Knowing Brings a New Battle

I learned something new earlier this week. It is something that I think I already knew, deep down somewhere. That inexplicable gut feeling. But, already ‘knowing’ didn’t lessen my reaction.

My DNA testing came back. I am a carrier for OTC. I had a feeling. Things in our family history were a little too coincidental for me. Still, hearing it, having it confirmed by professionals, was a bit like being punched in the stomach. I kind of stopped hearing the world around me. I moved and went through the checkup at Georgetown just like every other time. But I wasn’t really there. It was like some thick blanket of fog had descended.  On Monday, I walked around in a daze feeling just like I did that moment that the doctor came to my hospital room and said “Mrs. Long, we aren’t going to be able to send Matthew home with you right now.” I had just started to convince myself that everything he is going through isn’t my fault. But, here I am again, trying not to go back into that mode of apologizing to him for giving him this disorder. But I did. It’s right there on my X-chromosome. If you unravel it and sequence it you’ll see, right there in the urea cycle, OTC Deficiency, in big flashing neon letters. Caution. Having children is dangerous. Your X is poisonous.

Think back to high school science and those punnet squares. (Weren’t they fun? I really did love genetics.) Here’s what we have: A child gets one sex chromosome from each parent. Dad gives an x or a y. Mom only gives out x’s. One of my X’s carries OTC. So, My daughters could have the ‘good’ X or they, too, could be carriers. There is a 50/50 chance of any future son having OTC. Or, a 25% chance of a future child being a son with OTC.

Which brings us to a post that I was saving for a later date. Will there be any future children? It’s the question that everyone asks immediately after the birth of a child “Are you done now?” Well, here is the answer. Rusty and I have always gone with ‘let’s take it one at a time’. We have a child we love them and watch them grow and then (so far) at around a year or so of age we decide “hey, this isn’t so bad. Let’s do it again.” and decide to add one more to our family. Even after Matthew’s birth, I hadn’t totally ruled out the possibility of more children. Not knowing what his needs are going to be, we certainly haven’t planned to have more, but it wasn’t out of the realm of possibility. However, knowing that this wasn’t some chance mutation, I find myself wondering if we are ‘finally done’ having kids. Knowing that I’m a carrier means that a baby can be tested in the womb for OTC and we can start him immediately on proper formula and medication to prevent any brain injury. But will that child also need a liver transplant? Is it at all responsible of us to continue to have children knowing that one could need that type of drastic intervention to preserve its life? Is it silly to let the news change our plans? It’s not impossible for us to have a healthy son or daughter, after all.

There are so many things to consider now. And, maybe I shouldn’t be considering anything. I am certainly not at the point where I want another child. But, I am the crazy lady who truly enjoys being pregnant. And I love those sleepless nights of cuddling and nursing my baby. It’s weird to think that that might be behind me. But then again, as so many strangers like to tell me, I do “have my hands full” already. Erynn has told us over and over again (since before Matthew was born) that we were going to have one more baby ‘because Matthew needs a friend’. Maybe she knows something that we don’t know.

I need to sit back and relax. I need to enjoy what God has blessed me with and be content with what His plans are for the future. I certainly don’t know what He has planned. He may have 3 healthy boys in my future (although, I hope not!).

“Therefore do not worry about tomorrow, for tomorrow will worry about itself. Each day has enough trouble of its own.” Matthew 6:34



I’ve been in a dark place lately. I have been focused on all of the bad and haven’t been able to see any of the good. Seeing the good in things takes effort and I’ve been too tired for that. I’ve been trying to potty train Ruth, who has been more than a little bit defiant in the matter. Matthew has been spitting up after every feeding and crying in his sleep and up all hours of the night spitting up. It feels like I am constantly either changing a diaper, cleaning up a potty miss from the floor or mopping up spit up. Which leads to Erynn. The poor kid isn’t getting any positive attention because I’m so busy cleaning and correcting and consoling, so she’s acting out too.  I am too tired to keep busy and keep my mind focused on other things. So, lately, I’m only seeing the bad.

I’m only seeing that Matthew will no longer take a bottle. That he isn’t holding up his head properly. Or rolling over. That his arms are too stiff. That he doesn’t fully ever let his fingers out of a fist. He isn’t interested in toys. He doesn’t grasp them. He doesn’t put his hands in his mouth or grab his toes or play with toys. He isn’t making normal baby gibberish noises. He isn’t starting to talk. That he may never talk. That he may never be able to hold a pencil and write. I’m only seeing the impossible situation of :Things would be so much easier if I could just nurse my son. Instead of pushing medicine down his throat and then fighting with him to take a bottle and then pushing the bottle down his throat and then trying to find a time to pump breast milk (he’s allowed a tiny bit with his formulas). And somewhere in there I am supposed to eat something and feed the girls. By the time that is all done it is almost 12 and I’ve been at it since 6 and I’m still in my pj’s. Tired. So very tired. And so very focused on the wrong things.

