“Yes,” Dr. Sticks assured her. “His status will remain the same. He won’t be treated any differently just because you have signed a DNR.”
Ilene hesitated for a long time, mulling it over. Finally she said, “Okay then. If nothing is going to change. We’re only signing this document in the event of the worst of a worst-case scenario. I don’t want the doctors or the nurses or the staff or anyone to lessen their care because we’ve signed this form. We believe that he will recover if he is given proper care.”
“Nothing will change,” said Dr. Sticks reassuringly.
So she signed it. And I did too. And we had copies made to take home with us. This DNR order became like the handle on an airliner’s emergency exit door. We didn’t expect to ever have to use it.
But August’s de facto status did change. It was a subtle difference. It was as if his hospital room had sprung a slow leak of oxygen. In the same way that everyone interpreted Dr. Eugenides’s intervention to mean that August’s end was nigh, so it was with the DNR. This was especially true of the nurses. The DNR apparently depressed them—they seemed more somber knowing that it was in his chart. The nurses emotionally stepped back, preparing themselves for the worst. And I don’t blame them—the deaths of children are hard on them. No one wants to see a child die.
As if pulling us back from the brink, a gastroenterologist, Dr. Nassar, hypothesized that August might be suffering from superior mesenteric artery syndrome (or SMA). This was what may have been causing the arching. Not all of the doctors concerned with August’s care accepted Nassar’s diagnosis, but, lacking any better one, they more or less went along and began treating him for it. SMA is rare and extremely serious. Up to one-third of SMA patients die as a result of it. We fully understood at this moment that August’s life was in danger. SMA treatment required the patient to gain weight, specifically, in August’s case, to build up fat deposits in his lower stomach area.
The hospital’s main pediatric surgeon, Dr. Sherwood, surgically implanted a G-J (gastrostomy-jejunostomy) tube so that August’s amount of nutrition could be increased. When he opened August up to implant the G-J tube, what he saw inside convinced him that Dr. Nassar’s SMA diagnosis was valid. Because such matters were in his wheelhouse, I believed him, and I think he made the best call he could have made. Sherwood trusted me with his personal cell number and took my calls when I needed to speak with him. His own child had spent months in this same hospital, and the way he acted toward Ilene and me indicated that he understood what we were going through. He represented Bensalem-Salomon at its best.
August became wedded to a machine: he required continuous enteral feeding. An artificial opening had been created at his abdomen. This opening had two ports, the G-port (for medications going to his stomach) and the J-port (for nutrition—PediaSure—going to his jejunum, the middle part of the small intestine). We had to stay focused: medication went into the G-tube, liquid nutrition into the J-tube. Mixing this up would bring serious consequences.
Most of the day and into the night, August had to be fed this way. Every few seconds, a pump would inject a spurt of PediaSure through a transparent plastic tube to the J-port and then into the jejunum. This was called J-tube feeding. A small blue-and-white pump called a Kangaroo was the injecting mechanism. Slightly smaller than a brick, it attached midway up on an IV pole. With this nearly around-the-clock feeding, the sound of the little blue pump turning on and off every few seconds, with a several-second delay between each burst, was like white noise broken up by a predictable ripple. Hummmmmmm-click-grrrr! Hummmmmmm-click-grrrr! Hummmmmmm-click-grrrrr! This sequence of sounds provided a constant and hypnotizing rhythm to our lives.
On an October night while August was recovering, I sat beside his hospital bed, eyes glued to a TV set projecting from an arm high on the wall, watching Barack Obama debate Mitt Romney. Other nights that month I watched the San Francisco Giants sweep the Detroit Tigers in four games in the World Series. I thought of my dad and what joy this victory would have brought him. As with the presidential debates, so it was with the World Series: the little Kangaroo pump supplied the background clicking and whirring, constant electronic music in monotones.
Since August didn’t need to remain in Bensalem-Salomon for the treatment to proceed, we brought him home, fed him night and day using the Kangaroo pump, put weight on him, kept in touch with his doctors, and hoped for the best.
