WHAT-DO-YOU-KNOW?

Ever wondered what a child living with sickle cell disease thinks or knows about the condition? Well, I recently had the pleasure of attending the paediatric sickle cell clinic. It was an interesting place to be with a lot of knowledge to acquire. I certainly felt at home, with all the children around, although of course, I would rather not have so many children born with the sickle cell disease.

My experience at the paediatric sickle cell clinic was very educative. I actually saw a child with the rare haemoglobin genotype CC which technically is not sickle cell disease although, a haemoglobinopathy in its own right. Also I saw a child with bi-parietal bossing. He actually had bi-parietal, frontal and occipital bossing in addition to severe gnathopathy. Of course, I had read the literature, seen pictures in books, but never in person. And ohh, he also happens to be Hb SS/SD.

Thus, in one visit I had come across people with two rare genotypes which a regular sickling test will not detect. You can understand my emphasis on HB electrophoresis as the test of choice as opposed to sickling tests. The child with HbCC’s parents would have both tested negative for a regular sickling test, so will the HbAD parent of the child with HbSD. Thus, again I say, HB electrophoresis is the test to do, not just a sickling test.

By way of new information, well, I always knew I had an underbite. And for some reason thought it was normal. I just discovered, I do have gnathopathy, all though mild, thank God. Which makes me wonder, why did I have braces as a child? They did not seem to do much. I really could not see any improvement with them. And of course there go my hopes of getting new braces to fix the “remnants of my oro-facial problem.”

So now, what do children living with sickle cell disease thinks of their condition? Well, wait till you meet an 11 year old sickle cell disease patient in 3rd grade (3 years behind in his education because of frequent ill health, hospitalization and absence from school) who wants to be a pilot. Give him a pen and paper and this is what you come up with…

Transcribed as…
Sickle cell is a disease called Yellow eyes
When I come to reviews they tell me that I should buy Zincovite and Folic Acid.
When I come to hospital they tell me not to eat oily food.
When I am sick and I am vomiting, I should fill a bottle with Water and add ORS (oral rehydration salts)
When I am sick I feel like eating
When I am sick my stomach aches
When I am sick, when I walk a bit, I feel my heart beating fast
When I am sick I shiver
When I am sick I feel like being in school, but my mother does not allow me to go to school
When I am sick I sometimes don’t feel like eating
Long ago when I was sick my head was becoming big
This is what I know about sickle cell disease.

Addressing key points:
1. YELLOW EYES.
In some parts of my country, sickle cell disease is actually referred to as “yellow eyes”, due of course to the fact that jaundice is a prominent feature of sickle cell disease as a result of the constant breakdown of abnormal cells.
It is important to note that, “yellow eyes” is sometimes used as a derogatory expression to tease children living with sickle cell disease.

2. Medication.
As you may be aware, people living with sickle cell disease are expected to be on routine medication.
Commonly Folic acid, which is required in the production of new cells and is generally needed in higher quantities in people with sickle cell disease due to the high cell turnover.
Zinc, in formulations such as Zincovite is essential for stabilizing the red cell membrane and of course VItamin C.
In addition to these are important immunizations and other routine medication prescribed by health care providers to ensure a healthy or rather relatively healthy life for people living with sickle cell disease.

3. Nutrition.
Admittedly, I have not been advised to limit fat intake intake, specifically because of sickle cell disease nor have I heard anyone else give this advice routinely. However, with the increased risk of gallstones formation, I guess dietary fat limitation is in order. However we need to remember that the main reason for the gallstones is actually the increased breakdown of abnormal red blood cells.
Also a balanced diet is certainly useful in maintaining good health.

4. Hydration.
As you might be aware, dehydration is a common cause of crises in sickle cell disease. It is thus important to prevent dehydration at all cost.
People living with sickle cell disease are advised to drink large volumes of water daily to maintain good health.
In situation where the risk of dehydration is high, such as if a person is vomiting or has diarrhea, Oral Rehydration Salts come in handy and when oral fluid intake is not possible, intravenous fluids, to prevent crises.
Note that fluid therapy is a key component of management of sickle cell disease.

5. Pain
Vaso-occlusive crises could occur in any part of the body and could be triggered by just about anything. Heat, cold, stress, dehydration, infection etc. Long bone pain, acute chest syndrome and priapism are common, however, abdominal pain due to mesenteric infarction is also possible.

6. Anaemia
Anaemia is another key feature of sickle cell disease. Symptoms include palpitations, as described by this child as well as easy fatiguabilty, breathlessness on exertion, headaches, dizziness and poor concentration.
These are common complaints of most people living with sickle cell disease.

7. Infections
Febrile illness are common in sickle cell disease, mostly due to the lowered immunity. Infections in turn easily precipitate crises and lead to long periods of hospitalization. In the tropics, malaria is more common and severe in people living with sickle cell disease, thus extra effort is required in its prevention such as the use of insecticide treated bed nets.