Yesterday, I was scanning through one of the urea cycle pages that I follow, when my eye caught a post. It went something like this: “My son is 4 and isn’t talking yet. Anyone else having this issue?” Following that post was about 25 or 30 comments of “Yep, my son didn’t walk until he was 3” or “My son is 7 and he is just starting to put 2 words together” or “My daughter walked at 2 and is starting to talk at 5” I read every single one of them. And my heart sank lower and lower with each comment. I kept looking over at him and telling myself, “You’re right, Julie. He’s not going to talk. How are we going to get by if he can’t talk? I’m going to have to learn sign language and that didn’t go very well the last time I tried.” All morning and into the afternoon my mind was filled with thoughts of ‘he’s not going to talk until he’s 7!’ and “my poor baby! Why him? Why did this happen to him? Maybe we made the wrong decision.”

But then I was washing dishes and it hit me. (The kitchen sink seems to be the new place where God speaks to me) I was looking at it all wrong. I had just seen 25 instances where a parent was told that there was no hope. 25 instances  where a parent didn’t give up. 25 instances where a child’s brain had healed in some manner, on its own time. 25 children who were doing something that the doctors didn’t think that they would ever do. And do you know what? Matthew is going to be number 26. He is already doing so many things that we never expected him to be able to do. And he, too will heal. Not on my time, but in God’s time. We used to sing a song at church that said “Lord I believe. Help my unbelief.” It never made sense to me. How can you believe and not believe? But now I’m living it. Believing with all of my soul in something that my mind can’t understand.

When I think that I just can’t take any more. God gives me a little rainbow, a tiny reminder to hold on to His promises. (He’s also given me an amazing group of women that I can open up to and whose prayers remind me to focus upwards and encourage me to carry on)The little light bulb moment at the sink was one of them. Matthew’s spitting up gave me another.

His spit up problems have gotten worse and worse. Eating results in an hour of him being upset and spitting up and, more and more often, it has also resulted in his tube being pushed out. Talk of a G-tube resurfaced. I don’t know why this is so upsetting for me. I know that it may actually help him to take a bottle better and to improve in ability to sit up and tolerate tummy time, but I just don’t want to do that to him too. It’s irrational. I can’t explain it. I just want the tube to go away. So two days ago, when it came out AGAIN, I left it out. I thought “i’m going to make this kid take a bottle.” I tried and I tried. And I cried and I cried. And I begged and I begged. But, I barely got 10 ml into him by mouth. During out battle, a new NG arrived in the mail. Rusty put it in. We put Matthew to sleep and started his food. He slept all night without spitting up and have very few and very small spit up incidents the next day. Did I get my miraculous “look he’s taking everything by mouth and doesn’t need any tubes” event? Nope. But God gave me a nights rest. And He comforted Matthew. The spitting up is considerably less than it was 2 days ago and the need for a G-tube seems less urgent.

I’ll take it! From this storm cloud that I’ve been living in, those things are beautiful rainbows.



Status 1B

If you watch a crime drama long enough, you’ll see the episode where someones daughter can’t wait any longer for her father to get his heart transplant, so she starts killing off the 4 people ahead of him. Well, that’s not exactly how it works. The transplant list is more like transplant tiers. One tier is higher than another and the patients within that tier are somehow ranked. But, I don’t know what number Matthew is. His transplant team doesn’t even know what number he is. They don’t even know how many other 1B people he is in line with. It is all done through a special group that keeps things anonymous… you know.. to avoid some crazed parent killing off other sick people to save their child.



So. Status 1B. This is where Matthew is currently at. He was just ‘upgraded’ to this status a few days ago. If we were thinking about the transplant list as a pyramid, it would be right up there at the tippy top. The only tier higher than this is the point of the pyramid, Status 1A. 1A is where Matthew was going to be listed back when he was in the PICU; before he decided to stop tricking everyone and to miraculously stabilize. 1A is emergency transplant. Matthew is just below emergency status. I don’t believe that he will move to 1A unless he stops being able to maintain his levels or something else dangerous happens. Recently, Georgetown transplanted livers for 3 urea cycle children. They averaged being listed for 3-4 months before their livers came available. As our team has said, “There just aren’t that many infant livers out there.” We are waiting on an infant liver of his blood type, with matching arterial branches and I don’t know what else.. but it’s a big list. There is so much more to it than simply having the same blood type.


And so, we wait. Our ‘go bag’ is packed with everything except pants and shirts. The girls suitcase is sitting out next to their dresser. There is a game plan and a call list for when it is time to go. Phone lists and pack lists and a ‘the girls love this and hate this and need this for bed time” list is already made. All we need now is a call. One short phone call that changes everything. One phone call that I am scared to death of and anxious for all at the same time.



One of my “liver Mama” friends shared some information with me. The organization that lists transplant patients shares some information in anonymous form. It would appear that there are currently 3 children awaiting liver transplants in our region at Status 1B. Matthew is the only one under age 1. He is also the only one of his blood type. It doesn’t give us any idea of time frame, but it is still interesting to know.