Retinitis Pigmentosa

The pathology in the clinics still amazes me! I saw a 10 year-old boy this week whose father said he couldn't see well. The father gave the history that ever since the boy had been young, he couldn't see well in dim light. During the daytime, he was able to see quite well, but as soon as he was in low-light conditions, the child had to start feeling around for things and couldn't navigate well through rooms, etc. The child had never seen an eye doctor, and the family history was limited.

The father gave such a good history that I had an idea of the diagnosis before I even examined the child. Indeed, he had bone-spicule-like pigmentation in the periphery, consistent with a diagnosis of retinitis pigmentosa (RP). The child didn't have any other problems (such as hearing loss, difficulty walking, etc) to suggest that it was part of a syndromic disease, such as Usher syndrome.

Unfortunately, there isn't much to do for this child right now to actively improve his vision. However, we can explain the disease to the parents and continue to follow him.

Luckily, there is a lot of active research in this area, much of it at my home program, The University of Iowa. Dr. Ed Stone studies inherited retinal diseases and recently received a 25 million dollar gift from Stephen Wynn (owner of Wynn Hotels) to continue his research. Interestingly, Mr. Wynn also has retinitis pigmentosa, thus his great interest in supporting Dr. Stone's research.

You can check out the website for the Stephen A. Wynn Institute for Vision Research here:

http://www.wivr.uiowa.edu/

Angie's here!

Arrived in Siteki!

I need a pathologist!

It just so happens that many of my dear friends in the U.S. are pathologists, not to mention my very own sister. I guess they sort of adopted me into their group at Iowa. Well, they have NO idea how badly they are needed here in Swaziland... I would love to "phone a friend" right now.

Yesterday I went to Mbabane again to help out at the Mbabane Government Hospital Eye Clinic. "Mondays in Mbabane". Anyway, I met with Sharon for awhile, planning some projects here in Swaziland, planning a meeting with the Ministry of Health, etc. It was productive, but I am learning there are a lot of road blocks to the progress I hope to make. More on that another day. 

After meeting with Sharon, I went to help out the ophthalmologist at MGH, Dr. Msiska. One of the patients I saw in his clinic is a little three year old girl. She was wearing her little pink "winter" coat and had cute little pigtails. She came in with her father, who was holding on to her tightly. She was very well behaved and did not fuss at all when we examined her. The father spoke very little English.

She had the sweetest face, but the most obvious issue was her complete right sided ptosis (droopy eyelid). and her full-appearing orbit. Dr. Msiska said, "Well, the tumor must be growing."  Excuse me? 

It turns out, he met her over a month ago when her parents noticed "something wrong" with her right eye. She had an eye exam and a CT scan of her head. The exam and the CT scan were both concerning for retinoblastoma. The CT scan apparently showed an intraocular tumor with calcification and thickening of the optic nerve. So, with a working diagnosis of retinoblastoma, Dr. Msiska enucleated (surgically removed) her right eye one month ago.

She came into see us yesterday because last Friday (three days prior), she started having seizures. She had never had seizures before. This is concerning because it likely indicates intracranial spread of the tumor. (In the U.S. she would have already been started on systemic treatment for retinoblastoma, as dictated by an oncologist.)

Given the concern for intracranial spread, I asked if we could obtain an MRI. But, I quickly learned there is not an MRI in the entire country of Swaziland. It is a very small country (smaller than the size of New Jersey), with limited resources. So that was out.

Next, I asked the doctor what her current treatment regimen was. He stated that she needs to be sent to South Africa for further treatment, but they will not accept her as a patient until we have a formal tissue diagnosis. Because her eye had been enucleated and sent to the pathology lab over a month ago, I asked why we didn't have a formal diagnosis (again, likely retinoblastoma, but without pathology, a definitive diagnosis cannot be made). He said, "Well, the pathologist is ill." The only pathologist in the entire country. And I guess he is very ill and may not come back to work for quite awhile. So this child's eye has been sitting in formalin for a month, without a diagnosis, and therefore without treatment for retinoblastoma.

The doctor at University of Iowa who treats retinoblastoma cases always insists that children with suspected retinoblastoma be seen immediately. As in, the same day if possible. And the treatment begins immediately. There are very few true "ophthalmic emergencies", but this could be considered one of them. Or maybe it could be considered a case of  "ophthalmic urgency". At any rate, this poor child's (likely) retinoblastoma has been growing/spreading for the past month.

