Dubai: Deaths occurring due to thalassaemia and birth of children with thalassaemia major have been reduced to one in Dubai since 2017, Dubai Health Authority’s Thalassaemia Centre announced on Sunday.
This genetic blood disorder affects nearly 8.5 per cent of the local population, globally one of the highest rates of incidence.
A new kind of peripheral transfusion system for iron chelation that does not allow iron toxicity in patients has been the main reasons for reduction of mortality. Iron toxicity is one of the major causes of death of thalassaemia major patients. Education and genetic screening has helped in bringing down the birth of babies being born with this blood disorder.
Dr Khawla Belhoul, director of the Thalassaemia Centre, said that the mortality rate has been gradually declining with efforts of the centre’s staff. In 2013 eight patients out of 438 patients (on regular blood transfusion) died, in 2014 six out of 447, in 2015 three out 436 and in 2016 and 2017 only one patient died each year out of around 425. s
Speaking to Gulf News, Dr Essam Dohair, senior haematologist and coordinator at the centre, explained the manner in which the new iron chelation method worked. “The patients who come for transfusion are unable to avoid the iron build up that damages their organs and results in high rate of mortality. We are using the Peripherally Inserted Central Catheter (PICC) line. This catheter extracts the excessive iron 24 hours a day, seven days a week, not allowing iron toxicity in the patient’s heart and other critical organs. The catheter is able to transfuse iron chelation medication so that iron is pumped out and does not harm the heart muscles.”
Dr Dohair added that active education of couples with the dormant gene for thalassaemia and genetic counselling and screening of couples before marriage had helped avoid thalassaemia major cases in the last two years. “In addition to this the UAE government pays for bone marrow transplants of Emiratis suffering from thalassaemia. We have had excellent results with nearly 35 individuals with thalassaemia major being cured of their condition with bone marrow transplant, only last year.”
Dr Dohair said that the centre was also helping expatriate patients with the condition by helping raise money and liaising for their bone marrow transplants at the best centres across the world.
Furthermore, since 2007, the centre has begun exhaustive community outreach and awareness activities, which has directly resulted in reducing the number of thalassaemia cases in the emirate.
The X-ray department at Rashid Hospital also had a big role as they were the ones who conducted the iron test to check if there is iron in the heart. The Dubai Blood Donation Centre also ensured to supply the centre with the needed supply for the blood transfusion.
Dr Belhoul said that the centre was moving towards completely eliminating thalassaemia major. “Our main objective is try to bring the mortality rate to zero, improve the quality of life of thalassaemia patients and focus on a cure through gene therapy.”
DHA’s Thalassaemia Centre was the first to be established in the Middle East in 1989. It currently has 850 registered patients of which 429 are on regular blood transfusion.
What is thalassaemia?
Thalassaemia is genetically inherited blood disorder that affects an individual’s ability to produce haemoglobin and red blood cells resulting in a lower percentage of haemoglobin causing anaemia and fatigue. If the gene is a dormant one the child is said to have thalassaemia minor where he suffers from anaemia but does not require blood transfusions. If both parents of the child have the thalassaemia minor mutation then he has a 25 per cent chance of developing the major form of the condition. If either one parent has thalassaemia major then their child has a 50 per cent chance of inheriting thalassaemia major.
A child with thalassaemia major requires blood transfusion once a month to replenish his haemoglobin. He requires iron chelation therapy to reduce the iron load in the body, lifelong. Iron toxicity of organs causes early death and patients of thalassaemia major are also sickly and can have several other bone and physical deformities.