8.Absenteeism
This must have been a complaint of every child living with sickle cell disease at some point in their lives.
As I mentioned earlier, this kid is 3 years behind his colleagues due to constant ill health and hospitalizations.
This of course is one of the social complications of living with chronic medical conditions such as sickle cell.

9. Anorexia
I can certainly identify with this. Pain has an uncanny way of robbing you of the desire to eat or do anything else for that matter. When you don’t feel like eating when you are sick, and happen to be sick more days than not, this can only have a negative impact on your health and well being.

10. “My head was becoming big.”
This was in reference to bossing that had occurred during infancy, which his mom probably told him about. Frontal bossing as well as gnathopathy are some of the facial featuers observed in people living with sickle cell disease and for which a lot of children are teased.

This is what one kid living with sickle cell cell disease knows about it. please spend some time finding out what people living with sickle cell disease think/ know about the condition. It just might help you in coming to an informed decision about having a child with sickle cell disease.

Then of course, there is you. What do you know about sickle cell?
What does your partner know about sickle cell disease?
What do your friends, neighbors, family know about sickle cell disease?
This might be your chance to educate someone, please spread the word, share this post, tell someone about sickle cell disease.
Remember, SEPTEMBER is sickle cell AWARENESS month.

Be the change you want to see guys.
Peace,
Sefakor-Enam.

(Special thanks to my colleague Edem for this idea and this kid and his mom for giving me permission to share this information.)

DEEP THOUGHTS! WEIRD THOUGHTS!!!

Hmmm. so much to think about. If we would all play our part to be the change we want to see. The things that bother me. #sickkids #preventableillness #preventabledeaths

It is September, sickle cell awareness month. Yey! We get a whole month! If only we could affect policy, change mindsets and such.  We all do our bit, to brighten the corner where we are.  I have been doing a bit on social media, retweeting and sharing other people’s stories. It is quite refreshing, all the information available to anyone who will spend just a little time to read. I especially like all the stories about strong black women living with sickle cell disease, having successful carriers and families. Sickle cell is really not all gloomy. You just have to look to see that a good number of us live relatively normal lives despite all the challenges.

I was all excited about sickle cell awareness month, then out of nowhere, tragedy struck. I was hit with some sad news. I read about the death of a 4^th year university student. She was just a month to graduation. I carried out a little research, and turns out this lady was actually someone I attended elementary school with. She was a year ahead of me. On further investigating, I found out she passed suddenly after a short sickle cell crisis. Yeah, it does that, sometimes. I tried to get confirmation on this.  I really had no memory of her. I resorted to facebook, and it was evident her death was sudden. No condolences, nothing. She had actually recently updated her profile picture. Now, what struck me, going through her pictures; that could have been me! I am sure most people had no idea she had sickle cell disease. She didn’t have the typical look, but wait, what is the typical look?  That is a discussion for another day. She could have been some 90 or 100 kilos, full of life, fashionable, and suddenly she was dead. A month to graduation!!!

Of course, my condolences to her family, friends, loved ones, everyone who she was dear to. I can only imagine the anguish her parents must be feeling. A child, seen through all those years of life, education and just a month to graduation passes on. Sad! Then there is me, only child of my mother, father’s last child, medical student, big hopes and aspirations…Oh! Lord, spare me. I pray for long life and health. This is just one of the things we have to deal with, living with sickle cell. So much uncertainty!  Every time I hear of another sickle cell related death, I’m  shaken a bit. But hey, we will all die someday; let’s consider where we will spend eternity. Honestly though, tell me, when you hear a young person died suddenly, what do you think of? For me, first, road traffic accident, second, sickle cell. Definitely sickle cell.

A while back, a friend recounted her mom’s anger at another lady who had recently lost her son due to sickle cell disease. The boy was probably 10 years when he passed. As the mom of the deceased wailed uncontrollably, my friend’s mom was simply incensed. To her, this mother, was at fault. She caused her son’s demise. Subjected him to a life of disease and pain and ultimately death at a young age. I couldn’t understand why anyone will take such a stance. This mother needed sympathy not condemnation.  Especially not at that time.

While at home, during my current Va-K-Tion, my mom and I had a chat. You know those mother-daughter conversations that daughters generally hate?  Yeah!  Suffice it to say, I am not having one of those ever again!  So, we eventually got into a conversation about sickle cell, and my future. Her grand kids.  mmhmm.  Apparently, being the fussy person that she is, oh yeah, for those who know me, I truly am my mother’s daughter.  Quite fussy myself. Knowing her haemoglobin genotype, she actually took my dad to get tested. Story has it that, everyone, ‘by the way, they already knew her, my mom is quite a popular person’ said she was too fussy. She should let it go. My dad was too well looking and handsome ‘he really is, no bias’ and healthy and such, to have the disease or trait. Yes, so they didn’t test. Then what do you know, I am six months old, falling sick a lot, pain in my fingers (that would be sickle cell dactylitis (hand-foot syndrome) [-extreme pain involving the hands and feet due to a block in blood circulation. Often the first symptom of sickle cell in babies.] Detailed family history, labs, the works, this would later prove that I have sickle cell disease. Thus the journey begun.

