I want to share my story of how growing up as a SCD patient was for me. This is not a pity party please, it’s just to bring more awareness to you. I was a sickly child, and I got called all sort of names for that. I still remember Some of those names. My first 10years was manageable, but shit got real afterwards. I got into Jss1, I was in the hostel. I cant count how many times I had to be admitted in the school clinic. Sometimes I stay at my guardian’s place. For me, it was always bone pains, always.
That went on throughout secondary school. One day you are fine and kicking and the next day, you cant manage to get yourself to the loo without help because you are in severe pain. No word can describe a sickle cell crises. Excruciating is just skimming the top.
I found myself asking God why me? What did I do wrong to deserve this kinda pain. I blamed my parents secretly and openly one day when I couldnt take it anymore. I remember praying for death to take the pain away.Infact it was a regular prayer for me whrn I have crisis.
One day, my mum heard me and burst into tears. Then I got into medical school and it just was like, my condition worsened. I was always having one pain or the other. I didnt take life serious because I always had it at the back of my mind that I would soon die anyway. The talk then was that once you get to the 30th birthday mark then you have escaped sickle cell disease but I always thought I wouldn’t even get there.
Now am there and I know better. Sickle cell doesnt leave . It’s in the blood. I had a change of mind in 300level, I decided I didnt wanna die anymore, I stopped praying for death to come, I started taking my studies serious, I told myself even if I ended up dead, I want to make an impact helping others like me. My condition is not as bad as some others but that doesnt make it less painful.
When I tell people my genotype, the response is always you dont look like one, are you sure and I say I have Vasooclusive crisis to show for it. I remember falling ill while prepping for final exams. It’s still the worst crisis yet, I had stressed myself and pushed beyond my limit. I was on admission for several days. Doped up to the brim. Funny how those days are still on the list of my happiest moments in life, not because of the pain of course, but because I realised I had great friends and I was deeply loved by them.
I remember waking up from one of my opioid induced slumber to see @sogensis looking at me with soo much worry. I gave him a ‘high’ smile and said stop looking at me like I’m going to die, dont worry I ain’t dying just yet, I will be around to torment you for a long time @yakinzz12 was called ‘The owner’ because he was the one who stayed over every night. @Reasonabledoc would come over to take over by 5am with breakfast ofcourse. @delejosh @HaybeeMD I was able to pull through those times because of you guys.Hugging faceHugging faceHugging faceHugging faceHugging face. Thank you.
Things got a little better after school, I had fewer crisis then got myself my own personal physician and Le hubs @flayedDr, husband. Pregnancy was not without its troubles but yes I scaled through. Then labourSleepy faceSleepy faceSleepy face.
I started having the worst possible crisis in labour,I kept shouting that I was having bone pains and they all thought it was labour pain. I know the difference goddamit. Labour is super painful you would agree with me but this was more. Baby came out and that was when they believed me because I was still in pain. I got pain relief afterwards.
Over the years, I’ve learnt to manage the pain, avoid triggers and live the best life, kicking butts while at it. I’m not letting this limit me.
So, do you know your genotype?, what about your partner’s. Are they compatible? You dont wanna subject your children to this kind of pain believe me, you dont. And it’s not just the pain, a lot more can go wrong.
SICKLE CELL DISEASE and its accompanying complications.
SICKLE CELL DISEASE
It’s a genetic disorder where the body produces abnormal haemoglobin S or C instead of Haemoglobin (HbA) which is the normal one. HbS and HbC becomes deformed after it loses its oxygen content forming a sickle shape thus the name Sickle cell.
This makes it difficult for the cells to pass through small blood vessels. It causes blockage and reduced oxygen supply to the parts of the body after the blockage.Also end products of metabolism cannot be excreted because of this blockage. This is the major cause of pain in part the body where this happens.
This blockage can happen in any part of the body. The bones, the brain, the spleen, the penis. This is what is responsible for the different complications a sickle cell disease patient can suffer. So I will be taking the complication one by one…
This can start as early as after the 6month of life when the fetal Hb has changed to Adult Hb. In this group it usually starts in the hands and foot, can involve one or more fingers.(Dactylitis) it’s as a result of blockage of the tiny blood vessels in the bones…
As the child grows older, the other bones of the body become involved. Most times the pain starts in one limb and go on to involve the other limbs. Severity ranges for mild to very severe and most times will require ingestion of strong analgesics to relieve the pain.
The spleen is an important organ in the body which clears old senescent blood cells from the circulation. In this form of crisis, the spleen sees the deformed HbS/HbC as bad and it destroys them rapidly. This leads to a sudden drop in the blood levels and yellowness of the eyes, coke coloured urine. Please note that this process is going on daily in the body thus the yellow eyes and persistently low levels of blood in the sickle cell child, however it is further accentuated in presence of malaria or an infection causing an exaggerated breakdown of cells. Most children will require blood transfusion at this point to replace the blood cells that were broken down rapidly. Recurrent blood transfusion is not without its complications too.
Yup, you read that right. SCD is probably the most common cause of stroke in children. With SCD a child has young as 3yrs can have a stroke..
The pathophysiology is still the same, blockage of blood vessels in the brain by sickled cells. Most children will recover with or without complications. Problem is once a child has one stroke, the risk of having another one and another one becomes higher. Multiple strokes means multiple sequelae, some will have paralysis, some cannot talk again, seizures is also a common complication after a stroke.
ACUTE CHEST SYNDROME.
This involves the lungs, the lungs won’t be able to perform its function of oxygen exchange. The child complains of severe chest pain and difficulty in breathing. They will require oxygen support at this point. Some may end up in the Intensive care unit (ICU). I had this once and its quite distressful I would say. It’s more common in the older child especially during the teenage years. Its also a common cause of death. I lost a friend to this at 27yrs. (continue to test in peace dear).
ACUTE SEQUESTRATION CRISIS.
This occurs when the spleen starts trapping all the blood cells. There’s a rapid fall in the amount of blood in circulation which is available for proper functioning, the spleen grows rapidly causing abdominal discomfort. Most children will require blood transfusion to replace the blood that is trapped in the spleen. The spleen can be removed surgically to prevent recurrence. The condition can be fatal because of the rapid fall in blood levels.
Rory’s Mum, is a medical doctor. She had suffered and had survived the Sickle Cell disease. Sharing her story, is to enlighten you on the reason(s) why genotype test is extremely important. If you know your genotype, it is important to know about your partner too, and be sure of the compatibility. This will handle the risk of your children going through untold pain and lot more wrongs.
Reach Rory’s Mum, @chrystyn90 to learn more before you commit to that person. Her Dm is also open for related questions only.