Life Beyond Sickle Cell - A story of Love, Strength, and Faith
Posted on 2024-12-02 By : Abhulimen Ohiku
My country, Nigeria, was recently dubbed the “sickle cell capital” of the world. It has the highest number of sickle cell disease cases globally, with an estimated 150,000 babies born with this inherited blood disorder each year. About 50,000 of these babies sadly do not make it past their 5th year. Sickle Cell disease affects the shape and function of red blood cells, transforming their normal round form into rigid, sickle shapes due to abnormal haemoglobin, and as a result, these cells cannot flow smoothly through blood vessels, depriving organs and tissues of oxygen.
In children (mostly) and adults, this causes severe and recurring episodes of pain, known as vaso-occlusive crises, and damages organs over time. Complications often begin in childhood, which include frequent infections, acute chest syndrome, and delayed growth, all of which demand constant medical attention.
Despite all this, the care and awareness of this condition at the time I was growing up was limited at best, and lacking at most.
The 2nd of 3 brothers, I was born in June of ‘91 and diagnosed in March of ‘92, on my mum’s 30th birthday. And while I can’t speak to what went through her mind at the time, I can attest to what she made sure stayed in mine till this day. From the moment I could understand what was going on, she was crystal clear that I must define the disease and never the other way round. For a condition so widely misunderstood and incorrectly surmised, she emphasised the complete disregard of information that came from anyone without experience or expertise.
She made my health my responsibility from an early age. “Your dad and I will do everything we can, but the life you want is up to you”. I heard her loud and clear. They ensured I had access to the best possible treatment, nutrition, and care available to us, and I did my part, making sure I took my meds everyday, always stayed hydrated, and never overexerted myself as kids are prone to do.
Despite such privileged conditions, my experience with sickle cell was far from mild. I have vivid memories, countless, as a child, waking up in the middle of the night, screaming in agony, being rushed to the emergency room to be sedated with pain medication after my mother’s attempts to soothe me proved futile.
On good days, I was able to return home a few hours later with just enough pain to keep from crying. Other days turned to nights in the hospital, admitted and treated for one complication or the other. This was a recurring reality for almost three decades.
Despite a less-than-favourable outlook on the disease, I was never advised to limit myself to it. Not by anyone who mattered, anyway. I was taught to be careful and aware of the condition and how it manifested, but more importantly, I was encouraged, by mum and dad, by my doctors, by my brothers, to live in spite of it, so I did.
As a teenager I did very well in school; I was popular; I played football; I played basketball; I loved riding bikes; I read; I danced; I rapped. Later on, I moved to the UK to attend university. I met people; I studied; I partied; I learnt things; I fell in love; I got heartbroken; I attended music festivals; I travelled; I lived.
Oftentimes I would end up in excruciating pain and in some cases, hospitalised as a direct (or indirect) result of engaging in one or more of these activities, but in those moments, with the love of friends and family, I continually chose to live - through books; through movies, and best of all, through music.
While I was busy living, universal research and development concerning (the treatment of) sickle cell disease had made significant strides. As at 2018, potentially curative stem cell transplants that once seemed a distant hope, were now happening across the world, with favourable results, and unbeknown to me, my mum, in addition to juggling a full life and a very busy career of her own, had been keeping up with the studies the entire time. Through her findings, she connected with Dr. Gaurav Kharya, the Director of the Department of Cellular Therapies and Bone Marrow Transplant at Indraprastha Apollo Hospital in New Delhi, who expressed his confidence in my eligibility and suitability despite my age at the time.
She eventually brought me and my brothers up to speed towards the end of the year, as she felt it was a chance we had to take, but ultimately, it was my decision to make. I thought about it, we talked about it, and at the beginning of 2019, in memory of my dad and the 10th remembrance of his passing, it was that will to keep living he helped instil in me that motivated us a family to go ahead with the transplant. At about that time, my mum also shared with us, a vivid dream she had of seeing a white plaque on which was written ‘Something good is about to happen’ in my dad’s distinctive scrawl, with his signature underneath.
The HLA typing test kits sent over to the United States in December 2018 returned in early January of 2019 with the excellent news that my older brother was a 100% match and in July 2019, shortly after I turned 28, my mum and I travelled to India, where we lived for 5 months.
