A cruel fate

Ten year old Sanduni had been just a four month old baby when she was diagnosed with Thalassemia. Ever since then, she has been making monthly visits to the National Thalassemia Centre (NTC) in Kurunegala for her prescribed blood transfusion. Seeing the plight of this little girl lying in a ward- bed at NTC with a cannula taped to the back of her tiny palm was heartbreaking. This world seemed a cruel place for having brought the fate of a ten-year-old to a state where she would not have another choice than to undergo such pain for the rest of her life.

Sanduni’s story

“My husband and I became concerned of our new-born baby when we realized that she was considerably inactive in comparison to other babies of her age. She was pale and slow and she was even having difficulty sucking the milk when I breastfeed,” Sanduni’s mother P.A. Deepa Kumari said. So Kumari witnessing these symptoms turn worse by the day had consulted a doctor at the Dambulla hospital. 

This young mother says that her first and foremost concern when she learnt of her daughter’s condition was to find the funds for the required treatments and medications. “My husband is a mason so we don’t have the financial capability to afford high-cost treatments. I felt so helpless at this thought,” she recalled. A massive burden in Kumari’s mind had been relieved when she was told that all treatments and medicine required to treat Sanduni’s condition is provided free of charge by the NTC.

Sanduni attends the Beliyakanda Siriwajjana Vidyalaya. “My favourite subjects are Sinhala and Enviornmental Studies,” she said as a bright smile lit up her face. “My daughter is very keen on her studies but she misses school almost every week. On days which she uses the infusion pump with medication she is kept at home. Also each month she misses school on days she goes for the blood transfusion. Her performance at school is average and I feel very happy about it considering the circumstances,” her mother said.

Thilini’s story

Twenty year old Thilini Nisansala is expecting to sit for the GAQ examination at the Kelaniya University to continue her higher studies and also volunteers at the NTC as a librarian.
“I was diagnosed with Thalassemia when I was three months old. Since then I have been getting monthly transfusions and the daily dosage of drugs. The intake of drugs daily is a bit painful when the infusion pump is injected but now after years of it the body is adapting   to the pain,” she said adding that since she started work and studies she has started taking the medication in the nights.

Thilini is from Kurunegala and her father who works at the Co-operative fuel station is the breadwinner of the family. “Providing the drugs and blood transfusion services free of charge is a massive relief for families such as ours because we wouldn’t be able to afford these treatments if we had to pay,” she said.

One could not even know that Thilini was suffering from Thalassemia because she is very active and energetic.  She has many plans for the future but her main aspiration is to complete her higher education and get into a good profession. “I have a younger brother who also has Thalassemia so my parents have had a very rough life bringing us up. I want to someday give a happy comfortable life to my family,” Thilini said with certainty.

Dilemma at NTC

 There are hundreds more similar cases who visit the NTC daily for bloodtransfusions, Thalassemia detections as well as consultations. The transfusions and medications are provided to patients in order to maintain the normality in their bodies against the complications that occur due to Thalassemia. However, seven Thalassemia patients died during the past two months. Although this was reported asdue to  a ‘scarcity of drugs’, the NTC said that the actual rationale behind the situation was a completely different reason: a substitute drug.

“The patients are given a drug named Desferal (Desferoxxamine) for Iron Chelation – to reduce the Iron accumulation in the body due to frequent blood transfusions,” said Dr. Ashok Perera of NTC explaining the background of the situation. According to Dr. Perera the concentration of Iron in a normal human should range between 20 – 400 ng/ml. But this rate rises to about 4000 -5000 ng/ml in a Thalessemia patient due to frequent blood transfusions.

“The excess Iron accumulates mostly in various parts of the body and disrupts the functions of each of the organs,” Dr. Perera explained adding that if the patient does not go through proper Iron Chelation treatments they could suffer from heart and liver failure, pancreas failure which could result in chronic diabetes as well as various hormone -related diseases. 

 “Desferal should be taken 10 -12 hours daily through an infusion pump which is injected into the patients’ body. This situation arose partly as result of the substitute drug for Desferal as it was rejected by some patients due to various side effects such as itchiness, swelling and redness around the injection area. As a result, the Iron concentration of the bodies increase and the patients die of complications which arise due to high Iron concentration in the body,” explained Dr. Perera. 

