By Marissa Anto,

Children’s Hospital of Pennsylvania Neurology Volunteer

The new mama, Editha, tightly held her small six-week-old baby; only a tiny head visible, floating in a sea of pink ruffles from her dress collar and wrapped snuggly in a far too large fuchsia felt blanket. Just for context, it’s a balmy 80 degrees Fahrenheit outside. I notice baby Glory’s elongated almond eyes and immediately recognize the tell-tale sign of Down’s Syndrome. Before obtaining any history, I decide to examine the infant to confirm my suspicion. I find she has all the characteristic features including up-slanted palpebral fissures, transverse palmar crease on her hands, low-set ears, sandal gap toes and an increased skin fold at the nape of the neck. I start asking the young first-time mom questions about her pregnancy, the delivery and Glory’s first few weeks of life. Editha brought Glory in to see a neurologist at our mobile neuro clinic because she felt like the baby’s tone was low. Luckily, despite being small for gestational age (and with low tone), Glory was otherwise neurologically intact, feeding well and gaining weight. Yet I was concerned about her anterior fontanelle or soft spot on the top of her head because it was abnormally full and can signify hypothyroidism in a newborn. Hypothyroidism is a common hormonal abnormality that occurs in children with Down’s Syndrome and infants must be screened regularly for both this and blood count abnormalities as Down’s children also carry an increased risk for hematologic malignancy. Although Glory’s exam was largely reassuring, she needed to come to FAME ASAP for bloodwork (which couldn’t be done at her local clinic) to ensure she didn’t have any thyroid or blood abnormalities. Children with Down’s syndrome also carry a higher risk of congenital heart disease. Fortunately, Glory had a normal oxygen saturation and normal pink coloring without any appreciable heart murmurs. She would eventually need an echocardiogram or ultrasound of the heart to definitively rule out any heart defects.

As a pediatric neurologist, I am used to having tough conversations with families, explaining complicated and often distressing diagnoses. But how do you tell a new mom that her baby has Down’s syndrome when her language doesn’t have a word to describe the condition? In the United States, Down’s Syndrome or Trisomy 21 is fairly well-known to the public, with an estimated incidence of 1 out of every 700 babies born in the U.S. In Tanzania, this special population and the vocabulary used to describe them is virtually absent from mainstream society. I struggled to explain the condition and its common co-morbidities to Editha via an interpreter. Dr Anne tried looking up a Swahili word for Down’s but couldn’t find it. She asked one of our medical student interpreters, Abdulhamid, who also couldn’t come up with the appropriate terminology. I asked our social worker Kitashu, but he too said that, although he had previously seen some children with this condition, he had never known what it was called. Dr. Anne finally Googled a picture of children with Down’s and showed Editha, a look of understanding registering on her face. She said she now recognized what her baby has. 

Later that day, I searched online and found few posts on Down’s Syndrome in Tanzania. One was written by a mother of a child with Down’s Syndrome and was titled “There Is No Word for Down’s Syndrome in My Language.” In the article the mother details how there is no Swahili word to describe people with Down’s Syndrome and reflected that she had never known or seen children with Down’s Syndrome growing up in Tanzania. She speculated that families may have hidden children with Down’s or, even worse, completely neglected them due to the cultural stigma surrounding having children with special needs. She noted that there is virtually no data that exists regarding the incidence of Down’s Syndrome in Tanzania. The author of the article recounted that when her child was diagnosed with the condition, she unfortunately had very little support or guidance from the medical system in terms of what to do or expect for her child.

I wanted to make sure that this young mama before me did not feel the same way. I explained Down’s Syndrome to her and all the things we needed to screen Glory for at FAME. I explained that the baby’s fontanelle was full and that made me nervous, but the rest of her exam was reassuring. I gave precautions to return immediately should the baby develop any lethargy, vomiting, feeding intolerance or altered mental status prior to coming to FAME. Fortunately, Editha and Glory did come to FAME as instructed the following week. I was encouraged that Editha was able to make the two-hour journey to get her child the care she needed. Glory’s thyroid studies and blood count were both found to be normal. I reiterated all the things that would have to be routinely checked and what Editha should monitor for in baby Glory. I tried to reassure Editha that children with Down’s Syndrome can often lead very full, largely normal lives as long as their specific health care needs are met. Editha told me she only hoped that her baby would remain healthy and that she would do whatever she could to ensure this. This meant traveling two hours each way to FAME for a cardiac evaluation in a few months and making the long trip every time Glory needed blood work. I was reassured to see Editha so well-bonded to her baby, her affection and love obvious. She would do anything for this precious baby. And that is truly the most important thing a pediatrician can wish for any child but especially for a child with special needs.

