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"The Lives at Stake" - Sickle Cell Disease Awareness

Dr Adetuberu Dare

15th Mar, 2016

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Of this HbSS thing [Sickle Cell Disease]

"...a group of inherited red blood cell disorders"

This is intended for awareness, to be told in every area possible. The world's ends if possible. Herein lies my thoughts on the Sickle Cell Disease.

Up until now, what most folks may know may just be statistics, distant tales or vague warnings. Some however may have seen a relative or the other suffer from the disease and its complications.

"Lack of tissue Oxygen can cause attacks of sudden, severe pain - crisis"

It came close to me once. T'was my cousin. She died eventually. She did not reach her 21st birthday. It was during one of those crises that she died. Kudos to her parents; they cared so much for her, gave her their all till the very end. Only in the end, the disease overcame the best they could offer.

But I dare say that the inevitable could have been avoided. Love or one's fantasy may have one sticking out one's neck that one'd marry one's babe who is also an AS. One in 4; is all they sing all the way. One may love the other so much and deems it impossible to let go of a booming relationship.

They eventually get married.

"SCD - a lifelong illness"

It did not get close to me through blood anymore, but it sure did get close to me again. I found out she's a single mother; the father absconded. He just left home and that was all. Left her with the cute little girl who turned out to be my patient at the sickle cell clinic.

In a third world country with no job and high demand for proper care due to increased risk factors for crisis in our environment; yeah, Sickle Cell Disease brought me to such an emotional low when I considered this child's plight. I eventually had to admit the child and manage as a case of known HbSS in Vaso-Occlusive Crisis secondary to Severe Malaria and Sepsis with Hand and Foot Syndrome. That was needed, but the mum had no funds for management. And on whom would I place the responsibility? The man who had abandoned his child and his wife? The child's government? Of do I point fingers at a disease that has no predilection for beauty nor class?

"...in underdeveloped countries, 90% of patients won't live past the age of 5"

One other time was a shocker to me. At first sight, I had made a spot diagnosis of HbSS on seeing the classical features this woman's kid had. But it was the family history that dazed me.

Three of her children had died around the age of 5 years. Cause of death was usually fever, passage of dark coloured urine, yellowness of eyes and/ or bone pains. Well, that was all she could empirically lay hold on. The real cause was so far unidentified. She didn't know her genotype, in fact she guessed she was AA.

Here was a case of Ignorance.

I ordered for Hb genotype which confirmed HbSS and subsequently booked the child for clinic.

"Sickle cell anaemia; when a person has two HbS genes. HbSS is the commonest and most severe form of SCD"

It is either fate or we just found ourselves here. What we've gotta do is give them the best. Prevention of crisis is the end goal.

He thought she lied to him or she thinks she cannot deal with the aftermath... no please! These should never be the case. A divorce in view? Kindly forget that; a life is at stake here.

In the biggest hit in the last 6 months of my practice as a medical officer at this pediatric centre was this child at about 12 years. At the regular clinic we planned to admit and manage for mild VOC secondary to sepsis. But his father deferred admission till when he could find someone to stay with the child on admission. His mother had left him with his father since he was an infant. (Story for another day). A day later, the boy represented as an emergency. We had to battle with a case of Severe Sepsis with DIC. He didn't come out of it you know.

" I love this boy, I will do anything for him" his father kept saying...

"...maybe 1 in every 365 black child is born with SCD"

June 19; World Sickle Cell Day, Here's an awareness of sort.

Pre Marital Counselling is necessary for folks going in. And if you are in already , the deal is on you. Give all the love and care necessary. Forget a divorce if you are considering one (you had the chance to break up before you reached the altar, it is only cowardly of you to do so now after you are married and you already have a child with the disease).

And never miss your clinic visits.

Spread the gist, tell the tales of ones who're battling for their lives. Let it be known in distant places...for this is the sole reason for which this piece was written.

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Dare Adetuberu