I searched for Janel’s name in the medical record app. As the slow internet connection loaded his chart, I noticed the location in the hospital listed for him. It wasn’t the emergency room—it was the operating room. The pit of my stomach tightened as I read the notes in Janel’s chart. He’d had another seizure shortly after arriving in the emergency room and had immediately undergone a CT scan. The scan showed that his ventricles were massively enlarged again. His shunt had malfunctioned. Ian had taken him for emergency surgery to repair or replace it.
Not only were we lucky that this hadn’t happened on the plane, we were very lucky this hadn’t happened in Haiti. Janel could have died.
I wrote to Ian to see what he thought had happened. Could it have been an effect of the altitude as I had feared could occur on Janel’s initial flight from Haiti to Boston months earlier?
“Every once in a while the shunt valves go down,” he replied. “It is actually a good opportunity to remove it and do an ETV.”
ETV stands for endoscopic third ventriculostomy, an operation in which the neurosurgeon makes a small hole in the skull and passes a metal scope slightly larger than a drinking straw through it into the ventricles of the brain. The scope’s tiny camera relays images to a video monitor. The surgeon guides tools through the device to puncture a hole in the floor of one of the ventricles, allowing a new channel for egress of excess fluid. In some cases of hydrocephalus, this detour makes a shunt unnecessary. Because of Janel’s shunt failure and the fear of another shunt complication when he was back in Haiti, we hoped an ETV would spare him the need for a shunt.
The shunt removal and ETV procedure would be Janel’s fourth neurosurgical intervention.
* * *
It was my third trip of the year to Haiti to work with Martineau and the internal medicine residents. The strategy of working regularly and intensively with a small group of local doctors was paying off. They could now easily diagnose recurrent one-sided pulsating headaches accompanied by nausea, photophobia (aversion to bright light), and phonophobia (aversion to noise) as classic migraines. They knew the instant they saw the combination of a masklike face, stooped posture, slow gait, and resting tremor that the patient likely had Parkinson’s disease. They recognized the common story of patients falling unconscious with rhythmic, uncontrolled shaking followed by confusion as epileptic seizures. And they learned how to treat these conditions with locally available medications.
Under their care, patients’ migraines decreased in severity and frequency when they were prescribed low-dose amitriptyline and encouraged to stop their near-daily use of aspirin and Tylenol. Patients who had been stiff, slow, and statue-like from untreated Parkinson’s disease began to move more fluidly and smile more animatedly when they were prescribed carbidopa-levodopa. Children with epilepsy not only finally had their seizures controlled with locally available anti-epileptic medications, like carbamazepine or phenytoin, but were able to go back to school—local teachers in rural Haiti thought epilepsy was contagious and often refused to let children with seizures attend. Patients expressed enormous gratitude, but this was bittersweet—I was so happy to see them feeling better and returning to their normal lives, but I found it troubling to think about how long they had suffered from such easily treatable conditions.
Unfortunately, not all of the patients we saw had such simple solutions.
A thirty-two-year-old single mother named Nadège had been diagnosed with migraines, but her headaches continued to worsen. She had also noticed bulging of her right eye—the medical term is proptosis, which always sounded to me exactly how it appears. She was otherwise perfectly healthy and even still had normal vision in the proptotic eye. Her CT scan revealed a marble-size tumor just behind her eye, pushing it outward and compressing her optic nerve, the conduit of vision from the eye to the brain. Without surgery, the growing tumor would cause the eye to bulge out farther, and she could go blind.
A fifty-three-year-old woman named Jesula also had debilitating headaches. Her CT scan showed a huge meningioma—a tumor of the brain’s lining—just below the top of her skull. The tumor had grown so large that all of the fluid-filled spaces in and around her brain had completely collapsed to try to accommodate the mass. Amazingly, she had no symptoms beyond headaches, and her neurologic examination was completely normal. Meningiomas are almost always benign and so surgery would be curative. But without it, she would meet the same fate as Francky, dying when the pressure in her skull forced the brain downward, pinching off vital structures.
A forty-year-old pastor named Jean-Rémy had progressively lost vision in his right eye and then began to lose peripheral vision in his left eye. By the time we saw him, all he could see of the world was through the right half of his left eye. This pattern of visual loss suggested a tumor of the pituitary gland, which his CT scan confirmed. The tumor had grown so massive that it had extended from the gland’s compartment at the base of the brain far upward into the brain itself. Without surgery, he would become blind and suffer severe complications of hormonal imbalances as his pituitary gland failed.
All of these patients could be big saves with neurosurgery. Their tumors were inside the skull but not within the brain itself, causing neurologic symptoms by compressing important structures rather than by invasion of healthy brain tissue.
I remembered when I had first heard about Janel’s case from Martineau. It had struck me at the time as a unique instance in my two years working in Haiti when bringing a patient with a neurologic condition to the US would make a major difference. Part of the argument to our colleagues for bringing Janel to Boston had been that it wasn’t likely to be a common request. After all, brain tumors account for only about one percent of all tumors, and some types of tumors within the brain itself were unlikely to be potential big saves even with surgery.
Then there was Francky, who could have been a big save. Now these three patients could be big saves. The CT scanner at HUM had opened Pandora’s box: we were diagnosing more and more patients with conditions we couldn’t treat.
This is something I was accustomed to in neurology since there are several neurological conditions that have no effective treatment—diseases like ALS and Alzheimer’s disease. There’s nothing more heartbreaking than telling patients that I know exactly what’s causing their symptoms but have nothing to offer beyond helping them and their families come to terms with the diagnosis and prepare for an inevitable demise. Though I was comfortable diagnosing neurologic diseases that I couldn’t treat in the US, the CT scanner at HUM was leading to the diagnosis of conditions that were treatable, just not in Haiti.
I wrote to Ian about these three new patients and sent him cell phone snapshots of their CT scans. I concluded the message:
After everything that happened with Janel, I don’t know if Brigham will be excited to do another charitable neurosurgical case. Any ideas of other avenues to explore to get these patients the care they need? They have much better baseline function than Janel—all ambulatory young to middle-aged adults with normal cognition.
Ian replied:
I still think we should try to do these cases here. We will go nearly a lifetime without seeing another tumor like Janel’s—and he was so debilitated before. These other cases sound like they will not require anywhere near the hospital length of stay that Janel required.