Tuesday, September 14 – A problematic inpatient and a very interesting outpatient…

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A photo from Akash of a sunrise in Dar

Educational lectures occur every Tuesday and Thursday at 7:30 AM and are typically given by visiting volunteers of which there have been few over the last year and a half of the pandemic. We, meaning the neurology team, had been here in March 2020 when everything hit the fan and we had to scramble home in a rush before the borders closed. Despite having been safer in Tanzania than it was at home, the State Department announced that anyone overseas had to either return home or shelter in place, which really wasn’t an option for our team. The airlines had immediately cancelled everyone’s flights at the announcement meaning that we all had to rebook with some of the team having to pay exorbitant amounts for their new tickets. Worse yet, all the new flights flew directly into NYC, ground zero for the outbreak, at a time when there were very few restrictions in regard to personal protection. On arrival, we were all led through immigration in long lines with very little spacing and no one wearing masks. How we all made it back to Philadelphia with contracting COVID is pretty remarkable.

Dr. Kerry giving her lecture

I was back by myself in September/October of last year as the residents still were not allowed to travel and then we all returned last March as the first group of volunteers since the beginning of the pandemic. We were able to do some lectures then, but they were still restricting gatherings at that time. For this trip, though, now that everyone has been vaccinated including all of the fame staff after vaccinations finally reached Tanzania last month, we have been able to meet, albeit masked, for our normal educational lectures. Dr. Kerry’s lecture this morning dealt with the assessment and management of babies born with hypoxic-ischemic encephalopathy, or HIE, a common problem here for a number of reasons.

Kitashu, Angel, Leeyan, and Revo – our dedicated FAME Neuro team

Babies with HIE are typically very developmentally delayed and may often be dependent for life, something that must always be considered prior to beginning resuscitative efforts on these children. The long term prognosis is an issue given the lack of social services and support for these families beyond simple rehab which doesn’t really affect their overall outlook, but rather helps with managing problems rather than resolving the underlying issue at hand. Jumping in without having considered all of the ramifications of your actions and potential unintended consequences can often do more harm than good.

Our volunteer coordinator, Prosper, holding a friend, Esther.

After Kerry’s lecture, we learned that our patient who had presented with seizures and had also raised concern for COVID, had several more seizures overnight and into the morning. We were unable to fully evaluate him yesterday due to the COVID concern, but had asked that he be loaded on valproic acid, a common antiepileptic that we use here. Despite the load we had given him yesterday, he had continued having seizures. As most of the team began seeing our neurology clinic patients, a few went into the ward once it was determined that we didn’t have to worry about COVID and he was transferred to Ward 1. At that point, he was observed to have focal seizures of his left face and arm with minor suppression in his level of consciousness.

Dr. Anne and Denise charting on our inpatient with seizures.

His CT scan that was done the day before did demonstrate some extremely subtle fullness of the right parietal lobe with some cortical hyper density and it was difficult to tell whether this was a primary problem or something related to his seizures. We decided to repeat his valproic acid loads over the remainder of the day (we only have oral and no IV) and considered a benzodiazepine challenge as he was initially felt to be sedated and we wanted to rule out subclinical status epilepticus. When they went to do the challenge, though, he had improved and we held off. Overnight, his seizures stopped and by the following day, he thankfully remained seizure free on the higher valproate dosing. Unfortunately, we had no real explanation for his having seizures, and, given the clear focality of the episodes, this raised even greater concern for an irritable focus such as a tumor, infection or stroke. His original CT scan, though not entirely convincing, did have some subtle findings that were not unequivocally abnormal which left us a little bit in the lurch as how to proceed. In the end, his examination was entirely normal  from a neurologic standpoint making the issue a bit less urgent and we ultimately decided to discharge him home on valproic acid have him follow up with us in several months. Dr. Anne would see him and do a detailed neurologic exam at which point she could let us know her thoughts and we could always decide on whether to repeat a contrasted CT scan or try to get an MRI in Arusha, not always the easiest thing to do for many respects.

Revo, Cat, and Denise evaluating a patient

The remainder of the day was spent seeing our routine smattering of patients that also included someone with suspect occipital neuralgia. We see this often at home and mainly in patients that have chronic headaches associated with cervicalgia, or neck pain. This is often referred to or described as a “rams horn” headache as it radiates from the occipital region up and over the top of the head unilaterally or bilaterally at times. One therapy that has worked very well for these patients, both here and at home is an occipital block, something very simple to perform and will often resolve their symptoms for months, years, or even indefinitely in some cases. This is something we teach the residents to do back at home and have been doing here at FAME since we’ve been coming. I’m convinced that there is a tremendous amount of cervical pathology here that very likely stems from the culture of carrying heavy loads on their heads, whether it be to bring goods to the market, or carrying 5-gal buckets of cement on their heads when working hard labor. Overall, this contributes to their neck pain and headaches and keeps us quite busy with our neuro clinics in this regard. Job security 😉

