In occasion of the International Thalassaemia Day 2021, Ali Sattarpour, 45, France, Fédération SOS GLOBI Vice President and TIF’s Thalassaemia Patient Advocacy Group (T-PAG) Member, recounts a life of struggles, hopes and aspirations with thalassaemia and stresses the need for better recognition of this highly restrictive blood disorder.
He is not used to talking about his illness but wants it to be better recognized. Throughout his life, he managed to hide his pathology from his colleagues and friends. For fear of prejudice above all, he said. This inhabitant of Issy-les-Moulineaux (Hauts-de-Seine) suffers from this rare genetic disease that affects haemoglobin, a protein responsible for transporting oxygen in the blood. B-thalassaemia prevents the normal production of haemoglobin. People who have it (713 patients in France) are then severely anaemic and need blood transfusions every 3 to 6 weeks to survive.
“There was a time, in my childhood and adolescence, when transfusions were equated with AIDS, I thought that by telling people that I was transfused, they would believe that I had AIDS. That’s why at school, my friends didn’t know. There are still five or six years, nobody knew it”, tells Ali who is of Iranian origin.
With age, Ali has put his fears into perspective. He even became the Vice President of SOS Globi, the French Federation of Sickle Cell and Thalassaemia patients, which participated in the International Thalassaemia Day Its goal: to campaign for better recognition of this highly restrictive disease.
A whole life punctuated by transfusions
“I have to be transfused approximately every month, my whole life is punctuated by this,” explains the professional filmmaker. ”I am always very tired a week before and then I feel good again afterwards. When we have something important to do, we put it back after the transfusion, I also organize my trips… ”.
With the Covid and for the very first time in his life, Ali Sattarpour was afraid of running out of blood bags. “During the first confinement, we all wondered if we were going to have enough, but the EFS (Editor’s note: French blood establishment) reassured us by explaining that there were three months of reserve. “
Beyond transfusions, patients with thalassaemia must take many drugs daily to compensate for the lack of iron they cause. Since the 2000s, this treatment has improved considerably because before the arrival of the tablets, iron chelators (which reduce the iron level in the blood) had to be injected daily for ten hours. “The injections were done at night. When you’re a child and a teenager, psychologically it’s very difficult to live with”, he adds.
Despite regular episodes of fatigue and the inability to make great efforts, Ali Sattarpour has come to terms with all these constraints. “It didn’t stop me from living a normal life.“
Hope for a cure with gene therapy
If very few people in France are affected by this disease affecting red blood cells, it is particularly widespread in certain countries of South Asia, the Middle East or southern Europe where malaria was endemic. Around 100,000 children are born with the disease worldwide each year. It results from a mutation in a gene that preserves malaria. When two carriers of this mutant gene have a child, there is a one in four risk of developing β- thalassaemia.
This is what happened to Ali Sattarpour followed since the age of 10 by the Professor of Medical Genetics, Frédéric Galacteros, at the Henri-Mondor hospital in Créteil (Val-de-Marne). “Today, patients can live until a relatively old age – we are starting to have β-thalassaemia patients who are over 60 years old – but at the cost of regular visits to the hospital to be transfused”, underlines the specialist. The diagnosis is made very early, around the age of five to six months when anaemia is revealed.
Recently, great advances have been made in gene therapy using bone marrow cells from the patient himself. “This therapy offers real prospects for effectiveness. The patient’s marrow is recovered, treated and reinjected, it is very heavy, there is chemotherapy but the cost-benefit ratio must be measured”, underlines Professor Galacteros. This “enormous” hope has also made it possible to change the discourse of doctors: “Before, we used to say to our patients: You are fighting for your survival; now we can tell them: You are struggling to be in perfect shape for the time of gene therapy for true healing.’’