 NOTE: The names of mama and baby have been changed to protect their privacy

By UPenn Volunteer Neurologist, Mike Baer

I met my first, and only, Hadza patient several days after arriving at FAME. We had been told that she was presenting with a seizure and a headache, but when I met her the story was more concerning. She had collapsed suddenly two weeks prior, seized, and since then hadn’t spoken a word to her family. When I examined her she was mute and weak on the right side. A head CT showed that she had a large intraparenchymal hemorrhage extending from the left temporal lobe to the left frontal lobe, which we felt was caused by either a ruptured aneurysm or a mass that bled. Either way, the prognosis was poor. We started her on antiepileptic medications and hoped to gain more information from a CT scan with contrast, which could reveal an underlying mass. 

The Hadzabe, I learned, are an ancient tribe of hunter-gatherers, who are struggling for survival and live in the Lake Eyasi region, just an hour or so away from our home base in Karatu. They traditionally communicate with a “click language” and survive on hunting game and foraging for fruit and honey. There are no formal leaders and little social hierarchy, so most important decisions are made through group discussion. With the rise of agriculture, livestock herding, and tourism their ability to survive as hunter-gatherers has become tenuous, and today less than 1500 individuals remain, including our critically ill patient. 

Unfortunately, just two days into her hospital stay, the Hadza patient had a second seizure. She was extremely lethargic afterwards, and the head CT with contrast showed that the bleeding had extended into her ventricular system, which was beginning to expand to create hydrocephalus. Her prognosis, even with surgery to relieve the hydrocephalus, had gone from poor to grim. We spoke with Dr. Badyana, the physician in charge of her care, and together we sat down with the daughter, who had remained at the patient’s bedside. Dr. Badyana explained that the patient’s condition had worsened and that the bleeding would ultimately end her life. We showed the daughter the images from the CT scan. She didn’t say much and she remained calm but it was clear that the news affected her greatly. Her demeanor was one I had seen before, back in Philadelphia during some of my end of life discussions for similarly dramatic intracranial injuries. The Hadza woman passed away comfortably the following day. 

During our four weeks at FAME, I and the other neurology residents from the University of Pennsylvania had the opportunity to interact with and learn from members of a variety of other tribes. We visited Daniel Tewa, a member of the Iraqw tribe and local farmer and historian, who showed us a traditional house that he had constructed, modeled after the home he was born in. He taught us about the traditions and customs of his people, many of which were changing from the pressures imposed on the Iraqw tribe from outside forces. We also treated a large number of Maasai and one of our translators, Kitashu, a Maasai and a FAME social worker, was helpful in helping us understand Maasai culture and how it related to the counseling and treatments we provided to our patients. I was particularly struck by the close social circle I witnessed and the readiness of the community to help in times of need. 

Working at FAME was an incredibly valuable experience. The clinicians, already proficient in a wide range of domains, were eager to learn about neurological diseases, a topic not widely emphasized in Tanzanian medical education. While we did treat a large number of patients, the most rewarding aspect of my work at FAME was helping the medical staff strengthen their capacity to independently care for many of the neurology patients that we saw. I look forward to seeing how FAME and healthcare in Tanzania advances to address the unique needs of the country’s diverse population, from the Iraqw to the Hadzabe and the Maasai. 

By Volunteer Dr. Mike Rubenstein

Over the last several years, we have come up with a pretty good system for just how to schedule our neurology clinics during each of the months that I am here with the residents. We knew that to build the program we would have to come on a recurring basis (hence the visit every six months) to develop a trusting relationship with the clinicians here and the patients and families who come to see us and appreciate our care. Back in 2011, we had also developed the Neurology Mobile Clinics to serve some of the more remote areas of the Karatu District. This model seemed to work very well: The clinics were very well attended, and patients, who wouldn’t normally come to FAME for medical care, could continue seeing us in the villages, while also having access to FAME if necessary. What began in 2010 with my teaching the FAME clinicians how to do a neurological examination for patients, has now evolved into a well-organized, month-long clinic attracting nearly 300 patients each time we visit.

