REPORT: Sickle Cell Disease: Beyond Talking to Research

Children suffering from sickle cell disease are referred to as “Abiku” by the Yoruba and “Ogbanje” by the Igbo, which translate to mean “born to die” and children that come to cause suffering to their parents.    

A girl with a sickle cell

BY: David Arome

Sickle cell disease (SCD) is one of the silent trends in public health crises, predominately among the black race. SCD is very peculiar to the black race, with Nigeria still being the country with the largest population of SCA globally. It is estimated that the prevalence of SCD in Nigeria is 2% of every newborn with SCD, and 25% of the entire population has the sickle trait.  The potential for sickle cell disease is extremely high in Nigeria, which accounts for almost half of the global burden of the disease.   

In essence, sickle cell disease occurs when a child inherits faulty hemoglobin genes from both parents, usually characterized by typical symptoms of repeated pain crises and frequent fatigue. Sickle cell is a lifelong disorder that, to date, has no known affordable care.

In some cases, especially from a cultural perspective, sickle cell is defined as the way it manifests. Children suffering from sickle cell disease are referred to as “Abiku” by the Yoruba and “Ogbanje” by the Igbo, which translate to mean “born to die” and children that come to cause suffering to their parents.    

The hemoglobin gene is the gene in the body that produces the molecule that carries oxygen around the body. This gene is usually present in the red blood cells of all human beings, and so when somebody inherits a faulty gene that has mutated at a specific point for sickle cell disease, these faulty cells produce abnormal hemoglobin, and this hemoglobin cannot sufficiently interact with the lung. They are not able to carry enough oxygen to other parts of the tissues that need it. In so doing, those tissues need oxygen, and they are not getting it as efficiently as they ought to, which triggers a pain crisis. 

Nanret James is a fifteen-year-old girl and the only surviving child of her parents from Plateau State. She lost her two siblings at a very tender age to the cold hand of the sickle cell disease crisis.

Nanret is also a sickler and often experiences sickle cell crises, which affect her education in and out of school. Nanret, out of curiosity, went to ask a family doctor about sickle cell disease. The family doctor explained to her what sickle cell disease is all about, its accompanying complications, and the cost of managing the crisis. She felt so sad and could not hold back her tears.  

Nanret’s passion as a growing child is to become a medical doctor where she can help and support other sickle cell patients around her to stay alive and imbibe good practices of a healthy lifestyle. 

In a virtual interview with Dr. Idowu Aimola, an associate professor of biochemistry at Ahmadu Bello University, Zaria, a Cohort 1 Fellow of the African Postdoctoral Training Initiative (APTI), a programme implemented by the African Academy of Sciences (AAS) with the support of the National Institutes of Health and the Bill & Melinda Gates Foundation. The university don hinted that beyond the world sickle cell day, there is a need to own sickle cell research as a people and drive the possible solution permanently.  

Dr. Idowu said SCD is one of the only things the black race can be proud of because we own it. It belongs to us and will always continue to be with us throughout our lives, and the only way to live in peace with SCD is to find a way to manage it with those who actually have it.

When asked about the theme for this year’s World Sickle Cell Day, “Building and Strengthening Global Sickle Cell Communities, Formalizing Newborn Screening, and Knowing Your Sickle Cell Disease Status,” Dr. Idowu noted that the theme for this year’s World Sickle Cell Day is quite interesting. Looking at the economics of sickle cell disease, which causes great economic losses, financial burden, emotional issues, and social demographic issues for people living with the disease, getting a lasting cure for sickle cell should always be a drive for us beyond just the fanfare of having NGOs go out and raise awareness and all of that. 

Beyond World Sickle Cell Day, Dr. Idowu emphasized the need to constantly engage sickle cell patients, listen to their perspectives, and learn from them to build policies, plans, research, and campaigns around what they prefer. 

“The government should find a way to keep getting feedback from the sickle cell disease community itself. Also developed a pipeline in which to receive constant feedback from them. And from the feedback there, we can use it to drive policy, research, and management strategies to continue to make their lives more meaningful and rewarding. Socio-culturally, if you want to manage a disease, you must also manage its environment beyond giving drugs”. 

On the misconception about sickle cell disappearing when the patient crosses a certain age, Dr. Idowu said that actually, it never disappears; instead, what happens is that the damages become wider spread and more prominent, and the symptoms diversify and change as the patient continues to age.

When asked about the management strategies for handling the sickle cell crisis, Dr. Idowu noted that the current acceptable global cure for sickle disease is a bone marrow transplant. Though the cure, which is a bone marrow transplant, is not easily affordable by the teeming sickle cell patients due to its huge cost. “Accessibility to this kind of therapy is very difficult, especially for those of us living in this part of the world. A normal bone marrow transplant goes beyond $500,000, and those with sickle cell disease in Nigeria or Africa cannot afford the cost,” he said.

One of the widely used strategies within Nigeria and Africa is to manage the symptoms and prevent people from getting sick with infections, especially malaria. Most sickle cell patients are placed on medications that have to keep infections at bay while also encouraging healthy lifestyle practices among them.   

Ongoing research on Sickle cell Disease; 

Dr. Idowu noted that sickle cell is unique in the sense that those born with sickle cell disease surprisingly do not express the symptoms within six months of their lives. The reason is that within six months of birth, the child survives on the same type of blood as in the womb, called fetal hemoglobin. 

What happens is that within the first six months of birth, the body tends to switch on the Gamma-haemoglobin that helps in the production of fetal hemoglobin, such that at two years, all the genes that help the body produce fetal hemoglobin are turned off, whether you are born normal or sickle cell. So, you are unable to produce fetal hemoglobin as you grow older.

Further extensive studies have also established that patients with sickle disease who have a higher fetal hemoglobin seem to live longer and are healthier than those with a low fetal hemoglobin. 

Although sickle cell research can be challenging, more progress has been made. My research is still in the preclinical stage and is basically focused on understanding the way in which these genes carry out this function and seeing if we can identify a new therapeutic target that can reverse the switch in people with sickle cell disease. 

Currently, we are being founded by the Gate Foundation through an APTI grant and supported through training and capacity building by the National Institute of Health and the African Academy of Science to set up a research program in my institution. 

Using the funds provided, we have been able to enroll participants in the study and acquire state-of-the-art equipment to be able to answer those very unique questions as to how sickle cells keep their fetal hemoglobin while other cells keep theirs off. 

Across Nigeria, we are fully engaged and expanding. We started communication with the Centre of Excellence for Sickle Cell Research and Training, Abuja; Professor Obiagali Nnodu, on how to expand the study cohort that may be going through clinical trials in their own study center.

When asked how to make research translational beyond paper work, Dr. Idowu emphasized that there is a need to synergize and integrate the research sector into a community-embedded strategy that connects the clinical and bench sides as well as the patients. He further stressed the importance of employing transparency in all the steps of our research. Transparency: communicate the exact nature of what you are doing.

On data harmonization, he said, the government needs to own the data generated from its funded research through TETFUND, where the data are not just collated but adequately managed for economic and developmental purposes as well as informed decision-making.


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Jairus Awo

Jairus Awo is a Nigeiran Muiltimedia public interest journalist. He believes in the power of the media as a catalyst to development and societal growth. You can tip him an idea on [email protected]

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