Perspective...

"There's no such thing as simple. Simple is hard."
~Martin Scorsese


And what a tough pill to swallow in a world where the main objective is to make everything more simple. Simple ready-in-minutes meals for the active family. Super absorbent paper towels for quick and simple clean up. Online banking for fast, simple bill paying. Simply put, we go to so much trouble to make things simple for ourselves that we forget what it takes to put the work in for the ultimate outcome. I am not immune to wanting my life to be simple. It just isn't. When Coulby was born, simple redefined itself in my world. My life pre-Coulby now seems simple. My life when Coulby is healthy and metabolically stable seems simple. But in the times when every day is a struggle to keep up with what he needs to thrive, I realize how not-so-simple life truly is. And how not-so-simple his every day is, living with Citrullinemia. The last week has been one of those reminders that always snaps me back into reality. HARD! You would think that after (just days shy of) 6 years of living this UCD life that I would not get such whiplash every time we hit the metabolic wall. But it happens every time. Every. Single. Time.

Okay, so usually I allow myself to ignorantly believe that once we get through March, all of the nasty germs that kids spread back and forth to each other will kind of dissipate. And I know that must sound ridiculous to most, but it is my attempt to will away any illness from touching Coulby after the long Winter of colds, stomach viruses, flu, fevers...you get the point. You can imagine my disappointment when Coulby was sick, yet again and in April no less, with a stomach virus not so long after the last round that he so graciously shared with all of us. The stomach virus that landed him in the hospital for a 4-day stay from hell. *Shudder* I do not even like to think about it. Brings me back to a very bad place, mostly for Coulby, but also for myself. So anyway, Coulby was sick yet again.

He started not feeling well about a week and several days ago. Just not eating. Saying his stomach hurt. No details, but going to the bathroom a lot. Throwing up if we tried to push food on him to meet his daily protein and calorie requirements. The poor kid was just sick and there we were trying to force food and drinks on him from sun up to sun down. It does not make me feel good to say that. For all of Coulby's life I have mourned not being able to take care of him while he is sick the way a mom should. The way my mom always took care of me when I was not feeling well. Coulby might as well have me saying that 'sick' is a non-existent word in his vocabulary. That he might as well scratch it out because he cannot be sick. Not like other kids.

The not eating or wanting to drink anything other than water went on for about a week. Literally, we were just waiting for the moment when we would have to bring Coulby to Hopkins. I always wonder how long he can possibly go without meeting his metabolic requirements before he gets into trouble and needs medical attention to pull him out of it. It is scary. And I really believe it is that fear that makes going through each and every illness so stressful. It is that fear that people do not understand. And the struggle that no one can understand is having a sick kid and still having to get him to eat. What do you feed a kid with a stomach virus? A picky eater, no less. Do you want to eat anything when your stomach hurts? Now you see why I say that when Coulby is sick, life is not simple. Not at all. For the most part, our daily routine in caring for him has become second-nature. But we are reminded of the smallest things that we do every day to keep him alive and well when he is sick. Because that balance gets so thrown off and we so desperately try to gain it back.

Coulby finally met all of his protein requirements today for the first time in well over a week!! This is blog-worthy. And if you are a parent of a child with a UCD, you know why. Because it is that opportunity to kind of breathe again. To live again. Coulby is finally asking for food. And drinking milk. And tormenting his sister with a vengeance (ah, sibling rivalry). Coulby is back! Oh how sweet it is!

And now I can resume life under the illusion of simplicity. Go through the motions and slip into our "normal" routine again. Into the daily rituals that usually seem so simple, but become so hard when we are thrown a metabolic curve ball.

Here we go again...

My camera usually tags along wherever I go, and out of the hundreds of shots that I take, I usually only end up with a handful (if that) of photos worth sharing. Coulby is not the most cooperative subject, so I have to be quick with the camera if I want a decent photo of him. I have found that calling his name and snapping off a shot when he turns to respond is my best plan of attack. Hence the photo accompanying this post. This photo of my sick boy. Yeah, I said sick boy. As in he does not want to eat anything and throws up every time we try to push something on him. So what do we feed a kid who does not want to eat but HAS to get so much protein and so many calories and so many ounces of fluid in every day?


Honestly this week has been such a test of patience. I am failing miserably. It does not help that patience is not a virtue I am blessed with. Then to have to maintain patience with Coulby when he does not want to eat anything, and I mean ANYTHING, well...it is just next to impossible for me. And I know that he did not ask for any of this. This life that he was given: always having food pushed on him, always having to drink his concoction of formula and medicine, having monthly blood draws at Hopkins and enduring hospital stays when illnesses cannot be managed from home. The poor kid has been through so much in his short life.

Here is to hoping that after four days of not feeling well, Coulby will start to rebound and get back on metabolic track. It is emotionally and physically draining to be a parent of a child with a metabolic disorder any day, but especially when he gets sick. My impatience comes mostly from my fear of Coulby becoming hyperamonemic, with the possibility of brain damage, and ending up at Hopkins, confined to a bed with an IV in his arm for several days. I know that people cannot really know what this experience is like unless they live it, but I always hope that by blogging, those who do not live with a UCD day in and day out can at least learn what it means for those of us who do. And for those who do, I have the utmost respect for you because I know exactly what it is like!

Illness Be Gone!