Leeyan, Emily and Dr. Anne evaluating a patient

As is often the case, our most interesting patients will come late in the day and today was no exception. A 17-year-old boy with a history of predominantly motor cerebral palsy secondary to a birth injury who was otherwise cognitively intact and had successfully attended school presented with abnormal movements that had begun several months ago. The movements came on rather abruptly and were there throughout the day, but went away during the night. There were very disturbing for him and something they had never noticed before during his life. There was no history of infection or fever that they could remember and he had no other symptoms such as shortness of breath to suggest any cardiac involvement. When I went to look at the boy, he had very classic movements of his limbs and face that was essentially constant and something that I had seen here on two prior occasions. The movements were choreiform and, given his age and the sudden onset of the movements, quite classic for Sydenham’s chorea, a movement disorder occurring primarily in late childhood and adolescence and related to a Group A Streptococcal infection and often rheumatic fever, causing inflammation of the heart or damage to the heart valves. Cases of Sydenham’s chorea, though, may often occur in the absence of any clinical manifestations of an earlier infection, but the presumption is that one had occurred.

Revo and Anne watching Denise giving an occipital nerve block

Other than his choreiform movements that were again, constant, and his motor findings consistent with his prior diagnosis of  CP, his exam was unrevealing for any other pathology. All patients with Sydenham’s should have an echocardiogram to rule out any cardiac involvement, despite a lack of symptoms or history, as the major complication for this disorder is cardiac in nature with patients often requiring heart valve surgery or replacement. We were able to set him up for an echocardiogram the following morning, which did demonstrate some mild carditis, indicating inflammation of the heart and would impact our recommendations for antibiotic treatment. His erythrocyte sedimentation was normal, suggesting against any active infection, though the current recommendations were still to treat with 10 years of penicillin and using the monthly IM formulation to assure compliance. The purpose of continuing the antibiotics is to reduce the risk of recurrence with potential cardiac complications or recurrent chorea. The treatment of the movements, on the other hand, when necessary can be by either using steroids to treat the underlying autoimmune process or possibly other medications commonly used in the treatment of movement disorders.

A photo from Akash of his hotel in Dar

Sydenham’s chorea is no longer commonly seen in the United States, mainly due to the prompt, or often excessive, use of antibiotics in the western world. This was the third case that I’ve seen here at FAME. The first case that we saw was in 2013 when we (Danielle Becker and I, quite independently I should add) first diagnosed it by seeing a video of the patient, immediately recognizing the movements and history and then traveling here several months later to meet Roza, the young girl who we had seen in the video. She had an active infection and some valvular damage, but thankfully didn’t require anything more significant from a cardiac standpoint. It is a fascinating condition, both historically in the world of neurology and in regard to its crossing over into many specialties with neurologic, cardiac, infectious disease and even psychiatric manifestations that are possible. It is a diagnosis that many learn about in medical school, but never have a chance to see it in real life. Once you see it, though, you will never forget it.

Sean relaxing on one of our veranda hammocks.

We had asked Teddy, the seamstress we have used the last several years, to come up to FAME to do all of the fittings for all those who wanted clothes made. That included everyone except Akash and me. Even Dr. Grady had wanted to have a bowtie made. In addition to the fabric that everyone had purchased in town the day before, Teddy brought quite a number of additional fabrics to choose from. The many colors and designs of the typical Tanzanian fabrics are absolutely incredible, much as they are in many of the other East and West African countries. Teddy worked with each of the residents, Kerry and Sean individually, taking many, many measurements along the way and figuring out how many and what types of pieces to be made from each of the fabric pieces based on their design and size. She would write everything down in her notebook and attach a very small piece of fabric to remember given the number of pieces she would be working on for the group. Kerry would be leaving the coming weekend, as would Sean, so she would need to have their pieces ready before then, while there was no rush on the others items as we’d still be here for over two weeks.

Teddy and our group getting clothes made
Phillip selecting which fabric for which piece of clothing

It took well over an hour for Teddy to complete all of the ordering and get packed up. Dr. Anne had remained for the entire time to help out as Teddy doesn’t speak English well and I thought it only proper to offer the two of them a drive into town rather than having to call for their own driver which would have meant a bijaji or a bota bota and it was late. We hadn’t eaten dinner either and everyone was starving in addition to me. I drove them down to the junction at the tarmac where they each had a driver waiting for them, but the rides would be much shorter from there. As I drove back up the FAME road to my home away from home, I once again realized how fortunate we all are to be here, helping others and ourselves at the same time. Though Akash was still in Dar tonight, he would be getting his passport tomorrow and catching a flight back to Kilimanjaro where he’d have to spend the night at the KIA Lodge at the airport since his flight home would arriving after dark and the drive to Karatu is too dangerous at that time. We would hopefully have him back for clinic by Thursday morning as they were picking him up at 6:00 AM and everyone was looking forward to having our pediatric neurologist back with us as soon as possible.

A typical sunset scene from the veranda

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