In any case, August’s body had become the site of a proxy battle.
The operations were brutal for our son and hard on Ilene and me. Because UNF offered no paid family leave, and because Ilene and I were not able to take time off from work, we couldn’t be on hand during the surgeries. So we had come to rely on Katrina Johnson, who stayed with him during the day to keep him company and to take care of him after the procedures. When we first hired her, she was a UNF student, and for about six years she worked as August’s principal caregiver. She grew to love him like a brother, and August in return loved her. We considered her family, and so having her at the hospital was like one of us staying with him. Often she was the one present when he awoke from the anesthesia. In the post-surgery phases, she’d had to hold his head up as he vomited into a towel.
Six surgeries in all, each with general anesthesia, were performed over a period of four months. The first was to implant the pump. This took place on July 8, 2010. The second, third, and fourth surgeries were devoted to attempting to disinfect the device and thus prevent further infection. A fifth surgery would be performed to finally remove the pump. And then, because cerebrospinal fluid wouldn’t stop leaking out of the incision site at the base of the spine, a final surgery was undertaken to address that problem. After this last one, Dr. Joyce told me that he had done “something special” to bind up August’s back internally so that the oozing would stop, but he didn’t elaborate much concerning what this had been.
The infection finally abated with the removal of the pump, and the discharge of cerebrospinal fluid from the incision site stopped with the sixth and final surgery, but the pump’s aftereffects were like a listserv that doesn’t have a button for you to unsubscribe. August had undergone five hospitalizations in few months. His number of days as an inpatient totaled more than fifty.
In late October 2010, this period in our lives came to an end. When I brought August back home for the last time, I surveyed the damage. So many operations had left his lower back a crisscross of permanent scars.
VIII
BY THE LATE SPRING OF 2011, AUGUST HAD MOSTLY recovered from the six surgeries of the preceding summer and fall, so we were able to get him back outside again in his walking device, the Rifton. Before the 2010 procedures, he had so loved walking in it. With the pump no longer inside him, we assumed that he would return to doing what he had done previously—pacing jerkily up and down the driveway. As before, he became excited when we rolled him in his wheelchair out the kitchen door, down the ramp, through the carport, and out to the top of our long driveway. As before, he squealed exuberantly as we lifted him out of the wheelchair and dropped him vertically into the Rifton. But then, once inside the device, he made no attempt to walk. He just stood there. This was the way things became for him. Permanently. Just standing still. He now was like a statue, a happy stationary fixture cooing in the wind. Gone forever were the days of fevered striding and the jollity of adventure.
Also, and exponentially more serious than not walking, was something incredibly creepy that had started occurring several months earlier, in November 2010, less than thirty days following the last of Dr. Joyce’s six surgeries. This was behavior Ilene and I had never witnessed. Dr. Munodi had warned that Joyce wasn’t properly weighing benefit versus harm and that there could be unwanted secondary effects. And this indeed came to pass.
August had begun arching. Arching, also known as extension, meant that, for periods lasting for up to ten days, August would involuntarily bend backward and to the side in a twisted position and remain in it. His limbs would be distorted, his back bent, as if perpetually trying to glance over his shoulder at his heels, causing him to resemble a human pretzel. For days on end he would be stuck in this contortion. In ballet, there is a pose called attitude derrière, and August sort of looked like that except that his leg wouldn’t be up in midair, his head would be permanently cocked to the side, and he couldn’t get out of it. “Fixity of posture and muscular rigidity” was how this condition was clinically described. External stimuli didn’t seem to bring on an episode, so we had no idea what event, if any, would trigger one. For the next three years, arching became, like Russia for Winston Churchill, a “riddle wrapped in a mystery inside an enigma.”
