A Cold, Clinical Interlude

What is a double aortic arch?

double aortic arch

Diagram of a double aortic arch, courtesy of Medline.com

 

In a person with normal cardiac anatomy, the aorta (which is our body’s main artery) comes up out of heart toward the person’s back, travels down the chest parallel to the esophagus, and branches off into smaller arteries below. In a person with a double aortic arch, the aorta is shaped less like a simple tube and more like a tube with a ring at the top. In fact, a double aortic arch is just one variety of a group of congenital heart conditions called “vascular rings.”

If you look at the image above (borrowed from Penn Medicine), you can see that the “ring” created by the double aortic arch fits like a rubber band around both the trachea (through which we breathe, connecting the mouth and nose to the lungs) and the esophagus (through which food travels from the mouth to the stomach). As a person with this kind of utterly impractical anatomy grows, that ring may not.

With a band around your trachea, breathing is difficult.

With a band around your esophagus, eating is difficult.

This was the diagnosis Sammi received at 13 months old, with an order for surgery as soon as possible.

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Blame Needs a Target

When Sammi was a newborn, the sleep deprivation was expected and, though unpleasant, not unreasonable. Ronni had not slept well, and I was ready for Sammi not to sleep well. When her 90-minute-maximum sleep schedule extended into three months, and I was back at work, I gave in and hired a postpartum doula to come twice a week and give me five consecutive hours of night time sleep. I felt no end of guilt around this; other mothers could survive on this kind of broken sleep without help, and even if they were miserable, most of them could not afford the kind of help for which I was paying someone. I needed that sleep to function, and so I fought past my blame of my own body for needing it, and I slept for five consecutive hours, twice a week, for about six weeks, until I had to quit my job. Every night after that was broken into tiny fragments of light, fractured naps between feedings.

Still, this all felt temporary. Time crawled by as winter turned to spring. Sammi was growing more slowly, but she was not sleeping any better. Against the raw and crackling synapses in my brain that convinced me it was wrong, her new pediatrician and every other adult in my world convinced us to sleep-train her when she was eight months old. We opted for a method that seemed less cruel than others and promised she would be sleeping through the night in a maximum of twelve days.

floorNothing can describe the kind of screaming she maintained for hours every night. Following every lesson in the sleep book, I waited as far from her in the house as I could between scheduled “check-ins,” but the sound followed me. I cowered on the hardwood floor, hands over my ears, rocking like a traumatized child. I felt the screams vibrate through my heart. I was exhausted by months of sleep deprivation, fear of illness, and the completely new world I was inhabiting, but there was something more.

A mother understands the quality of her baby’s scream.

She was not screaming in frustration. She was screaming in pain. I knew it. I said it. I said it on day one and day four and day twelve and on day twenty-seven, when no one could believe how long it was taking to sleep-train Sammi.

Five months later, when she was finally diagnosed with a cardiac condition that involved, in part, her aorta wrapped around her airway, we were told that anytime her blood pressure rose, she would feel her airway constricting. It would hurt, they told us. Don’t let her get worked up until she’s had surgery.

Before we knew for sure, before a CT scan confirmed the diagnosis of double aortic arch, I blamed myself for being a poor parent who could not teach my baby to sleep. It must be, I thought, that she knew I didn’t really believe in sleep training; or that I was eating something that upset her stomach as it passed into my breastmilk; or that I hadn’t checked her bedroom well enough for drafts or spiders or wild wolves which must be charging at her crib as I rocked on the floor, listening to her screaming from the floor below.

After we knew for sure, I blamed myself for not pushing her doctors to find out what was wrong. I knew all along. I knew something was wrong; I knew that was screaming-in-pain, and I had not stopped it. I had not fixed it. All she needed was to be calmed, have her blood pressure drop so that her aorta would stop strangling her. Instead of holding her, I left her alone in there, to scream and choke all by herself.

Once I knew, I didn’t stop holding her. Not ever again. I am holding her still.

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Not Knowing and Knowing Are Tied for Worst

The otolaryngologist (who would prove to always know best, every damn time) warned us about colds for Sammi, but we didn’t know what that meant. As a baby, Ronni had never had a cold. Not ever. We had no idea how a baby with a cold should sound or look. When we left that doctor’s office with a diagnosis, Sammi was 6 weeks old and I was on maternity leave. When I went back to work six weeks later, she still sounded like Darth Vader, but it was her normal.