For the first month, I had to be tested frequently and thoroughly to give the doctor and his team a full and clear picture of what they would be working with. My brother (my donor) joined us for a few weeks at the required time for the harvesting of his stem cells. He was a real trooper throughout the harvesting process, and the doctors were able to secure more than enough stem cells for my impending transplant. He was nicknamed ‘Mr. Super Stem Cell’ by the medical team, as a result of the excellent quality of the stem cells retrieved from him.
After Dr. Gaurav (“Doc” as I now call him) was satisfied with the state of my health, he needed my consent before proceeding, but first, he took his time to discuss all the possible risks involved - from stem cell rejection to death. I was unbothered by most of them, but the one that hit me hard was the risk of infertility.
I love children, and for as long as I have known who and what dads are, I wanted to be one. The thought of not being able to experience that someday, really hurt. Doc emphasised that it was a possibility, not a certainty, but just incase, he recommended that I freeze sperm samples before moving ahead. I did, but with a heavy heart. I spoke with my mum, with other loved ones and I was encouraged to deal with what was right in front of me and not what may or may not lie ahead. So, I eventually accepted the situation and the sacrifice that it required, and I gave the green light.
My mum and I moved into the hospital ward, a completely sealed off and isolated room for a 30-day stay and I was put on an intense regimen which included chemotherapy in order to decimate my bone marrow in a way that facilitated rebuilding with the donor cells.
It worked. However, for a whole week, I could barely lift myself out of bed. My head had to be shaved - ahead of the hair falling off which may have been more traumatising - my hands and face had visibly dark streaks; there was blood whenever I coughed up anything, and I had a sore throat so bad I could barely drink, not to talk of eating, and yet I had to keep my nutrition level up because I had no other form of protection against infection. My immune system had been wiped out by the chemo.
After chemotherapy came the main event; the transplant itself which was done on the 23rd of August, 2019. Asides from the prayers of the medical team and my mother prior to the start of the process itself, the transplant turned out to be the least ceremonious phase of the entire procedure. It was akin to an IV flush, albeit with a large syringe filled with stem cells. It lasted all of 90 seconds, to my surprise and my mum’s. The medical team led by the head Nurse/Matron then presented me with a birthday card dated 23rd August 2019 - signifying my 2nd ‘birth date’.
This stem cell transplant was shortly followed by 3 sets of transfusions of blood platelets starting about a day or two later (spaced a day apart) to kick off a 30-day grafting period - the waiting period within which grafting or chimerism as it is also referred to, is expected to happen, all things working well.
For an entire month, my mom and I were isolated in a multiple-daily-disinfected room, with a round-the-clock rotation of nurses in masks and gloves to administer medication, draw blood for tests, check my weight, measure my girth (all very important for determining the medication to administer); measure my water intake against my urine output, and ensure I was diligent with my antiseptic sitz baths and throat gargles 5-6 times everyday. Those were absolutely critical. My mum had a notebook in which she recorded everything like clockwork.
I did all I was told to do; at times reluctantly, but never with petulance. I also intentionally steered clear of any research or hearsay as I didn’t want any information that could potentially cause me to contradict or question those who knew better.
Everything was monitored and assessed to the letter by Doc and his team, and I was given regular progress updates and encouragement till he felt confident enough that I could be released from the ward and continue my recovery as an outpatient for the next 3 months. Despite the positive forecast on the medical side, this was the most challenging phase on a personal level. It required a great deal of strength, fortitude, faith, patience, self-awareness, and day-dreaming to bear the physical, mental, and emotional weight brought on by the process. This was a rebirth and growing pains were part of the deal, not just for me but also for my mum who in her own way as a mother was experiencing the same ordeal.
I found clarity, comfort, and courage through journaling, meditating, drawing, Bible sermons, friends & family, my mum, and most of all, music. I can’t prove it, but I can confidently say that without the joy of music, this would be a completely different story; or there’d be no story at all. My love of and for music assures me of God’s love. It always has. All things considered, it’s an assurance I don’t need, but one I have anyway, and that’s what I cherish most about it.
That love and all the ways it manifests was a sustaining force those 5 months, through the good, the bad, the ugly, and eventually to the beautiful. At the start of December 2019, Doc confirmed the new stem cells were in full effect and my genotype was no longer SS, but now AS. Much to his palpable delight, the success of the procedure had exceeded his own expectations. It was nothing short of a miracle.
