Tuesday, September 10, 2019 – A wearing day for a pediatric neurologist….

Standard

Tuesdays are our educational days here that begin at 7:30 AM with a half hour lecture by one of the visiting volunteers typically. This would be our first of several lectures during our visit here and Marissa had volunteered to discuss Cerebral Palsy which was incredibly apropos to the day that she was about to have in clinic. The lectures here are sometimes challenging for a number of reasons, only one of which is the language barrier. Though every caregiver at FAME speaks English and both rounds and medical records are in English, it is often difficult to be certain that the language we are using, both the English and medical, are being perfectly understood by the doctors in the audience. Speakers tend to use medical terms that may not be common and it is natural for speakers to sometimes talk fast, both out of some level of anxiety and the time constraints that exist as we only have 30 minutes to cover a topic. I will often briefly stop the talk to make sure that everyone understands what the speaker said or to give the generic name instead of the brand name which is not used here. Over the years, I have become accustomed to doing this as it is not second nature and the clinicians here are far too polite to interrupt a speaker and ask them to clarify. They leave that to Frank and me.

Marissa’s talk was perfectly constructed to cover all of the various types of cerebral palsy, the typical causes and the treatments that can be offered (not many) in these patients. It was at the right level – remember, the clinicians that we work with here are either clinical officers (essentially independently practicing nurse practitioners or physicians assistants), assistant medical officers (two years of additional training beyond the clinical officer) and MDs who have only completed an internship and no residency. Residency trained physicians are more of a rarity here as there is no system to support education such as we have at home in Medicare. Our residency programs are support by Medicare which allows residents to be paid a salary whereas here, one must pay for the majority of residencies (when they are available), which is out of the realm of reality for most individuals, or they will have to have a sponsor. There are very few specialty residencies in the country, too boot.

After Marissa’s talk it was time for morning report and the very first patient presented, by nature of her room, was the young 2-year-old infant with the encephalopathy that we had been following. Her summary was so brief that I nearly missed and wasn’t entirely sure that I had heard it correctly, but 30 minutes before our lecture, she had vomited while her mother was feeding her and then coded after an apparent aspiration. Despite all attempts to resuscitate her, she died. I found all of this out after report, because as I said, I didn’t catch everything when it was originally presented. Marissa and I discussed it after we were certain that she had passed and were both very disappointed that we hadn’t been able to do more this child. She died without a definitive diagnosis which is always frustrating for us, but we had little else to look at. Her second LP had been very benign other than a possible elevated opening pressure which may have indicated elevated intracranial pressure related to whatever process what going on. She very likely died from the aspiration, but her increased ICP and the underlying process she was suffering from also very likely played a significant role in her death. It was very unfortunate and it had affected both Marissa and I as we were the two that had been involved with her case.

The family member of the gentleman with the subdural hematoma from yesterday came in this morning to speak to us, but the patient didn’t come as it was difficult for them to get him here. We explained everything to him and the fact that they would need to go to Arusha for surgery to evacuate the hemorrhage. He would very likely recover some of his function specifically in regard to his confusion and possibly his right sided weakness as well. I had reached out to the neurosurgeon in Arusha to make her aware of the patient and had also asked what funds they would need to bring with them in order to be treated at Arusha Lutheran Medical Center, where she worked. The amount was 500,000 Tanzanian shillings, or about $217, for the surgery and care that was required. The family indicated that he would go back to his village to raise the money and they would travel to Arusha the following day. As the patient had been stable for the last month, we didn’t feel that another day delay would be a huge issue.

Dr. Annie wearing her new piki piki helmet given to her by the last group here.

Marissa’s day was made up of almost all children with profound neurological disabilities secondary to either birth related injuries or childhood infections that had, unfortunately, occurred previously. Many of the children were cortically blind (typically related to occipital lobe involvement of their prior process) and had profound static encephalopathies, meaning that they had global impairment of cognitive function and were able to do very little, let alone anything for themselves. Most of their stories were very similar, whether their injury had occurred at birth or later, but the most significant fact was that their families had cared for each them impeccably with whatever resources they had. Most of these families bring their children to us with the hope that there is some cure, but unfortunately, that is not the case as there is no primary treatment for them other than to treat symptomatically – physical therapy, treat their epilepsy if it’s present, and possibly their spasticity with baclofen which we do have here. Several of the mothers were in tears when Marissa told them that their children would not recover, others had been aware of this without being told and took the news with far less emotion. Regardless, it was difficult for Marissa as she presented case after case of these children and felt that she had very little to offer them. I reassured her that they were far better off having seen her and that her care would now enable the families to focus on what could be done rather than continually searching for a cure that didn’t exist.