Dr. Msiska did say there is a private pathology laboratory in Mbabane (I think they mail things out to be read overseas). He suggested the father take the eye there. Unfortunately the cost is prohibitively expensive for this family (1000 Emalengeni or about $100). So... he couldn't secure the funds. (Typical daily wage here is 50 Emalengeni, or about $5). And furthermore, I asked the doctor how the pathologist would receive the specimen. Apparently the father would have had to hand-deliver the eye to the lab himself. I cannot imagine having a child with probable cancer, who has now lost his or her eye, and having to carry the specimen myself to the lab.

So what did I do? I did the only thing I could think of: I called Jono :) Jono to the rescue. I explained the complex situation. He said, "This is an emergency. This child will die from this disease." He heard the desperation in my voice and knew that I wanted to bring the eye back to Siteki with me so that we could send it for pathology read in the UK. Jono said, "Absolutely. I will pay for it."  I told him I was happy to pay for it too (it doesn't solve the 'systems' problem, but will help one child). So, we will mail the eye out to the UK, get a pathology read and diagnosis, and then the child can be referred to South Africa for treatment.

Meanwhile, Dr. Msiska mentioned there was yet another child on the eye ward with likely retinoblastoma. He said he usually sees about 3-4 cases/year, but he has had two in the past month. The two year old boy was to have his eye enucleated today. So Jono and I told Dr. Msiska to send that eye to Siteki, and we will send it to the UK as well.

Poor red reflex (right eye), concerning for retinoblastoma.

I love Swaziland. The country is beautiful. The people are welcoming. They are kind and loyal. They are gentle and trustworthy. They are hard workers.  BUT I just cannot wrap my head around all these barriers to quality eye care/healthcare.

I don't want this post to sound like a criticism of Swaziland. It isn't. I am just trying to make others aware of systems issues here in Swaziland. These issues are especially apparent in a country as small as Swaziland. It is a domino effect: the pathologist gets sick, so a tissue diagnosis cannot be made, so the child cannot be referred for treatment. There is no "escape" route (such as a second pathologist to read the specimen, or a way to send it to a South African pathologist).

We Americans must also remember: America has plenty of its own problems. And many of them relate to healthcare and access to healthcare as well. So it isn't just a problem here in Africa.

I did learn from this patient that "love is love". And this father, although he may not understand all the things the doctors are saying, he may not speak English, and he may not understand the complexity of his daughter's illness...he loves her just the same and wants the best for her. I could see in his eyes how deeply he loves her. So Jono and I must help, however we can.

Any of my pathology friends looking for a job in, oh say, Swaziland?!

Hlane Royal National Park

Angela and I went to Hlane Royal National Park this weekend and saw lots of wild animals. It was great fun, but we had to leave Mabuda Farm at 4:45 a.m. to make it in time for the sunrise. It was an early morning!

 

 

 

 

 

 

 

 

 

 

 

 

Praying mantis in Swaziland

The most amazing praying mantis. Angela found this on her porch one morning.



I think it looks hand-painted!

Jono does it all

As I have mentioned before, on Thursdays Jono is in the O.R. Yesterday, he had 19 surgeries! We started at about 9:30 a.m. and he was finished by 4:30 p.m.



The OR team having coffee before starting surgery

Operating "theater", as it is referred to here in Swaziland

One of the cases was ocular trauma in a small boy under the age of 5. I think it was another "stick to the eye". Anyway, we couldn't get a good exam in clinic, so we had to book him for an EUA (exam under anesthesia). I wondered when the anesthesiologist would arrive.

Next thing you know, Jono is administering anesthesia to the little boy! He anesthetized his own patient! For those of you wondering, he trained in anesthesia prior to taking up ophthalmology. It was so slick. The kid was under five minutes after getting into the O.R. He used an LMA (laryngeal mask airway) to secure the airway. We examined the child, found that the tiny corneal wound had self-sealed (common theme yesterday), and he was awakened from anesthesia. I was impressed.

