Sickle Cell Anemia - Inherited Red Blood Cell Disorder

I do not remember how old I was when I experienced my first Sickle Cell Crisis. I know I was very young and somewhere between 6 and 9 when I first became aware that something was wrong. By that time I was living with my grandmother on my mother’s side. She had no idea what was wrong with me and concluded that the reason why my arm was hurting me, was because my dead mothers’ ghost was holding on to my arm, trying to take me to the grave with her. As such my grandmother implemented a plan to deal with this Supernatural Event.

Back then in Jamaica, not much was known about Sickle Cell. In fact, we did not know that such a thing existed. My Grandmother was a victim of her situation and upbringing, she could only react the way she did. My early treatment for Sickle Cell Crisis involved Christian praying, a key said to be blessed by Oral Roberts and Hogwarts like Dark Magic of Obeah. I cannot remember ever taking a pain medication while living with my Grandmother on my mother’s side. To treat pain and to ward off the spirit of my mother, who died when I was 9 months, I was given Oral Roberts key to hold in the hand that was hurting. My arm was wrapped with several leaves from the almond tree, after it was rubbed with Kananga water and rum. While my Grandmother recited various scripture verses from either the bible and/or her little book of dark magic.

Not My Maddah but she reminded me of my Maddah: 

'Mother woman' Linda Grace of St Peter Healing Temple in St Thomas

After experiencing several painful Sickle Cell Crisis, my grandmother decided it was time to seek the help of a higher power, a true professional in the Dark Magics. One morning I was told to get dress and was taken to see an old woman who goes by the professional name of Maddah (Mother), down a lane. I had no idea where this lane was as I held my grandma’s hand, being led like a lamb to slaughter. I remember Maddah’s gate was made of rusting zinc and that she had goats in her yard and a big tamarind tree, which I was hoping to raid at some point during the visit. I remember sitting in her house looking around while they talked about my condition and my dead mother's spirit. 

 

Except mine was Blue and in a zinc wash tub

Then I was stripped naked and placed in a big wash tub and given what was called in Obeah terms, a “Bath”. The water was nice and warm, it was blue with slices of Seville oranges and various herbs and spices floating in it. I was washed and then oiled from head to toe with various sweet smelling warm obeah oils heated with a candle. As Maddah recited various Hogwarts like dark magic incantations, she massaged by small frame with forceful precision. After which I was held over a smoking coal stove and smoked like a stuffed pig. Maddah turned me around making sure the smoke permeated every part of my well-oiled body.

Various oil a... 

   
After everything was over, I was given a piece of rock candy and we were sent on our way. My grandmother looked satisfied and pleased with herself, in her head, it was problem solved. My mother, her daughter, would no longer try to hold on to her only son’s arm, the youngest of 3, trying to drag him into the grave with her.

One month later I was back holding Oral Roberts key and my arms again rubbed down with Kananga Water and rum then wrapped in Almond leaf, screaming in pain.

 

Eventually, news of my painful predicament reached my overseas aunt on my father’s side. She commanded my father to take me to the University Hospital in Papine, sometime in the early 70's, I think. There, I was eventually introduced to the Sickle Cell Unit at the Medical Research Council, headed by Doctor Graham Serjeant and his army of doctors and nurses. Or as I like to call it, my home, away from home and my part time family.

By this time my Sickle Cell Crisis was coming fast and furious. I do not remember my first visit to the Sickle Cell unit, but I remember most of the other visits while growing up, because I grew up in that clinic.

Most of my regular visits to the Sickle Cell clinic involved taking part in various studies. We were poked, prodded, and measured as they try to learn more about the disease. Our progress was tracked and charted. We were given pain medication in the form of Panadol for pain and Folic Acid and iron supplements to take daily daily. Eventually we were told not to take any iron supplement just Folic because of the risk of iron overload.

From all the Sickle Cell Crisis I experienced in Jamaica - I was never hospitalized nor was I given anything stronger than Panadol. I suffered greatly as a child, always in pain, at times begging the doctors, nurses or my father to cut my arm off, thinking that would stop the pain or begging for death. Most of my crisis were treated at home, in some cases I was taken to the hospital but in almost all cases it would have been better to stay at home instead of going to the Emergency department at UWI, at 2:00 AM in the mornings. It was a waste of time, nothing was done but it was a welcome distraction from the pain at times. I remember at one point the Clinic had a bus and they would pick me up from home or school, take me to the clinic and then drop me back home again.

One of my most defining, life changing moment came when I was between 8 to 11 and listening to one of the doctors talking to my father. The doctor was telling my father that I would not live past the age of 45. This had a profound effect on my development over the years, as I began to live as if I knew for sure the age of my demise.

