François looked up at me and smiled. “Spinal cord!” he exclaimed enthusiastically, proud of his newly acquired neurologic diagnostic skills.
“Exactly,” I replied, smiling back at him. “The combination of spastic quadriplegia, hyperreflexia, and bilateral Babinski signs with no problems above the neck suggests a problem in the cervical spinal cord. Now, in my experience in Haiti, non-traumatic paraplegia or quadriplegia is either Pott’s or it’s not.”
Pott’s disease is a tuberculosis infection of the spine that causes one or more of the vertebrae to collapse, which can lead to compression of the spinal cord. Tuberculosis (TB) is mistakenly considered a disease of the past by people who live in high-income countries, where it is exceedingly rare. But TB isn’t a disease of the past in the world’s poorest countries, where it continues to infect ten million people per year and kill more than one million people per year—more than AIDS. Haiti has the highest yearly rate of new tuberculosis cases in the Western Hemisphere.
“Let’s feel for any spinal deformity that would suggest a collapsed vertebra due to Pott’s,” I said to François.
As my hand approached Davidson’s neck, his eyes grew wider. And as I pushed on the bones of his cervical spine in the back of his neck, he grimaced in pain. “Sorry, sorry, almost done,” I said as I quickly walked my fingertips up and down his neck. Nothing felt out of the ordinary. I gently tried to bring his chin to his chest, but it was immovable, completely rigid. He started to cry softly, big warm tears streaming down his cheeks. “Okay, done. Sorry. I’m done now. Sorry.”
Enel wiped Davidson’s tears with his hospital gown while François and I stepped away to talk through his case.
“I don’t feel anything to suggest Pott’s,” I said, “but we still might see a bony defect on an x-ray or CT scan that could help us make the diagnosis.”
We sat down at the nearby nurses’ station to review Davidson’s CT scan on a computer. The scan didn’t show any problem with the bones of the spine, so Pott’s was off the list. I worried this meant we were very unlikely to make a diagnosis for this young boy. We had seen so many paralyzed patients with no diagnosis in Haiti that we had developed a name for the scenario: cryptogenic paraplegia (or quadriplegia)—paralysis of both legs (or all four limbs) of cryptic origin.
But Davidson’s case turned out not to be cryptogenic at all. At the point where his spinal cord arose from the base of the brain, his CT scan revealed a huge cyst compressing the spinal cord to the width of a thin string.
No wonder he kept his neck rigid and refused to move it. He was literally hanging on by a thread—a thread of spinal cord connecting his head to his body. Any further compression, perhaps even if he bent his neck the wrong way, could cut off the brain’s signals to his lungs to breathe. His neck muscles must have become rigid over time from not moving in order to guard his spinal cord.
It was hard to tell if the abnormality on Davidson’s CT scan was in the spinal cord itself or just compressing it from the outside. If it was just compression, maybe surgery could relieve the pressure and give him some of his motor function back. On the other hand, his spinal cord could be irreversibly damaged from three years of being so severely deformed.
Here we go again, I thought. In any rich country, this unfortunate child wouldn’t have had to wait three years for a diagnosis, let alone the diagnosis of a problem that might have been fixable before it got to this point. But if whatever this was kept growing, it could eventually affect his breathing. He would suffocate and die.
Should I try to get Davidson to the US for surgery? Removing the cyst might do nothing more than save him in this quadriplegic state, leading to a life of complete dependence on others. This would be a challenging existence anywhere in the world, but especially in a place like Haiti. Davidson would not have a power wheelchair that he could control with what little strength remained in his left hand. And even if he did, navigating the rocky, hilly dirt roads would be treacherous. There would be no accessible ramps to enter schools, which in any case would have no adaptive technology to help him learn in his paralyzed state. If his father died, he’d have no way to take care of himself. He would be as bad off as Janel—maybe even worse. At least Janel could move a little.
I looked over at this small boy in his big bed. His eyes were filled with intelligence as he scanned the room, calmly observing his surroundings. He was in a corner bed, the exact same spot in the adult ward where Francky had died a few months earlier. Wincing, I looked away, closed my eyes, and shook my head. The possibility of getting involved in another potentially tragic story seemed overwhelming. I felt like I had failed Francky, who had died with false hope that we would be able to help him. I had managed to get Janel to the US, but nothing had gone as planned and I wasn’t sure we had really helped him in the end. It was probably just too late in his case. It might already be too late for Davidson too.
As I began to feel depressed by the deep hopelessness of it all, I realized I was going have to make a strong effort not to let my past failures overinfluence how I approached this boy’s care. To start, I needed an expert opinion from a pediatric neurosurgeon to get a sense of whether there was any way to help Davidson. If there wasn’t, at least I could be straightforward with his father about this devastating prognosis. If it looked like surgery could help him . . . I decided I would cross that bridge when I came to it.
I Googled pediatric neurosurgeons specialized in spine surgery in Boston and found Dr. Mark Proctor, the chair of neurosurgery at Boston Children’s Hospital, which is just across the street from us at Brigham. I dashed off an email to him about Davidson’s case.
While I awaited Mark’s reply, François and I explained to Enel that the scan showed a problem at the top of his son’s spinal cord. We weren’t sure what it was. Whatever it might be, there was no treatment for it in Haiti. It was possible surgery might help, but we weren’t sure. And even if surgery could help, it couldn’t be done in Haiti, and we couldn’t promise we could get him to the US due to the complexities of costs and visas. Would he like us to look into that possibility further? Yes. Did they have means to afford surgery abroad? No. Did they have passports? No. We told him we would try to get a better understanding of what might be possible after consulting with some colleagues in the US.
Just as Ian had wanted an MRI to better understand Janel’s case before committing to surgery, Mark wrote back that he needed an MRI for Davidson before he could say whether he thought he could help him. I was headed home to Boston, but Martineau offered to help arrange this.
A few weeks later we received the MRI report, which confirmed our impression from the CT: there was a large cystic mass compressing the spinal cord. But it was unclear what it was. A tumor? A congenital malformation that hadn’t become symptomatic until early childhood? An infectious cyst from some type of parasite? I took screenshots of the key images and emailed them to Mark. A few moments later I received his surgically brief reply:
Really bizarre. Not sure what it is, but it seems like something that could be treated and afford the child great benefit. I would definitely like to proceed. We can use neurosurgery free care pool.
I began excitedly texting Anne that Davidson’s case was accepted for free care and that I looked forward to working together again. But then I remembered that an initial yes had been just the beginning of a months-long saga to obtain Janel’s passport. Since Martineau no longer had his government connections to help us, Enel and Davidson would need to go through the process on their own.