Monthly Archives: September 2014

Sesky Eysis. (Sexy Eyes)

Guys and girls, we are so different. Aptly stated, men are from Mars and women, from Venus. No kidding! Anytime I mention what I look at in a guy, my male friends go like, “guuurllll you are just weird.” Apparently, a typical guy has a keen interest in a woman’s well, “back pockets” and that-other-one. Females however, oh it is such a spectrum. Girls be looking at sideburns and toe nails and back to lips and and… There are just so many things different girls are interested in, an instant turn on or put off. Wondering why that date went so well but she didn’t want to see you again? Huh, maybe, just maybe it was the toe nails.

Now me, I am an eye girl. Yeah, quite apart from all the intangibles, I like eyes. I know, eyes? Yes eyes! Now, you are probably wondering, how-the-hell does a person like eyes? Well, let me explain.

It probably has to do with the fact that I happen to be one of those kids who wore those super thick spectacles with the rope behind the neck from a very early age. Oh, I was 7 and absolutely detested my glasses. All I wanted, was to see without them, like the other kids. I just did not understand why I could not see without those truly atrocious spectacles, which by the way made any attempts at sports, an absolute nightmare.

Turns out, I had a refractive error, myopia (that means I am short/near sighted). Very near sighted actually. No prizes for guessing how near sighted I am. Let’s just say, by the WHO classification, I am severely visually impaired. Thankfully my visual impairment is corrected. I happen to have high index, photochromic, anti-reflect lenses. These babies have to be a life saver of sorts. Thanx to them I don’t have to walk around with glasses as thick as the base of coke bottles, and in addition they serve the added role of protecting my eyes from the harsh tropical sun and the migraine inducing glare from all my devices, given that I do stare at screens for very long periods. Oh, and thank God for the whole geek-chic trend, I can still look trendy with my glasses and dress it up.

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Did I hear geek-chic, just me in my contact lenses and yeah my all time favorite spectacles. (Wearing these babies has never been cooler, *wink,

Back track, so, I was in second grade and grossly underperforming. Could have easily been diagnosed with one learning disorder or the other and my life would have followed a totally different course. All it was, I could not see. I could not understand anything in school because I could not see and I was writing a whole lot of jibberish because, you guessed, I could not see anything on the chalk board. So yeah, being about the only seven year old kid wearing ugly glasses and hating them and wishing I could just take them off, you can certainly understand why I was fascinated with eyes. Especially my ‘malfunctioning pair’. For some reason I always found myself staring into people’s eyes and wondering “why me?”

So you see, I am very interested in the eye as the organ of vision, and equally keen on preserving mine. Now to the present day, almost. Three years ago my haematologist, sent me off to see an ophthalmologist, because of frequent ‘eye complaints’ and thus the journey begun. Turns out, just by having sickle cell disease I could lose my vision. I know, it’s absurd! This sickle cell thing keeps cropping up in the weirdest of places. Even worse, being that my genotype is SC, I am at an increased risk of developing SICKLE CELL RETINOPATHY. (Slumps onto desk and screams, *insert expletives*) Ok now that we are calm, let’s get on with it…

Yes, I was in medical school, and somehow, of the many complications of sickle cell disease I had read, this one had simply escaped me. Call it small print. I really was in awe. Anyways, in addition to all the other medical appointments I had to keep, now there were the-painfully-long-and-blurry-vision-inducing biannual routine fundoscopy appointments with the ophthalmologists. Frankly, It was after my rotation in ophthalmology that I fully grasped what sickle cell retinopathy is and it’s life altering consequences, considering so many, even “the enlightened” are unaware of its existence.

So now you are probably wondering WHAT IS SICKLE CELL RETINOPATHY?

Well, retinopathy in general, is damage to the RETINA, (i.e. the “seeing” part of the eye which is located at the back and contains several layers which have nerves and small blood vessels coursing through them. The retina captures light and images so that you can see.)
Retinopathy is caused by several factors which may be acute or may have been occurring gradually over time (chronic).
It is usually seen as an ocular manifestation of systemic diseases such as diabetes, hypertension and of course SICKLE CELL DISEASE, it also occurs in some premature babies and acutely, after eye trauma.

