Earlier this week was our regularly scheduled appointment at Johns Hopkins. It had been almost 8 weeks since our last appointment but it felt like only a few since we made the trip. I still cannot imagine how we made that run weekly (and a few times twice a week) all of the way from Western Maryland with a screaming newborn in the car.
Our last appointment was in early April and at that visit we learned that he put on twice the expected weight from his previous appointment so his team lowered his calories a bit. I suppose we are going to have to watch Corrigan’s juice (and root beer-if I am being honest) intake because at this visit he gained three times the expected amount of weight since April. Granted, we are only talking grams here but while careful caloric management is super important for kids with Urea Cycle Disorders, I do not want his calories to be empty ones from soda.
While there I told them that I was worried about making sure that Corrigan was properly hydrated during these hot summer months. When we go to the playground, or anywhere that he runs around and gets heated, I take a few 60ml syringes of water or juice to bolus through his g-tube while we are there. I try to get him to take drinks from his sippy cup but he is so busy having fun that he doesn’t want to stop to drink, or if he does humor me with a sip it is just that…a sip.
Corrigan’s meds do a wonderful job scavenging the bad stuff from his system but without the proper amount of fluids the meds will not effectively whisk it from his body. He needs to pee for everything to work the way it is meant to work and if he is dehydrated from over exertion, or illness, then things get dangerous quickly. I thought that I was doing a good job of bolusing him a few ounces every hour that we were out playing but was surprised that his fluid intake goals were way higher than I was achieving.
Cor gets 44 ounces of fluids between his formula recipe, his medicine boluses and the ounces of water that we use to flush his tube 6 times (or more) a day. On top of those 44 ounces, on a regular day when he is not in the heat, they want him to have another 11-12 ounces of other liquid. Juice, soda or water. Heck, even popsicles count! However, on days when he is playing outdoors our goal, on top of his 44 ounces is now an extra 22 more ounces of liquids!!! Man, I wasn’t even close with my 3-4 ounces per hour at the playground.
How on Earth do I potty train a child that has to consume this much fluid per day?
(The above information pertains specifically to Corrigan and should not be taken as medical advice for your child. Proper hydration IS important for children with Urea Cycle Disorders though so consult your child’s dietitian for information on how much extra fluids your child may need during the hot summer months)
Corrigan’s ammonia, at this week’s appointment, was 61 which is about 20 points higher than his normal. After looking at all of his labs, and taking into consideration his recent weight gain, we made some changes that should help bring his number back down and likely changed course a possible hyperammonemic episode had we not gone down for a check-up. Yes, the nearly 3 hour runs to Baltimore are a pain in the butt but his appointment this week showed us why careful and close management by a metabolic team is so crucial to stability. If we had waited another week things might have turned hairy.
Speaking of pains in the butt. Do you know what is super fun?
Well let me tell you.
Baltimore in the summer.
Super fun! (not)
Due to our appointment time, Corrigan and I had to park on the very tippy-top of the parking garage and when it was time to leave, and after I heaved his stroller into the trunk, the stupid trunk would not close. In 97 degree heat I stood slamming the trunk over and over again, becoming more panicked as each minute passed. I was hours away from home, alone with just my toddler in the middle of Baltimore and no idea how to fix a stupid trunk latch.
I may or may not have called my husband and sobbed like an idiot. What did I think that he was going to do from hours away? My hot tears just made me more angry and then when I waited for nearly an hour for the parking garage security to come and assist and they never showed up, I was even more furious. What was I going to do?
I was getting sunburned and drenched in sweat. I stood there pressing the trunk button on my key fob and watched as the mechanism partially unlatched but the one piece that was stuck never moved. Finally, I angrily grabbed a pen from my purse and worked out the timing of pressing the button with my left hand while jamming the pen under the latch at the exact right moment. It took a few tries but it finally unlatched and I could have sobbed even more with relief.
Of course, by the time I got down to the parking garage exit my ticket time had expired (Hopkins only allows 15 minutes from the time your ticket is stamped and the time you leave the garage) so I had to back up from the gate (with a line of angry, horn-honking drivers behind me) and park again, get Corrigan back out of the car, carry him back to the ticket window and then stand in line all over again to pay the difference.
We must have looked like swamp rats because a lady in line behind us tapped me on the shoulder and said, “Here, you look like you need some cold water” and handed me the iciest, most delicious, H2O that I have ever drank in my life. That was a pretty cool gesture, right?
We made it home in good time, no traffic delays, and things have been good. We are not scheduled to head back down to Baltimore until the last week of July or first week of August. That appointment will be a bit more stressful because we also have to squeeze in a GI visit to check on his g-tube size but for now we are going to enjoy our summer and make some memories!
Thanks for checking in on Corrigan!