I was shocked, to say the least.  Really mom!  No really, am I the only one thinking SERIOUSLY?  Like for real? Don’t get me wrong. Of course I love my parents. They’ve given me more than most, ensured I live as close to normal a life as possible. But, since normal is relative, I’ll say I have a pretty good life. I still got to ask, seriously? I could not believe what my mom was telling me. So here is the thing, my mom, is a nurse. A good one at that. She is smart, learns really fast, my dad, also a smart guy. So now, two educated people with adequate knowledge actually set out to get tested but didn’t?  OK, so it was 1990, there wasn’t as much information blah blah blah, but my mom? She really knew better.

So will someone be right to be upset with my mom, if I happen to die young, just like the young lady I recently found out about, or that 10 year old boy? Well given this story I think they would. I now have a fair understanding of my friend’s mom’s reaction. I understand her, but really is there any justification for her reaction? My mom has done a lot to keep me healthy and I am the best judge of this.  So, hear this, ‘AINT NOBODY GOT THE RIGHT TO BE PISSED OFF AT MY MOM FOR ME OR WITH ME. I AINT ASKING FOR IT. I DON’T NEED IT. IF I WERE TO DIE YOUNG, WHAT MY MOM WILL NEED FROM YOU IS LOVE AND SUPPORT NOT JUDGEMENT. LET HIM WHO HAS NO “SIN” CAST THE FIRST STONE!’

The issue here is this; there are some kids who hate their parents, for well, this genetic gift. I can’t say I am pleased. I am certainly pissed, but, I don’t know, it is still unsettling to have someone judge a grieving mother.  Also, the love is definitely far more than the anger. My parents went all out for me. What if they couldn’t afford to keep me healthy? What if they could not pay for expensive schools, extra tuition to ensure I did not fall behind in school work even though I was hospitalized quite a bit? What if they couldn’t afford all the hospital bills and medication and expensive medical tests? Did not have adequate medical knowledge and access to provide the quality of life that I have? What if like others with sickle cell, it was viewed as a disability, an obstacle and prevented me from aiming high and making giant strides in my life? What if it was all sickness, and my parents weren’t supportive, will I feel the same way?

It is a tricky place to be as a child. Hate/ love for your parent. It is real though. There have been times, where in extreme pain, on admission I felt resentment for my parents, despite all their efforts. I hope sometime in the future, your child does not end up in a similar situation, especially at young age. They could just break your heart.  You know how younger kids are brutally honest. He could just tell you, mom, I hate you for making me sick. Especially so, if you do not have the finances, support system  and adequate knowledge required to enable your child, living with sickle cell to have a relatively healthy life.

Thing is, we all have different stories, you cannot be armed with information and decide to do nothing.  For those who are ignorant, they may be excused by some, but come on! We are in 2013. Ignorance stopped being an excuse a very long time ago. There is so much information out there. Saying you had no knowledge and thus subjecting your child to illness is unpardonable. There are options now. If you think you love that person too much to let them go. Well, find money for in-vitro fertilization (IVF) with genetic selection, or for a bone marrow transplant, or whatever else, and oh, these are some expensive stuff, no cheaper than the health, financial and emotional cost of raising a child living with sickle cell disease. Brace yourselves!  And, for those who want to PRAY AWAY the sickle cell, marry someone you have a high probability of having a child with the disease with, and have ‘faith’ that they won’t get the disease. Here is a quote a pastor once shared relating to issues concerning sickle cell disease, trait and marriage.” What I can do by intelligence, I don’t expect God to do by miracles…” (Of course this is open to varied interpretations)

Again, I love love love my parents.  Am grateful they have helped me get this far in life. For those who  think I am being critical, remember in the scenario I am the dead one. ‘aint nobody wanna be dead’ I got stuff to do. So help me GOD, I am going to live a long healthy life, and when am done with everything I have been called to do, by all means, I will rest in peace. As I said, we’ll all die. Honesty is good. It is sickle cell awareness month. A different perspective is always refreshing.

Again my condolences to the loved ones of the young lady who recently passed due to sickle cell. Please don’t judge parents of kids living with sickle cell, especially in their time of grief. I try not to. And please don’t judge people living with sickle cell. We need all the support we can get. Our parents need your support too. But for real, get information, get tested and do what is right, for you, your children, your family, lets even add on generations yet unborn.

DEUCES.