What I've Learned

People are funny. I include myself in this rather bland statement. Not "ha ha" funny. More like funny because their human nature makes them unpredictable. That kind of peculiar funny. Do you follow me, or am I making absolutely no sense? Maybe further explanation is required...

My mind was wandering the other day, as it often does, and I got to thinking about all of the people I have met or crossed paths with at different points in my life. Some I have formed close friendships with and we will forever be connected. Some became mere acquaintances. And then there are those with whom I have lost touch, re-connected, and either lost touch with again or formed steadfast connections. Funny how that works sometimes. Which brings me back to the initial "people are funny" statement. You think you know who will be the one person standing next to you through anything, and I mean ANYTHING in life: highs and lows, celebrating and grieving, moments of glory and shame, times of clarity and uncertainty. The one who will accept you for just what you are, no matter what. The one you could call in the middle of the night and know he/she will answer without irritability. That person who will take your hand when no one else will. But you never really know until your friends, family, acquaintances, co-workers, neighbors...you get the point...are put to the test. Because people are funny like that. And they will surprise you.

I speak about people in general, but more specifically, about people in my life in relation to being a parent of a child with a UCD. This encompasses those I knew before, and met as a result of, Coulby's birth and diagnosis of Citrullinemia. I can tell you that in the days of hell following Coulby's initial hospitalization at Johns Hopkins, I was surprised over and over again. Surprised by the outpouring of support; phone calls, cards, visitors, meals. Just the genuine care and concern that people had. But mostly surprised by who reached out. There were those that I knew, and always had known, would be there during tough times. But it was the people whom I would have never expected to be there that blew me away.

Human nature is to set ourselves up for disappointment. To put expectations on ourselves or someone else that are unrealistic or unattainable. In doing so, I was disappointed by several people in my life who were not there during such a scary, traumatic, stressful and helpless point in my life. But it is also human nature to judge, unfairly or otherwise. Judging sets us up for disappointment as well, but in ourselves and not others. This brings back memories of a woman I used to work with. She was gruff and seasoned in her job, which made her a bit of a know-it-all. Well, in my opinion she was a know-it-all, which is a judgment in and of itself. I was young and she always made sure I knew that she was far older and therefore wiser than myself. It was annoying. At that time in my life, I was almost always the youngest in the office, and I heard about it: "When I was your age (fill in the blank)," "I'm old enough to be your mother," "That was long before your time, I'm sure." Anyway, this woman was not my favorite person. But do you know who was one of the first people to call when Coulby was hospitalized? Yup...that annoying, know-it-all co-worker of mine! I NEVER would have expected her to call. I mean NEVER. It was shocking. And humbling. So much so that I remember it to this day. I will remember it always.

In this world of technology, places like Facebook can link us to all of the people we have ever met, or even come into contact with, in our lives. I have met some phenomenal people online. Other UCD families who share a story similar to my own and know exactly what it means to have those people you know you can count on. When these disorders threaten our children and life gets scary and crazy and often seems unfair, they are the ones who help us through it. I suppose that really goes for every facet of life, but especially a UCD life.

My hope for my children is that they have an abundance of people, in addition to myself, who will stand beside them through anything. I hope they form those steadfast friendships young and grow to appreciate the value of such. I hope that they, too, can be that to others. When I look beside me, I know who I will see. Do you? The people you expect to be there might disappoint you. Don't discredit the know-it-all co-worker. Expect the unexpected, because people are funny...

It ain't easy being cheesy!...OR...Say cheese!...OR...The adventures of Coulby and the twisted cheese curls!

Cheese curls are not my thing. I am more of a Cheetos girl. But that does not matter when Coulby is in a cheese curls phase. 'Cheese curls phase' = Coulby WILLINGLY eating excessive amounts of cheese curls. And this means that he is getting not only protein to easily help meet his daily protein requirements, but also all of the calories that cheese curls have to offer...which are a lot. Coulby liking cheese curls makes life a lot easier because we have a sure thing to feed him that makes the job of meeting his dietary needs easier. MUCH easier, truth be told.