NTC Consultant Dr. Dayarathne Bandara said due to these deaths  many other  patients too refused the intake of the substitute drug. “It was not a situation we could afford to risk. Fortunately the situation is under control now. The Ministry provided the Desferal drug once more and the patients are continuing their medications routinely,” he said.

Moreover, Dr. Perera said that the Ministry has also provided an oral drug for Iron Chelation named Asunra (Deferasirox). “The patients seem to prefer this drug as it does not require injection,” he added.

What is Thalassemia?

“Thalassemia is a genetic disease; in other words this disease is passed from one generation to the next,” Dr. Ashok Perera of NTC said.
The disease is caused through a defection in the 11th chromosome pair. When one chromosome in the pair is defected, such an individual carries a depressed, diseased gene. “These carriers don’t have the disease. But they carry the genetic defection in their bodies. Once such a pair marries, their children have a 25% possibility of being Thalassemia patients,” Dr. Perera said.

The ailment of this disease is linked to red blood cells. It causes a premature breakdown of red blood cells reducing the life span of an average red blood cell from 120 days to 30 -60 days.

Thalassemia is diagnosed at a very young age. The symptoms of the disease are clear as the children become anaemic. “In rural areas this condition was commonly referred to as the Kehethage Apale or Maandama. As the patients, mostly children were not given the necessary medication their lives were limited to 7 -8 years,” Dr. Perera said.
 
In Sri Lanka the majority suffers from Beta Thalassemia. The first patient had been diagnosed in 1945 by Professor C.B. de Silva. At the moment there are about 2000 Thalassemia major patients in the country and the NTC which is the largest Thalassemia centre in the country treats about 775 of those patients.

•  Account name: Thalassemia Prevention fund
  
•  Account number: 012-2-001-4-0007613
  
•  Bank: People’s Bank – Hospital Branch, Kurunegala Rehabilitation fund
  
•  Account name: Thalassemia rehabilitation fund
  
•  Account number: 012-2-001-0-6905993

•  Bank: People’s Bank – Hospital Branch, Kurunegala

Economic burden of Thalassemia

Dr. Ashok Perera says 5 – 7% of the funds of the health budget is allocated for Thalassemia patients annually. The Government spends nearly Rs.0.4 million on a patient each year. As patients age, the money spent increases to a maximum of Rs. one million.

NTC treats nearly 800 Thalassemia patients and about 50 patients are accommodated at NTC for blood transfusions daily. The drugs, the Iron Chelation device as well as blood transfusions are provided to patients free of charge by NTC. “The Iron Chelation device alone costs Rs. 50, 000 and is provided to each patient. It can be used 10 -15 years,” said Dr. Perera.  A vial of Desferal costs Rs. 350 and an average patient requires at least 100 vials per month. Therefore the cost of medicine alone for a patient is above Rs. 30,000 each month. “NTC requires about Rs. 7.5 million worth Desferal each month,” Dr. Perera added.

NTC provides an excellent blood transfusion service. An average patient requires 800 -900 ml of blood each month. Dr. Perera said that the total requirement of blood is about 650 pints each month.  Dr. Ashok Perera said that the high financial cost which the country has to bear is one of the main reasons to ensure the prevention of this disease and thereby reduce the number of new Thalassemia cases. “In 2007 we received 50 new Thalassemia patients. It was the highest number of new detections so far. Therefore, we launched a program which concentrated on Wayamba, North Western and North Central provinces to carry out prevention and awareness programs,” Dr. Perera said.

Prevention of Thalassemia can be done by preventing the marriage of two Thalassemia carriers or two patients. Undergoing a simple blood test to check the carrier status can determine the safeguard of the future generation from this disease. A full blood count and a chemical analysis of the red blood cells are carried out in order to determine the carrier status of an individual.  Dr. Perera says that it is vital for a couple to check the Thalassemia status in their blood before marriage. Once an individual goes through the Thalassemia screening test, they are provided with a green card if they do not bear a risk. The Thalassemia carriers are provided a pink card. 

“The pink card holders should marry ONLY a green card holder. That is the only method which we can use to ensure the prevention of Thalassemia,” Dr. Perera said.  He said that  about 150 individuals come to NTC centre daily to undergo the Thalassemia screening process.

“As a result of the awareness programs the number of new patients has decreased gradually. Last year we received only 30 patients. But this year once more it has increased slightly because so far we have detected 40 new patients. Our aim is to bring down this number to below ten,” he added. 

Thalassemia highly prevalent in Wayamba

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