The rains have come a bit earlier than they have in the past with showers, some heavy, now occurring on a daily basis. Though this is seen as a blessing to the Tanzanians, for it means a good planting and growing season to follow, it makes our lives messier given the fact there are few paved roads here, so travel becomes more difficult and merely walking to clinic each morning can be a chore. The timing of our spring trip has generally coincided with a break in the wet weather, between the short rains of January and the monsoons that typically begin in late April, but it seems that this year the rains may have decided to come earlier than normal. I’m not sure we can necessarily blame global warming, at least not for this one, as this is still within the realm of normalcy for the region, though there’s little question that global warming has affected all of Sub-Saharan Africa with wide ranging droughts in the recent years that has decimated many of the large livestock herds.
Travel here can be quite precarious in the rains as I’ve had the opportunity to experience on several occasions in the past. One of the main issues occurs with our mobile clinics to more remote villages in the Karatu district. Our travels to the Mbulumbulu region (Kambi ya Simba and Upper Kitete) have been seriously disrupted in the past as that road can only be likened to a Slip ‘n Slide (for those of you to young to recall the early Wham-O Slip ‘n Slide) in which keeping your vehicle on the road can be related more to luck than skill. In April 2011, our Land Rover (with me driving) slid off the road nearly onto its side, ending upright axle-deep in the thick mud requiring us to dig out each wheel to elevate the vehicle to where it could be yanked out by another Land Rover. After hours of work to free ourselves, clinic was obviously cancelled for the day as just getting back to the tarmac was a major undertaking. The road to Rift Valley Children’s Village, a site where we spend two days, can also be pretty challenging in the rains with the steep up and downhills that we have to navigate on the well-named “African Massage Road” that has very shear drop-offs one would prefer to avoid if at all possible – the trick is always to keep your wheels moving and not to touch your brakes as the minute you lose traction and begin to slide, you have no control of the vehicle. Needless to say, it’s as much of an art as it is a skill.
Last year’s adventure in the Southern Serengeti with Laura, Ashley, and Theandra, in which our vehicle became hopelessly stuck in the mud and water (perhaps it was more of a debacle than an adventure depending on one’s perspective) did have to do with the excessive rainfall that was localized to that region. The road became more and more submerged until we eventually found ourselves in the middle of what was essentially a swollen river with a foot of water on top of mud to the extent that there was absolutely no traction underneath. The rangers from the Conservation Area were looking for us, thanks to Dr. Annie, but with all the water, neither we or they were able to make visual contact despite our nearly hour-long walk with all our baggage through the mud and water in the dark with wild animals all around and swarming termites attracted to our lights to add insult to injury. After failing to find the rangers, or for them to find us, we headed back to the vehicle and were eventually rescued perhaps an hour later when they crossed the river to reach us, but it still required that a scary walk to their vehicle, and an even scarier drive to the Ndutu Safari Lodge where we were supposed to spend the night as it was far too late to return to FAME that night. We were driven to FAME by one of the rangers in his government truck the following morning while Vitalis, our guide, remained back in Ndutu to arrange pulling the Land Rover out of the muck and to safety. If one had tried, I don’t think it would have been possible to plan a better experience, though I’m not sure my fellow passengers felt that way at the time or today.
All of this discussion of the weather and the roads, of course, greatly affects what our activities will be here both for the neuro team (as far as our outreach mobile clinics) as well as for our group as we have plans to spend two nights and three days in the Central Serengeti. There were recent videos of hundreds of safari vehicles being stuck along the only road into the park due to impassable spots that were too risky for them to drive through. One positive for us is that we’ll be driving a Land Rover, a much more dependable vehicle than the Land Cruiser when it comes to rough roads, water and not getting stuck. Regardless, it’s always a bit of a gamble going out into the bush as you never know what you’ll find or what will come your way. Needless to say, it’s always an expedition.
We had a good volume of patients today – twenty-five in all – with a number of children to keep Yoon Ji happy. It’s always a good mix of disorders for us, though a fair amount of MSK, or musculoskeletal, patients always seem to sneak their way which is not all to dissimilar to what we see back home and much the bane of our existence. To those non-neurologists reading this, musculoskeletal pain, such as back or neck pain, other joint pain, is not a neurological problem, though it often finds its way to us much to our dismay – here we have physical therapy handouts to give patients while back at Penn, we refer these patients to the “spine center” where patients are seen either by orthopedics, neurosurgery, or physical medicine and rehabilitation, after they are triaged by a nurse coordinator. No such luck here as those specialties don’t exist and it is up to us to educate the patient and/or family.
One very unfortunate patient who we were asked to see at the end of the day, was a 47-year-old gentleman who was brought in after suffering sudden onset of left-sided weakness and depressed mental status a day after having had a severe headache and loss of consciousness. A CT scan was obtained shortly after his arrival and demonstrated a massive right hemispheric stroke involving all of the MCA territory as well as a portion of the ACA with early hydrocephalus and marked midline shift with early herniation. In a nutshell, it was unlikely that he would survive the stroke. On examination, he had already blown his ipsilateral pupil and had a dense left hemiplegia with no response to painful stimuli on that side and was overall obtunded and unable to follow any commands.
