Chiari Malformation: Facing the Pain
A father and son fight through the ordeal of multiple surgeries to repair the boy's skull.
In the fall of his fifth year, my son Charlie began complaining of brief, blinding bursts of head pain, which the pediatrician diagnosed as tension headaches. My wife was skeptical, but I believed the doctor, falling prey to the self-protective notion that children are never truly as sick as they say.
Charlie’s headaches eventually hit a peak of 20 per day. They interrupted his kindergarten activity so routinely that the school nurse began to prepare her small cot in advance for his many visits. Unusually independent and close to his own feelings, Charlie developed a ritual of self-hypnosis to deal with the pounding in his head and, later, the tingling sensation in his arms and legs.
An MRI eventually revealed Charlie’s problem: a condition called Chiari malformation. The back of his skull was slightly too small for his brain, constricting the crucial flow of cerebrospinal fluid between brain and spine. The tingling was the result of a growing fluid-filled cyst in his spine called a syrinx, also caused by the constriction. Left untreated—or undetected—the syndrome could cause paralysis.
We sent Charlie’s films to five renowned pediatric neurosurgeons. To our shock, they responded to our e-mails with completely different recommendations. Several of the proposals were so nightmarishly invasive it was hard to believe they could be contemplated, much less performed, by sane men. One surgeon, however, persuaded us to try a new, conservative approach that would “leave no hardware” in Charlie’s body. In October 2004, he and his team removed a sliver of my son’s skull to create more room for the fluid. Our hopes were trounced when the headaches returned within a month.
More doctors, more Websites, more message boards, more specialist e-mails, more desperate sobbing. I broke down in my boss’s office as I described being emotionally assaulted by fate, like riding blindfolded on a roller coaster.
Charlie’s original surgeon all but begged to try again, this time with a more aggressive plan. In the end, we saw him as the physician least likely to treat Charlie’s brain like a Thanksgiving turkey.
The surgical team went back into my son’s cranium and this time added a graft to expand the thin covering of his brain, the dura, like tailors letting out a pair of pants. Afterward Charlie looked like the victim of a terrible highway accident. But worse than the long bandage crawling up the back of his head, the many leads glued to his chest and the IV needles piercing each hand was the look of helpless confusion in his eyes. The way the adult-size bed swallowed his body reinforced the gross injustice of it all. I felt impotent and desperate. More than anything, I wanted to take his place.
The nurses gave Charlie a pain indicator card on which was printed an array of faces gradually becoming more anguished. I always expected him to point to the one most twisted in agony, but he usually chose the expression in the middle, a face not in terrible pain but resigned to a bleak reality.
After three nights in the hospital following the second procedure, we packed Charlie carefully into the minivan and drove home. The headaches persisted, but during the next few weeks their frequency diminished remarkably. His first 24-hour cycle with no pain reminded me of the first time he slept peacefully through the night as an infant. It’s the kind of primal relief that drops you to your knees.
Today the only physical reminder of this period is a soft, faint scar beneath the sandy hair on the back of Charlie’s head. Unlike my emotional scar, Charlie’s goes barely noticed or remembered. In that way, I do feel like I’ve taken on his pain—a bargain for which I’m forever thankful.
First Person originates at the other end of the stethoscope, presenting essays and commentary from patients, consumers and other medical outsiders. Proto invites your contributions; please send ideas to the editor.