Growing up, I was not allowed to do what other kids my age did. I was given a note for school to excuse me from PE (physical education) for the entire time I was in school. So, I spent most of my PE time sitting under a tree just looking at my friends play. I was always the child sitting on the veranda or looking out the window at everyone having fun outside. I was wrapped in cotton wool by my family. I was treated like a Gremlin who was not supposed to get wet, constant shouts of stay out of the rain, stay out of the pool, stay out of the sea, dry off quickly, only bathe in warn water rang through my ears. After a while even my best friends started to decide what I could and could not do, because I had Sickle Cell. Academically, I missed a lot of school in my early years, but I knew I had to make it up at home and I had friend who would bring me the schoolwork.

However, in my adolescent years and based on what that Doctor said, I began to change how I lived. If I was going to die by 45 then I would try by best to live life on my terms. Partying, drinking, smoking, and running with the pack. I was blessed with friends who knew my condition and was not afraid to take a stand against my excesses, even when I protested, they would sidelined me. They were always there when I am sick, if I could not “do road” with them, then they would come by my house and chill.  

 
One thing I never did was to use my sickness to manipulate others. Maybe because my family was so over protecting of me. I was always like a bull tied to a tree trying to break free. One of the positive things that came from hearing about my 45-year lifespan limit, was that professionally I was not afraid to take chances. I knew my physical limitation, so I quickly worked out what type of life I wanted to live. I did not want to suffer or be dependent on other people. So, I dedicated my life making sure I had a profession and the ability to earn a living.

When I was younger, I had lots of Sickle cell Crisis. Then the number of crisis diminished for a while and as I got older the frequency of crises increased again and got more serious. I began to experience more hospitalizations due to Acute Chest Syndrome, pneumonia, and mild strokes. I remember turning 45, at that time I was trying to climb up the corporate ladder as a software developer. Working long hours and playing hard. Nothing happened in that year but every year after, I felt I was living on borrowed time.

Life in any ER

At 47, I was in an Emergency Room, with pneumonia and coughing my guts out. I was alone and sick like a dog and thought I would not be leaving this hospital alive. My sickle cell doctor came to me and she looked at me and I think she knew my state of mind because she did not leave my side and took command of my treatment, bossing everyone around. She held my hand as I coughed up the contents of my lung and as I slumped back in the bed, I said “this is it then”.

She asked why I said that, and I explained what I heard the doctor said when I was a child, regarding my 45-years lifespan limit. She was very angry at the doctor and explained how unprofessional he was, no child should hear that. She said, yes that was the belief back in the 70’s and 80’s but this was a different time with modern medicine. She explained that she had patients older than I was and that it was not my time. My doctor stayed with me until I fell asleep.

I had several more Emergency Room visits and ended up in Intensive care unit once and honestly, I did not think I was going to make it out of the ICU but here I am. Sickle cell is still with me, now compounded with the treat of COVID-19. I am now more proactive, making sure I do what I need to do. I try to make trip to the ER the last resort because I implement preventive procedures at home when I feel a crisis coming on.

For most of my early Sickle Cell life the strongest pain medication I got was Panadol, Tylenol, and Ibuprofen. I experienced my first dose of Morphine in the early 90’s, while away from Jamaica. I had a really bad pain episode and went to the emergency room. I explained I had sickle cell and later a nurse came over with a needle and stabbed me in the leg. I screamed out in pain, then I was in La-La-Land, everything nice.

I went home but the pain continued and was admitted to hospital and they hooked me up to a Demerol dispenser. After a while I lost track of time and reality. I was in space, captured by aliens who wanted to clone me and like Jean-Luc Picard of Star Trek Next Gen, I had to escape. It was a classic escape, being chased by doctors, nurses, and security, I timed everything to perfection, from room to elevator, lobby where security was waiting, zigged right then left and slip through revolving doors. I jumped into the back of a taxi, laying down on the back seat and offered the driver $50 to drive me what was 5 minutes from the hospital to where I lived. After all I had no idea where I was or what time it was or what day, month, or year it was, but I was free from the aliens trying to clone me.

When they ask me if I am allergic to any medication, I always say Demerol. 

My doctor said once that is not an allergy, that is just Demerol. 

I made it home and called family members, who decided they were coming for me to take me back to another hospital. I thought they were aliens from the spaceship I had just escape from. Somehow, they found my relatives and cloned them. So, I went for a knife waiting for them to come through the door. It took them a long time to reach my apartment and by the time they got to my place the Demerol wore off and I came to my senses. Two weeks later I was discharged from the hospital they took me to. 

My friends will never let me forget this and take extra pleasure telling anyone who would listen about my ordeal. To this day I manage my pain meds, no matter what is prescribed. I will dictate when I need something stronger. I am not opposed to morphine, but it is the pain meds of last resort and I do not want it round the clock on automatic regardless of the pain levels. I find some hospitals love to keep patients sedated during a crisis , a tactic I always rejected. 

Normally I refused blood transfusions, even when various doctors pleaded with me that I needed it. I remember by first blood transfusion. After which I kept asking people who did not have sickle cell if this was how normal people feel. I never felt so strong and full of energy before in my life. I was going up stairs 3 steps at a time. I was on top of the world, feeling like the cow that jumped over the moon. after a while I was backed to my old self again, struggling to climb stairs at times. Now I understand why vampires need a fresh supply of blood.