Dr. Pons at work

Dr. Pons and Welcome ("Wellie")

Today (Friday), I took care of clinic while Jono met with some workers from CBM South Africa (Christian Blind Mission). They are located worldwide and help support Jono's mission and work at Good Shepherd Eye Clinic.

The post-op cataracts looked phenomenal! The corneas were crystal clear, the lenses well-centered, the IOP within normal limits, and the patients had minimal intraocular inflammation. I honestly couldn't tell a difference between the patients who had undergone phaco versus the patients who underwent small incision cataract surgery. It was incredible to witness patients whose vision had improved from "hand motions" or "light perception" to being able to see and navigate (unaided) around the exam room! This will sound super lame, but a smile needs no translation: despite the fact that I couldn't understand what the patients were saying, the huge grins on their faces gave it all away :)

I also saw a cute little baby in clinic with a limbal dermoid.

Limbal dermoid of left eye. For those of you who are ophthalmology residents: it is most commonly located inferotemporally, as in this photo. It can cause astigmatism leading to amblyopia. The patient may also have an associated eyelid coloboma. It can be associated with Goldenhar Syndrome (Oculo-Auriculo-Vertebral syndrome).



Limbal dermoid.



My cutest patient of the day :)

We are just moving out of winter here and it is slightly chilly, so I turned on the gas heater for a bit tonight. This is my "living room" at the beautiful Mabuda Farm.

My living room at Mabuda. The double doors lead to my patio that overlooks the Lobombo mountains.

Another gorgeous sunset at Mabuda

Eye trauma in Swaziland

I am not in Iowa anymore. In Iowa, I saw a lot of eye trauma secondary to farming accidents and bar fights.

Yesterday, I was in the operating room with Jono when I was summoned by one of the nurses. There was a 13 year-old boy out in the pre-op area who had lost vision in his right eye secondary to trauma. They asked that I come evaluate.

When I went to examine the boy, it was clear he didn't speak any English, he only spoke siSwati. Luckily the room was full of patients who were waiting for their surgery (Jono did 20 surgeries yesterday in about 6 hours!). This really kind lady sitting next to the boy helped me translate.

Me: "What happened?!"

Boy: "I was hitting a cow with a stick when the stick broke and it hit me in the eye."

Me (thinking): "Why on Earth were you hitting a cow with a stick?!"

Me (saying): "Oh no. I am so sorry. When did it happen?"

Boy: "Two days ago."

Me: "Why did you wait until today to come to the doctor?"

Boy: "I thought it would heal on its own."

I quickly checked the vision in his right eye. It was hand motions at 1 meter.

Before I move on to what happened to the little boy, I must tell you about hitting cows. I went to tell Jono the basic exam and that I was going to take the boy down to clinic to examine him. He asked how it happened. I said, "he was apparently hitting a cow with a stick when the stick broke..." And before I could even finish Jono said, "Oh. This is so common in Swaziland. And the reason that it happens this time of year is that the kids are on break from school." He was totally unfazed. I suppose if you want to draw a similarity between Iowa eye trauma and Swazi eye trauma, this accident could be considered a "farming" accident.

Kids help out on the farm and they hit the cows with the sticks to drive them into the pasture. According to Jono, this is one of the most frequent causes of eye trauma in kids here in Swaziland.

I took the boy downstairs. He had perfect vision in the left eye. He had no RAPD in either eye, which was a great sign. There was no hypopyon in the anterior chamber, very mild cell and flare, and no cataract. He had about a 5mm full-thickness corneal laceration running obliquely from the limbus at 5:00 to the central visual axis (which is unfortunate), but it was Seidel negative! His anterior chamber was deep and formed. There was no vitreous cell and I could see the optic nerve and retina pretty well and there was no obvious pathology. I gently checked an eye pressure, and it was 10mmHg (low end of normal), which told me the eye was holding a pressure and again supported the idea that the wound was self-sealed.



Corneal laceration

I went and conferred with Jono. We decided that since the eye was closed, it happened more than 48 hours prior to presentation, and there was absolutely NO sign of infection we would observe. If this had presented acutely, we would have definitely placed sutures in the cornea. Sutures hold the eye closed, but they can cause irregular astigmatism and furthermore, we don't like to put sutures right in the central visual axis.