During these recurring ten-day periods, it was as if a monstrous winged reptile were roosting atop our house. This was an enormous, shrieking bird from one million BC, a pterodactyl unlike the blithe variety we had known. This one was ominous and fearsome. Ilene and I experienced constant anxiety on account of it. If we weren’t dealing with its presence, we were anticipating its return. For perhaps fourteen days August would be his usual, relaxed, calm self, but then something inexplicable would trigger an episode. The first sign of it would be a brief, involuntary gesture, a jerk, a twist, an arm spasm, something we named “the Egyptian.” The Egyptian would consist of his left arm suddenly shooting out as in a stock image of an ancient Egyptian. It would be a momentary gesture, but it would be enough to let me know that the dreaded prehistoric bird had appeared again on the western horizon and was rapidly winging its way toward us.
The Egyptian would be followed by three to five days of gradual ramping up, of August’s ever-increasing inability to sleep, and more and more nervous, manic behavior. Then, following this build-up, for perhaps the next five to seven days, he would remain in a fixed, twisted posture, stiff as a board, the Full Pterodactyl. Ancient, even primordial, it was a locked-in-a-pretzel form in which August would remain petrified for days. During the Full Pterodactyl, he would go for forty, fifty, even sixty hours straight without sleeping.
From the way he pursed his lips during the Full Pterodactyl I knew that August was uncomfortable. All of his life I had argued to others—mainly to disability skeptics who automatically assumed that he must be living in torment and so should be taken out of his misery—that August wasn’t suffering. And he hadn’t been. But during these arching episodes I had to admit that he was going through pain and distress. It was not constant, but there were hours during which he would moan and groan. And then the Full Pterodactyl would slowly subside. Afterward he would experience a period of calm lasting for as long as two weeks.
People who say that God doesn’t send you anything you can’t handle have never encountered a situation like this. With each lengthy arching attack, August’s condition would be so horrible to behold, so awful and appalling, that it would become almost impossible for me to bear. In the morning I would become afraid to go into his room to perform his daily hygiene. My hand lingering on the doorknob, I would listen to his moaning and visualize his lips pursed, face wan, and body pretzelized. The mere thought of August’s hideously twisted shape would sweep me up like gale-force winds and carry me away. I wanted to escape, to move to some other state, to be some other person. But I was his father, so I would push through the dread and do my job. I would turn the knob and bound into the room and screech at him at the top of my lungs in the same Wicked Witch of the West voice that I’d always used to herald August’s day. Twisted as he would be, the little performance would still amuse him. And this, for a moment at least, would be the eye of the hurricane.
No one at Bensalem-Salomon had any idea what the problem was. Every doctor who weighed in on the matter did so from the perspective of his or her specialty. The gastroenterologist was the GERD Man. Whatever the symptoms or the question, his answer always seemed to be “GERD” (gastroesophageal reflux disease). The orthopedist suggested the extreme solution of “rodding”—inserting titanium rods into August’s crooked back to keep it straight. To his credit, this doctor, who I knew to be caring and conscientious, indicated through his body language that even he thought that this was a terrible idea. “I’m sorry,” he said, “but I just don’t have any other tools in my tool kit.”
Did the Full Pterodactyl emerge from the succession of six operations August had undergone over four months? Did it come from something undertaken in the last of them? The fifth surgery had remove
d the pump, but cerebrospinal fluid had continued oozing from the incision site at the base of the spine. So, Dr. Joyce had performed the sixth and last to stop the leak. Afterward, he had told me that he’d done something special to get the job done. There was a seal, a patch of some kind. But he must have patched the site of the leak too tightly because nothing resembling arching had occurred prior to that last surgery, and it started less than a month afterward. The facts pointed in that direction.
Ilene and I racked our brains trying to figure out what had brought on the arching. Did the sixth surgery inadvertently inflict neurological damage? Was any part of a bone in the spinal column removed, leaving a sharp edge? Or did the surgery cause scar tissue to form in or around the spinal cord? Or, in tightening the dura, did Dr. Joyce overdo it, inadvertently creating a tethered cord?
Perhaps a second Relaxanoid pump would help by subduing the arching. For the few months in the summer and fall of 2010 when August had the pump in his body, we had seen the device doing exactly what it was supposed to do, relieve spasticity. In and of itself, the device was effective. So I speculated that implanting another might address the arching. Dr. Sandemelir agreed that, indeed, another pump might address the problem and so was willing to have a second one put in.
