Blood, Guts and Glory: Thalassemia Patients Battle for a Better Life

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Blood, Guts and Glory: Thalassemia Patients Battle for a Better Life

When his company offered Vikram*, a systems analyst in Toronto, a transfer to Montreal with a promotion and pay raise, he was delighted but concerned.

The 31-year-old unmarried Vikram worked twice as hard in his job to make up for the time he took off for medical reasons. The promotion was a justified reward for his hard work. But the question bothering Vikram, the issue that would determine his decision whether to accept the posting, was: Will I find a Thalassemia care center in Montreal?

It was not the first time this unique blood disorder was the deciding factor for the choices Vikram made.

“Thalassemia makes all the decisions for me,” he said.

At nine months, Vikram was diagnosed with Thalassemia, a genetic non-communicable blood disorder. Born and raised in India, he spent his early years in and out of hospitals. His parents moved to Canada but Vikram had to stay back with his grandparents because his parents could not cope with earning a living and paying his medical bills.

“After my grandparents passed away, I struggled with the costs and poor facilities for Thalassemia care in Delhi before deciding to move to Canada,” said Vikram, who shifted to Toronto when he was in his twenties.

He has needed consistent and frequent blood transfusions, ranging from one unit (or bag) of blood every two months to one unit every seven days. Currently, he needs three units of blood every four weeks.

Vikram explained that he visits a clinic for the transfusion. Here, a pump pushes blood into his veins at a constant speed. Each unit takes about two hours to be transfused which means Vikram must spend from six to eight hours in the clinic. This transfusion gives his blood an iron overload.

Vikarm then has to take iron chelation (kee-LAY-shun) which involves infusions of the drug Desferal to remove excessive iron from his blood and organs for five full days.

The Desferal infusion, he added, is more painful. He has a portocathetor, which is a device under his skin in his chest. This is connected to a needle which is attached to a syringe pump every Monday to infuse the drug continuously for five days. The pump, which Vikram wears on his waist, is about the size of an oversized palm-pilot and weighs a pound-and-a-half. It buzzes every time it pushes the medicine.

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"Blood, Guts and Glory: Thalassemia Patients Battle for a Better Life." 24 Mar 2017

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Though he is supposed to take Desferal everyday, Vikram said he takes the pump off two days a week so that he can “be a normal human being and socialize a bit.”

He also takes folic acid, calcium supplements and anti-coagulants for infusion. Vikram said he has had a spleenectomy and several liver biopsies.

In short, Vikram has been sick and under medical care all his life.

Thalassemia patients like Vikram are a minority in North America. Also called Cooley’s Anemia, the disorder mostly affects people from the Mediterranean and South Asia, and found its way to the US through immigrants. About one to 40 per cent of the population in the Indian sub-continent and China is said to be a carrier.

Thalassemia causes reduced hemoglobin, which is the protein in red blood cells that carries oxygen to all parts of the body. A severe form of Thalassemia is usually diagnosed in early childhood and is a lifelong condition.

A person who inherits a Thalassemia gene or genes from one parent and normal genes from the other parent is called a carrier (Thalassemia trait). Carriers often do not show signs of illness other than mild anemia, but they can pass the variant genes to their children. A person who inherits both defective genes will suffer mild to severe anemia. Parents with Thalassemia trait could give their baby three possible combinations: two normal genes, one normal gene or two Thalassemia genes.

“Here is a disease that has no cure,” said Dr David G Nathan, who is a specialist in Thalassemia at the Children’s Hospital Boston’s Hematology/Oncology department. “You need life-long transfusions and there is a risk of getting Hepatitis C from blood transfusions.

“Bone marrow and gene therapy are curing people. But when will the treatment be risk free? Never. Patients need a donor, probably a sibling. The parents have to risk another child because there is only a 25 per cent chance that the child will not have Thalassemia too.

“Pre-natal diagnosis helped reduce the numbers in countries like Greece and Cyprus which had national policies for its cure. There is no national policy in the United States. Even if pre-natal diagnosis has helped to reduce new occurrences among babies, the number of Thalassemia patients has increased because of the growing immigrant population,” said Nathan, author of the book “Genes, Blood and Courage: A Boy called Immortal Sword” (1995) about a Thalassemia patient.

Life expectancy among Thalassemia patients is improving, according to Dr Ellis Neufeld, a researcher at Children’s Hospital. “People born in the last 15 years are doing much better than those born before 1960, for example.

“Treatment for Thalassemia started in the 1960s. By the late 1970s, excess iron in the blood could be removed. Hepatitis screening started in 1990. Patients who were then under 15 are now well into adulthood,” Neufeld added.

According to Cooley’s Anemia Foundation (CAF), there are currently 1081 patients in the United States though the Foundation does not know of every case. A 1993 survey by the National Institute of Health (NIH) had found 518 people suffering from Thalassemia in the country. The National Heart, Blood and Lung Institute (NHBLI), a department of NIH, reported 21 deaths due to Cooley’s Anemia in 2001, based on death certificates.

