Hope for parents as bone marrow transplant gives sickle cell disease patients new lease of life

Sickle cell disease is often seen as a death sentence. However, there is hope according to convalescent patients that KEHINDE AKINSEINDE-JAYEOBA met in Lagos.

It was celebration of life and hope for the future for parents and guardians of sickle cell disease convalescent patients at the weekend in Lagos. These patients who had undergone bone marrow transplant (BMT), a life transforming experience, came together to share stories of their experiences and to encourage patients to embrace the procedure.

It was indeed a bitter-sweet experience as parents recounted how they moved from a situation that was considered hopeless, to finding permanent solutions and renewing their hopes. They also shared the satisfaction that comes from giving their children the best medical treatment that is available.

Sickle cell disease (SCD) is the most common inherited blood disorder that affects one’s red blood cells. The symptoms and complications associated with sickle cell disease range from anaemia, which makes the patient feel dizzy, to shortness of breath, and tiredness. There is a painful ‘crisis’ that occurs in the chest, arms, and legs. This often occurs suddenly when the body is under stress from infection, fever or dehydration, stroke, jaundice, among others.

Former Minister of Health, Osagie Ehanire, said in 2022 that Nigeria had 66 percent of the cases of babies born annually with sickle cell disorders in sub-Saharan Africa. Quoting the World Health Organisation (WHO), he said, over 300,000 babies globally were born annually with sickle cell disorders with over 75 percent cases in Sub-Saharan Africa.

According to a sickle cell disease advocate, Ayoola Olajide, there are a lot of problems associated with the disease, paramount of which is lack of support system for the patient.

Olajide, a sickle cell disease patient, who is about to clock 60 years of age in a couple of months, explained that in Africa and Nigeria in particular, there is a huge burden on the patients and their families, noting emphatically that the country is an uncaring society for sickle cell disease patients.

“In Nigeria, if you are a sickle cell disease patient, you’re completely on your own. Nobody except your family cares about you.

“In schools, one finds it difficult to cope with studies especially during the periods when there is crisis. There is no social support for sickle cell disease patients in the country unlike some other countries where there are subventions from government for people with the disease,” he said.

According to him, sickle cell disease patients are not empowered in their early years as fear of the unknown usually makes the parents to leave them uneducated.

“It is so heartbreaking to see sickle cell disease patients in their 30s and 40s begging for alms to take care of themselves as they are not educated and without any form of livelihood.

“Some were even abandoned by their parents, having yielded to advice that the child will die, therefore, there is no need to waste money on them.”

However, all hope may not be lost. Though coming at a price, sickle cell disease patients can experience better days ahead for themselves and their loved ones.

According to a parent, who simply identified herself as Mrs Osakwe, the journey to giving her daughter a new lease of life free of fear and uncertainty was not entirely easy.

However, looking back, she believes it was worth it. Her daughter, Lilian, was diagnosed of sickle cell disease at nine months of age. She went through a period of grief, self-rejection and even unbelief, thinking to herself how could it be?

“I used to see my daughter as an egg that I need to handle carefully so that it doesn’t fall and break. I panicked at the thought of what the future held for my daughter. We pampered her and excluded her from a normal childhood because of her condition. However, she never stopped being hopeful that something could be done about the condition,” Mrs Osakwe said.

In the course of doing research, she found out that a bone marrow transplant (BMT) can actually save her daughter.

Mrs Osakwe, with the help of a doctor, reached out to a community of survivors in the country. They shared their success stories with her and this led her to Dr Gaurav Kharya, a bone marrow transplant senior consultant.

She found out that though it might cost a lot, but with BMT, a new lease of life awaited her daughter.

Last year, she took her daughter for BMT at Apollo Hospital, Sarita Viihar, Delhi, India. Speaking on her daughter’s journey to receiving the transplant, Mrs Osakwe noted it was not easy, but the hope of a better future for her daughter pushed her through.

“I was tested with everything. My patience was tested, my finances were tested, even my faith was tested. There were days all I wanted to do was just to scream at the doctors. Other days I would just want to sit down and cry. There were times I would want to scream at my daughter for refusing to eat the specified diet and there were times I’ll just want to hold her close to me and give her the world, or even if it’s my body she desired to eat,” she recalled.

Looking back, she said she is filled with gratitude to God. “I am at peace now. My daughter is so full of energy. Though we are not yet at the 365 days mark after the transplant, but my daughter is vibrant. I’m no longer scared of sending her to carry out chores.”

For Mrs Ngozi Odigboh, her daughter was diagnosed at six years for sickle cell disease. She said she was shocked because she believed her genotype was AA as revealed by a test she did before her marriage.

