James Bryan Herrick recorded the first clinical findings on a sickle cell patient in 1910. A hundred and ten (110) years down the line, this same disease, as rough as the edge of a sickle, came to cut through the hearts of everyone that has ever held Ismaila Barry dear. Sickle cell anaemia brought us so much sorrow on the 16th of June, 2020 as though it was eager to remind us that World Sickle Cell Day was just around the corner. How could we have missed 2020’s commemoration when the reminder we had was the loss of a special one? How could I forget those involuntary tears and haphazard painful smiles I served when I heard of his demise? I mean, we don’t have angels walking around anymore, but we do have those few set of people who bring along a package of kindness, selflessness, humour, and the likes to whoever they cross paths with regardless of who you are to them. If this is not your description of Ismaila, then I’m sorry, but perhaps, you knew the wrong person. We might not have you around again, but we continue to find solace in your altruistic nature and the kindness you did spread during the short time you lived here with us. And until we meet again, we pray for Allah’s Mercy on you and all the departed souls. Aameen
With a lesson learnt from your demise, I hope to inform as many people as I can about the battle you fought to the very end!
What is sickle cell anaemia?
What more can I say about it, than it being the disease of the innocent. It is never acquired but simply inherited, and the only crime of its victims is to be born with a gene they never even asked for. With that being said, let’s talk about this disease already because at this point, it’s way past ignorance o’clock!
To first clear a misconception, sickle cell disease doesn’t necessarily mean sickle cell anaemia. Rather, sickle cell disease is a broad term that refers to a number of disorders, one of which is the sickle cell anaemia. Sickle cell anaemia is an inherited red blood cell disorder in which there is a deficiency of healthy red blood cells to carry oxygen throughout the body. Usually, red blood cells are disc shaped and flexible thus able to move easily through blood vessels. However, in sickle cell anaemia, the red blood cells are shaped like sickles or crescent moons. These abnormally shaped red blood cells which become rigid and sticky lose their easy conformability and can therefore get stuck in small blood vessels leading to a slowing down or even worse, blockage of blood flow and oxygen to parts of the body.
Cause of sickle cell anaemia
Like earlier stated, this is a disease present at birth and the primary cause of the red blood cells becoming sickle-shaped in the first place is due to a mutation. People with sickle cell anaemia (medically termed as Haemoglobin SS) inherit two sickle (S) genes, one from each parent. Usually, it is the most severe form of the sickle cell diseases. In a case where a child inherits one normal gene from one parent, and a defective gene from the other parent, then they become carriers of the disease but generally do not have any symptoms. However, they are capable of passing the genes onto their own children.
Symptoms of sickle cell anaemia
This disease usually manifests itself around 5 to 6 months of age. Symptoms do vary from person to person and could change over time. These symptoms can include:
o Anaemia: Sickle shaped cells break apart easily and die, leaving a person with very few red blood cells. Normally, red blood cells live for about a 120 days before they need a replacement. But sickle cells die very much earlier in about 10-20 days causing a shortage of red blood cells (anaemia).
o Fatigue: Red blood cells are very vital in making sure the body gets sufficient oxygen so when they become deficient, the body is definitely deprived of enough oxygen leading to fatigue.
o Episodes of pain: Pain is a major symptom of sickle cell anaemia and occurs periodically thus the term “pain crises”. It develops as a result of the sickle-shaped cells blocking significant blood flow through small blood vessels to vital regions such as the abdomen, joints, or chest. Others may equally feel pain in the bones.
Intensities of pain and the duration for which they last vary from a few hours to a few weeks. Some people might have a few pain crises a year whilst others could have frequent crises that are so severe requiring them to be hospitalized frequently.
o Frequent infections: At an early stage in life, sickle cells clog the blood vessels in the spleen (an organ which acts as a filter for the blood). This leads to damage and poor protection against infections.
o Delayed growth and puberty: This is also due to a shortage of red blood cells as they are equally responsible for providing the body with necessary nutrients for growth. Their deficiency therefore causes slow growth in affected children and delayed puberty in teenagers.
o Swelling of hands and feet: Occurs as a result of the sickle-shaped red blood cells blocking blood flow to the hands and feet.
o Vision problems: The small blood vessels that supply the eyes are prone to being plugged with sickle cells which could damage the retina and cause vision problems.
Risk factor of sickle cell anaemia
There is only one main risk factor involved and that is being born to parents who are both carriers of the sickle cell gene.
Complications of sickle cell anaemia
Imagine a complication giving rise to other complications? Well, that is sickle cell anaemia for us. Sickle cell anaemia in itself is complicated; the symptoms and painful experiences it is associated with are complicated enough yet still, it could lead to other complications such as:
o Organ damage
o Acute chest syndrome which is life threatening as it could be associated with breathing difficulty
o Leg ulcers (open sores on the leg)
o Complications in pregnancy
o And others.
Is sickle cell anaemia treatable?
Well, a stem cell transplant is the only known cure however; its cost ranges from thousands of dollars to hundreds of thousands of dollars. In a nutshell, it is almost an impossible mission for many especially in our part of the world. Management of sickle cell anaemia is therefore centred on avoiding episodes of pain, relieving symptoms, and preventing complications.
How do we collectively prevent sickle cell anaemia?
Sickle cell anaemia is very much preventable! It is high time our generation took a deeper look into health counselling for the benefit of ourselves and our unborn kids. If one is a carrier of a sickle cell gene, then making kids with another carrier of the sickle cell gene is completely risky because there is every chance of bringing forth a child who suffers from the disease. So the way out is counselling and proper match making. Knowing your genotype and that of your partner doesn’t kill. It only saves you from unforeseen pain. Make an effort to know your status.
As for the living sickle cell warriors, here are a few tips to keep you healthy on a daily basis:
o Take your supplements regularly. Your life depends on it.
o Stay hydrated especially in hot and dry climates, and after exercise.
o Avoid extreme temperatures (cold or heat).
o Moderate exercises are essential but, never overdo it.
o Smoking is your enemy! Treat it as such.
To the rest of the public, please take sickle cell seriously and do your best to prevent it. There is no point in bringing forth kids that would undergo so much pain during their lifetime when it could be simply avoided. And best believe, you will have a fair share of their pain at the end of the day.
“Love is blind has been constantly resorted to in an effort to make excuses for ignoring non-matching genotypes. Perhaps, it is time to lend love a pair of glasses with which it could see the pain the future holds due to its blindness. Maybe then, it might start seeing clearly.”