Happy to be HOME!

Coulby is home! He was discharged from Hopkins yesterday and is happy to be home and reunited with his trains. The discharge orders were to make sure Coulby drinks lots and lots of fluids, and gets as many calories as we can get into him. And of course, he must drink his formula. So far, he has done well drinking his formula, with some resistance, but has been stubborn with eating anything. I do not think it is related to his illness at all--it seems to be driven more by his stubborn streak and need for independence. Coulby wants to be in control and make his own decisions, rather than having someone always telling him what he has to do. Has to eat. Has to drink. Has to get his medications. You get the point. I know this is typical 4-year-old behavior, but I also attribute it to the fact that Coulby has had no control over much in his short life. From the moment of his Citrullinemia diagnosis, most of his decisions have been made for him. Now he gets the chance to control some of that. Frustrating now, but something that will definitely help in his future.

Last night Coulby was reluctant to sleep alone, so he took over my place in my bed and slept with daddy. I slept in the guest bedroom in our basement, but do not plan to make a regular habit of doing so. We just figured that Coulby had a big last two days and needed a little extra TLC upon coming home. He slept through the night (minus the multiple times getting up to go pee), and most importantly, was fever free! I am not sure if I had mentioned earlier that the docs did say that Coulby has an ear infection that might have been contributing to his high fever. He also was/is fighting a virus that has been seen in the hospital and is characteristically accompanied by a fever for 4-5 days in most children affected. Anyone else noticing the potency of the viruses going around this sick season? Nasty bugs that seem to hold on forever! (no, my congestion has not dissipated yet either--it has been over a week now!)

This morning Coulby woke in good spirits with his usual endless supply of energy. I am sure not having a fever has helped in that department. You would never know Coulby just spent time at Hopkins, hooked up to an IV with a temperature alternating between normal and 103! A miracle, my boy! That is all I can say. And while it has not been fun to have Coulby sick with multiple colds and other bugs this winter, I am so amazed at how well he has weathered each one. God is good!

Thank you all for your prayers and continued support through our journey with Citrullinemia. You help us to stay strong when things get tough! Please continue to keep us in your thoughts as we work to get Coulby back on track and eating like a champ again.

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!

News from Hopkins...

Coulby will be spending the night at the hospital. I knew he was not feeling well from the second that he first woke up this morning just by looking at his face. On the way out the door to head to Hopkins, he was in decent spirits despite a temp. of 101. Coulby was still chatty and walking around and being his cute little self. But the fact that he had a fever again, after just coming off of an illness, bothered me enough to think he needed to have blood drawn and his ammonia checked.

Coulby arrived at the hospital with daddy, waited around for someone to finally draw his blood, and then had to wait some more to get the ammonia results. (Same old Hopkins routine every time.) In the meantime, his temp. had reached 103, so the Motrin clearly was not doing the trick to bring the fever down. They gave him some Tylenol (which, by the way, we NEVER give him because of the potential liver damage it can cause...he does not need anything else going on with his liver!), and that brought his temp. back down to normal after a little while.

While waiting for results of Coulby's ammonia, my nerves started to get the best of me, so when the phone rang and it was my husband calling, my heart started pounding a mile a minute! His ammonia: a grand 45!! I was relieved, to say the least. The doctors were going to let Coulby come home after about 6 hours of being hooked up to IV fluids (to hydrate him and get some calories into his body), and as long as he could eat something and drink his medicated formula. He downed a bag of Fritos, drank his drink, and then proceeded to throw up! A set back for sure.

I know that Coulby had been complaining about his stomach hurting since this morning, although he did not seem sick to his stomach at all. But he is the kind of kid who does not want to eat a thing when he is really not feeling well, and I think the Fritos and formula were just a little too much for his tummy. It was too late, though, because the docs agreed that Coulby should stay for at least the next 24 hours to continue to receive fluids and calories. They worried that we would not be able to get anything into him if he came home. It was a bummer for us, but I know it is what Coulby needs right now.