I went back to work on a Monday, leaving her at day care for the first time. My plan was to work from home two days a week and have our girls home with a sitter one day a week, leaving them in day care just two days per week. I was home Tuesday. They went to daycare Wednesday. The sitter came on Thursday. I came home on Thursday evening and could hear Sammi’s breathing when the door opened, and she was three rooms away.

She hadn’t wanted to take the afternoon bottle I’d left with the sitter. She was breathing louder, and faster, Darth Vader on a treadmill. She smelled like spit-up, though she had not actually spit up anything. After several hours of worrying, our fear overcame my discomfort with asking for help.

I knocked on the door of our neighbor, a nurse practitioner. When she answered, I hung my head and asked, “Can you come over and tell us if we should take Sammi to the ER?”

She showed us how to recognize chest retractions. We went to the ER. It was my first course in Hospital Bag 101.

Now I knew a word I could use, a powerful sword to brandish at triage nurses. It gave me the power, every time I had to take her to the ER, to command a position at the front of the line, a quick path to the oxygen mask and the steroid shot. It got the job done: “I have a three month old baby whose chest is retracting. We need to see a doctor right away.”

nebulizer mask“I have a four month old baby whose chest is retracting.”

“I have a five month old baby whose chest is retracting.”

“I have a one year old baby whose chest is retracting.”

What I didn’t have was the knowledge that a bronchoscopy and a laryngoscopy were two different tests. The question we got in the ER, time after time, was “has she been scoped?” I said yes, but that wasn’t what they meant, and the difference would turn out to be monumentally important.

What I knew made everything scary.

What I didn’t know made everything scary, too.

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It’s Hard Until It’s Harder

When I had my first daughter, Ronni, everything was new and surprising, just as it is with any brand new parent. We took her to the pediatrician at 10 weeks old because she was pulling on her ears, which we thought meant she had an ear infection. He was avuncular and had seen it all. He humored us by looking at our round-cheeked, dimpled baby girl, peering into her ears, and then telling us casually that she was pulling on her ears because she had discovered that she had ears.

Humbled, and fighting my own demons that told me everyone knew better than I did, I took her home and treated her like a healthy, normal baby. Luckily, she was.

Three years later, Sammi’s birth was a shocking, week-late affair involving big terms like “intrauterine growth retardation” and an unplanned caesarean section. She came home, all four pounds and eleven ounces of her, and we expected her to be little but healthy. No one had seen anything wrong with her once her blood sugar stabilized. Because her sister had yet to experience any ailments beyond pink-eye, we had no sense of what a normal respiratory infection in a baby should sound like.

Even so, something continued to haunt me about her. She was a relatively easy newborn, but there were things that seemed off. I was exhausted, but that hadn’t stopped me after Ronni’s birth from falling instantly and deeply in love with her. Sammi seemed…off. Not right. I couldn’t put my finger on it. I loved her, but it felt instinctual rather than emotional. I was incredibly ashamed, and tried not to think about it.

One day I took her to the pediatrician because she was breathing very quickly. I’d consulted our dusty copy of Dr. Spock, and she was breathing about a third more quickly than she was supposed to. And she was asleep. The doctor declared it “rapid breathing of the newborn” and sent me home.

Ronni, age 3, started calling Sammi “baby snort-squiggle,” because she squirmed and spluttered and breathed like someone with a constant cold. At Sammi’s one-month checkup, her pediatrician offhandedly mentioned “tracheomalacia.” I made him write it down.

“Oh, you’re going to go home and look it up on the internet?,” he said to me with a wink. “Sure. Go ahead. It’s nothing. She’ll grow out of it.”

I looked it up. It worried me.

Against the advice of our pediatrician, we took Sammi to see a pediatric otolaryngologist (also known as an ear, nose, and throat doctor) at the local children’s hospital. I’d been following the journey of a parent on the Mothering.com forum, a woman whose daughter wasn’t diagnosed with severe reflux until the mother took the drastic step of videotaping her baby nursing. The video showed clearly that the baby would arch her back wildly by the end of a feeding, a known symptom of this type of reflux. That video moved that baby’s diagnosis forward far more than any pleas from her mother.

