But this doctor was concerned. Hiccups could come out of nowhere and stay, but when they lasted this long, you had to at least consider other possibilities. A hiccup is a spasm of the diaphragm, the muscle that separates the chest from the abdomen. They are usually triggered by overeating or reflux or other common phenomena that irritate the diaphragm or the nerve that powers it. A chest X-ray didn’t show anything on the diaphragm itself, so the E.R. doctor was concerned that the problem might originate in the nerve, or even in the brain. He gave the couple a list of neurologists and more chlorpromazine. The first available neurologist appointment was 10 days away. A few days after leaving the hospital, the increasingly frightened, exhausted, still hiccupping man and his wife returned once more.
Mark Goldin was the doctor on call that day. The man explained that the numbness and tingling that he had in his left arm for the past couple of years had suddenly spread to the other arm. His legs were numb, and he was having trouble walking. Examining him, Goldin found deficits that were subtle but real. When he asked the patient to reach out and touch the doctor’s finger with his own, he missed. His left arm was much weaker than his right, and his reflexes everywhere were abnormally exaggerated. The combination of hiccups and odd neurological symptoms worried Goldin. Was there an infection in the patient’s brain? A tumor?
A head CT scan showed that the patient had a small mass right where the brain joined the spinal cord. The brain tissue surrounding the tiny mass looked different from the other surrounding tissue. But it was hard to see just what was going on because of the bone at the back of the skull. An M.R.I. allowed them to see that the mass was encased in a sac of fluid that extended into the spinal cord like a tadpole’s tail. Goldin had never seen anything like it. Was this a collection of tumors — metastasized from a cancer somewhere else in the body? Or was it one big, strange tumor?
Goldin phoned a neurosurgeon on call, Ahmad Latefi, with the results. The surgeon was quiet as he reviewed the M.R.I. on his own screen. He recognized the tumor immediately. This is a hemangioblastoma, he said flatly. The man needs surgery.
Pressure on the Diaphragm
Hemangioblastomas are rare, slow-growing tumors usually found at the back of the brain or within the spinal cord. This type of tumor stimulates the growth of blood vessels to supply it with the high volume of oxygen and nutrients necessary for its growth. The additional vessels and blood give the tumor a reddish-orange color, a striking contrast to the surrounding gray tissue of the brain. Most of the symptoms patients experience derive not from the mass itself but from the fluid-filled cyst that surrounds it, which presses upon the spinal cord, limiting its blood flow and crushing the delicate nerve tissues, causing, in this patient, the hiccups as well as the numbness, the tingling and the weakness. If not treated promptly, the damage to the cord would be permanent. And if not treated at all, the patient would certainly die.
The patient was young and healthy, but the surgery required to remove the mass was still quite risky, and the chance of causing paralysis or death was high, Latefi explained. But not having the surgery was riskier still.
In the operating room, Latefi gently removed the bony layer of the spine, where he knew the tumor was located. Once past the bone, the surgeon moved his microscope into place. He cut through the dura, the thick protective sack around the cord. He had to get past the long fibers that make up the spinal column without cutting them to reach the tumor. He gently separated the fibers connecting brain to body to reveal a bright orange, marble-size blob. Each of the many blood vessels nourishing the mass had to be individually cut before it could be removed. With that accomplished, the fluid-filled cyst would be resorbed by the body. The surgery lasted nearly six hours.
The next morning, two physical therapists came into the patient’s room. Could he walk? Let’s find out, he said. They helped him to his feet. Flanked by the therapists and supported by a walker, he made his way down the hall. The nurses cheered. One took pictures of him on his feet, less than 24 hours after surgery, to send to the team.
The man went home after five days. The hiccups persisted — it takes time for the nerves to recover. In the hospital, a nurse had taught him a trick that worked as well as any medication. If he poured a packet of sugar under his tongue, by the time it dissolved, the hiccups would be gone. It was remarkably effective — no one knows why. At home he didn’t use sugar. His wife bought him a cake to celebrate his recovery, and every time the hiccups came back, he took a bite of cake. Finally, after a week, the hiccups stopped for good. That was two and a half years ago. He’s had the hiccups once or twice since then. And the sugar trick still works.
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