About five in the afternoon on a weekday trauma shift we got a call from Native Air, a helicopter medical transport service, about a 17-year-old they were bringing us with multiple fractures sustained in an ATV crash. The accident happened way up in the northern part of the state near St. Johns, a ranching community that borders the Navajo reservation. His vital signs were stable, and they had medicated him for pain and would arrive in 10 minutes.
The trauma team assembled in the bay closest to the elevator from the helipad. About 9 minutes later the elevator doors opened and the helicopter crew wheeled our patient in on their flight gurney.
I took one look at the patient and cursed under my breath. He had a triangular face with a broad forehead and narrow jaw and chin. His chest was wide and deep, barrel-shaped is the term. His limbs were painfully thin with knobby joints and marked curvature of the long bones, those that weren’t already splinted. His eyes were striking—deep blue, and the sclera, the white of the eyes, were the color of a new robin’s egg. All the markers of Osteogenesis Imperfecta. What the hell was he doing on an ATV?
Osteogenesis Imperfecta, also known as ‘brittle bone disease,’ is a genetic disorder, a gene mutation that causes defective collagen synthesis. It may vary in severity, but the classic expression causes weak bones that can break under the patient’s own weight. A sneeze can break ribs. A simple stumble can result in a broken hip or ankle. Patients usually end up confined to wheelchairs by their mid-teens, and although they may have a normal lifespan, the repeated fractures lead to short limbs and the characteristic facial appearance. So what monumental stupidity would lead someone who could break a leg just standing on level ground to get on an ATV and ride it around? Then he told me the machine had been a gift from his parents for his birthday, just a couple of weeks earlier.
“A special risk assessment parents constantly face is the risk of harm to our children versus the need to allow them to learn independence and find their own freedom.”
I almost went ballistic. This wasn’t just stupidity, this was child abuse. What kind of parent would willfully place a child with this disease in that kind of danger?
It took a couple of hours to get his x-rays done, get all the fractures identified and splinted, talk to the orthopedic surgeon, and get his pain under at least marginal control. He had fractures of both femurs, five ribs on the right, the right radius and ulna, and his left ankle. All were fragmented, would need operative repair, and were at high risk for nonunion.
Just as we were getting him ready to go to the OR for the first of many expected procedures, the charge nurse told me his mother was in the waiting area. St. Johns is a long way from our trauma center, and she had come by car rather than air. I stalked from the trauma bay, struggling to control my anger.
I found her in the quiet room just off of the main ER. She was in jeans, heavy work boots, and a flannel shirt. Her hair was pulled back, and her face was dirty with dark soil and sweat. I introduced myself rather coldly and outlined her son’s injuries, the plan for his immediate surgery, and some of the future procedures he would need. She listened calmly but there were unshed tears in her eyes.
“He’ll be OK, won’t he?” she asked.
“He’ll live, but he may not walk and there’s a good chance some of those bones won’t heal,” I said. Then my self-control broke. “How could you let him ride that thing?” I demanded.
Then she did cry. “He was so happy,” she said. “Finally he could come down to the corrals and be with us during the day.”
She explained that they lived on a ranch 3 miles from their nearest neighbor. Her son had an all-terrain wheelchair, but the path to the corrals where they raised horses was too rough even for that. He had been confined to the house and front yard for several years, while the rest of the family worked the ranch. He’d been terribly lonely. They knew the risk when they got him the ATV, but he had begged to be able to come down to the corral and watch her work with the animals.
I was now ashamed of my outburst. I did my best to reassure her that her son was in the best hands and that we would do everything possible to help him. Then I showed her how to get to the preop area so she could see her child.
Surgeons are acutely aware of risk. We make judgments every day about the relative risk versus benefit of our procedures, of the patient’s ability to tolerate an operation, of the chance that complications will outweigh potential benefits. Those are calculations that we try to base on our best understanding of physiology, our own abilities and the patient’s health. But sometimes we have to accept that the risk of our actions will be high but that the patient has no other option. When the consequences of inaction are not acceptable, we (and our patients) must accept the risk and do our best
There are other risk assessments that people make every day. We take risks when we love someone, when we get married, when we have children. A special risk assessment parents constantly face is the risk of harm to our children versus the need to allow them to learn independence and find their own freedom. What had it cost this mother to make that judgment and come up wrong? I hope I never have to learn that for myself.
Read Dr. Davis’ related blog, Handling Risk.
Bruce Davis, MD, is a Mesa AZ based general and trauma surgeon. He finished medical school at the University of Illinois College of Medicine in Chicago way back in the 1970’s and did his surgical residency at Bethesda Naval Hospital. After 14 years on active duty that included overseas duty with the Seabees, time on large grey boats and a tour with the Marines during the First Gulf War, he went into private practice near Phoenix. He is part of that dying breed of dinosaurs, the solo general surgeon. He also is a writer of science fiction novels. His works include the YA novel Queen Mab Courtesy, published by CWG press (and recently reissued by AKW Books as the e-book Blanktown). Also published through AKW Books are his military science fiction novel That Which Is Human and the Profit Logbook series, including Glowgems For Profit and Thieves Profit.