Early in the week of March 30th, I met with the nurse practitioner that had been managing J’s ICU stay for the previous weeks. We were women with a plan. J was progressing really well, had been weaned nearly to home oxygen levels during the day, and after a lot of finagling around we finally found a BiPap mask that worked and was comfortable for him overnight; it was time to get him home.
The last step was fixing the suspected microaspiration problem. When kids have lungs like J did, any illness causes a huge amount of lung secretions. If they also suffer from reflux (which J did, sometimes badly), it can cause small amounts of milk or saliva to be aspirated from below. These small aspirations, if frequent enough, can cause further respiratory distress and eventually pneumonia. Stop the aspiration, and hopefully stop James’ cycle of illness and constant hospital stays.
J was already being fed by G tube, which reduces aspiration since he was not swallowing food. The proposal was to temporarily change from a G to a GJ tube, a straightforward and noninvasive procedure done with x-ray guidance to swap feeding appliances in the existing access point in J’s tummy. With the GJ tube, J would get his food into his jejunum (i.e. the beginning of his small intestine), further limiting his aspiration risk by not putting any liquid into his stomach at all. Then, once he stabilized and stayed out of the hospital for a few months, we could switch back to the G and hopefully at that point at least partially feed him by mouth.
ICU Dad and I went back and forth on whether to do this. We have tried so hard to limit the amount of interventions for James, but the logic was undeniable: J was just going to keep getting sick, unless we made a change.
The procedure was scheduled for Tuesday, March 31. ICU Dad stayed with J Monday overnight, and I arrived Tuesday morning to relieve him and accompany J to the procedure. We squabbled with anesthesiology over whether he really needed to be intubated and under general anesthetic for it (we lost), and then waited around for most of the day for the interventional radiology room to free up.
J was not happy that day. His food was turned off in anticipation of the procedure, and he was hungry (and a kid that is continuously fed is not used to the sensation of hunger, so really, he was pissed). Eventually, he was only calm held close to my chest in the rocking chair, and we sat that way for hours until they finally called for him in the late afternoon.
I took a picture of him before the procedure as I always have. Signed the consent form. Did the long walk alone back up to our ICU room to wait.
Before every single surgery that J has ever had, I’ve always had terrible sinking feelings. There is something deeply unsettling about handing your child over to a doctor and just walking away. The feeling that maybe you will never see them again. I have usually made them bring him back to me to give him one more kiss.
Not on March 31st. I can’t believe that, in hindsight. I was worrying about the long-term effects of yet another use of general anesthesia, as I always have, but the whole thing was just so … routine.
He was finished in 30 minutes. The radiologist called me in James’ ICU room: “Very straightforward, once he was under it took less than 10 minutes, and he did great. You can tell the nurses they can start feeding him again right away.”
J was brought upstairs, extubated to BiPap, food started, and then he slept contentedly. I went out for dinner, then came back and turned in early. It was not a bad night; he fussed here and there, but no more than normal. He was sleeping peacefully when I left the hospital for work early on Wednesday morning. The NP stopped by before I left to talk about resuming our home nursing services for Friday and other discharge planning stuff. Routine.
I texted P, our nanny, around 10 that morning from the office, our usual exchange: “How’s J?”
“Great, we’re playing and he’s laughing so much today.”
“Good, give him a kiss for me.”
Then at 11:30, she texted again: “Something is wrong.”
To be clear, this is a woman who kept her shit together when J’s oxygen tank failed on her while she was out with him picking Max up at preschool in the fall. The woman does not say “something is wrong” unless something is really f*cking wrong.
I called her. Told her to find our NP, find the resident or the fellow and tell them. They all knew P well, and they would listen to her.
I heard minimal updates for a couple of hours. Then around 2pm: “I have to leave now to get Max, but I can’t leave J like this.”
I picked up my bag and walked out of the office and onto the train. As soon as I got to the hospital and saw J, I went into a full on ICU Mom tear. James was not James. He was clearly in pain, with a panicked look on his face. His heart rate was over 200, and had been for 2 hours. His blood pressure was dropping. I was marching around the unit, talking to the doctors, telling them to look again at the test results, repeating over and over “Something is really wrong.”
I held him for a few minutes, hoping it would help. I know now that it must have been excruciatingly painful for him to be held by me at that moment, but he didn’t cry. In fact, he just nuzzled his face into my neck with relief. For the last time.
Then he started to crash and the doctors were pushing into the room. IV access, centerline access, intubation, dopamine, epinephrine, x-rays, consults. I texted ICU Dad and told him to come – he was walking out of his office in seconds. When you live at the extremes like we have, you know the difference between a routine bad day and a Bad. Day.
Then diagnosis: the GJ tube had perforated James’ small intestine, and the resulting leak of formula and intestinal fluid had sent him into septic shock.
Surgery arrived shortly thereafter. The head of pediatric surgery was on call; incidentally, and perhaps poetically, the same wonderful man who performed James’ bowel surgery on his second day of life. We had seen him and spoken with him many times since that first day. He looked rather stricken, but maintained an icy demeanor as he explained the procedure. At one point I had to ask him to stop and restart because I had become unable to process auditory inputs. I thought to myself a few times that I might be so sleep deprived that I was actually having a nightmare.