Our visits are now comprised of an approximately week-long, clinic that we do here at FAME, a week of mobile clinics that entail travel to more remote villages in the district, and then several days of clinic back at FAME, where we see follow-up patients or patients who missed us the first time around. News of the clinics, including the mobile clinics in the villages, is well disseminated to the community by FAME staff. They travel throughout the district announcing our arrival a month in advance and inform potential patients of the types of disorders we treat. In addition, FAME’s social worker, Angel, has a list of patients who had visited FAME in the time between our visits with neurological disorders and would need to return to see us.

The first few days after our arrival are reserved for those patients Angel will call. This structure allows the new residents to get a good feel of how things run here. Every patient is seen with an interpreter (who might also be a clinician) or possibly two interpreters if the patient doesn’t speak Swahili, which is not uncommon among some of the local Iraqw and Maasai populations. The medications we use are limited and some are different than those we have in the US. The doses are different than what we are used to and the options for testing are considerably different. Additionally, the types of therapies we have at our disposal are much more limited than those in the US. However, in the end, it is still mostly the same disorders we are treating here, though the differentials may vary quite a bit. The principles of medicine are still the same, and it is all a matter of taking a good history, performing a good examination, and developing your differential. It is actually medicine the way medicine was meant to be practiced – an all too uncommon event in today’s world.

Today was our first announced day and what we encountered outside the emergency ward (having been taken over this month to be our neurology clinic) was a bit of a mob scene, with patients everywhere waiting for Angel to get them registered. They would then proceed to have their vitals taken so we could begin to see them. Our first patient at morning report was a young gentleman, who had arrived during the night with new onset left hemiplegia following left neck pain. He was also found to be HIV positive – a new diagnosis. He would need to be seen in consultation by one of the residents.

We had three exam “rooms” for patient visits, and I told Angel that we could start by registering thirty patients for the day, as I knew we would inevitably see more than that. Later in the morning, the residents suggested setting up a fourth station to see patients waiting in the hallway adjacent to one of the other stations. So, it was with this arrangement that we began to plod away seeing patients for our first “announced” clinic day of the season. The number of patients we would see would quickly increase well beyond the thirty we had originally registered.

Ståle, a gentleman who runs a home in Mto wa Mbu and whom I have known for several years, came this morning with his car full of neurologically impaired children. Somehow, he has been tasked with caring for a number of young men with muscular dystrophy, most of whom we diagnosed and have treated for several years. He brings all of them on the same day packed into his Land Rover with their wheelchairs tied to the top.

Every resident who has met Ståle and the children he cares for has been changed for the better after seeing the dedication he has to these kids and the wonderful outlook these children have, despite knowing the condition they have and what the future holds for them. With this in mind, we saw all the kids, most of them with Duchenne’s muscular dystrophy, and continued on with our incredibly busy day.

One of his young boys, who had recently moved to Ståle’s home and had been assumed to have Duchenne’s muscular dystrophy, was clearly different from the other children. I saw him with Amisha, a neurology resident from Children’s Hospital of Philadelphia, and, unfortunately, we really had no history as the boy couldn’t give us any and Ståle had no family members to contact. The boy was unable to walk, but had no pseudohypertrophy of his calves and the majority of his atrophy was in his shoulders and arms. He also had some mild wasting of his temporalis muscles. Given this constellation of findings, it became clear to us that he had either fascio-scapulo-humeral or limb-girdle dystrophy, both of which have a tremendously better overall prognosis than Duchenne’s as they have a normal life span, albeit with significant disability. Still, it was a bit of good news we were able to relay.

Finally, in the midst of our incredibly busy clinic, I found that a patient had been brought in to see us on a stretcher and was promptly placed in the emergency room. Dr. Gabriel had mentioned to me previously that there was a patient he wanted us to see (and appropriately so), who had been in the hospital here in July with presumed encephalitis and wasn’t recovering. Unfortunately, after bringing the patient in, he promptly began with focal seizures that appeared to be epilepsia partialis continua – something that can often be difficult to treat. According to his family, the seizures had begun about a week prior and were essentially occurring on a regular basis. His original presentation was such that he was found unresponsive by his family at home and then had been brought into a local hospital where he remained unresponsive for about another 48 hours before being transferred to FAME. Here, it was clear that he had an encephalitis and underwent an LP and was placed on antibiotics and acyclovir. He eventually had a CT scan showing numerous large early hypodensities in the brain and more specifically, the bilateral temporal lobes, the most common location for injuries that occur in herpes encephalitis.