And these are no ordinary cheese curls. They are TWISTED cheese curls. These spiral things that look cool and take up a lot of room in the bag to make it look full even though it is not (one of the most annoying things about any bag of chips, really). 28g of cheese curls = 1.8g of protein and 160 calories. Coulby can eat about 75g or so in one sitting...about 429 calories! *Gasp* Guess most parents would be horrified that their child was consuming that many calories from cheese curls. But not parents of children with UCDs. The more calories the better. Kind of a foreign concept in this world of over-consumption and gluttony.



Not only does Coulby love cheese curls...twisted...but he makes sure he gets every last little bit of cheesy coating at the bottom of the bag. Kinda gross, but whatever works. Hence the above photo. That was Coulby post-empty-cheese-curl-bag. He had this cheesy beard and mustache going on that, while disgusting, was also very photo-worthy. I did not capture it well enough to do it justice, but the cheese was coated on his face, fingers and body pretty good. I could not be happier that there is something that I KNOW he will eat if nothing else. Until he gets tired of the twisted cheese curls, they will stock the pantry shelves.



On another note, Coulby had a Hopkins appointment yesterday. And his ammonia was...(drum roll)...29!! Yay! A number we can work with for sure! He has gained weight (thanks to twisted cheese curls, no doubt) and had an overall good check-up. Good news is always nice to report. A relief. A reminder of Coulby's miraculous life.

My life turned upside down...sideways...inside out

April 29, 2010: If I allow myself, I will live the moment all over again. A moment in time when such a simple thing as a phone call sent me hurdling into a world I never knew existed within the world. A world in which an enzyme slacking on its job can upset a seemingly simple internal human cycle. A UCD world. Six years ago today I crossed the threshold, only looking back to grieve the loss of the world I knew. Being honest with myself, I still do sometimes. Think about what could have been. And then I look at Coulby. Having been diagnosed with Citrullinemia at 4 days fresh, he has never known any other existence. And he smiles. Laughs. Plays. Pretends. He is happy. Content with the life he has been given. Citrullinemia and all.

*May 5, 2010: I started this blog on the 6 year anniversary of Coulby's diagnosis and could not finish it. Not because my emotions overcame me. Not because I found it difficult to talk about how my life was turned inside out by Citrullinemia. But because it is truly a task to convey what it feels like to go from having a seemingly "normal" newborn to one who is diagnosed with a potentially fatal disorder. Those who have never experienced this just have no idea how it feels, to no fault of their own. My hope was to recap that day in such a way that everyone, UCD families or not, could experience what I felt. And I just do not know if I can do that. Taking a break from the post does not seem to have sparked any creativity.

I remember a lot about April 29, 2004, but I can honestly say that certain details are blurred. I am not sure if that is my way of protecting myself from feeling the despair all over again, but my brain was overloaded that day with words not even remotely close to those existing in my vocabulary. Coulby was so tiny and perfect. So innocent. And he was a pincushion. 4-days-old with an IV in his tiny veins. I did not know if he would make it through the night. I did not know if he had any chance at a normal future. I did not know if he had suffered any brain damage. Everything that I ever thought I knew or had any control over ceased to exist that day. And it has never, ever been the same since.

Coulby has grown into the most amazing little man. He has fought his battles and overcome all of them with grace and a smile. He goes to public school (something I was not sure he would ever do), he has gotten through illnesses at home, he can eat anything as long as it is in moderation, he laughs, he plays, he talks (a lot...and loudly), he walks, he writes his own name, he spells small words, he comprehends...all things that a 6-year-old little boy should do. He has come a long way from the screaming 4-day-old baby hooked up to an IV and wires. And I have come a long way from the new mom whose world seemed to rip apart at the seams in an instant. While this is not necessarily the life I would have chosen for my baby boy, it is the life he has been given; I have been given. And I draw from his strength to keep me going during the best of times, and during the worst of times. Coulby is my hero. My little miracle. My inspiration.

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!

Getting into the Groove...

...literally and figuratively speaking! My blog has kind of fallen by the wayside lately. Giving in to being lazy and putting it off are the only excuses I have for my lag between posts. But here I am! Back again! And really, I do not have anything new and exciting to report...which is always good in our UCD life. I like it boring. I like it monotonous. I like saying, "Nothing," when someone asks what is new. It means Coulby is healthy, stable and, most importantly, home.

