(The second question is always, “Why haven’t you transplanted him?” I will touch on that soon.)
Corrigan’s story from birth to diagnosis is so similar to many other family’s stories. The vomiting, the lip-smacking, the tongue-rolling, “seizure-like activity is observed,” rapid breathing…coma. We all have that number though, tucked into our heads, the one that we pin the hopes of our child’s future on.
“How damaged is his brain from the ammonia?”
If fortunate enough, we are sent home after the neonatal battle for our child’s life with scales, powerful meds, amino formulas and fear in every single breath that we take. That number, that highest number still lingering.
We get home and struggle to find a routine amongst the weighing and measuring, the difficult feeding, the fussy infant and our constant need to watch every single movement their little bodies make. Every spit-up makes our stomach lurch into our throats. Not because if the unappetizing nature that spit-up is, but because we just do not know…
“Is this a sign that his ammonia is rising again?”
We forgo our own sleep and sit beside their crib or cradle, watching their tiny chest’s rise and fall. We whisper prayers to Higher Beings as tears roll down our cheeks.
“Please show me a clear sign when he is in crisis. PLEASE LET HIM WAKE TODAY…TOMORROW…please?”
We search the web looking for answers. We make ourselves read all of it, the research findings, the personal stories of triumph and tragedy. We know that we shouldn’t compare one child to another. We should simply be thankful that our child is still alive but that number. That highest number is still in our head and when we find someone that is sharing their story, whether on a blog like this one, or in a Facebook group, we find ourselves reaching out.
“My daughter has ASA and is 4 months old. How is your son doing? What was his highest ammonia?”
You cannot help it. You compare. You know that C has the same disorder and is in elementary school, doing so very well. His ammonia never got too high because he was lucky enough to be diagnosed on Newborn screening before the sneaky tendrils of ammonia kissed his brain. You know about B who’s ammonia was 1000 when diagnosed, went on to have a liver transplant but seems happy…healthy…alive, thank God. You read the story of J who lost his battle with OTC and cry hot tears at the beautiful words his family wrote for his funeral. You celebrate the fact that L’s highest ammonia was near two thousand yet is now meeting all of her milestones. Is talking. Communicating. On track.
“So-and-so’s child died with an ammonia of 400. Yet L lives, and thrives, on 1900…what does this mean for my child?”
Our child’s future…that number. And every single number from every single blood draw, the hundreds of them that they have had taken in their short lives, feels just as important. We cheer for one another when we learn of our UCD friend’s checkup…we aren’t looking for developmental updates, we are not concerned about anything else that took place during that appointment other than the number. We wait to hear the news. We cheer, no lie-my body jumps with joy, when I hear that Lynlee pulled a 37. And we all shiver, and pray fiercely, when we learn that our friend’s little guy is sick, admitted and she posts..
“It was 223″
Normal ammonia levels fall somewhere between 5 and 40. Our kids, especially the younger ones, can run in the 50’s and 60’s without an issue. Some even a bit higher. Though you have to wonder what running double of what is “normal” really does to them. We feel uncomfortable when the lab sends back a 77. Yet the team deems everything “fine” and send us home. We hate sitting anywhere above 65.
For Corrigan, and many other genetics teams, anything over 100 means admission. It means that something is in flux. Something is wrong. The urgency of the nurses and doctors in the ER, once that number above 100 comes back feels like electricity in the air. No one takes >100 lightly. I have heard conflicting stories as to what point, what number, does the brain start to be damaged from increased ammonia. I will not speculate here. I do not have that answer.
“Please let the emergency meds work quickly. Are the labs back yet? Can you check again, please?”
Corrigan was transferred from our local rural hospital to a more trauma-capable hospital at the end of his third day of life. Looking back, knowing everything that we know now, it seems so clear. How did they not recognize the symptoms? How did they read those labs and not put two and two together?
“Hindsight is 20/20″
Our story is the same as yours. The way it began is probably so close to the same it is weird. But maybe you live near a bigger city, or were transferred to a Children’s Hospital that understood the symptoms+labs. Maybe your Newborn screenings came back super-fast and you got a call from your pediatrician, or the State, before your child was showing any symptoms. That was not the case for Corrigan.
Oh, hindsight truly is 20/20. Mark and I knew nothing about ammonia. Or what is considered normal and safe and what is abnormal. There are notes upon notes in a book that I kept from the moment that we arrived at the second hospital. I scratched down every lab result they threw at me. I hadn’t a clue what any of it meant. I assumed that the doctor’s, at this better hospital, would read them and interpret them. Why wouldn’t they?
“We have no idea what is wrong, we will just have to wait for Corrigan to wake and tell us”
Eventually, after 4 1/2 days at the second hospital, Corrigan was diagnosed. Over the phone, really…by doctors at a hospital you might have heard about. And that helicopter came and took my very sick baby away from me and saved his life. I am grateful, please do not misunderstand, to have him here at all. Perhaps one more day, maybe two, hooked up to thousands of dollars of state-of-the-art equipment, surrounded by highly-regarded Pediatric physicians, watched lovingly by top-notch NICU nurses-AT THAT SECOND HOSPITAL-would have meant a much bleaker outcome for Corrigan. I might have buried my baby.
