No Boundaries

Two roads diverged in a wood, and I,
I took the one less traveled by,
And that has made all the difference.
–Robert Frost

It’s been 18 days since Ben’s surgery at Boston Children’s Hospital, which involved attaching titanium rods to his T1 and T12 vertebrae to create tension to straighten his thoracic spine. By all accounts, and despite some unexpected issues with Ben’s bones, the surgery was spectacularly successful. Ben’s scoliosis has been dramatically reduced from 75 to 25 degrees, and this improvement should become greater with every successive spine surgery he has, every 6-9 months, as the rods are extended to allow for spine growth and the tension to straighten the vertebrae increases.

Ben's sisters visiting in ICU, April 5, 2013

To say the words “spectacularly successful” following any of Ben’s surgeries is a wish I never allow myself. My coping strategy is to prepare for the worst, and then be pleasantly surprised if that doesn’t occur. But the best? I never prepare for that.

What’s been even more spectacular is Ben’s rapid recovery. Each surgery is a road newly traveled for us; it’s impossible to know what to expect, because no surgeon, anywhere, is able to tell us “well, on the last 20 children with Schwartz-Jampel syndrome that I performed this surgery on, this is what it was like”. Because there aren’t enough people with SJS to know this. Because not one physician that Ben sees has another patient with his same condition. I can’t say that every child with SJS would recover from spinal growing rod surgery as well as Ben, but at least it’s one data point.

So each significant surgery is novel, because it’s done on Ben. It’s risky, it feels like an experiment, and the outcomes are largely unknown because Ben is the guinea pig. Only 8 days before the surgery, because I took Ben to see his hip surgeon, the spine surgeon and hip surgeon started discussing the growing rod impacts on Ben’s hips. The hip surgeon and the physical therapist thought that the rods should not be extended into the lumbar spine, because then Ben would no longer be able to sit. Only 2 days before the surgery, our extended team made a definitive plan that only the thoracic spine would be operated on, a lumbar brace would likely be needed to address this part of the scoliosis that isn’t being treated, that when Ben is between 16-18 years old he should undergo a total hip replacement, which would give him enough flexibility in his hips to sit once the lumbar spine was fused with the thoracic spine, following the hip replacement.

Ben's growing rods

It was a 6 month conversation condensed into a few days. It felt very uncomfortable, but at least it happened.

Our road traveled has led to us becoming more insistent that doctors talk to one another, as was amplified during Ben’s hospital stay. When blood-tinged mucous started coming out of his trach during post-op suctioning, something that had only happened twice before, I insisted that Ben’s otolaryngologist (ORL/ENT) doctor be called. When Ben’s breathing became rapid and fierce, I called his pulmonologist directly, to bypass the critical care team who wanted to handle it all by themselves. When the pulmonologist and ORL docs, who have been caring for Ben for nearly 11 years, visited him in the ICU, the attending said, “I didn’t know Ben had so many friends”. Yes, they feel like friends, but more importantly, they know his baseline, what our family is capable of, and they can judge his decline and his recovery needs in a more accurate way as a result of this knowledge. Why not bring them into the picture?

I teach a session on care coordination in my PM755 class to BU School of Public Health students, most of whom are working towards their MPH. It’s a class where I introduce Ben’s needs and our family. I first tell the students about the goals of care coordination, why it is needed and the innovations that exist as part of the Affordable Care Act. But then I need to introduce them to reality, and examples like the one above illustrate my point. My goal is not to make every parent, sister, son, daughter, brother a patient advocate (although that would help); my goal is to illustrate all the reasons why care coordination is such a difficult concept to achieve, even in our nation’s best hospitals.

Having a little hospital fun

Ben’s future now involves traveling down a 5-7 year road of repeat spine surgeries for extending the rods (Ben hopes for every 9 instead of 6 months), and once he is no longer growing, a total hip replacement, followed by a definitive fusion of the thoracic and lumbar spine. But we can’t take our eyes off Ben’s knees, elbows, wrists and eyes, and as always, his respiratory status is vulnerable and needs our attention too.

High school will have some ups and downs, for certain. But right now, we’re looking forward to getting Ben back to middle school, where he can finish up a fantastic 6th grade year, return to the band, and hang out with his friends. We are so thankful that his recovery road, this time, is so much shorter than any of us, including his surgeons, imagined.