My son, Wade, was born with Pfeiffer Syndrome, a genetic disorder that affects development of the skull. It requires repeated surgeries as he grows to reshape his skull, allowing room for his brain to grow. Little Wade had a pretty rough start - more than 50 surgeries in the first 5 years with accompanying hospital stays for a myriad of reasons aside from his skull. He would quit breathing without explanation and we wouldn't know what to do, except hope that the CPR and rescue breaths we were giving him would be sufficient to keep him alive until help arrived. We'd call the doctors, rush him to the hospital, try different medicines, position him differently, try different foods. Anything they said to do, we did. It got so bad that he was life-flighted six times in three months.
Each time we were life flighted, we were told the same thing, "His MRI looks fine." But Wade was not fine. Being life-flighted multiple times for not breathing isn’t what kids who are fine do – even kids with special needs. I couldn't convey to his doctors that yes, he does have special needs, but they only see him when he's sick. They don't see him when he's happy and playing with his toys and grabbing his feet to put in his mouth.
Trying to find answers, we took Wade to Seattle, Cleveland, Dallas, and then back to Houston. Then we found Dr. Dan Curry, a neurosurgeon at Texas Children's Hospital. We went to see him, and it started out like all the other appointments: me explaining in my mom language that Wade would just quit breathing sometimes. Dr. Curry saying, "His MRI is unremarkable. It's what I would expect for someone with Pfeiffer Syndrome."
“I’m telling you something is wrong”
One of Wade’s other diagnosis’ is hydrocephalus, which is basically extra fluid in the brain that has to be continuously removed by a drain that’s placed internally from his head to his belly, called a shunt. By now, I had surmised that his shunt wasn't working properly. So I told Dr. Curry, I understand that his MRI looks fine. But I'm telling you that something is wrong. I explained that when Wade lays down he's fine, but when I sit him up he starts crying and pushing back to lay down. He starts hitting his head.
This time, I also asked if we could do an intracranial pressure (ICP) monitor to see if there's pressure in his brain. Parents have been my greatest resource and have taught me most everything, including the words to use when talking to the doctors. They are the ones that suggested maybe it was his shunt and the only way to truly know was by inserting an ICP monitor into his brain to check the pressure inside his skull. Dr. Curry surprised me by agreeing. "If I know one thing, it's that I don't know everything and these kids always surprise me,” he said. “So let's just see what we find."
“Do you see that number?!”
We did the test the next day. It’s important to point out that this is not a simple test, like drawing blood. It requires drilling a hole in the skull and inserting a monitor. When Dr. Curry came to check on Wade after the monitor was placed, he walked in and went to the ICP monitor display at the head of the bed. He calculated some numbers and said, "Do you see that number?! That number is 90. His ICP is 90! Normal is 0-20, 20 when you're laughing, but it goes right back down." He hurried away and we were immediately scheduled for emergency surgery to replace Wade's shunt.
It was in that moment that it all came into focus for me: I had been watching my baby suffer. For years. Four years actually. I remembered all the times I had no idea if what I was seeing from Wade was a headache, sinuses, seizures, upset stomach, stubborn toddler, shunt malfunction, or death knocking on his door. I remembered all the times I had made him go to school even though he was crying and hitting his head. I had known all along that something was wrong, but I didn’t believe in myself, in my voice. I didn’t know how to explain what I was seeing in language that doctors would listen to.
Today, Wade is happy. He is a jokester who loves going to school and seeing his friends. None of that would have been possible if it weren't for Dr. Curry hearing my concerns as a parent and taking a chance. And none of it would have been possible if I hadn’t insisted on being heard, taking a chance in suggesting that Dr. Curry test his intracranial pressure.
Many years after this has happened, I still come back to the times that I felt so powerless that the only thing I could do was kneel at Wade’s bed and weep. But don’t ever give up. I learned that even if it takes years, if you know inside that something isn’t right - believe it, keep fighting, and speak up.
Becky went on to become a neonatal intensive care unit (NICU) nurse, applying her experiences as a parent in the NICU and through all Wade’s subsequent medical needs to her own practice as a clinician. Read more about how she is working to improve patient-centeredness at Texas Children’s Hospital in a forthcoming blog.