Once August was back home, Ilene and I found ourselves again running a home hospital. It was as if we had become triage nurses. No one at Bensalem-Salomon had suggested to Ilene and me that we should apply for home nursing.
Translucent plastic bags of PediaSure hung like ripening fruit from an adjustable transfusion pole. At night, the bags glowed like faces. The pump—with its rhythmic clicks and whirrs—operated night and day, a toiling midget. And the suction device (to clear August’s airway) lay ready at hand.
To keep track of feeding and medicating, we had to chart each step every hour of the day. This was our guide seven days a week, and this went on for weeks and months and then a year. Ilene and I quickly became exhausted. We were working our regular full-time jobs, raising Clio, and taking care of a very sick child. We could not afford for either of us to stop working: because of August’s medical needs, the wolf was always at our door.
By early December 2012, August had gained a lot of weight, which was the purpose of the Kangaroo pump and the incessant J-tube feeding. We had been told that if he built up fat deposits just below the stomach, then, in all likelihood, the arching should cease. And if the arching went away, he could resume feeding by mouth rather than through a J-tube. We could go back to living the life we had been enjoying up to July 7, 2010, the day before the first Relaxanoid pump was implanted. Happy days would return.
But in the middle of that month, the Egyptian showed up again, followed by the shrieking bird from one million BC flying toward us from the west. When it arrived, it circled the house several times before landing on the roof.
The massive doses of pain medication that Dr. Eugenides was prescribing accomplished nothing. But more importantly, this latest episode of the Full Pterodactyl ruled out Dr. Nassar’s SMA diagnosis, the one that Dr. Sherwood thought he had verified. Whatever its cause, the arching wasn’t brought on by SMA.
No explanation for the arching remained. None of the medical professionals had a clue. For almost three years, we had shown August in his state of extension to an array of doctors at Bensalem-Salomon. Now Ilene and I were back to square one. And we were alone. We acutely felt the accuracy of item fifteen on Roy Ellis’s 30 Signs You May Have a Disabled Child: “It dawns on you one day you know more than your doctor.”
One particularly bad night during the December relapse, August was so extended that Ilene and I could hardly change his poopy diaper, even with the two of us working together. He was stiff and twisted, like a big piece of driftwood. Afterward, as I was lying in bed, a thought struck me. At 3 a.m. I got up and padded as quietly as I could through our house of creaking floors. I turned on the computer and sent an email to Dr. Sandemelir, telling her that we wanted her to turn off the Relaxanoid pump to see if that might help. It was counterintuitive, but perhaps diminishing the dose of Relaxanoid to nothing might relieve the arching. There was nothing else left. Stopping treatment—we should try that! Up until then, no one had suggested or even thought of turning the pump off.
The next morning, I drove August to Dr. Sandemelir’s clinic, and we started turning down the flow of Relaxanoid. This reduction had to be accomplished over a period of many weeks, otherwise August would go into withdrawal. Ilene and I also began weaning him off the magic potions that Dr. Eugenides had ordered. Eventually we managed to take August off all of the drugs. By late February 2013, the elixirs were leaving his system, and the pump was administering only about one-third its former dose of Relaxanoid. We observed clonus, or shaky legs (involuntary, rhythmic, muscular contractions), return, but the cramping—the charley hor
ses—didn’t. But we still couldn’t go back to feeding August by mouth, which is what we were aiming to do. We were stuck now feeding him through a J-tube, using the little blue Kangaroo pump day and night. He had to be tied to this machine almost all of the time.
As August became drug-free, his lively little personality reawakened. It was as though he had been living within a shroud, but now it had been torn open, and we could see his little cheery face inside. During this period, our goal was to return him to the relatively healthy state he’d enjoyed up to July 7, 2010. Progress or regress was measured by how far or near we were to getting him to eat food by mouth and by how near or far we were to the Nevermore Pterodactyl departing forever.