He had been treated with a 21-day course of oral acyclovir, but had never really woken up. He had also been placed on carbamazepine for his seizures, but at a relatively low dose considering the injuries to his brain and his propensity to have seizures for the rest of his life. As he lay on the emergency room gurney, unresponsive with continuous jerking of his left face, arm and leg, his family stood by patiently awaiting our input and whether we had anything at all to offer their 28-year-old family member. There was no issue with handling the seizure part of the equation, but having our very own infectious disease specialist here was certainly a blessing. We have no confirmatory tests here such as a PCR on the spinal fluid, so the diagnosis would be based on the clinical features that were quite suspicious for HSV encephalitis with treatment that had been delayed by at least several days. Herpes simplex encephalitis is something that must be treated immediately upon consideration of the diagnosis, as the virus rapidly multiplies and the damage it causes becomes more extensive. It is also irreversible once it occurs. The mortality rate for HSV encephalitis can be very high even when treated and the morbidity is great with chronic seizures and very common severe cognitive deficits.

We had to tell the family that he would not recover any of his function and would almost unquestionably remain unresponsive if he did not succumb to some complication. Yet, we could possibly improve his seizures by increasing his carbamazepine and, if that did not work, we could add phenobarbital later.

Our other patients were the typical mix of epilepsy, Parkinson’s disease, headaches, and back pain, to name but a few. Thankfully, we had no plans for the night as we had seen a total of 42 patients – the largest number we had seen in a single day.

Dr. Michael with a little patient, Photo by Ali Mendelson

By Neurologist, Dr. Michael Rubenstein

I am taking today's blog title from an amazing book by Tracy Kidder about a young medical student who survives the genocide in Burundi to find himself in the United States. It's a true story and so as not to give away too much I will suggest that you find a copy and read it. The inspiration for today's blog, though, is another amazing story of strength and hope here in Northern Tanzania.

Our very first patient of the day was a young woman who had recently been diagnosed with epilepsy and was on a medication that she was not taking on a regular basis. Thus, she was continuing to have seizures. After taking our history and examining her, though, we had some concern for the type of epilepsy she had and felt that an EEG would help to sort it out as the medication one uses is often dictated by the type of underlying epilepsy the patient has. We brought her over to the ER which is serving as our makeshift EEG lab for the time being and the epilepsy team hooked her up for a study. She was patient No. 1 for this groundbreaking technology at FAME. Amazingly, she had exactly the type of epilepsy that IS NOT well treated by the medication she was taking and she was converted over to a medication more effective for her condition. In fact, the medication she was on can sometimes worsen seizures for patients with her condition. This could certainly make the difference between well controlled and poorly controlled epilepsy which would make all the difference in the world for this young woman.

We saw an early tremor dominant Parkinson's patient who we had not seen before - Dr. Thu was incredibly excited since she will be going into a movement disorder fellowship next year and this was right up her alley. We saw another stroke patient who I first saw in 2011 and continue to follow up with on a regular basis even though he has continued to do well. The hardest thing here is to get patients to remain on their long term medications, though, as it is just something they aren't used to doing. We had wanted him to remain on aspirin but he had unfortunately stopped it several months ago after his prescription ran out. It is not due to non-compliance, or lack of adherence as they now refer to it, but rather that it's just something that isn't build into their culture at the present time. We'll continue to work on that.

So now for the story that inspired the title for today. A woman was brought to FAME today by her friend and eldest son. She is 47-years-old and the single mother of five children and the primary caregiver for her elderly mother. One year ago, during the night and for no apparent reason, people came into her home and threw acid onto her face. Those responsible have never been caught and there was no clear motive to suggest why it may have happened in the first place. She spent two days in a local dispensary (about three hours from FAME) before they realized that she needed more extensive care. She was then transferred to another hospital where she spent only three days. Antibiotics and bandages were applied and she was sent home. When she arrived home she found that many of her possessions there had been stolen. The acid has disfigured her face to such a degree that she has lost both of her eyes and is now blind and all that remains of her nose are two small holes for nostrils.

Despite this horrendous injury and disfigurement, she has persevered and has a remarkable attitude. When asked about any sad thoughts, she does admit to some concerns as to how she will continue to care for her children and her elderly mother, but says that she has accepted what has happened to her and is ready to move on. Her only complaint to us was a minor headache. She was an incredibly lovely woman and when she spoke it was quite easy to forget her disfigurement or the ordeal that she had been through. At the end of our visit, she asked if she could have a photo of her with Thu and myself. I think all of us wondered if we would have that amount of strength had we been put through a similar situation. And, almost to add insult to injury, we found today that she also has diabetes as her blood sugar was extremely elevated and that this will also need to be dealt with. As I walked beside her to the lab for her blood work, I could feel the strength and livelihood emanating from this woman who made me realize that in the depth of our struggle for survival, there are always those unlikely individuals who have demonstrated an even superior strength to have risen from further depths and will always give us eternal hope that we may do the same. It is these unlikely encounters that remind of us of our internal strengths. 

