September is sickle cell awareness month in the United States. Here in Nigeria, Samira Sanusi and the rest of her team dedicate the month to creating awareness of Sickle cell disease (SCD), and the millions of Nigerians living with it. But there is still much to be done to properly sensitise Nigerians on this disease.
What is Sickle cell disease?
It is a blood cell disorder that causes the red blood cells to take on a sickle or moon shape, due to an abnormality in the oxygen-carrying protein called Haemoglobin. Because of their shape, these cells stick to vessel walls and block the flow of blood, preventing proper circulation of oxygen. The result is that the sufferer does not have enough healthy red blood cells.
According to Amoto Sani, a doctor in Osun state, the condition is inherited by people born with two sickle genes (S gene), one from each parent. “Anyone with the AS genotype is a sickle cell carrier. This means that the person does not have SCD but carries one of the genes that cause the condition,” he adds.
Basically, carriers of sickle cell won’t necessarily develop the disease but are at risk of having a child with the condition.
This is why the medical industry has advised that anyone with the AS genotype should find partners with the AA genotype as that ensures that none of their children will have sickle cell disease.
We know what SCD is, but what’s it like living with it?
“Like a roller coaster ride. You have healthy and happy days, but you are also not sure what part of your day or activity will trigger a crisis,” Samira states.
A crisis is the term used to describe the painful complications of SCD. The most common are sudden episodes of severe pain that can afflict any part of the body, with the duration, severity, and frequency varying for each sufferer.
Others are strokes, paralysis, and ulcers. Other complications often interfere with a patient’s education, career, and psychological development. And sickle cell disease is a lifelong disease.
Growing up with the disease, Samira Sanusi, now a sickle cell advocate, remembers these episodes of pain, anaemia, and infections. “I was constantly sick as a child. I could not play with my friends as much as I wanted to and was in and out of the hospital,” she explains. “But I never really understood what was happening until I turned 15,” she finishes.
At 15, Samira suffered complications that would change her life forever. She lost the use of her legs and could not sit on her own; it took up to five nurses to help her change position.
“In 2003, my complications grew worse, and I had to be flown to Saudi Arabia for better treatment,” she recalls with sadness. An exhausted Samira spent seven years going in and out of the hospital, receiving various treatments for the complications that came with her crises. She confesses that she went through twenty-eight surgeries and was lonely because she was away from her friends and family.
Armed with this experience, Samira helps SCD sufferers understand how to avoid triggering a crisis. One repeated recommendation is staying hydrated, as water eases the flow of blood around the body.
A healthy diet is also important as SCD is sometimes associated with reduced appetite, low calcium intake and vitamin D deficiency that can lead to delayed growth, especially among children. To ensure healthier growth and better immunity against infections, most sickle cell patients include a lot of whole grains, fruits and legumes in their meal plans.
Lack of awareness
With 100,000 babies born with the disease each year, Nigeria has the highest rate of sickle cell disease in the world. One in two babies born with SCD is Nigerian.
But many Nigerians know little about the disease, according to the Sickle Cell Aid Foundation (SCAF), a not-for-profit organisation raising awareness on sickle cell disorder. “People don’t know about genotypes. Even educated people don’t seem to have enough information,” explains SCAF Vice-President Yejide Adewakun.
Checking for sickle cell is simple. A genotype test will show whether a person has the carrier gene (AS), and as long as carriers do not partner up with each other, no child will be born with sickle cell. And you can check your genotype through a blood test in any medical centre.
This sounds simple enough, so why is there still a gap?
Yejide opines that most people dismiss SCD because it is not contagious like the flu or HIV. “They hardly pay attention because it is not up in their face like other glaring diseases,” she adds.
But some of the blame lies with the abject state of healthcare in Nigeria. Many rural communities don’t have access to health facilities, making it difficult for them to learn about genotypes or sickle cell disease. Yejide explains that situation is worsened by the fact that the government pays little attention to the disease, stating, “There is no clear government policy on Sickle Cell like there is with cancer or HIV.”
This revelation becomes alarming when we consider that only 5% of children born with SCD live past the age of 10 in Nigeria. In the United States, where the government even set aside a month for awareness of the disease, life expectancy for those with sickle cell is 42.
In 2017, a bill to make genotype testing before marriage compulsory scaled the second reading in the Senate. It was slated for public hearing after the National Assembly’s recess last year, but till now there has been no word on it.
Is there a cure for SCD?
The only known cure for sickle cell disease is a bone marrow transplant.
Also known as a stem cell transplant, it involves replacing damaged cells in the body with healthy ones. Samira Sanusi, who underwent a successful transplant in Austria, explains that it is not a surgical procedure. “It works by transferring blood from the donor to the recipient. But in this case, new cells go into the bloodstream through an intravenous line (IV),” she explains.
In 2003, she was moved from a hospital in Saudi Arabia. “The doctors told me there was nothing else they could do for me, but my parents wouldn’t give up. They researched the cure for the disorder and moved me to Austria where there was ongoing research on transplants,” she recalls.
Samira confesses that the process of getting the transplant is difficult and believes she was just lucky. “You have to find a donor that is a match. Doctors usually advice starting from family and friends, but there’s only a 30% chance of getting a match,” she sighs. And if you are lucky enough to get a match your body can still relapse and reject the new narrow. “I had the transplant in 2004, and sometime after, the doctors hinted that my body was fighting it. I had to go on anti-rejection medicine,” Samira says. Even with all this, a bone marrow transplant costs thousands of dollars, making it one of the most expensive medical procedures.
Stakeholders have been advocating for more support for bone marrow transplants in Nigeria. Samira does so through her foundation, Samira Sanusi Sickle Cell Foundation. She knows that government help is needed given the cost of the procedure and the expertise required.
The good news is that the Lagos University Teaching Hospital (LUTH), in partnership with Sickle Cell Foundation, is working on establishing a bone marrow transplant centre. The aim is to provide affordable access to treatment for the disease, and at the moment, funds are still being gathered to bring the centre to life. This is one crucial step in the battle against sickle cell in Nigeria.