Our NICU Graduation story — Persistent Pulmonary Hypertension In Newborns

by JazChazDad ~ April 22, 2008

Our son Charlie was born with amniotic fluid in his lungs. It’s a fairly routine thing. It’s why the doctor spanks a newborn baby to get him to cry; the baby needs that one good breath which clears out the lungs and fills them with air.

Unfortunately, Charlie never got that one good breath.

The fluid in his lungs led to him getting severe pneumonia. His condition deteriorated so rapidly it seemed to be in free-fall: five days after he was born both of his lungs had collapsed and he had so many tubes and wires plugged into him that he looked like the back of your VCR. The doctors gave him antibiotics for the pneumonia, performed emergency surgery to implant a tube in his chest to help re-inflate his lungs.

But Charlie was still dying.

And they didn’t know why.

This went on hour after hour, day after day, test after test — each one requiring him to be stuck with a needle. The scale of this needle in relation to Charlie’s tiny arm seemed roughly approximate to driving a tent pole into mine.

“Do they have to do that?” I asked Charlie’s doctor, Dr. Karen Hendricks-Munoz, as we watched a nurse struggling to find a vein to tap. Eventually the ones in Charlie’s arms wore out and they had to start piercing his scalp.

“Sorry. We need to measure his oxygen levels,” Dr. Hendricks-Munoz said. “Dr. H-M” as everyone calls her, is the Chief of Neonatology for both NYU Medical Center and Bellvue Hospital Center. She is a tall, attractive woman who smiles easily even when her eyes betray that she’s had less sleep than you can possibly imagine. She’s the nicest person you never want to meet because, if you get to know her, chances are it’s because your baby is very, very sick.

“I thought the vital signs monitor told you about the oxygen levels,” I said.

“That gives us the blood oxygen saturation level but we need the blood tests to know the P-sat level — how much of the available oxygen is really being metabolized by his tissues. See, blood is essentially the body’s delivery system — ”

“Like Fed Ex?”

“Sure. The sat level on the monitor tells us how much oxygen is available for delivery in the blood stream and the p-sat level here on this lab report tells how much oxygen actually gets where it’s supposed to go in the tissues of the body.”

“So, the sat level on the monitor is how full the Fed Ex trucks are and the p-sat level on that slip from the lab is how many signatures they got for on time delivery?”

“Congratulations, you just passed Hematology 101,” Dr. H-M said.

I smiled as I pictured fleets of bright, shiny Fed Ex trucks racing to bring Charlie oxygen. When it absolutely, positively has to get there overnight.

“May I?” I said, gesturing to the lab slip, about the size of an ATM receipt.

Dr. H-M smiled, handed it to me. We were colleagues now.

“Ninety-nine percent — looks like our patient’s getting an A, doctor.” I said, handing back the slip.

“Actually, the p-sat level is a four-hundred point scale. For a patient on a respirator receiving pure oxygen, a p-sat number of ninety-nine is actually extremely sub-optimal, I’m sorry to say. ” Dr. H-M placed a comforting hand on my shoulder. As she went on about her rounds, I just stood there, shaking my head.

The glass wasn’t half-full. It wasn’t even half empty. It was extremely sub-optimal.

My fleet of happy Fed Ex trucks crashed, replaced by a tableau of utter hopelessness — in my mind’s eye I saw the hundreds of idling ambulances I remembered seeing on the west side of Manhattan in the early morning hours of September 12th, 2001. These were all volunteers, ambulance crews who had come from as far away as Pennsylvania to be there, waiting for the call that never came. To save people there has to be survivors.

What if Charlie didn’t make it?

Charlie’s tests continued to be inconclusive. We like to think of medicine as being as uniformly efficacious as a white lab coat. But medicine, a life science, is sometimes a lot more like life than it is like science. There’s guesswork, gut-calls, prayer. Real men and women wear that white coat and the difference between doctors who are merely good and those who are truly gifted is like the difference between knowing which notes to play and being a concert pianist.

Dr. H-M’s hunch was that Charlie was suffering from a condition called Persistent Pulmonary Hypertension in Newborns (PPHN), a fairly rare condition for which, even just a few years ago, there was no name, let alone cure.

PPHN develops when babies have fluid in their lungs at birth. When the baby is in the womb, the major arteries that branch off from the heart are significantly constricted. This performs a very important function en utero. It allows the mother’s cardiovascular system to regulate blood circulation in the baby, helping to ensure that his developing heart is not overwhelmed. The reason that they want the baby to cry as just soon as he comes out of the womb is because the shock of that one good breath is what tells the baby’s body that he is alive in this world. It’s time for him to breathe his own air, pump his own blood. Essentially, Charlie was dying because his cardiovascular system still thought that he was inside the womb; that he hadn’t been born.

Charlie was dying because he was lost.

With PPHN, the baby’s heart pumps, his blood flows, but because the major arteries leading away from the heart are constricted, oxygenated blood gets shunted back into the heart where it mixes with un-oxygenated blood.  Key organs don’t get the oxygen they need, toxins build up because they’re not being circulated out of the tissues. When the blood’s delicate PH balance has been lost, life-giving medications no longer work. The fragile newborn’s organs fail one by one until his entire system crashes and the baby dies.

During a critical period, Raelle found Dr. H-M seated by Charlie’s crib, watching over him. I had been the primary conduit for all the doctors.  I knew exactly how serious it was.  While I hadn’t kept my wife, Raelle, in the dark, I had tried to stay as positive as possible — she had just given birth and I wanted her to be able to rest.

Raelle asked Dr. H-M if Charlie’s condition was life threatening.

Dr. H-M, not unkindly, said, “I wouldn’t be sitting here if it wasn’t.” She was the head of Neonatology. As many as fifty critically ill babies were under her care at any one time and, out all of those bedsides, the place she felt her personal attention was most needed was here, with our Charlie. The most common complaint I hear about going to the hospital is how long it takes to see a doctor. I can tell you from personal experience — you do not want to the one they feel requires their complete and undivided attention.

The longer Dr. H-M stayed at Charlie’s side, the more nervous I became.

“What are you doing?” Raelle finally asked.

“I’m trying to figure out what kind of guy Charlie is,” Dr. H-M said.

If Charlie was a fighter, it made more sense to keep him at the NYU Neonatal Intensive Care Unit and try to keep him stable long enough for Charlie to find himself, realize he’d been born, duke it out with death on his own.

But if there was any doubt about his spirit, the prudent thing was to transfer him to a facility that had a heart-lung machine — total life support, the last stop before death’s door. Dr. H-M had been preparing to transfer Charlie but this truly a last resort because, in his fragile condition, the added stress of transporting him and performing this invasive procedure to hook his heart and lungs up to a machine risked doing as much harm as good.

“My gut tells me Charlie’s a fighter,” Dr. Hendricks-Munoz said finally. “I’m going to cancel the transfer order.”

She was right about Charlie.

His act as a person in this world was to grab the breathing tube that been shoved down his throat and rip it out. He did the same with another tube that had been surgically implanted in his chest to help re-inflate his lungs. He ripped that tube out three times before Dr. H-M and her team finally got the message.

Charlie was in the house.

He had no more use for their tubes and wires. He had places to be, a life to live.