Abstract
By Thanksgiving, we knew that Mariah could not survive. Two years earlier, in 2006, she had been diagnosed with dermatomyositis, an autoimmune disorder, which kept her from sports and made climbing stairs difficult, yet she mostly felt well. She enjoyed school and boy bands with a bevy of girlfriends. But in September 2008, she presented to a small hospital in far northern Vermont with apparent appendicitis. Instead, the surgeon there found diffuse intestinal inflammation and a perforated cecum. He closed the perforation, placed a temporary drain, and treated her with antibiotics. Within days, she bounced back to her bubbly, 13-year-old self and was discharged.
Instead of continuing to get better, however, over the next few weeks, nagging lower abdominal discomfort and slight fevers foreshadowed the coming disaster. Exploratory surgery at our university hospital revealed extensive intestinal vasculitis with multiple small perforations.
Tests confirmed that dermatomyositis was the culprit. The rheumatologists went to work, while the rest of the clinicians involved, myself included, waited for the vasculitis to settle down. Halloween came and went as Mariah lived on intravenous nutrition; her abdominal incision remained open, packed with surgical dressings that were changed under anesthesia three times a week. Our palliative care team helped manage Mariah’s pain, and we worked with Child Life specialists and physical therapists to prevent deconditioning and support her emotionally.
Although her life was clearly threatened, on any given day, Mariah wasn’t dying. She was mostly comfortable, didn’t look sick, and on weekends when her brother or friends visited, we saw flashes of her impish humor. Temporizing was better than whatever the alternative might be. I invested time and attention to banking relational capital, aware that she and her family would need doctors they could trust.
Then, in late November, a day came when Mariah was dying. Full-blown sepsis rendered her hypotensive, intubated, and sedated. Emergency surgery confirmed new intra-abdominal perforations and abscesses. Findings included “pan-intestinal vasculitis with nonviable small and large bowel.” Thankfully, within a few days, her blood pressure improved, and she tolerated nasal oxygen. Still, it was time to reconsider goals of care, perhaps at least avoiding CPR, so that if—or when—the time came, Mariah could die gently. Her parents faced the unthinkable task of confronting medicine’s limitations while clinging to hope.
Let me pause here to clarify that Mariah didn’t die, and that’s mostly because of her mother, Kerry.
I’d met Kerry during our initial palliative care consultation and liked her right away. Friendly, inquisitive, and detail-oriented, she partnered with her daughter’s doctors and nurses to make sure Mariah did whatever was needed to get better. Kerry had a presence that commanded authority.
With Mariah on life support in the pediatric intensive care unit (PICU), Kerry grew uncharacteristically quiet. She understood the gravity of the situation. She listened intently to what the doctors said to one another, or the nurses, and wasn’t shy about asking tough questions.
I dropped by the PICU frequently during those days. One afternoon, I told Kerry that I was worried that Mariah might not be able to survive and wondered if we should consider setting some limits on aggressive treatments. Kerry looked sad and responded softly, without anger, but also without equivocation, “That won’t happen. You watch, that won’t happen,” adding, “Mariah is not ready to give up and neither am I.”
The following morning, I joined PICU rounds. As the pediatric intensivist explained why sewing up all the intestinal perforations wouldn’t fix the problem, Kerry moved to within inches of his face, “I’ve heard what you can’t do,” she said. “Now tell me what you’re going to do to cure Mariah.”
In situations of this sort, I’ve learned to avoid repeating bad news. Mother Nature, over time, speaking through the accumulating burdens of illness, is usually better at conveying hard truths. In the meantime, with Kerry’s insistence, we persisted, creating care plans to keep Mariah alive.
Mariah gradually stabilized and, weeks later, was discharged home. Unable to eat, she was sustained on intravenous nutrition, with her open abdomen requiring twice-weekly dressing changes under anesthesia at the university hospital. To keep his family safe, her father, Steve, drove every one of those five-hour round trips over roads that in winter were sometimes nearly impassable. Whenever possible, I joined the dressing changes in the outpatient procedure room, checking in with Mariah and her family, and helped to adjust her home-based symptom management regimen.
Months became years. Finally, in the spring of 2012, Mariah’s liver began to fail. Once again, she was dying.
Dr. Daniel Croitoru, her meticulous, tenacious surgeon, presented Mariah’s case to several transplant centers, all of which declined to admit her. Hope seemed lost until Riley Children’s Hospital in Indianapolis agreed to evaluate Mariah for a multiorgan transplantation.
And so, in September of 2012, Mariah received a new stomach, liver, pancreas, and small and large intestines from a four-year-old donor and, a day later, an abdominal wall from a second donor. When the transplant surgeon explained details of the operation to Mariah, she was overjoyed to learn that she had a belly button again! On postop day 15, she ate ice cream for the first time in over four years.
It required many months of physical therapy for Mariah to stand and many more for her to walk. But she stood before a packed auditorium to receive her high school diploma. She was eager to get on with life. In 2023, Mariah graduated with honors from the University of New Hampshire.
My wife, Yvonne, and I attended a Saturday evening graduation party for Mariah that her family threw at the Newport, Vermont, community center. Dr. Croitoru was there, along with nearly 20 members of her clinical teams. We were all misty watching Mariah dance with James, her serious beau.
Before heading to the airport the next morning, Yvonne and I had breakfast with Mariah and James. We chatted about her future—she said she hopes to be a writer—and their relationship, which they think is going to last. I asked only one health-related question. “Mariah, I’m curious, do you actually get hungry?” Before she could respond, James snorted, “Hungry?! If I’m not careful, she gets ‘hangry’!” Mariah smirked while giving James side-eyes and then speared his home fries with her fork.
Later, during our drive to Logan Airport, I called Kerry to hear her reflections on the graduation party and Mariah’s startling well-being. She said that she’s thrilled with all that Mariah’s been able to accomplish and happy that she has James in her life. Though she misses not having her daughter around.
As our call was ending, I summoned the courage to ask for a personal favor. “Kerry,” I said. “If Mariah and James do decide to get married, please don’t make me crash their wedding.”
She laughed, “I promise.”
After several miles passed in silence, Yvonne asked for my thoughts. I said, I guess there are times when Nature herself will stand aside out of respect for another mother’s love.