Dr. Cohen again attempted to dissuade me. As I expected, he was adamant. One year following the last set of surgeries, he insisted, was not enough time for August’s body to fully recover. He needed more rest before another major surgery. This time I disregarded Cohen’s advice for a different reason than before: Ilene and I now were beside ourselves, sick with worry, almost frantic. By this time we had returned to the regimen of Botox injections and oral Relaxanoid, but they did nothing to relieve the arching, which had become an overwhelming problem driving us to distraction. Dr. Sandemelir advised us that another Relaxanoid pump might help. She didn’t promise that the pump would stop the arching, but she didn’t dissuade us from thinking that it might. We asked for a different doctor in the pediatric neurosurgery program to implant the second one, and, in the summer of 2011, a neurosurgeon much younger than Dr. Joyce, Dr. Tom Gordon, did so. The Yellow Man was on hand once again, monitoring the situation, present in the operating room, but I didn’t speak with him.
No infection occurred this time, and, somewhat surprisingly, by January 2012 our son was back for brief periods in his Rifton, standing at the top of the driveway in the shade of a small oak tree. Now August had the pump inside him without us having to worry about infection, so we could see what it could do. But as had started happening the year before with the first pump, August just stood in his Rifton, not attempting to move. Was the amount of Relaxanoid the pump was dispensing into his system the correct dose? Was it too high? Too low? If August had been verbal, he might have been able to help by telling us what he was feeling. Dr. Sandemelir had assured me that she intended to rely on clinical observation to determine the right dose. But August was a black box, a Neil Armstrong without radio communication.
By this point the golden visions that Dr. Sandemelir had conjured up when first pitching the pump to us had vanished. She had suggested not only that August would walk more gracefully but also that he might be able to feed himself. But with the second pump, attempts to help him do the latter made no progress. After a number of months of working with him, the occupational therapists at Mt. Herman told me that the pump had done absolutely nothing to improve his prospects. No possibility in the foreseeable future existed that he would be able to feed himself.
Ilene and I had requested the second Relaxanoid pump to be implanted to address the arching, but we found that it did nothing to alleviate that. August’s cycles of arching actually accelerated. But the pump did one thing well: it relieved August of charley horses. With the second pump, not once did he push up from his wheelchair’s foot pedals and scream out as he had done much earlier. This indeed was a benefit, but it was one that could also have been achieved by using small doses of oral Relaxanoid together with Botox injections.
One time while an arching episode coincided with a clinic visit, Dr. Sandemelir looked over August as he lay pretzeled on her exam table. She said that she had no idea what was causing the arching and no insight concerning what to do. Never did she indicate that this phenomenon had a name, and she acted as if she had never seen it before. But what was most troubling was that she exhibited minimal curiosity about it.
“I can’t find any fault with the pump,” she said reassuringly during one of August’s visits to her clinic. “It’s functioning as it should.”
“You don’t think it might have something to do with the arching?” I said.
“No, not really. The pump is not implicated.”
Her job, she intimated, was simply to manage the Relaxanoid pump, nothing more. From her vantage point, the device was functioning perfectly well, and that was all that mattered. We should find some other doctor to deal with the arching, she advised, and she recommended that we see a neurologist. The neurologist we had once met with in Bensalem-Salomon was hopelessly disability-phobic, so we couldn’t go to him. Observing August, Dr. Sandemelir sighed and said, “I wish I was smarter.”
During subsequent visits to her clinic I brought the matter up again and again. I was doing this more frequently because the arching was worsening.
“If you are not happy with the pump,” she said once, “we can remove it.”
“I’m not unhappy with the pump,” I replied. “I’m unhappy with the arching. It’s making his life and ours unbearable.”
“As I’ve said, the pump is not implicated in the arching.” Her tone was pleasant but defensive.