This is Coulby's new look...what do you think? He has been sick yet again with a cough, runny nose and an on and off fever for four days straight. Not fun. It seems like we have one healthy week for every three sick weeks. Not fair. Since Coulby had a fever even yesterday morning, we decided we should take him to see our local pediatrician. This was not something that required a rush to Hopkins (whew!), and in such cases we see the pediatrician and consult with the geneticist and dietitian.

Honestly I did not expect the pediatrician to be able to do anything for a persistent cough, so when my husband called and said the doctor thought Coulby has pneumonia, it took me a minute to process what he said. Coulby was given Albuterol via a nebulizer, and prescribed two antibiotics to wipe it out.

Of course I spoke to Coulby's geneticist about his diagnosis and prescriptions, and she seemed to think that from a metabolic standpoint, Coulby was handling this illness just fine. She was not concerned, therefore I was not concerned. It is just another illness to deal with, and we have had our fair share this season. I think we deserve a break! But I am thankful that Coulby is HOME through the whole ordeal and not in a hospital bed. Another blessing. I never thought my son would be able to weather so much from the comforts of home.

As for the photo, Coulby absolutely LOVES his new mask, and thinks it is cool that he can blow smoke "like a train." In fact, he keeps asking to "do his mask." At least it is a novelty for him...for now!

My boy

Before I laid down to sleep last night, I talked to my husband, who told me that Coulby's ammonia was at 33 (woo hoo!), but his temp. was back up to 103 (boo!). He was awaiting a dose of Motrin. You would not think it would involve all that much to get a dose of Motrin into a kid with such a high fever, but when you are in the hospital, such a simple thing can take, literally, HOURS! You know, the order has to be written for the Motrin, then has to be sent on to be filled, then it goes through several more hands before it finally makes it into Coulby's mouth. It can be extremely frustrating! Unfortunately, as parents, we have no pull in speeding up the process.

Coulby arrived at Hopkins yesterday morning and did not actually get placed in a room until 7:30pm! I wish someone could tell me what takes so long! Especially when, at 3:30, my husband was told that Coulby would get into a room in an hour and a half. Then, around 4:45, it was that he would be in the room in another hour. No, you do not need a refresher math course-it just does not add up! AND Coulby went 2 hours not being hooked up to the IV fluids! Not good for a metabolic patient with a temp. of 103! Who dropped the ball there?? During all of this, Coulby's formula was supposed to be mixed and ready for him when he arrived at the PCRU (the floor on which he spends his hospitalizations). The orders for the formula had been written up around 4:00. Because things were not running smoothly in the ER (and yes, this is Hopkins we are talking about), the doc did not want anything more to be done for Coulby until he was at the PCRU. He wanted to ensure that things would run more smoothly and be done in a more efficient manner than had been in the ER. Coulby was due to get (2) 3 oz. doses of formula with meds. before bed time. This would make sure that he had some medication on board, since he was not getting them through IV.

So, when Coulby got to the PCRU (at 7:30, remember), there was no sign of Coulby's formula, and still no Motrin. By 8:37pm, Coulby still had not received any formula, and was ready for bed. There would be no way he would get 2 doses in before he fell asleep! I did not know what to do, so I called Coulby's dietitian at home. Not something I normally do. She had to call the PCRU to get them moving and figure out what had happened to Coulby's formula that she had ordered to be mixed. And then, miraculously, they found it! Coulby finally got his Motrin, which did in fact keep the fever down throughout the night. But the disappearing formula was re-mixed and Coulby was given one dose before bed. At least he got some of his medication on board.

The story continues...while in the ER, Coulby had a blood draw, from which they took a sample of blood to be cultured. This would help determine what Coulby was fighting and why he kept getting a fever. The nurses at the PCRU informed my husband that they would be taking a sample of blood to be cultured. Confused, my husband said this had already been done in the ER. There was no record of this! After some confusion, the blood was found--sitting in the ER! It had never been sent to be cultured. After solving yet another mystery, the blood was then sent off to its original destination to be cultured.

If you are confused, you can imagine how confused we have been! There are so many little things that seem to always happen when Coulby is at the hospital, and it is exhausting because we cannot let our guard down for a second! We learned that early on. But I would think that Hopkins, of all places, would have a much more efficient way of running things. As Coulby's dietitian said, they (they=those who dropped the ball with his formula AND kept Coulby off of IV fluids for 2 hours while in the ER!) need to get themselves together because they are compromising Coulby's metabolic health! He cannot afford to be the victim of such dumb mistakes. It really is frightening. The whole situation.

As of this morning, Coulby seemed to be himself, despite the hospitalization and the lack of sleep last night. It is kind of hard to sleep with people coming in and out of the room all night long. But his temp. had started to go back up at 99.1. His ammonia, on the other hand, was at 26! Some good news! The verdict is that Coulby can go home today as long as he can eat and drink AND keep it down. Ideally he will get about half of his normal protein intake. When I got off of the phone, Coulby had finished his formula (although it took him a little while), had several bites of Rice Krispies, and had drank several ounces of milk. Not great, but a start.

I will be heading to the hospital around noon if Coulby needs to stay another night. I am praying that he will find his appetite long enough to be cleared to come home. I think he would be much more comfortable here, surrounded by his family...and of course, his trains!

I am sure he would love any words of encouragement, so feel free to comment! I will pass them along to my little trooper!