Corrigan has had a tough stretch, metabolically, with two hospitalizations in just two months. He hasn’t had this kind of instability since he was an infant and it’s been frustrating trying to pinpoint the issue exactly. I don’t want to get into too much detail about the “why” because honestly, they’re just theories but here’s a review…
On Labor Day weekend, he woke with terrible tooth pain and as soon as the dental office opened that Tuesday morning, they had him in to find the problem. Because he’s not an oral eater, the situation underneath the baby teeth, in the gums, is quite the disaster (chewing helps push up adult teeth and remove baby teeth) and the dentist removed four teeth to make room for the permenant ones.
The visit was incredibly traumatic for Corrigan and we believe it led to an ammonia spike that landed him in the hospital locally for a day and a half. That starting ammonia was reletively low (under 125) and was under 40 after just 90-minutes of rescue med bolus.
(For review, non-UCD patients- like you and I- likely have ammonias under 25. Corrigan’s baseline is around 35. He can tolerate up to about 65 without issue. We hospitalize and begin rescue at 100 or higher. Anything approaching 200 is believed to be “brain damaging” levels and we transport to Hopkins. Anything under 200 we stay locally. However that may change as we’ve heard they’re getting rid of the Pediatric unit locally)
Last week, there was an event at school where Corrigan collapsed and was not able to be roused for several minutes. An ambulance was called and he was taken to our local hospital, where several errors in his emergency protocol were made, rescue was delayed FOUR HOURS and his ammonia rose to 270 before his first drop of Ammonul was delivered. (I’ll address protocol here in another post)
He was then life-flighted to John’s Hopkins in the middle of the night. Again, it is believed that the stress from his collapse triggered the hyperammonemia, though again, it’s just a theory. He had a ton of tests at JH and his heart is beautiful and healthy and we have NO idea why he fell out and no idea if it will happen again.
Thankfully, his ammonia did recede, as it always does Thank God, to under 60 after his 90-minute rescue bolus locally, but we still transported due to the loss of consciousness. We wanted his teams eyes and hands on him after something so crazy and out-of-the-ordinary occurred.
It’s been a rough stretch with all of this turmoil on his brain, his behaviors at school have ramped up to very concerning levels. He doesn’t smile much anymore, he’s angry all of the time and very easily triggered, he’s frustrated and has very little energy to do active things.
He’s struggling mightily.
It’s hard to know exactly how all of this makes him feel. I’m thankful to belong to several UCD support groups with affected adults, who willingly share what elevated ammonia feels like and what recovery from those levels feel like as well.
They tell of the feeling of bugs on their skin as ammonia climbs. Hallucinations and disorientation. Temperature fluctuations that feel more extreme than what register on a thermometer. Shaking. Headaches. Tremendous pain behind the eyes. Eyes turning. Mouth swelling. Aggression and restraints can be needed as ammonia rises.
And the recovery after rescue whisks away the ammonia? “Like recovering from a ten-day booze bender” one member told me.
This is what my boy has to deal with while also trying to work around the brain damage and developmental delay that his outrageous newborn elevations caused before he was properly diagnosed. And the agitation on the surface of the brain that it is believed is caused all of the time, each and every day, by metabolites floating around also because of his disorder. Metabolites that we have no way of measuring or removing. Just floating around and pissing him off forty-nine times in 24 hours.
We send him to a school that loves and supports him the best they can, who carefully watch for signs of crisis (they’re the ones that called for me to get him checked after the teeth were pulled, and we went straight from school to the ER and they were DEAD RIGHT) and they have a strong history of good special education in our county, but Corrigan- I believe- is challenging even the most tenured teacher and aides this year.
I don’t like to share negative things here, I realize that many UCD families read these posts and new families often find this spot in Google searches so I’ve made a concerted effort over the years to keep things positive. But things aren’t positive at this point in the journey, and they haven’t been really all that great for awhile.
He has outbursts, all day long in school. Some seem to be related to the task at hand. We believe that he’s now at a cognitive level that he understands now that he’s different. And he also feels shame when he fails. Before, he was oblivious to expectations and never cared what others thought. Now he understands that he’s not comprehending something and often becomes upset before even trying something asked of him. It’s easier to throw a fit and be removed, than to try something and fail.
Oftentimes, his outbursts are inexplicable. He can be enjoying music or something fun and just start crying. He may stand up and start yelling. This last weekend, he was at a birthday party for his cousin, surrounded by tons of fun and toys and games and a bounce house and candy…yet he was miserable for most of it.
