Small: Life and Death on the Front Lines of Pediatric Surgery

5 | Going to Extremes

going to extremes Baby X.’s honeymoon is about to end, sooner than expected. We crowd the blood gas analyzer, watching as it spits out white snippets of paper stippled with fateful numbers . His oxygen level is drifting lower, from mere sag to full-on collapse, while the carbon dioxide climbs like the Dow before the crash. There are some reassuring signs. He still has a heartbeat. His blood pressure is holding. Heavily sedated, wearing eye patches to block out ambient light, Baby X.’s chest accordions back and forth, the rhythmic blast of the ventilator flexing his sparrow ribs. But what becomes increasingly obvious with each waning report is that the ventilator, now fully maxed out, is not going to be enough. Baby X.’s color has faded from pink to gray. Life is seeping out of him, and soon there will be no more. How much is left? Will our next move rally him to survival? We don’t know, but there is only one thing left to try—the most invasive, high-risk therapy that modern technology can muster. What we do next will either save him or prod him to a certain death. Baby X. is going on ecmo. Shortly after birth, when Baby X. first had difficulty breathing, his chest X‑ray showed what looked like a mass of air bubbles in his left chest. His intestines had slipped up inside his chest, creating ( 64 ) s m a l l multiple air pockets we could see on X-ray. This simple but classic finding tipped his doctors off to the diagnosis of a diaphragmatic hernia—a hole in the diaphragm. The diaphragm is the dome-shaped muscular shield that separates the abdomen from the chest and aids in respiration, allowing a person to take a deep breath and expand the rib cage. Normally the diaphragm is formed by the fusion of several membranes by the seventh week of gestation. If the diaphragm fails to completely form, leaving a hole between the chest and abdomen, the abdominal contents—the small and large intestine, spleen, stomach, and liver—can slip up into the chest cavity, where they will stay for the remainder of gestation. Consequently, the developing lung bud on the affected side will be compressed. Instead of the lung fully branching out, filling the chest with a spray of spongy pink air sacs, only an underdeveloped bonsai stub of a lung will be present at birth. When Baby X.’s pediatric surgeon at a nearby hospital operated on him shortly after birth, he found a hole so big that the edges of the diaphragm could not simply be sewn together. He trimmed a patch of Gore-Tex fabric to fit in the shape of the missing muscle and sewed it in place around the circumference of the hole, anchoring it to the ribs along the back edge of the baby’s chest because there was only a sparse rim of muscle. The operation was not particularly long or complicated, but no matter how well the pediatric surgeon had done his job, Baby X. still had only a 40 percent chance of surviving because of the underdeveloped lung. The stable interval between an operation to repair a newborn’s diaphragmatic hernia and the almost certain nosedive that follows is referred to as “the honeymoon.” No one knows how long the reprieve will last. Even with the intestines placed back in the abdomen, Baby X. was still susceptible to respiratory failure from pulmonary hypoplasia , or not having enough lung tissue at birth. Adults could live with only one lung, but a baby might not survive with his one going to extremes ( 65 ) normal-size lung plus an underdeveloped piece on the opposite side for two reasons. Not only was the amount of lung tissue deficient for gas exchange (oxygen for carbon dioxide), but also the amount of blood intended to flow through two full lungs would flood the smaller, underdeveloped lung, causing the blood vessels to the lungs to reflexively constrict in both lungs. This phenomenon, known as pulmonary hypertension, could be unpredictable and overwhelming, more difficult to manage than a ten-foot storm surge after a hurricane, and just as deadly. Bright light, loud noise, too much fluid, too little fluid, touching, jostling, pain—any number of factors could cause the pulmonary arteries to squeeze, choking off the flow of blood trying to get back...