My mum and I returned to Lagos just in time for the festive holidays, after a week’s stopover in Dubai to decompress; joined by my other brother who had anxiously remained in the UK all through our Indian sojourn. We had so much to be thankful for as a family. Yes, I still had a year of progressive recovery ahead of me, but we were working with a new level of strength and faith, so the testimony was only ever going to get better, and in the 5 years since then, it has undoubtedly gotten better.
The 2020 pandemic served as an unwanted and unwelcome extension to the medical seclusion I experienced in India, but I chose to make the best of it. I took the time to learn music theory and teach myself to play the piano. I decided I was going to bet on that love for music, and I did. In June of 2021, a few days shy of my 30th birthday, I auditioned for the IMEP Paris College of Music in France and was accepted to study Jazz piano and performance. This added another highlight to what had already been quite the story so far. I was over the moon.
In March 2021 accompanied by my mum, I went back to India for the first in-person check up since leaving in December 2019. All seemed well. Fast forward few months and as my immune system adapted to the new cells, I suffered a case of chronic graft-versus-host disease (GVHD), a common post-transplant complication which affected my eyes, skin, and joints (shoulders and knees). The severity, albeit relatively mild, required an urgent return to India in July 2021, where I was treated by Doc and his team for 2 weeks. This new regimen consisted mostly of corticosteroids, and I was assured that it was simply a setback, not a reversal. With proper care and consideration, the symptoms would improve and eventually resolve over time. I trusted Doc and though these additional challenges were not welcome, I accepted them as chapters still being written.
I recently returned to India, just over 3 years after the last visit, in celebration of my 5th year post-transplant. My GVHD symptoms have all improved significantly, and I wanted a full medical assessment of my progress so far. Doc prescribed the usual blood tests for liver/kidney functions, a full hormone panel, and sperm analysis. I had to relive the anxiety of 5 years ago when I was initially informed of the possibility of infertility. However, I was working with a new level of faith now. I was a sickle cell patient in July 2019, but not anymore! A recurring reminder of what can happen when you are brave enough, despite fear and uncertainty, to go ahead, leaving just enough room for miracles.
The tests came back with excellent numbers, and my fertility was not only preserved, but improved. I was speechless. Doc was visibly over the moon. He revealed to me that he had planned my treatment carefully to protect this aspect of my future; information I was not aware of at the time. I didn’t need to be. I just needed to believe and trust that there was more to this story yet unfolding.
Everyday I wake up is the healthiest I’ve ever been. Not once in 5 years have I had to worry about or deal with a pain crisis. That alone would be a worthy summation of the journey so far, but in addition to that, I have also completed 3 years of music school in Paris finishing with a Distinction. I have performed with bands on campus and at various venues. I have been named by the European Recording Orchestra as ‘one of the top emerging composers in 2023’. I have conducted a symphony orchestra in a formal concert in Bulgaria. I have travelled the world, learnt new things, met wonderful people, including The Bhaskars who are now the Indian extension of my family. And I have done all of this with an openness and an appreciation of a life renewed by the transplant.
As a young boy growing up in Nigeria, I could never have imagined this reality, this story. I hope it inspires and encourages many - those dealing with this blood disorder, their families, loved ones and those who are truly invested in providing the care and succour badly needed. I hope it challenges the leadership of Nigeria at the highest level, and to our numerous philanthropists to commit more to tackling this public health issue very decidedly. More work needs to be done on a national level regarding the administrative, medical, and personal concerns and demands of sickle cell disease in the country.
I have been inexplicably favoured in my life, and I write this with a profound and immense sense of gratitude. What once seemed a burden has become my privilege, and with that, a strong sense of responsibility to be of service to those equally afflicted, but less fortunate. The Sickle Cell Foundation of Nigeria, which my mum has recently been appointed a member of the Board of Directors, has renewed its commitment to improving and maintaining a system that fully supports the needs of patients and their loved ones, and I hope to be an active part of this effort as a counsellor to, and an advocate for those who need it the most. I cannot think of a more worthy way to give back than to help in my own little way, to keep my story going by making it a more familiar one.