It’s hard for us, as parents, because we are helpless to know exactly what we can do to help, because he doesn’t have enough words to tell us what the problem is in the first place. There is no stop-valve in Corrigan’s brain, if he feels it, it comes out of him and it’s rarely pretty.
We can’t parent him like we did our firstborn, neurotypical son. You cannot spank a child with brain damage! You can’t possibly know what he means or doesn’t mean and he certainly isn’t going to understand why I tell him not to hit and then up and spank him when I’m upset. I don’t agree with spanking anyway, after all my research, but people still think a “good spanking” is all a kid like Corrigan needs.
He leaves for school each day hopeful and armed with all of my words. “Be a good citizen” and “Make good choices!” I write notes to remind him how to behave and let him know that I expect him to attempt to control his temper, and he promises he will, but it all falls apart the moment a negative emotion hits him. And when he gets off of the bus, my heart breaks because I can see his shoulders slumped. His sad eyes. His defeated demeanor as he says “I’m sorry Mommy” before he’s off of the last step on the bus. “I said all da bad words today. I go to jail now.”
His teachers are struggling. He is struggling. We are struggling. Everyone is struggling. We all see so much potential in our boy, he has moments of such sweetness and he sooooo wants to be “good” but he has a lot stacked against him that prevents him from that goal.
Mark and I will weep at times, talking out all of the concerns we have for Corrigan, it breaks our hearts that his life is so difficult. We are not able to provide much fun for him. We aren’t able take vacations. He doesn’t get many toys. He has never seen the ocean or Disney World. He can barely keep it together at our little local fun amusement park or a simple birthday party, so we are fearful to attempt bigger things even if we could afford them.
He doesn’t eat in restaurants because the smells make him nauseated. Meat, cooked or raw, we’ve been told by adult affected patients, is an overwhelming and nauseating odor for Urea Cycle patients, and he doesn’t eat anyway, nor have much patience for waiting.
He’s never been able to sit through a full 2 hour movie so I’ve never attempted a movie theater. He can’t play sports because we can’t seem to keep him balanced enough with the calories vs energy expended. He misses out on so many fun things, because of this disorder and the damage it has caused, it isn’t fair.
I’ve reached out several times to Make a Wish, but they never ever reply, even though every UCD kid we know has been gifted a Wish. But even if something like that came through for Corrigan, could he handle it? Would we be stressing his system by being off schedule? By wearing him out walking? Or swimming? Can being too excited lead to a crisis? Am I seriously living a life where too much happiness is a danger to my boys brain?
We do a lot of “outdoorsy” things, which basically translates to “we take lots of low-stress walks”. He’s calmed by being outdoors. He loves the trees. He’s fascinated with moving water sources and bugs. He finds his peace sitting in the grass or staring up at the stars.
After last weeks stay, I was told that he’s starting to talk about “death” and “dying” in school and this weekend, I finally heard him talking like that too. It’s startling. I can’t swear to you that he understands death. Honestly, I don’t think he possibly could? But nontheless he’s learned the terms and he’s using it appropriately and it worries me to my soul.
He can’t tell me what it felt like to pass out. Is he afraid it’ll happen again? Did he feel it happening? He only spoke three words to me during the ambulance ride.
“I’m sorry Mommy”
The EMT nearly cried. I did for both of us.
If you ask him how he feels he tells you he’s sad.
He’s 9.5 years old this week and while many things HAVE gotten so much better, many things are getting much worse now. Add in the fact that I’ve lost complete faith and trust in our local hospital, that puberty is around the corner which means more instability, and that we can’t seem to get his aminos properly balanced, and it’s all a powder-keg.
I don’t know exactly how Corrigan feels, but I can tell you that it isn’t happy. He had the best August. He smiled so much his face must have hurt. He was bright and learning rapidly and was even-tempered and funny. Watching his sense of humor develop was amazing but that’s all been lost to the insult of two back-to-back elevated ammonia events.
Will he recover? Yes, his team believes so. These events are caught quickly and because he responds so well to rescue boluses, we have reason to believe that his regression and anger is not set in stone, but these are speed bumps on an already potholed road from his birth crisis.
We’ve had a pretty great stretch of stability but things are about to get hairy as he enters puberty, when all of the growing and hormones makes managing any metabolic disorder scary, and I don’t know what the future holds. So I’m getting my thoughts out here again as they come, as I used to when he was a baby and so unstable. That time is upon us again- I’m not ready. He’s not ready. Why is our cure not here yet?