J survived the surgery. He was returned to ICU with his abdomen open and a vacuum-sealed dressing overtop; the doctors were expecting a continuation of the extreme inflammatory response, so to close him early would be fatal.
He was on the ventilator and heavily sedated. ICU Dad and I sat together for a while, and then around midnight I got on the train home to catch a few hours of sleep and to see Max off to school in the morning (I hadn’t seen him in 2 days at that point).
There was no sleep to be had for either ICU Dad or me, unfortunately. Anxiety does not lend itself to rest. Then, around 6am, I got a text from ICU Dad that I will never forget. “Rough night. J had a fever of 108.”
So, I did not know fevers went that high. You may recall, James never really got fevers, even when he had HLH (where a high fever is usually the first symptom). Several ICU nurses and doctors later said it was one of the highest they’d ever seen. I knew enough to know it meant nothing good.
Max sent off to preschool, P and I drove to the hospital. Well, I think we did. In honesty, I have no memory of that morning. I do remember a panicked feeling of: “GET THERE NOW.”
The next 18 hours were a blur. ICU Dad and I stood together in the eye of James’ storm. First, the attending Dr. W. told us J was “hour to hour.” Then at midday we were downgraded to “minute by minute.” He developed a clot around an arterial line in his arm and the chubby, perfect little fingers on his left hand turned purple. I surrounded them with heat packs and breathed warm breath on them to try to stimulate circulation … the same as I have always done for Max in the cold weather. But so, so not the same, because this time it was my pathetically desperate attempt to save J’s fingers from amputation.
By the evening, as Dr. W. came off service, he told us we were “hour to hour” again. We felt okay. The afternoon was not as scary as the morning, and everyone seemed calmer, so we were, too. We asked the nurse if she thought we could step out for food, and she said yes. Our mood at dinner was optimistic. In the prior 15 months we had seen J turn SO many corners that seemed impossible only hours before. He had this. We were sure of it.
As we walked back onto the ICU floor, my phone started ringing. It was the ICU staff. J had taken a turn. His blood gas was not looking good. His platelets were extremely low. In the 90 minutes we had been at dinner, his feet and his other hand had started to turn purple.
We heard some familiar and some very unfamiliar words over the next few hours as we took turns sitting beside him. The mood was changing. And then, the attending arrived at the same time as the oscillator.
In 15 months, I have not once lost it in the hospital. Literally, not once. I have calmly instructed doctors to please ignore the tears running uncontrollably down my face as I asked rational and painful questions in a number of surreal conversations:
“The treatment begins with 8 weeks of chemotherapy.”
“There is severe brain damage in his right occipital lobe from an unknown cause.”
“The good news is, he doesn’t need a bone marrow transplant. He has a defect on his MLL2 gene, and the diagnosis is something known as Kabuki Syndrome.”
But when I saw that oscillator, I knew. I knew exactly where this would end. I felt like I was outside my own body, watching myself shriek and pull my own hair like a complete lunatic. Pushing away anyone who tried to touch me. I collapsed into a ball on the floor beside J’s crib and howled.
ICU Dad did what a good ICU parent should do in that situation: he completely ignored me and the nurses who were telling him to come and get his wife. He walked out of the room with the attending and the fellow and got a handle on the situation. And then once he was done, he walked back into J’s room, picked me up and looked me right in the eye and said “I can’t do this by myself. Get it together.”
For once, I couldn’t speak. Once I got my breathing under control, I walked silently out of the unit, out of the building and to the sidewalk beside the hospital. It was very windy, and the cold air on my face brought me back. “Stop it.” I repeated that over and over for twenty minutes until I knew I was under control.
When I got back to J’s room, as soon as ICU Dad saw that I had my wits about me again, he collapsed on the couch. It wasn’t sleep. It was exactly what I had just done, in a different form.
The attending was waiting for me. “I apologize,” he said. “I had forgotten that he was on the oscillator at the outside hospital before you got here last winter.”
He laid it out for me, bluntly, as would only be appropriate if the parent on the receiving end was a truly hardened ICU parent: ARDS. Extreme inflammation. Disseminated intravascular coagulation. Purpura fulminans. Organ damage. Extremity damage. Potential brain damage. We would know how things would play out in the next few hours.
I sat holding J’s hand for hours, until I couldn’t balance on the chair any longer. Then I woke up ICU Dad and he took my post. We stayed that way until the morning, both knowing but not wanting to say out loud what was coming.
Around 8 am, Dr. W., that wonderful, soft-spoken ICU attending who had cared for J so many times over the past 15 months, came into the room and closed the sliding doors.
“You have always asked me to be honest with you about James…”
James died at 2:15 in the afternoon on April 3, 2015. He was peaceful, dressed in a favorite pair of pajamas, not attached to any machines, and cuddled close between his Mommy and his Daddy.