Excuse me miss. I like your structure. Can we be friends? No kidding, a guy actually walked up to me with that. Now, if that was an attempt at a pick up line, all I can say is, weak! Seriously dude? I like your structure? Can we be friends? So, I just looked at him with a straight face, said no thank-you, and walked away. Thinking back, how did I manage to keep a straight face saying that? Oh and he was with a friend. I know! I just can’t help it sometimes. Poor guy, of course he liked my structure, hell, I love my structure!
There is this thing that keeps happening though. Often, when I mention to people that I have sickle cell disease, the response is, you don’t look like a ‘sickler’. Two things here, I don’t know whether that is meant to be a compliment of some kind, but really is it? How do ‘sicklers’ look? Then there is, who are ‘sicklers’? We are people living with sickle cell disease. I really don’t mind what people choose to call me, but for Africans and African-Americans. What do you think about being called a nigger? This really is how strongly some people living with sickle cell feel about being called ‘sicklers’. You need to know this. Not all black people take issue with being called niggers, but you really don’t want to call the wrong black person a nigger.
People have a perception of how persons living with sickle cell should look. In one word, sickly! If you “ain’t got the look you probably don’t have the disease” . Stereotypes! I hate them! People can be sick and not look sickly because they take good care of themselves or because of the stage of the disease, or because of access to medication, health care services and a million other reasons. You need to remember that! I for one, am anything but sickly. A good number of people living with sickle cell really do not look sickly. Absurd as this may sound, I actually got discriminated against because I didn’t have the look. I guess I did not sell it well enough. I get punished for having a disease, and I also get punished for not looking like I have it. What do you people want from me/us? Did you cringe, there, ’ you people’? Put that there for effect, no one likes being called names, or being stereotyped. You know, being called a ‘sickler’? Same thing.
So in high school, it is common knowledge that juniors do most of the work. I really didn’t mind. I always had chores at home, within limits of my health of course. Some things are just common sense. In school, I presented my health report, to keep the authorities in the loop, keep me safe, just the general stuff. Only problem, I did not look the part. I could not sell it well enough. So a senior, one of those who are not ashamed to display their ignorance, tells me I don’t look like a sickler and gives me a plot to work on daily. Sweeping? No problem! Except, for about a month, I kept falling sick, back to back. Would be in the ER for three days, get discharged, go back to school and be back in the ER the next week. Thus, the assigned work was not being done. I guess I finally sold the ‘sickler thing’. I was relieved of my duties. I really did not mind sweeping, but apparently, I could have been excused from the work. I think it makes sense. If a student is sick and spending a lot of time outside the classroom, in the hospital, I don’t see anything wrong with giving them a break in some areas. Just so they can have a fair playing field. The issue here is with the stereotype.
A colleague of mine, she really was bone thin, (damn, I wish I had her body, except, with my height I will look anorexic, so, I guess am ok with my weight). Anyway, so, on asking around, I found out she did not have sickle cell. Maybe she had some other condition that no one knew about. But somehow, she was excused from all duties, even got to use the seniors’ showers which were within the dormitory, while I had to use the juniors’ showers, which were about 100metres form the dormitory. This, at 4:30am, in a school that was not willing to provide warm water. Yeah! Thank God for that one house mistress who offered to provide me hot water daily. Why? Am guessing it was probably because I did not look like a ‘sickler’! They could not believe I had sickle cell disease, despite my health report. Wow! What did they want? An autopsy report saying died of… secondary to sickle cell disease? Side note; the colleague I mentioned, is now strutting her stuff on TV and in movies. She is just a thin person, making use of her lovely structure. Apparently her look was more “sickle cell appropriate” while mine wasn’t.
Now really, is there any justification for this stereotype? Any merit for the prejudice? In the medical profession, the appearance of a patient provides vital information. Priceless information you can get within seconds of meeting your patient as opposed to lab results that can take days and sometimes weeks to get to you. Life saving knowledge. The kind of stuff you want your doctor to know in its entirety and not just in bits and pieces. Persons living with different diseases have peculiar habitus –[general appearance, physical state, posture of a patient] in relation to the disease condition. This is true for sickle cell disease as well. There are a lot of scholarly articles online that seek to give reasons for these. Do make time to read them, you will find them insightful.
Typically, patients living with sickle cell disease are described as being small for age. This is generally true. The human body adapts, does what it can to survive. For a baby who is mostly unwell, unable to maintain a healthy appetite and has a body constantly fighting off infections, it is not surprising that they will be unable to meet developmental milestones. It is just simple science. Some studies have it that, the small stature may be due to infarcts at the epiphysis of the bone. Simply, the part of bones that enables us to grow taller gets blocked and the cells die because of the presence of sickle cells. Thus, the growth process cannot occur in its entirety, these children cannot attain their actual height. And as I already mentioned, it is really difficult to put on weight when you are constantly unwell. Little wonder they are small for age.
At the opposite end of the spectrum however, are the very tall ones. Some of my friends try to attribute my height to sickle cell. I say, it is just my dad’s good genes. They don’t seem to buy it; they want to give sickle cell credit for this too. I am not having any of that. So this is what we will go with, a couple of elves came to my room when I was a kid and stretched my limbs, made me taller. That is a reasonable compromise, don’t you say? Some studies have it that, the tall stature may also be as a result of the body trying to adapt. Red blood cells are formed in the bone marrow of long and flat bones. In the child, before the epiphyses fuse, these bones continue to elongate. In a person whose red cells are not functioning effectively and thus being broken down at a faster rate than normal i.e. in sickle cell disease. The body does what it knows to do best. Keep things in equilibrium, increase production. Remember, production occurs in the bone marrow of these bones, thus they elongate faster to enlarge the area for production of the red blood cells. As you know, weight is a function of genetics and environmental factors, thus the super tall may be super skinny in addition, be of average or above average weight.
Gnatopathy-[forward protrusion of the maxilla (upper jaw bone)] and frontal bossing- [protrusion of the middle part of the face] are some of the orofacial manifestations of sickle cell disease.
Jaundice is also a common presentation in sickle cell disease. Yellowing of mucous membranes such as the sclera-[The white part of the eye]. In sickle cell disease this is due to increased breakdown of those sickle cells.
So there is actually some validity to common expectations of how people living with sickle cell disease should look. There are a few more I could add but those wonder into the realms of the ridiculous, so I’ll rather not wade into those waters today. Thing is, even in medical circles, our habitus, is basically two extremes with normal looking persons living with sickle cell in-between. It is just a simple Gaussian distribution, like most things in life. We don’t all have gnathopathy, but if a doctor notices it in combination with other features that is vital information. We are not all jaundiced, at least not always, and there are a people with jaundice from other causes apart from haemolysis due to sickle cell. Even for doctors it gets tricky sometimes. It is certainly unnecessary for everyday life. No need for the sickle cell profiling. It is just unnecessary. Maybe you can take my word for it when I say I have sickle cell disease and not expect me to put up a show for you. That will be very helpful. Thank you.
I’ll love to know what you think about this post. Thanx