I had the nurses give him a tetanus shot and I placed him on antibiotic eye drops and put a clear eye shield over his eye to protect it and remind him not to touch or rub the eye. The visual prognosis is still unknown, but as long as the eye does not become infected, he will only have to deal with the corneal scar.

I told the child and his mother that he needed to be a "couch potato" for at least this weekend. I asked them if they had T.V. (No). I asked them if they had some coloring books for him to use. (No). I asked them if they had any games or magazines or books. (No). I asked the translator to ask him what he liked to do besides play outside. (Answer: play outside). I realized this may be a losing battle.

So, this morning before I saw him back in clinic, I drove into Siteki and bought him some crayons, a coloring book, and a blank notebook (total cost was about 250 Emalangeni, or about $2.50). I just couldn't bear the though of this child sitting at home bored out of his mind. They were very excited with the "gifts" today and it brought a huge smile to his face.

I have noticed there isn't a lot for kids to do in the hospital here. I know in the peds ward they apparently do have some activities. But if the child is a teenager, they get put in the general ward. This was the case with my patient who had orbital cellulitis. She was in a room with 8 beds. She had no books, T.V., magazines, or toys. Her mother had to work days and did not live nearby, so she couldn't visit often. I remember asking my patient, "What do you do all day?" and she replied "I sleep". I think we could use some Swazi "Child Life Specialists" to help keep the kids entertained.

Kids in the U.S. probably are over-stimulated (T.V., iPad, loads of toys, etc), but kids here do not seem to have enough stimulation. We should probably meet in the middle somewhere.

Putting things in perspective...

Well, I took all my paperwork for my Swazi Medical License to the administrator yesterday, and apparently my $100 background check is not sufficient. They really need a $5 letter from my LOCAL police station saying I have no offenses in Iowa City.

At any rate, I called the Iowa City Police Department and they can help me. They just need some basic information and they can print it out and give it to a friend or family member, who can mail it to me here in Swaziland. I emailed my boyfriend and sister yesterday, both of whom live in Iowa City, and both of whom are very generous and I was sure would be willing to help. Anyway, the subject line in my email that I sent in "desperation" last night was: "I just found out I need HELLLP!" 

They both wrote back really nice emails, but my sister said in the last part of her email:

     "Also in the future, please leave "HELLLP" out of your subject line unless you are dying from Ebola or kidnapped." 

And then in reference to the paperwork that I am stressing about, both she and Cory said something to the effect of,

     "I know [this] seems like a big deal but it will be fine."

I guess that put things in perspective :-) And I will refrain from putting "HELLLP" in all future subject lines, unless I am dire straits. I guess little phrases like this that we may use in our home countries can be misinterpreted when abroad. I am still thinking like an American but living like a Swazi!

Our 80 patient clinic day

Panorama of Good Shepherd Eye Clinic
Siteki, Swaziland
The eye clinic is on the lower level.
Level two is where the OR is located and the overnight wards for the surgical patients.
Level three does not belong to the eye clinic. It is the pediatric inpatient ward for Good Shepherd Hospital.

Today was the first Wednesday that Jono was back in clinic. He had been doing work in the U.S. for the previous three weeks. Wednesday is our busiest clinic day. Anyway, word got out that Dr. Pons was back in town and so everyone decided to show up.

We don't really schedule appointments for clinic. We do have follow-ups and we tell them what day to come back. But new patients can just show up whenever. So...I showed up today with an entire waiting room full of patients. Every seat was taken, there were people standing, and they were streaming out the door. Needless to say, it was a bit daunting.

I started seeing patients immediately and worked until about 1:30 PM, alongside Dr. Pons. Then we hopped in his car, drove quickly back to Mabuda Farm (about 2 kilometers), and his wife and his family members (visiting from America and South Africa) had arranged a delicious lunch on the rocks near the lily pond. It was perfect weather, great food, and great company. It was a nice break from all the chaos in clinic.



Lunch on the rocks at Mabuda Farm with Jono's extended family.



Helen and Jono's new stove for cooking. It is fueled by sticks of wood instead of gas or charcoal. We boiled water for tea/coffee on it and it worked well!

We promptly returned to the clinic and continued seeing patients until about 6:30 PM. We literally did not stop!  Jono figures we saw about 80 patients in clinic today. EIGHTY. But it was fun, and it taught me that I am capable of getting through these days! And I feel like everyone got the time and attention that they needed.