By Pediatric Neurologist Volunteer,

Doug Smith

Remember the days when you didn’t need an alarm to wake up?  When you woke with the sun rise, and your body was so anxious to rid itself of sleep that it was insufferable to stay in bed?  The song in your head is not one you heard on the radio and can’t shed, rather it’s the background music of your life.  You knew that the day ahead was one to remember.  One where you will experience the payoff of weeks, months, or perhaps even years of planning and anticipation.  Possibly one in which you will meet challenges or opportunities you have never had before, ones you feel prepared to overcome, with meaningful experiences as your reward.

That’s what this morning was like.  That’s what nearly every morning has been like during my time in Africa.  At night, the darkness is absolute, an almost palpable heaviness to the air.  The lack of sensory input gently and slowly turns the brain off.  It is disarmingly relaxing, and I find myself in bed and asleep well before I had intended to rest.  Come morning, the energy of the world changes with the sun rise.  The light calls to action dozens of songbirds and crowing roosters.  You can nearly feel the world waking up, and the call is hard to deny.  Then you remember what you’re here to do, and it really is impossible to stay asleep.

This is my view while I eat breakfast. I can literally see where my morning coffee was grown.

The medical experience thus far dwarfs my time in Botswana.  Morning starts with 8:30AM rounds.  FAME Medical has just 6 general inpatient medical rooms, each with 2 beds.  Despite the low numbers, the acuity and complexity of patients is impressive.  Currently, over half of the admitted patients are children.  We spend a particularly long amount of time with one child who became neurologically devastated over the past three months; I come up with some answers, but without significant testing abilities, I’ll never know if I’m actually right.  The father, a proud warrior whose stretched earlobes and traditional garb could just as easily grace the cover of National Geographic, tears up at my explanation.  He will not be the last person to receive bad news today.

5 kids from a nearby orphanage, awaiting their turnto see me.

The rest of the medical load, while significant, is African-casual paced.  Today, two orphanages delivered half their children to our doors, having heard that for the first time ever, a pediatrician has come to town.  I see five of them today.  The experience is exactly what I have come for.  

For two of the boys, I can make a diagnosis: muscular dystrophy.  They will never walk again. It is not all bad news, though.  A large percentage of families here do get the answers they've been seeking for a long time.  Down syndrome, Ohtahara syndrome; in these cases, I can offer a somewhat clear picture of the future, even if it is not the one they had hoped.  For others, they get confirmation of what they had suspected: that their severely weak, "disabled" child in fact has a full mind, capable of learning.

This is no small reward.  You see, schools here are very limited in their resources.  Any child with any somewhat significant problem is refused entry to school.  As an example, one 10 year-old boy was refused entrance to school his whole life because of a very subtle tremor, and they came to meet me to get a note saying he was medically cleared for school.  After TEN years!  In a more heartbreaking example, a boy with severely dystonic cerebral palsy, where he has basically lost control over all of his muscles (including his mouth and face), has a very full cognition, as far as I can tell.  When I asked his caretaker what I can help with, the boy replied, "I am a cripple."

Where schools have failed, the generosity of others have succeeded.  He was taken in by a Norwegian man, whose name I can't pronounce, let alone spell (it sounds like Skroli, but I'm sure there's an umlaut or two in there).  He has spent every cent he owns and has raised money from Norway to build an orphanage of sorts for children like him, children with significant physical handicaps but a lesser extent of cognitive impairment.  He takes them in and teaches them, not just basic schooling but also life skills, with the hopes of one day reintroducing them to their families.  So far, I have seen 7 of his sickest, and have given him half the money in my wallet to continue doing what he's doing.

Were it not for the FAME clinic here in Karatu and for Dr. Mike Rubenstein, the Penn neurologist who introduced me to the facility and is here with me now, none of these neurologically impaired children would have ever found the care they need.  Thanks to their charity, they are on appropriate medications now, and being cared for by people with the right mentality towards disability.

Skroli will have many more children for me in the coming days.  I hope I have the emotional wherewithal to handle them, and that I brought enough money to leave with a clear conscience.