My younger son was in the world about 24 hours before a doctor said something might be wrong. The baby had just finished his first bath and then, there it was, released into the hospital room like an animal from a cage: a worry made only wilder by how aggressively we chased it. I was panicked and disbelieving and angry all day. By night, I felt something like subdued. I remember staring down at him while he ate and thinking, Well, here we are again.
In a literal way, we were not there again, not at all. My first son was born almost four years ago in New York; my second, this past summer in California. One reason we moved here is the opportunity for our older son to live near the ocean, which is nice for anyone, but especially if you have the life-shortening disease cystic fibrosis. His treatment plan includes hypertonic saline solution, which is a sterilized version of what the surfers who jog down our block breathe in while out among the waves.
My older son is thin, but you’d never guess there’s something wrong just by looking at him. The problem is in his cells, which don’t handle sodium chloride correctly, thanks to the faulty genes his father and I both passed down to him. While my husband and I each have one good gene and one bad, our older son has two bad ones. He spends about two hours every day hooked up to machines that try to ward off what his body is trying to do to him, which is coat his lungs in mucus so thick he drowns. One of the medications he’s on, the one we’re told is his best chance at a longer life, was developed by a pharmaceutical company that set its list price at $311,000 a year.
With my second son, the problem was visible. If you looked at the baby’s head from above, you could see how it tapered to a point in the front. His temples were hollow, his eyes seemed a little close together, and, most noticeably, a bony ridge stuck out from his forehead. Search the internet for those symptoms, as we did, and you’ll find photos of babies who look exactly like ours. Still, when we met with a neurosurgeon a few weeks later, I thought there might be tests to do, an X-ray or a detailed and drawn-out physical exam.
Later, I found out the neurosurgery department squeezed us into the surgeon’s schedule after finding out our first baby was born with cystic fibrosis. My husband and I kept saying that to anyone who’d listen, even though one baby’s condition has nothing to do with the other. I think we wanted to make two things clear: that we could take whatever it was the surgeon was going to say, and that we could not believe the ferocity of our bad luck. Unsurprisingly, none of that seemed to be on the surgeon’s mind when he looked at our baby from across the room and barely paused to diagnose him before explaining treatment options. But wait, I interrupted, was he sure the baby had it? He was.
Normal babies are born with their skulls in separate pieces connected by fibrous joints called sutures, most of which don’t fully fuse together for years. Babies with craniosynostosis — like my younger son — have one suture, sometimes more, that have fused too early. The majority of the time, this happens in utero, in secret, undetected until after birth. Some cases of craniosynostosis are associated with genetic syndromes, but most aren’t, meaning that for now, the cause is largely a secret as well.
That last part was explained to us by a genetic counselor, who was just as patient and kind as all the others I’ve talked to since my older son was born. This second baby was conceived in a fertility clinic, we told her, using an embryo we had tested to make sure what happened to our first baby wouldn’t happen to our second. Why did something happen to this one too? I wish I knew.
Treatment options for craniosynostosis include surgery (to unfuse the suture) and helmet therapy (to shape the skull), sometimes one or the other, sometimes both. If left untreated, the brain doesn’t have enough room to grow, and the resulting pressure can cause developmental problems, blindness, and, in rare cases, death. At least one study has shown that even with treatment, kids with craniosynostosis are more likely than their unaffected peers to have developmental delays and struggle in school.
The baby was born in June; he was diagnosed in July. Draped in Northern Californian fog, the summer staggered on. We took our older son to his regular doctors’ appointments, and we took our younger son to be examined by his own expanding set of nurses and doctors. A few appointments in, one of them passed along the phone number of a woman whose baby had also been diagnosed with craniosynostosis.
This woman turned out to be one of those people you meet during a medical experience and never quite know how to thank. She still texts me sometimes. The first time we talked, we spoke for almost an hour. I wish I could say I never bristled at her, even if it was just once. “Oh, I don’t know why I bothered to ask how you are,” she’d said. “I know how you are — you’re in hell.” I don’t know, I thought, am I? What I felt most in the first months after my second baby’s birth was a twisted sort of luck. In the years since my older son was born, no one has ever said the kinds of things medical professionals were saying to me now: This is a problem with a solution. Ultimately, your baby is normal, and healthy. This might seem hard now, but it’ll be over before you know it.
It was still dark out when we brought our younger son to have surgery on his skull. After checking in on the hospital’s outpatient side, we were led to a curtained-off section of a larger room. There, his father dressed him in a blue gown that didn’t even come close to fitting an 8-week-old, and I watched.
I said I was keeping my distance because the baby was hungry — he’d been fasting since the middle of the night — but that’s not the entire reason I stood three feet away and shook a brightly colored toy peacock while people with clipboards came in and out. The baby cried just a bit before settling, quieter than a hungry baby should have been, and peeked over my husband’s shoulder to stare right at me. Between his look and his stillness, he seemed wise and almost accusatory, like maybe he understood that despite what she’d been saying, his mother was afraid.