Jono and I had a discussion about why we are able to see more patients in a day here in Swaziland. He has four reasons: 1.)The patients don't expect a long conversation/discussion with the doctor. They want to know what is wrong and if/how it can be fixed. 2) We can't do much "extra" testing here such as automated visual field testing or imaging of the macula and nerve. Therefore, we save time by not ordering a bunch of specialized tests. 3) The patients all sit on a bench in the same exam room and just scoot down the bench until they arrive at the chair to be seen by the doctor. Therefore, "turnover" time between patients is about 10 seconds. Much less private but much more efficient than in the United States. And finally 4) I am not practicing "defensive" medicine here. I am truly doing for each patient what I am able and what I would do for my own friends/family. Therefore, we avoid lots of unnecessary things that might otherwise slow me down. Don't get me wrong--I try to be very judicious in the U.S. about testing, etc, but I also spent A LOT of time documenting, making sure that I have written down absolutely EVERY detail about the exam/consultation. Here, I write a very accurate and complete, but succinct note on a paper chart. There is no electronic health record (EHR). There is no ridiculous amount of coding to be done. There is no "best practices" things that have to be checked and double checked in the EHR. It isn't better or worse, just different. There are pros and cons to both.

After the clinic patients were seen, we did biometry (lens calculations) for the pre-op patients. The surgical patients for the week come in the day before surgery, stay overnight in the clinic ward, and have surgery the next day. They stay overnight one more night, see us post-operatively the next day and then go home. The lens calculations (deciding the intraocular lens to be placed in the eye) occurs after clinic the night before. All of the lodging, calculations and surgery is included in the price of the surgery, which is 600 Emalangeni (equivalent to 600 South African Rand--both currencies are accepted in Swaziland and are of equivalent value). That is about $55 in the U.S. This may seem expensive, but it helps the clinic stay open. And for those that are destitute and have no money, we find funding to cover their surgery.




Dr. Pons doing biometry for patients that are to undergo cataract surgery the next day



Where the surgical patients sleep the night before and night of surgery. There are about 5 people to a room. There are two rooms with "private beds" and a bathroom for patients that pay more money.



Swazi currency: Emalangeni

Photo for the day:

Beautiful Swaziland

A taste of clinics in Swaziland...

Just a few photos from the clinics:

Child with congenital glaucoma and buphthalmos ("ox eye") of the right eye. In children with raised intraocular pressure, their eyes can get very large.

Mother of the child with aniridia. She came in for an exam this week and it was incredibly how strikingly similar their exams were! Genetics is fascinating!

 

Child with aniridia that was seen last week.
 

Squamous cell carcinoma in a person who is HIV positive.

This is the third recurrence (s/p excision with mitomycin C and cautery x 2). It appears that the patient may now have scleral invasion, which is not a good sign. No enlarged lymph nodes were appreciated on exam. This is a "6/6" eye (our equivalent of 20/20 in the metric system), and this patient will likely need an enucleation (removal of eye), to stop the spread. Squamous cell carcinoma tends to travel along nerves ("perineural invasion") which can make it quite aggressive. It is quite common in the HIV positive population. Squamous cell carcinoma in the United States is often seen in quite elderly people who have a long history of significant sun exposure. We often see it in very young people here (20s, 30s), secondary to their HIV+ status/immunocompromise.

Same patient, both eyes.

 

Patient who is in mid-thirties who is HIV positive and has residual scarring after herpes zoster ("shingles") in the V1 distribution. He also had involvement of the left eye (herpes zoster ophthalmicus). This photo does a great job of illustrating that herpes zoster follows a dermatomal distribution and does not cross midline. He had full ocular motility, but did have resultant ptosis (droopy eyelid) on the left.

 

Patient with vernal keratoconjunctivitis or "warm weather conjunctivitis". Vernal keratoconjunctivitis is incredibly common amongst children in Swaziland, and it has periodic seasonal incidence (summer). On exam, the patient had Horner Trantas dots (not easily seen in this photo). She also had large papillae on her superior tarsal conjunctiva, bilaterally. The most notable feature here is the pannus/anterior stromal scarring of the inferior cornea. If not treated, this would continue to encroach on her central cornea, eventually involving her visual axis.