By Sola Ogundipe

There is renewed hope for sickle cell patients in Nigeria and West Africa as the first comprehensive bone marrow transplant center dedicated to sickle cell disease is set to offer a potential cure.

This center established by the Sickle Cell Foundation Nigeria, SCFN, in partnership with the Lagos University Teaching Hospital, LUTH,  is designed to provide 24/7 care for patients undergoing the procedure.

It prioritises children under 18 for transplants because their bodies are less likely to reject the donated marrow. Adults, on the other hand, may experience a strong rejection response.

With an estimated 150,000 babies born with sickle cell annually, from which about 100,000 die before their fifth birthday from complications and a lack of proper treatment, Nigeria has the highest burden of sickle cell disease globally.

The National Director/ CEO of the SCFN, Dr. Annette Akinsete,  in a chat, observed that although bone marrow transplant is not a major surgery, it is similar to receiving a blood transfusion and generally involves finding a compatible donor who is a sibling who shares a closer genetic makeup with the recipient.

Noting the bone marrow center’s commitment to quality and safety, Akinsete said by focusing on children and sibling donors, the potential risk of rejection is minimised to the advantage of successful outcomes, thus enabling many sickle cell patients to look forward to a brighter future.

“We do a cure for sickle cell because we have the bone marrow transplant center which helps us to cure the sickle cell disease. We are doing the bone marrow transplant here in Nigeria, and we have a partnership with the Lagos University Teaching Hospital which has a facility for 24/7 care.

“Bone marrow transplant is not a major surgery, it’s like a blood transfusion. It’s not marrow from a compatible donor and we are keeping it very simple. Donors are siblings, brothers, and sisters because they are closer in the gene pool, even closer to each other than they are to their father and mother.

“As an individual, you are 50 percent of your father and 50 percent of your mother. But for you and your siblings, you are like having 100 percent compatibility, so that is 100 percent donation. We keep only children aged 18 years and under because children are not likely to reject the donated marrow because they’re not yet what we call entrenched in their own system.

“Whereas adults are so entrenched in who we are so the body will mount severe responses that can even be fatal. So we keep it simple and take only children and sibling donors. So it’s quality, quality, quality, safety, safety, safety. These are very important.”

Noting that the centre had not started doing transplants, Akinsete however said patients are already online and being counseled.

“It’s not what you start today and do immediately. You need a quality vision. Our patients are already online, so they’ve been online since the last quarter of last year. We have three patients already online for transplantation, but we have the potential for more. We are starting out targeting six transplants in one year.”

Explaining that the Vanderbilt University Medical Center in the US is the technical partner of the SCFN as regards the bone marrow transplant initiative, Akinsete said the Nigerian system is being fashioned after theirs.

“If you look at their own floor, the plan, ours is just like that, so they’ve got people who’ve adapted to suit our local situation. We do 100 percent matching, in America; you can do 50 percent matching. Your father can do as a grown-up, and that’s 50 percent, but we don’t do that. If the sampling is less than 100 percent, we don’t do it, it has to be a 100 percent match.”

The SCFN CEO said one of the reasons why it takes a long time to get to the point of transplantation has to do with the patient’s medical history.

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“We have to take a very long detailed history. Part of the management of sickle cell is blood transfusions, and if you’ve had frequent blood transfusions, you’re not a good candidate for the transplant because your body already has antibodies, There will be graft-resistant disease or rejection and that can be fatal.”

On the origin of the S gene, she said that it came up as nature’s way of protecting the human species from malaria.

“This is because A and S genes coming together is protective against malaria. If you look at the sickle cell map of the world and superimpose it on the malaria map of the world, you’ll see that they are one and the same. So it’s where you have malaria that’s where you have sickle cell disease.  But these days with travel, immigration, migration, civil services, we are having sickle cell disease in places such as New Zealand and Australia.”

Further, Akinsete said a lot of research that has yielded fruit today, by way of hydroxyurea, and bone marrow transplantation, came from research in America.

“We have developed a booklet for doctors and nurses, like a ready reference for the use of the drug, it is a very minute dose that we use for certain cells, and it was based on years of research. I’m very happy to say it makes a big difference.

 “So a child who’s taking hydroxyurea begins like an AS patient who is not ill, because hemoglobin A and hemoglobin F (fetal hemoglobin), which is what hydroxyurea raises for you,  are just one of the same. That’s the kind of hemoglobin you have when you’re in your mother’s womb.

“But when you’re born, because the baby that’s born today looks the same as any other baby, even if you have sickle cell, there’s no difference.

“So the time is only when they’re around six, seven months old that they begin to show symptoms, by which time that hemoglobin F begins to wane, and then the baby’s own genetic block comes up. So you now see sickle cell showing swollen hands, swollen feet, pain, etc. That’s beginning.”

Further, the CEO noted: “We do prenatal diagnosis in very early pregnancy, so when the child is born, we just begin to institute preventative medicine, formal immunisations, and antibiotics and monitor these infections that tend to be lethal.

“AS and AS must come for counseling. Eight out of 10 couples who are AS-AS, after proper counseling, go their separate ways, that’s what my medical experience tells me, but two out of 10 will still be there, so we must share other options that are available to us.

“In technology nowadays, there’s IVF, that helps, and what they do is they came up with pre-implantation genetic diagnosis. Pre-implantation, just as the name explains, pre-implantation of genetic diagnosis. So the big diagnosis in the laboratory, the spermatozoa cells, and the egg cells come up with embryos, are formed, then they test the embryos, and the ones that do not have sickle cell are selected for fertilization.

But you can imagine this is fraught with some ethical and religious consequences. Apart from that, the monetary aspect is also the issue. It’s not everybody can access that, but that is what technology has given us.

“The next thing is genetic engineering. It’s like correcting the wrong spelling because this thing called the sickle cell came from the fact that there’s a wrong spelling of the amino acids. That’s a way to put it very simply. So genetic engineering, as we call it, gene therapy or gene editing, edits the spelling and puts it back together. So there’s no donor. They use your cells, your marrow to correct the thing and put it back.”

On the question about whether sickle cell can be eradicated, Akinsete answered in the negative.

“The simple answer is no. Eradication is a big definition, very bold definition, that’s to mean you cannot see it anymore. I remember when I was a director for HIV and AIDS in Abuja, and then some of our legislators at times said, why don’t we gather all those who have HIV and put them on one island? And they won’t infect anybody. That’s so simplistic. And the same goes for sickle cell. They’ll still be here with us. But as a matter of fact, for instance, HIV, the one that said, we can reduce the prevalence.

“Prevalence means the number of people who have the condition. But more and more are living longer and longer. So we are actually having a higher prevalence. But they are living well. Ditto sickle cell, because they’re living well, we’re able to treat them with better drugs, so they’re living longer and prevalence is actually increasing, and we’re not going to kill them. We’re not going to put them on any island or somewhere. That’s so simplistic and it won’t work. We have to work with churches because they won’t marry some couples that don’t show a certificate that says they are compatible as well. That is simplistic, but the business of sickle cell is quite complicated, very complicated.