I will stay home with Caroline tonight while Coulby has his daddy by his side for comfort, and then (Heaven forbid) if he is there for another night, we will switch. It is always a balancing act. And these times can be very stressful, so please say some prayers for everyone, but especially Coulby.

I will be spending my night in a state of partial sleep, waiting for the Hopkins updates from my hubby. And I will be kneeling and praying for my brave boy. The house is definitely way too quiet without him!

Where is Coulby?

My house is quiet this morning. There is no shrieking. No whining. No train whistles or rhythmic "chug, chugs." Caroline is not crying because her big brother is not tormenting her. Because he is not here. He is at Hopkins as I am typing, in the ER, having his blood drawn. And probably getting an IV for hydration and calories.

As you know from previous posts, Coulby was sick last week. He was pretty miserable--stuffed up, fever, loss of appetite, and lack of sleep. We pumped him full of fluids, made sure he did drink his formula to get his meds., and treated him with Motrin to keep his fever down. Coulby made it through the illness, after passing it on to the rest of the family, and seemed to be getting better each day. We kept him out of school all week last week, and by Monday this week, he seemed to be okay to go back. Coulby absolutely loves school, so he was thrilled. And I was thrilled that he was feeling so much better. Asking for food, drinking willingly, and meeting full protein requirements again. I always feel like I can breathe better when Coulby is well.

Then he woke up this morning whimpering and whining. He whines quite often anyway, but something about this whine told me that he was not feeling well again. I felt his forehead, and it seemed like he had a fever again. Out of nowhere! One day he is on the mend, the next day he feels sick and has a fever again! How frustrating! I took his temperature, and sure enough, he had a fever of 100.2. I took it again, thinking maybe I had not gotten it under his arm very well, and I got the same read. I swear I just deflated right there and then.

Daddy seems to be the man of the hour when Coulby is not feeling well, and he managed to get Coulby to settle down a bit and stop crying. He put him in a nice warm shower, which seemed to rejuvenate Coulby, and he even looked better. I paged Coulby's treating geneticist, told her what was happening when she returned my page, and we both agreed that Coulby should be seen, especially with just "getting over" an illness and now having a fever AGAIN! Coulby and daddy headed out to Hopkins. When Coulby left the house, he was bright-eyed and chatty, so I felt good about that. But his head was warm, so I knew he still had a fever. I had hoped the Motrin would have kicked in by then.

So that is where we are now. Coulby just had his blood drawn about 20 minutes ago, and was a champ (as usual!). I am praying that even if Coulby is still sick, or getting sick again, that he will be able to come home and we can help him get well from here. Please say some prayers for Coulby. I will post more as I know more!

On the mend...

Well, Coulby is doing much better now! He got through yet another illness here at home, to which I say, "YAY!!!!!" This one was a nasty cold, with a cough, major congestion, and a fever. Coulby was pretty miserable for the first few days, but seems to be getting back to normal now. The fact that he is playing with trains again is a sure sign that he is feeling better. Coulby is ALL about trains!

Shortly after Coulby got sick, so did Caroline. Same thing, but she managed it better than her big bro. She still has a cough and snotty nose, but is fine. Both my husband and I ended up with it, too. I cannot even breath out of my nose right now and need to blow my nose every second of the day. It is more annoying than anything! And I cannot tell you how many boxes of Kleenex we have emptied in the last week.

During all of this, I went away for a much needed weekend away to scrapbook! I felt a little reluctant to leave with Coulby just getting over an illness and Caroline starting with it, but I was assured everything would be under control at home without me. And it was. I checked in about ten million times, but everyone got through the weekend without a hitch. And I finally got myself caught up on some of my scrapbooking. I will post some of my completed pages in the next few days.

Thank you all for praying for Coulby and thinking of us while he was sick. It means a lot to have such support during difficult times.