I have spoken on several occasions in the past of Ståle Anda, a wonderful Norwegian man who runs an orphanage in Mto wa Mbu, and cares for many neurologically impaired Maasai children, a number of who have muscular dystrophy and we have seen over the years. Ståle came here several years ago and, like everyone else here, decided to stay and open his orphanage to care for these children whether it was in his orphanage or whether he would assist with their care with them living at home. Either way, he has served as a saint here for many children who would otherwise have been abandoned and died. Ståle couldn’t make it today, but we saw several patients that he sent up including one young man with a muscular dystrophy or congenital myopathy. Unfortunately, the boy was now well into his teens with severe muscle wasting that was mostly proximal as one would expect. Thankfully, this is one of Ray’s areas of interest so he chose to tackle the case with Mike Baer, who is planning to go into neuromuscular disease. There was very little of his history or family history that was obtainable as he was now in the orphanage and given his advanced disease it was very difficult to further characterize his progression of disease over the years that would have been essential to make the diagnosis in the absence of other information such as genetic testing. Ray noted that his best guess in this patient would an autosomal recessive limb-girdle dystrophy, but, unfortunately, that would be the best we could do with the information at hand.

During the day, Frank advised me that a patient was traveling here from Arusha to be seen and that he wanted our help with them as they had developed monocular visual impairment the day prior. The patient was in his mid-fifties with diabetes and hypertension and reported that he had been driving the day before when he suddenly lost most of the vision in his right eye. Frank had Sehewa, our nurse anesthetist/optometrist see him first, and he dilated his eye and did a brief examination, but we felt the problem was going to be vascular and more likely in our realm. He had some hemorrhage on his fundoscopic examination raising the question of central retinal vein occlusion, though it was not florid enough to warrant the “blood and thunder” description that is most often used for this entity. After thoroughly analyzing the case, it was felt that the patient had an ischemic optic neuropathy, which is an ischemic event of the optic nerve, so there was very little else to do at this point other than placing him on aspirin and providing further secondary risk management with a high dose statin and good blood pressure control. The prognosis for his condition would be that 40% of these patients have some recovery of their vision so he would very likely be left with some deficit going forward.

Towards the end of the day, Kyra had an interesting elderly gentleman who presented to clinic, brought by his son who I know, who has had a month-long history of difficulty with gait and back pain. His bowel and bladder function were normal. He had had no prior evaluations and on examination was found to have left leg weakness and right leg sensory changes with about a T7 sensory level making us concerned for a thoracic cord process. Though he did not have any clear symptoms to suggest systemic or pulmonary TB, we frequently worry about Pott’s disease here as we have seen several acute cases and many patients with the sequelae of this very disabling condition. Pott’s disease, or tuberculous involvement of the vertebrae (tuberculous spondylitis), can often present with neurological symptoms due to the erosion and collapse of the vertebral body as well as extension of the infection into the spinal canal, both causing cord compression.

We have had patients come to see us, as with many other catastrophic neurological disorders, many years after their bout with Pott’s disease, who are in a wheelchair with a paraplegia and chronic back pain. We have little to offer these patients other than to treat their pain as the neurological deficits once present are rarely reversible in the condition as with many other conditions affecting the spinal cord. We decided to obtain a plain film of the thoracic spine first as if this study demonstrated bony pathology in the spine it would have answered our question without going to additional testing, though a CT scan at that point would also have been helpful to know the extent of destruction, perhaps. The study, though, was negative, essentially ruling out the condition in regard to it causing his neurologic deficits, and now he would need to undergo an MRI scan of the spine to further visualize what was causing the issue and, hopefully, it would treatable. They had indicated that they would try to go to Arusha to have the MRI done to get the answer.

We made it home that evening after a very long day in clinic and Marissa have seen a tremendous number of devastated children. Ray’s first question for me as I walked through the door was whether we had any Zoloft handy for her. It would be impossible for her, or anyone else for that matter, to have seen that many children with these heart-wrenching stories and not to have been affected as she was. Regardless of the fact that we are doing unquestionable good here, we must also resolve ourselves to the fact much of what we see we are unable to treat, most that would otherwise be untreatable at home, though some that are the result of the limited resources available here and the reality of the region in which we are practicing.

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