Coulby is having a great Winter (knock on wood!). While he has brought home colds and nasty viruses accompanied by fever, and one VERY persistent virus that he so graciously passed on to all of us, he has been metabolically stable. *Celebrate!*

Coulby's last several Hopkins appointments have gone well and yielded nice, low ammonias and fairly stable amino acids. A few minor adjustments to his diet and medication and he has been good to go. We have been managing to get all required protein and calories into him each day without too much of a struggle. Because we increased the one medication a little bit, Coulby can really taste more medication than formula now, and lets us know it. He will very firmly tell us, "I hate my formula because it tastes nasty!" It makes me feel badly because I know how awful it is, and I do not want to give him something so gross, but he has to have it. There is nothing I can do but tell him that I am sorry he has to drink it, but it keeps him healthy and home. Coulby seems to get that.

Coulby is doing well in Kindergarten, although he has a VERY short attention span and LOTS of energy. It makes it difficult for him to complete every task and stay focused. He loves going to school though. He is disappointed on the weekends when he asks if it is a school day and I say no. I get the, "Ohhhh, nuts!" A Coulbyism. I like that he is excited about school...how long will that excitement last? I do not think he will be getting on the bus to go to high school so happily and willingly.

So things here have been fairly quiet...not literally. Two kids can make a lot more noise than I ever imagined. Especially a brother and sister quarrelling! We have more and more of that in our house. I feel like I should be wearing black and white with a whistle around my neck! But when Coulby and Caroline play well together, it is so much fun to watch and hear. Their make-believe worlds sound like a great escape from reality.

Now that I am back in the world of blogging, I am going to make it a point to post more regularly. (Do you hear that, Mindy?) My main goal behind establishing my blog was to educate those unfamiliar with UCDs. To raise an awareness by telling Coulby's story. And I still believe in that. So look for more posts soon. Until then...

SAFETY/RISK

What does it mean to serve and protect? Do any of us really know who have not taken that solemn oath to do so? Other than the families they leave behind every time they walk out the door, only to breath again when they return?

A lifeline for my little man, and so many others like him who live their lives with a UCD. The difference between life and death, health and illness, safety and risk. Take the time to learn about these disorders, if for no other reason but to become aware of something you never even knew was around you.

What does it take to walk the walk? To fill those shoes? Those are some mighty fine feet, my friends, and some shoes that have seen some action!

The latest photo assignment: SAFETY/RISK. I know, kind of a tough one, huh? But it is funny, because the more I thought about it, the more I realized that we live our lives each and every day with both safety and risk. Each and every one of us. It is just that some live with more risk than others, whether because of lifestyle choices, careers or chronic disorders, to name a few. And the chronic disorders and career examples affect my life every day, so they seemed the most obvious choices to use to complete this photo assignment. I did not have to wrack my brain to come up with something to photograph after all...I only needed to represent what I live with every day.

Where would you have pointed your camera if given this assignment?

Prayers for Corrigan

Corrigan needs your prayers. He is currently hospitalized at JH due to a metabolic episode that presented itself yesterday. Mindy spent much of her day at her local ER as they poked and prodded at Corrigan in order to test ammonia levels. Due to an elevated ammonia on the rise, he was transported via ambulance to Baltimore. There is no saying how long he will be hospitalized. These are the sad realities of this disorder.

Please keep Corrigan, as well as Mindy, Mark and Connor, in your thoughts and prayers. Pray for healing and a short hospital stay. The hospital experience is less than glamorous, and is emotionally and physically exhausting for baby and parents.

Such a sweet little boy, who has been through so much in his short life, Corrigan is one of the brave children living life with a UCD. Every time he goes into the hospital, I cannot help but feel the anguish I feel every time Coulby is hospitalized. And these kids are so brave! I know Corrigan will come out of the whole experience still smiling and making others smile!

I am thinking about you, little angel, and cannot wait until you are back in the comforts of your own home. Mindy, my dear friend, I am praying for you and yours, and you know I am available for whatever you need! God bless you all!