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