In the US, he would have been taken immediately for a hemicraniectomy, possibly, as long as it was felt that he was salvageable, and the family understood that it was merely a lifesaving procedure and would not in any way improve his functional outcome – that is, he had infarcted most of his right hemisphere and that side of his brain was irretrievable. In essence, a hemicraniectomy is a procedure in which half the cranium is removed, relieving the elevated intracranial pressure and the mass effect the swollen infarcted hemisphere is causing that is compressing the healthy intact hemisphere. In fact, by removing the side of the cranium over the infarcted hemisphere, the flood gates for increased edema in that side of the brain are now open and you are sacrificing that hemisphere for the healthy one. Again, it is only performed as a lifesaving procedure and in the correct circumstances where the outcome is well-understood by everyone.
After a discussion with family and informing them of the likely consequences of the patients massive stroke (i.e. complete dependence if he were even to survive), they agreed with a conservative course and palliative care. As to our suspicion for a cause of the patient’s stroke, given his headache and unresponsive episode the day prior, we were reasonably confident that he had suffered a carotid dissection and was having thromboembolic events with the last one occluding his right MCA and most of his ACA. Again, at home, one may have considered mechanical thrombectomy, though the likelihood of its benefit would have been significantly limited and it’s doubtful that it would have been offered.
Heartbreakingly, Elyssa Weisman, my long-time patient, passed away in her sleep last July. I had first met Elyssa and her parents, Robert and Judith Weisman, in November of 2014 as part of my work with Children’s Hospital of Philadelphia and their trisomy 21, or Down syndrome, program. I had been asked to help out from a neurological standpoint with the trisomy 21 patients as they very often have neurological conditions and it was felt that these patients would benefit from having a neurological evaluation as they graduated from the care at Children’s Hospital and entered adulthood, at least medically. There are certain conditions that should be screened regularly, such as hypothyroidism, and others that are common enough to keep an eye on, such as dementia with the same pathology as Alzheimer’s disease, atlanto-occipital subluxation, and cerebrovascular disease. They can also develop a rare, poorly understood disorder called Down Syndrome Regression Disorder (DSRD) that is often mistaken for the early onset of Alzheimer’s disease seen in trisomy 21 patients.
When I first met Elyssa, she was 37 years old and had a history of complete AV canal repair, mitral regurgitation, and paroxysmal atrial fibrillation that were all closely followed closely by cardiology, hypothyroidism, and obstructive sleep apnea, and I had been asked to see her for abnormal behavior and motor tics that were very Tourette-like. It wasn’t long before I had formed a very close relationship with Elyssa and her parents and slowly became their primary contact in the healthcare world. Early on, I had noted that Elyssa had significant difficulties with her ambulation that I felt were unrelated to her body habitus, and after a short evaluation, discovered that she had a high cervical panus formation (as a result of her atlanto-occipital subluxation) that was causing severe cervical spinal stenosis and compressing her high cervical spine. Essentially, she had a cervical myelopathy that was affecting her gait and making her very unsteady.
After lengthy discussions with Robert and Judith, and a consultation with Dr. Sean Grady, chair of neurosurgery at Penn, it was clear to all that it would have been very difficult for Elyssa to have tolerated the type of surgery and fusion required to fix her problem, and the decision was made to monitor her clinically. Over time, as her gait continued to worsen and she began to occasionally fall, she also started to show signs of the early dementia seen with trisomy 21, further complicating the issue. Judith and I repeatedly revisited the decision to forego surgery for her cervical spine, reassuring ourselves that we had made the correct decision. Through all of this, Elyssa continued to persevere, as did her parents, and life continued as it had, with Elyssa bringing joy to everyone she met and maintaining her happy, cheerful self.
Though it seems inevitable that all of these medical issues would eventually have caught up with her, it was still unexpected when Judith had texted me that Elyssa had passed away quietly in her sleep. Of course, it was clear to all that this was a blessing in disguise considering the hardships Elyssa had endured throughout her life, but she had never really complained, and now she was gone. I sat with Judith, Robert, and other members of the family for shiva and grieved the loss of such a special person. For any of us who have cared for trisomy 21 patients, as I have over the years, it is self-evident that these individuals, as well as their families, all have a special place reserved for them in heaven or whatever they believe the afterlife to be. Shortly before our departure for this trip, Jill and I had the opportunity to visit the Weisman home and Elyssa’s room to see if there were things of hers that might be worth bringing with us to Tanzania. As we sifted through her many things, deciding what was feasible to bring, I felt her presence as if she were there offering many of her favorite Disney jigsaw puzzles and books for other children to enjoy.