I have posted the latest photo assignment below...BEFORE and AFTER. Mindy and I decided to use new or old photos and use software to bring them to their full potential...or try to! With Coulby being sick last week, and then going away for the weekend, I used old photos, but I hope you enjoy looking at them just the same!

BEFORE and AFTER

Sweet little Maggie living the life of ease! I caught her mid-yawn, and absolutely LOVE this photo. My favorite part, aside from the great yawn, is her whiskers sticking out every which way!

Our Japanese Maple is one of my favorite trees that we have planted around our home. I love the vibrant color of its leaves and its elegance. I was taking some photos after the rain, and happened to capture this drop hanging on to the maple leaf. I think it could be a little more focused, but I still like it!

This was another photo for Christmas gift tags. It is a rare photo of the four of us...where we all look halfway decent, that is. I liked the surrounding image, but really wanted most of the focus to be on us.

Last Christmas I wanted to make photo gift tags. I liked the look of this winter wreath that I had made, and zoomed in on it. I obviously had to brighten it up.

I took this great photo of Caroline...just being herself...and wanted to play around with the image. I really liked keeping the red hat! It seemed to really play up my drama queen!

Sick...again!

"Now make sure you keep your hands really clean, do not get near sick friends, wash your hands after playing in centers, and remember to use lots and lots of Purell." Coulby has heard me say this so many times that he has actually started reciting parts of it to me right before he gets on to the school bus to enter the germy world of public schools. In some part of my brain, I foolishly believed that this daily recitation would prevent Coulby from participating in the 'pass-the-virus-on' preschool game, from which he has brought home colds and other assorted illnesses. I suppose Coulby would have to live in a bubble in order to avoid ever getting sick, which we all know is not even an option.

What is my point? Coulby is sick...yet again! He was just sick about a month ago with some nasty stomach virus, which was hard on him, stressful on us, but still managed at home. In the last few days, Coulby has gone from having a runny nose, to being stuffed up, to a nasty cough, fever, and loss of appetite. Dealing with the cold symptoms: piece of cake. Dealing with the fever and loss of appetite: well, that on top of his Citrullinemia is extremely stressful and exhausting. For all of us.

Coulby's body can react to a fever by drawing stores from his body, which can cause excess protein in his system and possible hyperammonemia. We have to treat the fever with round-the-clock Motrin, and also make sure his protein intake is less than his expected daily intake. We also have to make sure that Coulby is drinking plenty of fluids, preferably those with calories. This will ensure ideal hydration and flushing of his system. By keeping caloric intake up, Coulby's body will be less likely to draw from its own protein stores to replenish his system and make him get better.

With all of this said, you can see why we dread Coulby being sick. There is the constant care to make sure he is getting what he needs to not only get better, but also to maintain his metabolic stability. Fear of hospitalization triggers stress. When he does not want to eat, he is not getting what he needs to prevent hospitalization. When he does not drink, he is not keeping himself hydrated and can end up becoming hyperammonemic. The average cold turns into a nightmare.

I pray that we can get Coulby through this cold here at home, and we are doing everything in our power to do just that. Please pray for him. For an appetite. For no more fever. For healing.

Valentine's Day photos

The heart of...

Happy Valentine's Day...well, a day late! Valentine's day: the most estrogen laden holiday of the year. Hearts. Chocolates. Flowers. Jewelry. Cards professing undying love. Um, I do not know how many of you out there get these things each year on the big V-day, but I do not. Not because my husband does not love me. He shows me how much he loves me each and every day. By bringing me a coffee right when I need one. Bringing home a calorie loaded breakfast of Dunkin Donuts. (Yum!) Indulging me. Putting up with my swinging moods and spoiled tendencies and restless nature. Plus, he covered himself for quite a few Valentine's Days when he proposed to me years ago on...big shocker here...February 14!

