Warriors and Survivors - Children Cancer Stories by Rukh Yusuf - Blog # 15

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 – 2

Six years old Sidra visits hospital regularly with her grandparents to get her treatment. She is patient of Beta Thalassemia major since her birth. Poor soul lives in Sheikhupura and have to travel to Lahore twice or sometimes three times a month for her treatment including blood transfusion.

She started looking pale when she was few months old. Initially, she was advised haematinics and other vitamins and later was referred to tertiary care hospital. Here she was investigated and diagnosed as Beta Thalassemia Major.

About her family, Sidra’s parents are first cousins; there is a trend of cousin marriage in her family. The siblings of her parents are married to their first, or second cousins. Her father passed away couple of years ago. Sidra’s mother looks after other two children and not healthy enough to travel this much for treatment of her daughter. That is why grand parents took the responsibility of her treatment.

Thalassemia is an inherited blood disorder in which the body makes an abnormal form or inadequate amount of hemoglobin. The disorder results in large numbers of red blood cells being destroyed, which leads to anemia. Hemoglobin is made of two proteins: Alpha globin, and Beta globin.

Thalassemia occurs when there is a defect in a gene that helps control production of one of these proteins. There are two main types of thalassemia: Alpha thalassemia occurs when a gene or genes related to the alpha globin protein are missing or changed (mutated). Beta thalassemia occurs when similar gene defects affect production of the beta globin protein. Both alpha and beta thalassemia include Thalassemia major and Thalassemia minor. There are many forms of thalassemia. Each type has many different subtypes.

Alpha thalassemia occurs most often in people from Southeast Asia, the Middle East, China, and in people of African descent. Beta thalassemia occurs most often in people of Mediterranean origin. To an extent Chinese, Asians, and African Americans can be affected.

The patients inherit the gene defect from both parents to develop thalassemia major. Thalassemia minor occurs if patients receive the faulty gene from only one parent. People with this form of the disorder are carriers of the disease. Most of the time, they do not have symptoms. If both parents are carriers (more chances in case of consanguineous marriage) for beta thalassemia, there is a 25% chance their babies have the disease, 50% chance their babies are carriers for the disease and 25% chance their babies don't have the disease or the gene.  In rare instances, beta thalassemia can be passed to a child if only one parent has the gene.

For moderate to severe thalassemia, treatments might include all these:  Frequent blood transfusions. More severe forms of thalassemia often require frequent blood transfusions, possibly every few weeks. Over time, blood transfusions cause a buildup of iron in blood, which can damage heart, liver, and other organs.

Chelation therapy. This is the treatment to remove excess iron from blood. Iron can build up as a result of regular transfusions. Some people with thalassemia who don't have regular transfusions can also develop excess iron. Removing the excess iron is vital for health. To help rid the body of the extra iron, the patients might need to take an oral medication, such as deferasirox or deferiprone. Another drug, deferoxamine, is given intravenously.

Stem cell transplant. Also called a bone marrow transplant, a stem cell transplant might be an option in some cases. For children with severe thalassemia, it can eliminate the need for lifelong blood transfusions and drugs to control iron overload.

Unaware of her disease and possible outcome, Sidra has to come to hospital for her blood transfusion and chelation therapy at least twice a month. Sometimes she needs an early transfusion from her city when her hemoglobin drops suddenly. It is quite difficult for her grandparents to find matched and safe blood. It is not even easy for them to travel to Lahore so frequently in this age. Sidra”s health status is another concern, with passing time her blood transfusion duration is decreasing.

I shall further discuss about Sidra, thalassemia diagnosis and cousin marriage in next blog,

 

 

 

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

 

Note: Names have been changed to protect identity.