It’s Called A Crisis


I’m seated in a coffee house in Lavington waiting for the man of the hour to arrive.  He walks in suavely through the front door decked in a burgundy blazer. I raise my hand to signal him towards me but he instantly makes a bee line in my direction. “How did you recognize me so fast?” I ask as we pound fists and he settles in, “that bald head hasn’t changed since high school!” he replied erupting into laughter mid-sentence. I remember he didn’t appreciate small talk back then, so we get right into it.

“When did you know you had sickle cell?”

“I actually didn’t, it was my parents who discovered that I did. When I was six months old, I was crying constantly. I’m sure it was the lack of sleep that finally prompted my parents to seek help, hehe. So during the visit, the doctor decided to have a sickling test done once he heard my name. That’s the other thing about sickle cell, it’s a genetic disease that runs in families – and largely found in individuals from Western Kenya.”
“Why specific to Western?”

“Well, sickle cell is actually due to an adaptive mutation – you know the way that malaria is common in the Western parts of Kenya? The way that malaria works is that the parasites infect the red blood cells and cause them to deform and eventually rupture. With the sickle cell mutation, the red blood cell shape is altered which makes it harder for the parasites to infect the red blood cell. So this mutation effectively protects the individuals from the more adverse effects of infection with the malaria parasite. Sickle cell mutation thus developed and selected for people in the Western regions of Kenya as it gave them an advantage before the advent of modern medicine.”

“And then entered the marvels of modern medicine”

“Right? It turned my superhuman advantage on it’s head.”

“So then your parents also have this mutation?”

“My parents are actually carriers, they don’t have the full mutation, but they can transfer the genes to me. Actually, I have 7 siblings and 2 of us have sickle cell, the others we suspect are carriers because they’ve never had a crisis.”

“A crisis?”

“A crisis in sickle cell is essentially these episodes of intense pain that can occur at any point within the body – the chest, the limbs, the abdomen. For me, it felt like there were ants crawling within my veins, or more like pins and needles projecting from the inside. The first crisis that I can remember happening occurred when i was about 13 years old. By this age my mother had already began explaining some of the trigger factors for the episodes and being a mother, had taken steps throughout my life without my knowledge to prevent that from happening. She used to source and brew drinks from mwarobaini (Neem tree). Have you ever had a drink from the plant? Nasty stuff to say the least, but it was effective, quite effective. She also began slowly teaching me about the risk factors that could trigger a crisis such as cold conditions, stress, infections, dehydration, very extraneous exercise. So to prevent all these I largely avoided swimming, whenever I had any symptoms of an infection we promptly got a doctor consult to allow for early diagnosis and treatment of the disease. The mwarobaini served a two fold purpose – it protected against dehydration, alongside the numerous fruit smoothies that were prepared, as well as provided some modicum of protection against infections. Entering teenage-hood, i remember I went out swimming with my friends one day, that night was probably one of the longest nights I have ever had. The pain was excruciating, and I spent majority of it in the hospital being managed with pain medication and fluids.

What happens when a person with sickle cell is exposed to these adverse conditions of cold, or stress, is that the same mutation that protects the red blood cell from infection with malaria undergoes a change of shape in these conditions. When this happens, the cells clog the capillaries and prevent flow of blood and subsequently the flow of oxygen to the tissues. This lack of oxygen is the primary cause of the pain – essentially causing ischemia – which essentially means that blood flow is insufficient to meet the oxygen demands of the tissues.”

“So how do you manage the disease?”

“currently, the best way to manage the disease is to prevent a crisis episode from occurring by avoiding cold as best as possible with warm clothing, staying hydrated and well nourished by having a balanced diet consisting of large helpings of fruits and vegetables. In the case that a crisis does occur, hydration and management of the pain by prescribed medication are the most effective ways of managing the condition. In acute conditions, blood transfusions may be required especially if a large number of the red blood cells is deformed. This is usually determined by the doctor guided by the severity of the current episode. Being a genetic disease, stem cell or bone marrow transplants are the only definitive treatment. However they are rarely done because of the risks involved such as severe infections, immune system problems, or rejection of the transplanted cells

“I’m tempted to say that you have as much medical knowledge as I do!”

“HA! I should have been the one to go to med school! But in my experience, I have to remain on top of things.”

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