Turning down the speed of the pump and thereby lowering the dose of Relaxanoid seemed to help. Over time we became cautiously optimistic. And then we became more optimistic. And then a little more. No Egyptian, no Full Pterodactyl appeared. We’re out of the woods! was a thought that Ilene and I began to entertain.
About this time, I started noticing hair developing on August’s body. Fuzz was appearing on his upper lip. I remembered Ronnie, August’s friend, the young man I’d met eight years earlier at the 2005 Spirit of the ADA march. I also sadly recalled that Ronnie was no longer alive. August’s body was becoming like a man’s. His shoulders were broadening, his jaw was becoming more defined, and his Adam’s apple was more protruding.
Then it returned. It was a long hiatus, the longest of any up until that time. But in the spring of 2013, the Egyptian appeared, and then, a few days later, the flying reptile was back, the Full Pterodactyl. When she saw him arching for the first time in a long time, Katrina burst into tears.
The cycle of the shrieking creature again went into full swing. When the ancient bird was upon us, it screeched all day, and at night it slept on the roof. After a week or nine days, the sound of its leathery wings could be heard as it flew back toward the western horizon. We would be free again—until the next visit a few weeks later.
One afternoon Ilene broke down in tears while speaking on the phone with a caseworker. She was at the end of her rope. Fortunately, the woman on the other end of the line realized that we were a family in distress. Wheels turned, and this woman helped set us up with a home-nursing agency. Evidently, we now qualified for home nursing.
The young, fresh-faced representatives of the for-profit home-care agency who came to our house promised us the moon. When the promises weren’t fulfilled and we called, we were told that these representatives had been transferred to an office in a distant city. Nurses were in our house now from eight to twelve hours a day—that is, if they showed up for work. Unlike our regular college-age caregivers at the time, Amber, Katrina, and Jobeth, they were strangers to us. The younger ones were immature and uninterested. They were nurses who, for the most part, and for whatever reason, couldn’t get hired at a hospital or convalescent home. Almost all of them were trying to find more permanent positions or transitioning to move somewhere else. All of them had only two years of nursing training (AA degrees) at community colleges. The turnover was great. We would be working with one nurse (in the rotation) for a few weeks, and then, without warning, someone new would show up in her place (always a her). With each unexpected replacement, I would have to take an hour out of an already busy schedule to orient the new person to the job.
These home-care nurses were prone to make mistakes. Several confused what went into the G-tube (medications) with what went into the J-tube (PediaSure). I had to keep a close eye on each nurse. I will never forget the one who set the Kangaroo at a pace to pump PediaSure into August at ten times the proper rate. I still shudder when I think about it. Who knows what would have happened if I hadn’t caught her mistake in time.
In “The Learning Curve,” a 2002 article in the New Yorker, Atul Gawande writes about the need for medical organizations to sometimes test ethical limits when training tomorrow’s doctors or working out the kinks of new, potentially life-saving procedures. Similarly, when a new program is getting off the ground, it often has to lower its standards in order to enroll enough patients so that it can run. Ilene had seen this happen at Loma Prieta when she was working there. The medical center had inaugurated a heart-transplant program and had taken in patients who shouldn’t have been in it, people with histories of drug or alcohol abuse or other compromised conditions who died post-surgery. Dr. Joyce’s pump program had to start from scratch and operate in a related, somewhat “sketchy” way in its first years until it was fully on its feet.
On the morning of Monday, October 14, 2013, August was back in Bensalem-Salomon due to a serious incident the preceding Saturday. We were not assigned to Five West this time, but to the sixth floor of the Tower. This was the same part of the hospital where I had spent two weeks with August in December 2001 as the physicians struggled to diagnose his Stevens-Johnson syndrome. Once again, I was sitting beside August’s hospital bed. This time, though, I was trying to put it all together. Ever since he had begun arching in late November 2010, I had been wondering why Dr. Sandemelir had promoted the pump so vigorously over the safer and equally effective low-tech alternative. I suddenly had two intertwining insights. First, from Dr. Sandemelir’s perspective—that of a young academic physician—low-tech Botox injections were humdrum. The Relaxanoid pump, by contrast, was high-tech and prestigious. Dr. Joyce had built a considerable publishing record around it. Like any young academic, she needed to publish in order to earn promotion. Second, I saw that those behind the relatively new pump program had needed to get its numbers up. The program had to have patients to grow. Like any medical organization trying to establish itself, it was pressed to maintain cash flow and institutional support. That’s why it had taken August into the program despite the fact that his being nonverbal made him a bad candidate.
