Stand Tall on the Quarterdeck
The following is an excerpt from Chapter 3 of Tiny Medicine, available on Kindle and in paperback in your local bookstore:
Life brings all of us at least a handful of spectacularly ephemeral encounters with great triumph. They can be individual or team-based, personal or professional, very private or very public. By triumph I don’t just mean “good” or happy experiences – I mean real victory in the face of long odds, a hard-fought battle that ends with right prevailing over wrong.
It took one of these triumphs for me to finally turn the corner in my first year of NICU fellowship, involving treating an infant with a condition called persistent pulmonary hypertension (PPHN). Every victory against PPHN – a disease that has given me (and most neonatologists) more nightmares, heartburn, and gray hair than all others combined – is a small triumph, but to fully appreciate why, I need to tell you more about the condition itself.
When a baby is born, a miraculous combination of physiologic events happens that forces developmental fetal fluid out of the lungs and fills them instead with oxygen. The influx of oxygen opens wide the baby’s pulmonary blood vessels, allowing the life-long exchange of oxygen and carbon dioxide to begin. With the rapid influx of oxygen, the baby’s blood reddens and his skin quickly fills with the pinkish flush of life.
Recent evidence suggests that this nearly magical sequence of events happens normally in about 998 out of 1,000 births. The other 0.2% of the time something else happens, leaving the pulmonary blood vessels tightly constricted. Constricted blood vessels don’t carry much blood, owing to the principle first described by 19th century French physiologist Jean Léonard Marie Poiseuille. He discovered a 4th-power exponential relationship between the radius of a blood vessel and the vessel’s resistance to blood flow.
In plain English, this means a blood vessel that is 1/4th its usual size will only have 1/64th its usual flow. A 64-fold change is equivalent to a 98.4% drop, or roughly the same as taking the entire Mississippi River at its widest point near Bena, Minnesota, and squeezing its corpulent 11-mile girth down into a channel the size of a high school running track.
This is a really important concept in neonatal pulmonary mechanics, because it means a small change in the size of a constricted pulmonary blood vessel has a very large impact on how much blood can actually flow through it. No blood flow means no exchanging oxygen for carbon dioxide, and no oxygen means a very blue baby. This is more or less what happens with PPHN.
Babies with PPHN often need to be intubated in the delivery room and wind up quickly needing 100% oxygen mixed with a special medication called nitric oxide that seems to help selectively dilate pulmonary blood vessels. Their first chest x-rays look like a car’s windshield in the middle of a blizzard: total white-out. They have oxygenation problems, ventilation problems, agitation problems, and blood pressure problems, any one of which can send the baby’s entire physiologic balance into a downward spiral.
Treating PPHN must feel similar to the way carnival plate spinners feel once they get all 12 plates going at once – eyes and hands darting from plate to plate, knowing that if you lose the rhythm even for a second to save one plate, the rest will come crashing to the ground.
Except for a baby with PPHN, losing that balance doesn’t lead to broken chinaware – it leads to death.
Some of my greatest private triumphs in medicine have come at the bedside of babies with PPHN, including the one that followed soon after Dr. Goldberg’s pep talk. On that particular call night, I wound up staying at a baby’s bedside for what must have been 14 hours straight. His name was Alex* (name and other details changed to preserve anonymity) and while I’d cared for several babies with PPHN over the first nine months of fellowship, he was without a doubt the sickest.
Alex was born exceptionally ill – the delivery room team spent 45 minutes resuscitating him before his heart rate was high enough to stop CPR, and when he was admitted to the NICU, he was immediately placed on the therapeutic hypothermia protocol. Therapeutic hypothermia is a treatment used for newborns whose bodies and brains are temporarily deprived of oxygen, most often before or during the delivery process. Pioneering clinical trials in the early 2000s demonstrated that intentionally cooling babies whose symptoms are consistent with temporary oxygen deprivation down to 33 degrees Celsius for 72 hours improves their chances of avoiding severe neurologic and developmental issues.
I’d treated numerous infants with therapeutic hypothermia, but Alex’s case was different because he also had profound PPHN. Managing PPHN is hard enough at a normal body temperature – it’s nearly impossible when an already irritable baby is now both shivering and splenetic.
I spent hour after hour with Alex, leveraging every aspect of my clinical brain to stay one step ahead of his disease. After five cups of coffee and hundreds of small adjustments, we finally “captured” him around midnight, reaching a state of relative stability in the midst of his critical illness on significantly high ventilator settings, ungodly doses of multiple blood pressure medications, and a cocktail of sedative and paralytic medications that kept him both relaxed and quiet. We even put a pair of noise-canceling headphones over his ears, knowing even the slightest disturbance to his restful state could restart the downward spiral we had spent so many hours to arrest.
Alex was the first newborn whose bedside I quite literally kept vigil over all day, all night, and through dawn until the morning team arrived at 7 a.m. to take over the next watch. I remember sitting in a swivel chair, elbows resting on the nurse's table, chin resting on my knuckles. I watched the digital shimmers of his monitor screen like a hawk – the pulsatile wave of the blue pulse oximetry line, the regular and repetitive morphology of the green electrocardiogram line, the red continuous arterial blood pressure line's nearly sinusoidal progression, and the sawtooth-like bounce of the yellow line intended to monitor respiratory rate but totally useless with a high-frequency ventilator vibrating the boy’s chest eight times each second. Every few minutes we’d tweak some parameter ever so slightly, inching one of the blood pressure medications up just a hair or one of 15 different ventilator settings down a nudge, always trying to stay at least one step ahead of his disease and keep all the plates spinning.
In the grand scheme of things, keeping Alex alive until morning may have been a small triumph, but for him, his family, and for me, it was much more. Alex would ultimately survive his PPHN, spend several months in the NICU convalescing, and be discharged home later that year. For me, I finally felt like I could chalk up a real triumph, a baby I believed was meaningfully given a better chance at surviving because he had me on call as his NICU fellow that night. Not that any other doctor couldn’t have gotten him through the battle, but I knew I had given him the fullest measure of my devotion and had come out on top.
Success breeds success, and my victory that night against the dueling evils of PPHN and therapeutic hypothermia bolstered my confidence and righted the trajectory of my path as a neonatology fellow...
Read more in Tiny Medicine, available on Kindle and in paperback in your local bookstore.
Dr. Chris DeRienzo is a physician from Asheville, NC and author of the book Tiny Medicine – One Doctor’s Biggest Lessons from His Smallest Patients, available for purchase on Amazon and at your local bookstore. Follow him on Twitter at @ChrisDeRienzoMD, on Instagram at @TinyMedicineMD and here on LinkedIn.
Clinical Pharmacologist & Toxicologist at David M. Benjamin, Ph.D.
5yIt's about having depth of soul and resilience!
Dear Chris, This is truly inspirational. Taking care of tiniest defines. You set a great example to the medical profession. We are proud of you. Anant