But then, in the middle of 2012, Dr. Sandemelir and her physician assistant, Ms. Bosman, began to suspect that perhaps the pump catheter was leaking or damaged or kinking and that maybe one of these had something to do with the arching. Yet another major surgery was scheduled, a procedure known as a catheter revision. It would replace not the pump itself but only the catheter. This was the line that went from the pump on the side of August’s lower belly around his body to the base of spinal column and then upward to a point about midway up his back. So, on the morning of August 1, 2012—Ilene’s and my twentieth wedding anniversary—Dr. Gordon undertook the procedure.
In the evening after the catheter-revision surgery, Ilene and I had just enough time to meet for glasses of champagne and salads at Bistro Aix, a trendy restaurant. She had found someone to watch Clio for a couple of hours. Not even having had a chance to change clothes, I bought a small bouquet at the hospital gift shop.
We sat at the bar because we were rushed: I had to get back to August’s bedside. The celebration would last just twenty minutes, one minute for each of our twenty years. There was a couple seated to our left, probably in their early twenties. I saw that the guy was wearing a Dead Kennedys–Holiday in Cambodia T-shirt. This was another madeleine dipped in tea and caused me to reflect, He could have been me thirty years ago.
From where we were positioned we could see the restaurant kitchen, noisy but fun to watch. Our waiter—a tall, skinny, well-inked fellow with a serious set of ear gauges—took our orders: glasses of 2008 Schramsberg Extra Brut, a small calamari salad for me, and an heirloom beet salad for her. We told the waiter that I needed to hurry, to get back to the hospital. He nodded approvingly, probably assuming that I was a doctor who would tip him well.
We had honeymooned for a week in the Napa wine country, where we visited, among many places, Schramsberg. After our glasses of sparkling wine arrived, we sat for a moment contemplating them, watching the bubbles rise.
“Whoever would have thought we’d be celebrating our twentieth with a child in the hospital,” I said.
“I would never have imagined it,” Ilene said.
“So here’s to the best twenty years of my life,” I said as I raised my glass.
“And here’s to the best twenty years of my life,” she said, raising hers.
“And may we have at least twenty more
good years.”
“At least twenty.”
“L’chaim!” I said as we clinked our champagne glasses together.
“L’chaim!”
And we sipped our drinks.
“Do you remember Our Song?” said Ilene. “‘Losing My Religion’?”
“R.E.M. Of course. It’s been a long time. ‘Oh, life is bigger,’ I sang, the opening line.
“No, that’s not how the song begins,” she corrected. “It’s ‘O life! It’s bigger.’”
“I really don’t think so,” I countered. “It starts with an ‘Oh.’”
“No, there’s no ‘Oh.’ Just ‘O.’ There’s no h. Seriously! There’s just ‘O.’ A vocative O, the O when addressing someone, as in ‘O ye, of little faith.’ The song goes, ‘O life! It’s bigger—.’”
“No way,” I said. “It’s got to be an Oh. Oh is an interjection, and the singer is using it to express his pain, surprise, frustration, and disappointment. It’s ‘Oooohhhhhh, life is bigger.’ The song’s about unrequited love. The person he’s speaking to doesn’t love him back, but he’s saying to her—I assume it’s a her—it could be a him—that it doesn’t matter, that life is bigger than that person’s love. It’s really about sour grapes. It’s a the-fact-that-you-are-not-loving-me-back-is-not-important-to-me argument. And love itself is not that important. It’s like the circles in a Venn diagram. Life overlaps and covers over most of the smaller one, love.”
Ilene thought about this for a moment and then shot back, “But the relative sizes of the circles in your Venn diagram are inverted. Because it’s O, love’s the bigger circle. It overlaps and covers over most of the smaller one, life. If the line is, ‘O life! It’s bigger,’ then the point is, the love circle is bigger, not the other way around. Love is all, or almost all. That’s why he’s saying, ‘O life! It’s bigger.’ ‘It’ here equals love, and love is bigger than life.”
A Life Beyond Reason Page 15