I am perched on the end of Aisha’s bed. Aisha is eight years old, and has βeta Thalassemia Major, the most severe and life-threatening type of the blood disorder thalassemia. Aisha is halfway through her first unit of blood for the day, her little body frail, yellowed with jaundice and a big, tense and distended tummy.
Aisha is curled up on the mattress. She has a beautiful smile despite having just lost her two front teeth. Aisha and her family fled Raqqa City last month. Despite the challenges, they have managed to sustain Aisha’s need for regular blood transfusions, relying on family members for frequent donations of blood, finding and buying the medical supplies and laboratory items in the market, and finding functioning laboratories and clinics to administer the blood as Aisha needs it. What they describe sounds heroic; they did everything they could to continue to get Aisha the care she needed, taking many, many risks in doing so.
A blood transfusion in the hospital in Menbij, Syria. Photo: MSF
When I was working in Syria in late 2013, we knew that there were many children and young people in Northern Syria affected with transfusion-dependent thalassemia and that they were facing increasing difficulty accessing the essential and life-extending services they needed to manage their regular blood transfusion and medication needs. Médecins Sans Frontières had plans to intervene, to start a dedicated medical program to offer these kids the care they needed, but the worsening of the conflict and security situation in Syria relegated medical teams to the sidelines. We have been impatiently waiting for a moment when we might once again access the population in Syria and revisit patients, like Aisha, living with thalassemia.
Thalassemia is not a single disorder but refers to a group of related genetic blood disorders that affect the body in different ways, ranging from mild to severe, and life-threatening conditions. For those affected with the severe form of thalassemia, the haemoglobin in their red blood cells is malformed and less effective at carrying oxygen around the body. This results in life-long anaemia that is often diagnosed in early childhood. In Syria, an estimated 5% of the total population have or carry the thalassemia trait.
Simply put, the blood that is keeping Aisha alive is also hurting her
Aisha is fully dependent on the red blood cells generously given by her family, but that gift is also rich in iron, the metal in our blood that makes our red blood cells function. Without regular monitoring of Aisha’s iron levels and regular administration of specific medication to bind to that excess iron in her body and excrete it, it accumulates to dangerous levels.
A high level of iron risks immense harm to Aisha's internal organs, as it damages the heart, kidneys, and liver, and can cause life-threatening complications. Simply put, the blood that is keeping Aisha alive is also simultaneously hurting her. She urgently needs to restart her medications, called 'chelators'. It’s been nearly three years since Aisha last had regular access to the chelation drugs. Her parents tell me how rare and expensive they were to buy, and that they didn’t have the equipment to administer it. Aisha has renal failure now, even without the blood tests to prove it, her little body is showing me all the signs.
MSF is providing vaccination services to children coming out of Raqqa city as well as surrounding villages. Photo: MSF.
Chronic conditions such as thalassemia are largely invisible in war. After six years of chaos across many parts of Syria, the conflict has resulted in a crumbling and dysfunctional health system, unable to care for people as before. We have seen the reemergence of diseases such as polio, frequent outbreaks of measles, whooping cough and hepatitis A. The declining access to primary, secondary and specialist care has resulted in untold preventable disability, morbidity and mortality. Too many hospitals have been abandoned and damaged by the fighting and the health staff now long gone. The indirect consequences of war on health of this population are immeasurable and will be seen and felt for decades. Children like Aisha, living with conditions that demand regular and specialised care have been forgotten and lost.
Chronic conditions such as thalassemia are largely invisible in war
I despair for kids like Aisha, whose short life has been made even shorter because of this bloody war and conflict, robbing her of a childhood that would have always consisted of blood transfusions and chelation treatment, but one where she might have felt well enough to be able to go to school, to have energy and strength to play with her friends – to live the best way she can for as long as she can.
Today, the task ahead is daunting. To resume chelation for patients like Aisha requires greater resources in laboratory, medication, supplies and trained medical staff. We have never set this kind of care up before, certainly not in a conflict setting.
Chelation can often involve lengthy daily infusions of medication into the skin, usually done at home overnight by nurses or family members. This life-extending service needs to be established and run every day so that families of children with transfusion-dependent thalassemia can rely on it, knowing that the staff and medications will always be there. It’s so clear we need to do this urgently for Aisha and for the hundreds of other children the Médecins Sans Frontières team have identified over the last few months.
Every day with her daughter is precious
I think about the war plane I heard last night and I wonder whether we can promise that we are here to stay; the sound of the jets a stark reminder of the uncertainty of this conflict and its ongoing ability to interfere with people’s right to access healthcare.
I talk with Aisha’s family about what life has been like since leaving Raqqa and their plans ahead, knowing how fragile life is here. I put my hand on her mum’s shoulder and we exchange a glance; she knows how sick Aisha is, how every day with her daughter is precious. I know we might not be able to prolong Aisha’s life; we are late, maybe too late, but we will try, and try to help as many others faced with this same reality. I tickle Aisha’s feet, we joke about being a vampire, she grins.
Names and details have been changed.