Eye problems caused by the sickle red blood cells can be found in all types of sickle cell disease but are MORE COMMON in older children and adults with HbSC and HbSbetaThalassemia. These eye problems, beyond retinopathy, include cataract, hyphema (bleeding into the front part of the eye), iris infarcts and atrophy.

CAUSES:
The ocular manifestations of sickle cell disease are due to vascular occlusion (blockage of vessels) as occurs in other parts of the body. Sickle red blood cells can get trapped inside the small blood vessels in the retina. This can in turn cause:
1. A decrease in blood flow, leading to damage of the retina which may be permanent.
2. Bleeding in the eye.
– When blood vessels are blocked, the eye makes new vessels to replace the blocked vessels.
– These new vessels are thinner and weaker and may easily bleed.
– The bleeding causes damage to the retina. Bleeding can also cause the retina to become loose from the rest of the eye. This is called RETINAL DETACHMENT

NB. Damage to the retina MAY cause changes in eyesight, which if not treated, may result in BLINDNESS.

Sickle cell retinopathy unlike the typical vaso-occlusive crises most patients have come to be accustomed to may be asymptomatic at the onset. Symptoms generally manifest in later stages, it is thus essential for persons living with sickle cell disease to see an ophthalmologist routinely to prevent the sudden vision loss that occurs in later stages.

When retinopathy becomes worse,symptoms that may occur include:
– Flashes and dark shadows in some parts of your vision (floaters)
– Blurred vision
– Sudden loss of vision
– Pain in the eyes

REMEMBER, you don’t want to wait to start experiencing symptoms before you see your ophthalmologist (because this may indicate advanced disease).

If however your experience changes in your vision, or any of the other symptoms you need to go to the hospital immediately. You may either go to your ophthalmologist (if you have one) or your haematologist (which you should) or to the ER.
Also, changes in vision can occur with a stroke (which people living with sickle cell disease are at increased risk of) so you are a lot safer getting examined by a doctor.

Recently, I had what I thought was a floater so I went to the ophthalmologists. Apparently, it was “nothing.” However, there had been some changes from my last fundoscopy examination. A few golden sun bursts -these are non-proliferative changes in sickle cell retinopathy. Actually, not a surprising find. The consensus, we’ll consider laser photo coagulation therapy later. It is just good information to have. It helps in planning and such.

The fundoscopy examination, is itself brief, eye drops are instilled into the eyes to dilate the pupils.This allows the doctor to see the entire eye better. Honestly, the dilation is the part I detest most. With your eyes closed for God-knows-how-long and, if you happen to be me, times two of that, because my pupils just won’t dilate as expected, you just patiently wait till your eyes are fully dilated so the ophthalmologist can examine your retina.

My tip for routine fundoscopy examination, sunglasses. Nothing like the tropical sun stinging your fully dilated eyes. It feels like the dilated pupils allow every ray of light to go through them, it hurts and is very uncomfortable. Of course with dilated pupils your vision is blurry, it usually clears after about a day, so another tip, don’t schedule your examination for a day you absolutely have to read, eg, the day before an exam. Trust me you’ll be frustrated on two counts, waiting forever for your pupils to dilate and then your inability to read after. I’ve been there. Very unpleasant experience.

Treatment:
Treatment is required if the retina show signs of damage. If caught early, most problems can be treated by the ophthalmologist. Frequent eye examinations will be required as well. Depending on the severity of retinopathy laser treatment can be done and alternatively, surgery after which strict follow up care will need to be adhered to.

Prevention:
(Really is better than cure/treatment) and which I am a staunch believer in is simple.
As stated above, early stages of sickle cell retinopathy do not normally cause changes in eyesight. Prevention of long term complications can be done by ROUTINE FUNDOSCOPY. I.e. getting your eyes checked regularly even if you don’t have symptoms, so that any changes can be picked up early and treatment initiated.

NB.Treatments work better if the eye changes are found early.
Also, tips for maintaining general health in sickle cell disease such as increased daily fluid intake are essential to help keep the sickled cells from sticking together and causing problems.

Retinopathy is just another one of the many complication of sickle cell disease. Please spread the word, pass this on and lets get more people informed about sickle cell disease.

Sickle cell disease is preventable, please make an informed choice.

Regards,
Sefakor-Enam.

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…

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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.)