Taking a cue from that experience, David and I recorded the sound of Sammi’s breathing before, during, and after a feeding. After any feeding, she sounded like Darth Vader. We played it for the otolaryngologist, who thanked us and insisted on a laryngoscopy. At the time, I thought watching my six week old baby have a laryngoscopy was the worst thing I’d ever see. In the years that followed, I would find that I was wrong.

The otolaryngologist diagnosed Sammi with GERD (reflux), and also not tracheomalacia but laryngomalacia.

“She’ll outgrow it,” he said, “but she’ll have a much harder time with colds until she does.”

Those words, and that diagnosis, were as prophetic as they were incomplete. There was much more to come, but it was my first tiny moment of validation. I had never needed to guard Ronni fiercely from dismissal by a doctor — she was four before she even had her first dose of antibiotics — but I knew, in my prehistoric mother brain, that our winking pediatrician was not taking me seriously.

Instinct is powerful. I learned to respect it, one doctor at a time.

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Hospital Bag 101

shower headThere is an altogether different quality to the time spent in an evening with a baby whose breathing is compromised. Friends with asthmatic and croup-susceptible children tell me that this slowed-down, stretched chronology is the same for them, with details changed but the timeline largely unshakeable. I remember, as a little girl, waking in the night to find my mother, dressed in pajamas and a terrycloth robe, sitting on the closed toilet lid with my wheezing baby brother, the shower running so hot that a soft rain would fall from the dripping ceiling. He was a croupy toddler. My Sammi was less easily categorized.

Diagnosed with laryngomalacia at 6 weeks old, all we knew was that tissue at the base of her voicebox was floppy. Her gurgling was never cute. It meant fluid was collecting there, in the folds of her loose larynx, and germs would find a happy home in the never-clear, always warm and moist soft tissue just below her throat. During a respiratory infection — others call them “colds” — you could hear this eleven-pound baby breathing from three rooms away.

As a “cold” took hold of my baby, nursing became a fight between air and food. Eat or breathe? At three months, at four months, at five months, at thirteen months, those were her choices.

Dictates of the evening:

9pm: The sound of her breathing would be so loud that we would need to turn up the television if she was in the room.

9:15pm: Nursing her at bedtime, I would watch her chest, right between the ribs, for signs it was sinking in, pulling toward the back. Similarly, I could see the space below her chin, where the neck meets the chest, was doing the same thing. It was like she was breathing in reverse, someone pulling air out of her like a vacuum, making her concave.

9:50pm: Gently, so gently, so slowly and quietly and carefully, I would lay the sleeping, wheezing, whistling baby in her crib.

10:00pm: Hovering on the edge of the chair in front of the computer, I would google “wheezing in baby,” “baby pneumonia,” “what to do when your baby isn’t breathing right.”

10:20pm: Heart sinking, I would pick up the crying baby from her crib and repeat all the steps from 9:15pm, feeling my pulse quicken and starting to make mental lists.

11:00pm: Concerned for the rhythm of her breath, and remembering my mother those many years ago, I would run a hot shower. Hold her on my lap in the bathroom with steam curling around us both, I would wrap a curl of her soft hair around my finger and watch her eyes as they watched me, fluttered closed, and startled open, over and over.

11:30pm: When the indentations in her neck and chest became big enough for shadows, David would hold her while I packed a bag for the ER.

  1. Extra baby pajamas
  2. Bottles
  3. Breast pump
  4. Extra baby blankets
  5. Baby sling
  6. Sweatshirt, sweatpants, socks, t-shirt for me
  7. Bag of granola
  8. Apple
  9. Book
  10. Phone & charger

11:35pm: I would buckle her into her car seat and open all the car windows. Yes, even in December.

11:40-11:50pm: I drove with one hand on the wheel, one hand shaking the carseat, yelling “Sammi?! Sammi sunshine?! You ok?” until she’d cry, proving she was breathing. Repeat. Repeat. Repeat. It was a ten minute drive, or less when I was really scared, and I would pull over more than once to check her breath.

I didn’t offer myself the luxurious option of loving her during any early part of the night. Love is frivolous. Love is weak and emotional. That was why I never hated taking her to the hospital; in the hospital, I could love her. In the hospital, it was always someone else’s job to keep her alive — other people were more qualified than I, other people knew more about sick babies than I did. Once she was in my lap on a bed in the emergency room, nebulizer mask on her face blowing steroids into her lungs, eyes drooping as finding oxygen became less work, only then would I find myself compelled to kiss her on the top of her sweaty head.

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