This epiphany allowed me to form a coherent picture. For Ilene and me, the pump had been a horror story, an episode worthy of the TV series Black Mirror, one for which there was no fast-forward button. We had to live through every second of it without any respite. Ever since the implantation of the first pump in 2010, August’s bedroom had become the scene of crisis after crisis. Excitement, panic, and adrenaline rush had occurred at the worst part of each turn of the cycle. And the wheel just wouldn’t stop turning. Our world would become anxiety-laden and frantic as we struggled to deactivate the bomb that had been planted inside of our son.
Disenchanted, I now wanted the second pump out. I became a man with a mission, convinced that it was going to kill August. That morning I spoke to the floor’s attending physician and anyone else who would listen. I asked, requested, commanded, ordered, implored, entreated, beseeched, and then begged that it be removed. I was a man running around with his hair on fire. I’m surprised now that no one called security. The attending physician tried to settle me down. He cautioned me that, given his state at the time, August couldn’t be operated on anyway.
In the middle of that Monday afternoon, Dr. Sandemelir came from her clinic, again blowing through the door like a bitter wind, eager to reconvert an apostate. Again she was alone—no sign of the Yellow Man, the Hippocrates company rep. It was obvious by this point that, despite his commitment to accompany us “on each step of the journey,” he had bailed.
Ever since November 2010, Dr. Sandemelir had dismissed the Full Pterodactyl as a problem that she didn’t cause, so it wasn’t hers to solve. On this particular Monday, once again she and I found ourselves facing one another over this same issue, she and I on opposite sides of August’s bed.
“He’s going to take this pump with him to the grave,” I predicted.
“Pardon me?” she said, eyebrows raised and staring at me.
“The pump has to come out.”
“Removing the pump will do nothing to resolve the arching,” she said calmly.
“No, I want it to come out. He’s going to take this pump with him to the grave!” I repeated.
She looked at me hard, baffled that I was bein
g uncooperative. For about twenty seconds we remained silent. “Removing the pump won’t change anything,” she finally said.
“He’s going to take this pump with him to the grave,” I said a third time.
“Why do you keep saying that? Removing the pump won’t solve the problem.”
“Why?”
“Because,” and then she leaned over the bed railing and waved her hand over August’s distorted form, announcing, “this is dystonia.”
I will never know why she waited until that moment to drop this bomb. Dystonia itself meant nothing to me; the issue was that—at this critical juncture—she suddenly and opportunistically had an answer to what August’s problem was. Whatever her reason for saying this word now, I was confounded, and the sensation came like feeling a first shudder of the San Andreas. Dystonia was a new word. Dystonia. It sounded like dystopia. The two could form a rhyme in a poem.
Why had dystonia—whatever it was—never been mentioned to me before? August had been suffering from the Full Pterodactyl off and on for nearly three years. In summer of 2012, he had spent sixty-four days in Bensalem-Salomon, twisted up into a jumble. More than two dozen physicians had trooped through his hospital room and viewed his arching. Not one of them though had pronounced the word dystonia.
“What is happening?” I said.
She looked at me blankly. “What?”
“Why did you wait until now to tell me this?”
“What do you mean?”
“Dystonia,” I said. “You waited.”
“Waited?”
“How long have you known? You waited until I wanted the pump to be removed to tell me that his condition was dystonia.”
“No.”
“Hell, yes. I see it now. If I hadn’t forced your hand, you would’ve never said anything.”
A Life Beyond Reason Page 17