The Sickle Cell Chronicles Part 1

My life changed significantly when I was 12 years old.

There I was lying in a hospital bed in crippling pain thinking what the hell is happening to me? Why does it feel like every bone in my body is being crushed repeatedly?

Less than 24 hours earlier I was bouncing around, playing basketball and enjoying life, completely care free.

Out of nowhere I was completely incapacitated in a strange environment, feeling pain I'd never known while on mind altering drugs.

It didn't feel real, it was as though I'd swapped bodies with an alternate universe version of myself. I prayed a mysterious stranger would appear out of thin air and send me back.

I spent over a week in hospital recovering from the devastation of my first sickle cell crisis. Even after learning what had happened it just didn’t make sense that I could suffer so much pain without any visible injury.

What is this invisible villain?

The lore runs deep, in fact it would be impossible for me to explain everything at once without boring you.

Therefore this will be more of a spider web of tales, ideas and reflections based on 33 years experience of living with sickle cell. And some medical knowledge based on 15 years of study.

Sickle cell anaemia is a genetic condition you’re born with caused by a mutation in the gene that codes for haemoglobin.

Unfortunately this mutation does not give you superpowers, much to the dismay of 12 year old me hoping to see Professor X roll into my hospital room and invite me to join the X-men.

Haemoglobin is the stuff inside our red blood cells that is responsible for carrying vital oxygen as they move around the body to deliver it where its needed.

The altered haemoglobin produced in sickle cell is called ‘Sickle Haemoglobin’ or HbS. Normal haemoglobin is called HbA.

Sickle Haemoglobin is problematic for a number of reasons and can cause complications which effect every organ in the body.

This week we will focus on the most common reason for hospitalisation which is pain.

The Sickle Cell Crisis

What happens as a result of a simple change in shape of a red blood cell is the trump card of sickle cells nefarious plan to ruin peoples lives.

• As these red bloods cells move through the bodies tiniest blood vessels they can get stuck due to the awkward shape.

• This causes a traffic jam, blocking the flow of blood and therefore oxygen to the body area supplied by that vessel.

• The most common areas this happens is in the bones and joints.

• Without oxygen arriving to the cells of the bones and joints the person will experience a severe acute pain crisis in these areas.

Hence 12 year old me thinking my bones were being crushed due to the intensity of the pain I felt. Between conciousness I imagined my bones might be replaced with the fictional metal alloy adamantium making me indestructible like Wolverine of the x-men.

When I think about it having an active imagination was extremely useful to me growing up with sickle cell. My favourite superheroes were always the people that had overcome great adversity, they never gave up fighting even when things seemed hopeless they would pull through bloodied and battered and still figure out a way to save the day. It gave me a lot of strength to align myself with them.

To this day witnessing acts of true courage deeply moves me, as I’m transported back to my paediatric hospital bed fighting my own hopeless battle.

Pain and Prejudice

The pain happens spontaneously at a cellular level so unless you have microscopic vision you would not see any reason for the person to be reporting such agony.

This is important when it comes to racial prejudice and bias in the treatment of sickle cell patients.

Analgesia is a relatively straight forward element of managing an acute pain crisis. However as sickle cell is a disease that primarily affects people of Black and Ethnic Minority heritage, negative stereotypes result in effective pain relief being closely guarded.

Unfortunately in the eyes of the world, if you are black and above the age of 18 and are asking for morphine to treat your sickle cell pain crisis, you are a drug addict.

Racism should be seen as a clinical feature of Sickle Cell Anaemia based on the magnitude of the effect it has on patient suffering.

Systemic racism and sickle cell are two invisible forces that combine to try and destroy you physically and mentally.

At 18 the bright colourful paediatric wards full of rainbows and butterflies fade away into the bleak, dull, misery of the adult world full of hate and evil doers.

The beginners tutorial ends and this is where you start the real game on maximum difficulty with a miniscule health bar against enemies with maxed out (passive) aggression levels.

Playing the game

I’ve been thinking more and more about the role sickle cell plays in my current life. On the surface I appear to function completely normally. I haven’t been admitted to hospital for the past 8 years. I’ve maintained a good career, relationship and social life and I’m pretty content on the whole.

As much as I’m grateful for the life I have I can’t help but think about how much easier it would have been without sickle cell.

I have a chronic disease but for the vast majority of the time I don’t experience any illness. I constantly have to deal with conflicting choices between how I feel in the moment and the potential consequences of triggering a disease process that puts me in crippling pain.

This is the game of sickle cell that I’ve become a master of playing from the age of 12 to 33. People often comment that I’m a very disciplined person as if its an independent personality trait that I was gifted with. The truth is I just can’t afford to not be disciplined. They only see me when I’m doing well and making all the right choices but one wrong move and the kryptonite within my body activates and destroys me.

Over the last 8 years I’ve learned to accept sickle cell as a part of who I am. It’s a chicken and egg dilemma, did I become mentally strong because of sickle cell or did I end up thriving with sickle cell because I was mentally strong. Either way it doesn’t matter. What matters to me now is sharing my authentic self, strengths, weaknesses and learning with others in the hope of helping them become masters of their own game.

That’s all for now.

Lewis

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Check part 2 of the sickle cell chronicles here where I talk about exercising with sickle cell anaemia and how I keep in shape.