Later, much later, I requested medical records. After years of waiting on that number to come back to tell me if my child’s brain was in danger, through many ER visits and over a dozen hospitalizations, I had a clear understanding of the weight of what it all meant.
“Based on his neo-natal history we do not expect his IQ to be above 65″ they told us a few months ago.
Jotted down, nearly sideways in my notebook, under the title “May 31,2008” it simply says, “Ammonia 201” That was the date of his admission to the second hospital. Corrigan was three days old. A lab result returned less than two hours after the ambulance brought him to the NICU.
“Two-hundred and one”
One time, when Corrigan was under a year of age, Mark and I had raced him to ER fearful that his ammonia was rising because he didn’t seem like himself. I do not recall the specifics of the symptoms that compelled us to race him to the hospital like that but I remember sitting there, holding my baby (who was, by then, smiling and acting totally fine…figures!) and waiting on the doctor to come back with the ammonia results.
Instead, a nurse walked in right around the time the labs should have been returned with a look on her face that made my heart pound. Behind her walked in another nurse who looked equally disturbed.
“Just tell me, what is the number? How bad is it?”
And she looked like she was about to tell me something horrible. I braced myself for 250. Or 300…but I glanced down at my smiling, spit-bubbled baby and was confused. She took a breath and said, “I am so sorry…the ammonia came back as 71” (I can’t recall the exact number, to be honest, but I remember it was higher than his “normal” but well under the 100 that would get him flown by Medivac to Johns Hopkins)
I looked over at Mark and…we started laughing. Relief makes you do funny things sometimes. We had to explain that 70, for Corrigan, was not bad. She was confused. Everything that she had been taught, in nursing school, told her that someone with an ammonia twice the normal level was in danger.
“She got it. She knew the importance of that number. Why did the doctors at the second hospital not understand 201?”
The next morning, during rounds on June 1st, the team ran through his early morning labs for Corrigan and I jotted it down again. “Ammonia 209” A slight bump in a number that meant nothing to me. Corrigan had been NPO for a few days and they decided to start him on TPN. He needed nutrition, they said.
In the records that I requested, from Johns Hopkins, the intake report made it all too clear. “Infant presented to (second hospital) and initial ammonia 201. Subsequent ammonia 209. Early am on June 2, ammonia was returned at 325. Repeat ammonia brought back 383. By the time that the phone call went out for assistance to the mystery that was Corrigan, on June 4th, 2008, his ammonia was 622 and still rising.
The last lab drawn at the second hospital was in the upper 700’s but we were told that the sample sat for an extended period before the lab put it in the machine so it could not be verified as “accurate.”
So we count Corrigan’s highest ammonia as 622. Pretty good compared to L’s near-2000! Right? I mean, L is doing GREAT! Look at her talking. Attending pre-school. What a blessing.
“Except that Corrigan is not talking. He is not progressing. He is not improving at an appropriate pace”
Because his little brain stewed for four days in ammonia levels that we know can cause brain damage. That he was studied, and poked, and prodded by a Pediatric Neurosurgeon at “Hospital #2” and that his primary physician was the Head of the Neonatal Intensive Care Unit. You know, professionals that should have recognized that an ammonia of 201, 209, 325,383, etc…an increasing ammonia level over a period of days…was a sign of something that should have caused more research, more of an answer than “We will just have to wait and see what Corrigan tells us is wrong.”
That ER nurse in our rural hospital. She understood what was normal ammonia and what was not. She was properly concerned to get a number back that was double what is normal. Why were these doctors unable to see the importance of a number that was five times higher than normal?
“It is what it is, Mindy. You have to let it go.”
I will. I have. For the most part. I have grieved for Corrigan. For us. I try not to compare him anymore. I know now that Corrigan’s 622 was far more devastating than the kids that had double, or triple that number, but were treated far quicker than Corrigan was. Am I jealous? Sometimes. I am hurt. I am saddened beyond words. I get angry. My heart is broken.
“It is not necessarily how high the number was, but how long were they at that number, as to how it affects the brain.”
It is hard though, sometimes, to see the kids with UCD’s that I have grown to love doing so well. Does that make me a sucky person? It feels like it makes me a sucky person. I am truly joyful to see each of them meeting milestones. I am. But someone messed up. They missed signs that could have prevented this struggle for my little boy.
If that 201 had been taken as seriously, at “Hospital #2,” as a 71 is at my local-yokel, rural hospital (who we, to this day, get impeccable care for Corrigan) he might be talking right now. Oh, he can say a few words but it has been months since he has learned a new one. No two-word sentences. No understanding that anyone in his life has a name.
If not for that 4-day stalemate, He might be able to identify me. Do you KNOW what I would give for him to look at me and call me Mommy?
He might be able to go to the mall without a meltdown because he doesn’t understand change. Because he has sensory issues, and sleep issues and rage issues. He might not slap himself out of frustration at not being able to convey his thoughts and feelings. He might be starting to potty-train. He might be able to do a simple 2-piece puzzle. He might understand the mechanics of drinking out of a cup instead of still out of a bottle-or regularly using a fork and a spoon. He might be scribbling shapes or pretending to be characters in Disney movies. He might be about to go to pre-school.
“Corrigan is “self-injurious” and is recommended for intensive behavioral therapy”
A 4-day bomb to his brain with a number that peaked at 622.
Transplanting his liver won’t change the past. What is done. Is Done.
and now you know.