Eventually an OR nurse led us out down a hallway, and over a sky bridge connecting the outpatient building to the inpatient one. My husband held the baby and I held the car seat while she chatted brightly about her own children, how they were in college and it would all go so fast, that soon enough our two sons would be that age. Of course I did not say what I was thinking, that some kids with cystic fibrosis still die in their teens and 20s, how when I see kids with CF who are of college age, I feel almost choked by hope.
The sky was blue in Oakland that day, a blue we don’t see often in the part of San Francisco where I live. Gliding over the sky bridge felt like maybe we were crossing into another plane of existence, and the nurse sped up her chatter, probably reading the shifts on our faces as we walked into the inpatient facility. At the end of a hallway, we reached the door where parents could go no farther. Should his car seat go with him? She wasn’t sure. We could go down to the cafeteria, though, and she would call us if the surgeons wanted us to bring it back. She held out her arms, waiting for us to hand him over.
My husband headed to the elevators, and I headed to pump in a windowless room with breastfeeding propaganda plastered all over the walls. The bottles, two for each side, filled, and I promptly spilled all four. When I found him in the cafeteria afterward, my husband was in a booth, surrounded by a strange array of snacks: natural tortilla chips, Red Vines, fruit wrapped in plastic. It was 30 seconds and 30 years in that booth, before two surgeons appeared in front of us, both with sweat on their faces, both saying it had gone well, the baby was doing beautifully. Everyone babbled the right words, and then, victory still on their faces, the doctors ran out of things to say and trailed off to get in the hot-food line for lunch.
While we prepared to go up to the pediatric ward, my older son was 20 miles away, swallowing spoonfuls of peanut butter spiked with a single dose of medication that costs $426. “Yes,” he’d said when we explained his brother would be gone for a little while. “His head is an oval. It needs to be a circle.” He nodded without looking up from his drawing, seemingly confident the doctors could fix the problem in one go.
This was in August, and we had no idea that in October, the FDA would approve a new drug therapy to treat cystic fibrosis. This therapy is a combination of three drugs (one of which my son already takes), and it’s a revelation, expected to work for a much, much larger number of patients with CF than previous drugs. The price tag, however, is familiar — over $300,000 annually. I’m hopeful about it. I’m still scared all the time. There are so many ways to lose access to a drug that a pharmaceutical company says is worth $311,000 a year.
Unlike pediatric intensive-care units I’ve been to with my older son, whose lungs are too vulnerable for the bacterial-infection risks of a group setting, the one his little brother was in was a single large room. Beds lined the walls, their privacy curtains partway drawn. A nurse explained the protocols while she led us to a nicely windowed section with views of the freeway and the bright East Bay sky. I stopped listening once I saw him: a small, bleating thing in the center of a large bed. The tubes snaking alongside the cardboard holding his limbs straight seemed like too much; the white-mesh covering on his head, like not enough. Everything I’d been saying about how this was nothing compared to our older son’s problems, how this would be easy, how we knew it could be so much worse — gone, destroyed. For that searing moment, all the terror and all the dread I’d been keeping at bay came on full force.
But that was only how I felt. The bone removed from my son’s skull was approximately three by five centimeters, nothing metaphorical about it.
The inpatient facility was being renovated, and we’d been warned about the construction noise, but we hadn’t really understood what that meant: jackhammers, lots of them, coming from what seemed like directly below the baby’s bed. To tell the truth, it helped that the noise was nearly unbearable because when I looked at him — or heard his half-hearted cries, or hovered my torso over the hospital bed and tried to feed him — what I felt was worse.
That first night after surgery, I took a lot of trips down the hall to the bathroom, past the elbow-shaped room where the family members of sick children ate takeout and charged their phones. Every time, I wished I could just keep walking and not stop until I was well outside the hospital doors. Perhaps the baby could meet me there, and I could carry him far enough away that neither of us would ever have a reason to turn back. It’s an urge I’ve felt ever since my firstborn was a few weeks old: to somehow bring us to a place not where things can be fixed, but so far from reality that they never needed fixing in the first place.
Our baby seemed more comfortable in the morning. I took a video of him sleeping peacefully amid the tubes and the cardboard still taped to his limbs. He looked even more like me then, when his face was swollen from having part of his skull removed. At some point he seemed to giggle at the construction noise beneath his bed. “Would you look at that!” a nurse said. “That’s amazing.” I looked, wanting to see more of what she saw — a baby who, for the moment at least, was okay. I think I did. I’m trying to see that a lot more these days. I’m trying to see it when I look at both of my children.
The sky was bright when the doctors decided my younger son was ready to go home. In Brooklyn, we lived so close to the hospital we could walk home after being discharged. Here, I clicked the baby’s car seat into our minivan and checked to see how long it would take to drive over the bridge. We were thousands of miles from where we started, and we were right back there, the same but not, different but not, as if we’d never sat in any of those doctors’ exam rooms, discussing our sons’ fates. As if we’d been doing this our whole lives, and as if we were just getting started.