My memories of Valentine's Days past are good ones. I remember the excitement of picking out cards to hand out at school, and all of the goodies we got to indulge in during classroom parties. And that is still the best part of Valentine's Day--having an excuse to bake all of those fattening cakes and cupcakes and assorted desserts. Oh, and the chocolate! It was always just a day that meant fun. My mom always made sure my brother and I had something at our places at the kitchen table, and she would often make sausage, egg and cheese muffins for breakfast, with the eggs cut out in the shape of hearts. Cutesy, I know, but it was always a great way to start the day. It lead right into the fun at school.

Now it is my turn to give my kids that same excitement every February 14. Coulby had his first school Valentine's Day party last Friday, and I made sure I was there to experience his excitement. The kids all decorated paper lunch bags with self-adhesive foam hearts, heart cut-outs, and crayons. Then they each got to put their Valentine's in each other's bags. You could just feel the childhood excitement of it all emanating off of them. Coulby has carried his bag around the house with him, examining each card over and over again, as if each is a prize he cannot believe he won.

So when I think of Valentine's Day, I admit to thinking about all of the frou-frou gifts we women so love receiving, but I also think of love--the love I have for my husband, and the two loves of my life, my children. They are my true Valentine's!

In honor of February, specifically Valentine's Day, Mindy and I have tackled a photo assignment: The heart of... Above are my interpretations of the assignment. Look for future photo assignments! Happy Valentine's Day everyone!

WELCOME HOME, Corrigan!!

Welcome home, brave little man! After a LONG and rocky hospital stay, enjoy the comforts of your home, surrounded by your family! Breath in the fresh air, and enjoy the freedom of going wireless! Although you did not emerge unscathed, your spirit is unbroken. Sending you hugs, prayers, and many blessings!

Old Man Winter...go away!

There is so much more to living with a Urea Cycle Disorder than originally meets the eye. Sure, there is everything I have mentioned to this point, but there are several "little" things that, in relation to UCD's, can be catastrophic to the metabolic balance. Some people love winter, some could skip right over it, and I am somewhere in between. I love the beauty of the season, and the many wonderful photo opportunities it provides, but I can live without the cold...and the GERMS!

We have always tried to limit Coulby's exposure to germs that can wreak havoc on his body and land him in the hospital, but germs are everywhere! At the grocery store. At home. In the classroom. Everywhere. I go out in public and when I hear someone coughing, I hold my breath and quickly steer Coulby in the opposite direction. People sneeze and I cringe. Conversations not meant for me grab my attention when there is any mention of "flu," "stomach virus," or "Oh, I am just getting over (fill in the blank)." And when someone in the family is sick, they know not to come anywhere near our house. They will be left out in the cold! We just cannot afford to risk Coulby getting sick. It could end in a hospitalization. And you bet we all get our flu shot every year!

I guess most people do not understand, but anyone living with a UCD knows that illness can cause excess build up of protein in the body, which, when maintaining a balance of a low protein, high calorie diet, can devastate that balance and lead to hyperammonemia. So we dodge the germs, or try to, and routinely use the many varieties of hand sanitizers on the market these days. And anti-bacterial cleaning sprays, wipes, and otherwise. You can say we are germophobic. I am a big fan of the new Germ-X foaming hand sanitizer. And Clorox Anywhere Spray. And Clorox wipes (they are great for wiping down public eating surfaces and germ-infested grocery carts).

So I love the beauty of winter, but it forces everyone to stay indoors and spread the germs around. You can imagine, then, how anxious we were when Coulby started Pre-K last fall. And he has had more than his share of illness since he started school, but we have been blessed to be able to manage each one from home. Not that it has been easy, especially during his most recent illness, a stomach virus, that stole his appetite for over a week and lead to one trip to Hopkins ER (to check his ammonia, which turned out to be fine) and many phone calls to his nutritionist and geneticist.

I read the disappointing news that Punxsutawney Phil saw his shadow today, so there are six more weeks of winter in our future. Ugh! I, for one, will be anxiously awaiting spring and the fresh air that comes with it. The circulated air. The not-so-germ-infested-air!