“The revelation that my daughter is sickle cell disease patient was so shocking that I wanted to take my life. I’ve had bad experiences. I had a very close friend way back in school who died of sickle cell disease. I also had a cousin who also died of complications from sickle cell disease. To hear that my daughter had sickle cell disease was a big blow. That day, I thought I would kill myself.

“And then she had her first crises. I panicked and the nurses told me that it wasn’t even a crisis, and that there was more to a crisis.

“We spent 11 days in the hospital and the nurses were telling me that it was just a mild one. There and then I told my mother that I can’t beat the thought of my daughter going through any pain more than that.”

Mrs Odigboh carried the huge burden with her for years. “I couldn’t tell anyone that my daughter has sickle cell disease because it was like a stigma to me that as educated as I was, my daughter had sickle cell disease. How could I make such mistake?

“I never knew that genotype test shouldn’t be one-off but something you do over and over again to erase mistake. I felt like a failure despite my personal achievements. I was ashamed and couldn’t tell anyone except my mum. Anytime I saw my child, I just burst into tears because honestly when I saw her, I saw death. My mother encouraged me to keep up the faith, but my faith wasn’t that strong. I just felt my daughter would definitely die someday.

“On this particular day I was reading the papers and I saw Dr Gaurav on Instagram. I told my husband that there’s this India man that said there’s a treatment for sickle cell disease, my husband doubted it.”

Three years later her daughter had another crisis which was a major one. At this point she searched for Dr Gaurav again and connected with Sickle Cell Foundation to confirm the claim of a possible treatment for SCD. They did confirm that BMT was indeed a possibility. “In my research, I found out it can be done in US and UK because I did not want to go to India. But the cost was on the high side so I contacted Dr Gaurav,” she said.

The consultation with the specialist was the beginning of the journey for her. She got lots of testimonials from patients in Nigeria which buoyed her faith.

“It’s a lot to deal with psychologically. I was sceptical because I’ve never seen anyone that has done BMT before. But by the time I got testimonials from people referred by the doctor, I felt light. By the time she did the first chemo, I was confident that I was in the right place because of the changes I saw in her. Now I’m happy. Though it was a tough journey but now we are in a better place. I’m no longer afraid that my daughter will die. I am eternally grateful that I can give my daughter this gift of life.

“The doctor really helped; he was empathetic. He knows his onions. He was professional and talked us through the procedure. Even when my daughter had complications from a virus, he was calm and walked us through it. He even referred to a patient who had similar virus and of same age as my daughter, so that we’ll be rest assured that the phase will pass. His predictions were apt. When he said we should expect fever, it would indeed come.”

Speaking on the procedure, Dr Gaurav Kharya explained that bone marrow transplant was a procedure by which defective bone marrow are replaced with a healthy bone marrow.

“It is called as an allogenic bone marrow transplant where we need a donor. The donor needs to be healthy donor. The donor can be related family donor or in some cases or unrelated donor. We have to check the genetic compatibility between the patient and the donor okay which is done by doing this test which is called as a child.

“Once we have looked at the compatibility, then we choose the best possible donor among two or three donors. We have donor options.

“So once the patient is with us, we do some basic tests to understand what is the physical condition of the child liver function, liver function tests, kidney function tests heart assessment and all. Then subsequently, we start preparing the patient. We give two cycles of chemotherapy so that we can suppress the very aggressive or hyperactive bone marrow and also suppress the immune system. This helps to allow us graft the new system which we’ll be putting in to settle down nicely. This is called pre-transplant preparation phase. This lasts for around six weeks.

“Then, we take the patient for bone marrow transplant which lasts for four weeks. First of all, we need to get rid of this defective bone marrow, which is done by giving chemotherapy for six to seven days. Then we give the patient the cells which we collect from the healthy donor. This is done like a blood transfusion.

“Subsequently, we just allow these cells, which are called the seeds or stem cells to settle down in the bone marrow and start working again, which takes around two weeks or so.

“Once the new bone marrow has started working, the patient is observed for two weeks and then discharged. Patients are to go for regular follow ups on a weekly basis for another 10 to 12 weeks till they reach the 100 days mark to decide the success of transplant for a number of diseases, especially sickle cell disease.”

Dr Gaurav, however, noted that there might be complications. “There are some complications associated with every procedure and so there are with bone marrow transplant. But majority of them are sort of manageable. The success rates have improved significantly. As you might have heard the success rates at our centre are approximately 90 to 95 percent. Fortunately, we have been able to manage the largest number of patients in the world.”