A child born with Thalassemia will have to endure a life time of monthly blood transfusions but, thanks to medical advancement, the once fatal disease is now a chronic illness.

Gargi Pahuja, who recently graduated from a law school in New York, was diagnosed with Thalassemia at 17 months. Now 30 years old, Pahuja was born in the U.S. after her parents emigrated from India in the 1960s. Neither of her parents had heard of the disorder before their first child was born.

Like Vikram, Pahuja spent a lifetime receiving blood transfusions. The disease has hindered her education more times than she can remember. She often had to postpone exams and assignments though she was able to “catch up most of the times.”

Pahuja has biweekly transfusions and chelation therapy for 12 hours, four days a week. She says that the only time she felt like she was dying was when she was hospitalized for 10 days in her senior year of college.

“At the end of that ordeal, I knew there was a possibility I would not survive,” she said. Support came from her parents and the New York-based Thalassemia Action Group (TAG).

“Being a part of a group has given me a tremendous sense of self. It is humbling to know that there are others worse off than you are — it puts your struggles in perspective. It has helped me realize that my purpose is to help others like me fulfill their goals,” said Pahuja, who lives in New York.

Pahuja was allowed as normal a life as anyone else till she went on Desferal. “I couldn’t go to other people’s houses for sleepovers, and I couldn’t explain when people asked why I missed school every two weeks. For the most part though, I was a normal kid,” she said.

“How people reacted (to my condition) depended a lot on how old I was. As an adult, I am open about everything, except maybe with my employers.” Pahuja, who is one of TAG’s presidents, took the Bar for New York and New Jersey a few weeks ago and does not have a job yet.

Vikram was luckier with his previous employers, who were supportive of his frequent need to stay off work. “I would volunteer for projects and work during weekends especially when the others in the team were unwilling to do so. I would work extra hours to make up. I am on probation in my current job and have not told my manager about my problem because I believe he is not likely to take this news well. I will tell them once I am off probation.”

Other than discretion in workplace, Nathan explained the psychological effect of the disorder on patients. “They used to be of short stature, their bones would be distorted… Now we can keep them looking normal. But kids suffer from the knowledge that they are always dependent and permanently on medical care.”

Vikram, who has worked as a volunteer for the Thalassemia Foundation of Canada since 2000, said he knew of cases where parents have been overprotective of their Thalassemic child and neglected their other children.

Permanent care also leads to high costs. Neufeld estimates annual expenses to average “tens of thousands of dollars”. “The 500mg vial (to remove excessive iron instead of the painful chelation therapy) costs $15 and you have to take four every day. So that alone is an expense of $60 a day.”

Pahuja estimates that Thalassemia treatment costs her about $1000 a month, taken care of by insurance and her parents. Vikram’s expenses are covered by his work insurance but he said if he had to pay the costs himself, it would be Canadian $200 to $500 a month.

Since pre-natal diagnosis is feasible, the elimination of the disorder is not impossible, but improbable. In Britain’s Northeast Thames region for example, the number of Indian and Cypriot children born with Thalassemia fell by 78 per cent after prenatal tests became available in the 1970s, according to the 1990 winter issue of Newsweek. It helped people decide if they wanted to proceed with the pregnancy or not.

According to Rudi Viscomi of the CAF Massachusetts Chapter, the New York area reported five deaths last year due to Thalassemia while Boston has not had any Thalassemia fatalities in four years.

The Centre for Disease Control and Prevention (CDC) received $2.5 million from federal funding for research over five years to ensure that people with Thalassemia are “identified, and have access to appropriate information, treatment and prevention services.”

CDC’s National Thalassemia Program identifies five federally funded centers in the U.S. Neufeld works for one of them, the Children’s Hospital in Boston, as part of the NIH-sponsored National Clinical Research Network funded by the NHLBI for treating heart diseases resulting from Thalassemia.

Pete Sampras, of Greek parentage and once the world’s best tennis player, suffered from mild Thalassemia, as reported by the Boston Globe a few years ago. It used to tire him sometimes but did not stop him from winning 14 Grand Slam titles.

Pahuja has a similar attitude — of not letting the disease bog her down. “I don’t know if I would call a chronic illness an inconvenience. It is a struggle, but no more than any other chronic illness. I could always do anything I wanted. I was active in sport too. I have led a normal life…”

Nathan gives credit to patients of Thalassemia.

“Health researchers owe these patients a great deal. They have been the models for clinical research. Thalassemia and sickle cell anemia have given birth to molecular medicine.”

Both confidence and support can give the patients comfort. As Pahuja said, parents need to know that as long as their child feels loved and accepted, there is nothing a family can’t handle. “A good attitude goes a long way. Be more educated than your doctor about your child’s condition and teach your child that,” she said.

* Last name withheld on person’s request

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