genetic disease is 4-7%. For the general population, it’s 3-4%. But here’s the catch: There may be  thousands of defective genes for malignancies and blood disorders that could be hiding in family tree and there are chances that these defective gene express themselves in case of cousin marriage.

Moreover, if kids also marry their first cousins and their kids marry their first cousins it’s a recipe for disaster. Because instead of introducing new, potentially helpful genes into the family gene pool, they are recycling the old  and possibly dangerous ones.

As per a study conducted in UAE population about Consanguinity in Leukemia and Lymphomas by Bener et al. The consanguinity rate in the families of patients with ALL (Acute Lymphocytic Leukemia) is significantly higher than those with NHL and HL.

In 'Consanguinity in Context,' medical geneticist Alan H. Bittles of University of Australia examines and explains common misconceptions about cousin marriage from legal, cultural, religious and medical perspectives. Bittles writes, there's no doubt that children whose parents are close biological relatives are at a greater average risk of inheriting genetic disorders. But the risks of illness and early death are three to four percent higher than in the rest of the population.

In native population of Pakistan, the rate of consanguineous marriages is high. Pediatric Cancer prevalence is increasing. Pakistani population and Government need to realize the consequences of cousin marriage and consider legislation as per medical perspective. As a rule, cousin marriage should not be a tradition to avoid genetic mutations and deformities. We shall see Thalassemia and its relation to cousin marriage in next blog.

 

We all pray for Abdul Ghani’s recovery and ease for his family. Aameen

 

Note: Names have been changed to protect identity.

Ref: Business Insiders’s article written by Gene Kim and Shira Polan in 4 Aug 2018

 

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Blog Post # 03 by Rukh Yusuf