Happy Birthday!

I have to say I have never been so thankful for a birthday!  Braylon turned one year old on August 13th, and I cried!  This last year has given a whole new meaning, for me at least, to what it means to have a birthday!  Usually we celebrate being another year older, which is still true in Braylon’s case, however I felt as if we were celebrating living.  When the doctors told us he may not live or we didn’t know what kind of life he would have, it was devastating; and now here we are, a year later, living well; and not just that, thriving while living.  What a difference a year can make.  Braylon has been in for his routine check-ups with, knock on wood, a flawless record, he has had zero hospital stays and started walking, before his sister did, at one year old!  What a miracle!  I look back to the awful day of his diagnosis and can’t help but still feel a little overwhelmed on how we manage ever day.  I then reflect on all the milestones we have been blessed with.  Just an update,  since I haven’t been on since December, and its September, yikes; Braylon had his six month neuro psych test at six months old and came out of the gates with the intelligence of a ten month old!… again I cried.  What a wonderful time to celebrate!  Of course, we new he was doing great, and that he was “normal” but to hear it out loud and think of what could have been or the unknown from the early days when we were first at the U… I became emotional thinking my baby is as smart as a ten month old!  LOL.  I almost felt like “see I knew my baby would be okay”  however I am grounded by the fact that I know that there is no end in sight, this is a real disease, that we have to never let our gaurd down with, and  never stop fighting.  After the neuro psych test, Braylon has done great, he started to crawl, he feeds himself with a hardy appetite, he is up to 8 grams of protein/day.  He still drinks three bottles of cyclinex and enfamil mixed per day as well.  He makes an annoying “dumb and dumber” movie noise, the one that goes “eehhhhhhh” when he wants something, he laughs, he smiles, he cries when you tell him no, he’s learning and playing and growing everyday.  I can’t, unfortunately, say that everyone is as lucky as us, we are so blessed to have come this far, may our experience, if anything, give people hope that in the face of adversity anything is possible.  Out of all of his challenges, we have found opportunity to live, to grow, to have perspective and to truly celebrate life.  Happy Birthday Braylon!  We love you!

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December update

I haven’t  made the time to be on here lately so I thought I would finely get on and type an update.  First, I should say thank you to the out pouring of support we have had for Braylon.  I am glad to report he is doing well!  I always wondering what that means, especially kids with this disorder, when people say they are doing well?  For us it means, he is completely normal.  He is the all smiles, all the time, he is using his hands to grasp toys and pull them to his mouth and he is trying to roll over, bless his little heart; in short he is acting like a four-month old.  Every time he smiles, I sometimes stop and think, that there was a time that it was made to seem that we may never be able to see that smile or hear that laugh and it takes me to a grateful place, that only someone who has come close to losing their child, I imagine, can go to.  But I think how wonderful it is to see him smile to see him thriving.  Then I see my daughter run by with no diaper on screaming, stop to lick the window and then crawl across the floor grunting, and I look back at Braylon and think, you were suppose to be the one with problems 😉  So life is good, knock on wood, we have had no ammonia crisis’ since the diagnosis and only one cold.  Who would have thought the common cold could evoke a totally body numbing experience.  I remember the moment where he was sniffling and it dawned on me that he had his first cold, and my whole body went numb and I felt the blood rush out of my face and all I could do was sit there paralyzed by the thought that, this is it, he’s sick, he’s going to get  a fever, we are going to end up in the hospital with high ammonia…I mean this is what the doctors had said might happen, they prepared us.  So I sat there on the edge of my seat, waiting, feeling for a fever, evaluating his every move, every breath…and then nothing.  Just a cold, and deep breath exhale with relief.  He made it through, a cold…sounds silly but that is our life now.  And so it goes, sometimes, I think back to those awful, gut wrenching, tear soaked first days and I am reminded that he is not completely healthy, not completely normal and he never will be, but my every wish remains that he will stay healthy and remain none the wiser to his condition.  It is with that, that we will celebrate his first Christmas and welcome 2011 with open arms.  We are ready for whatever the future holds and thankful for our blessings we have experienced this year.  We wish all of you out there reading this a very Merry Christmas and a Happy New Year, from our family to yours God Bless.

A bruised heart

A poem from a friend from an unknow author, thought I would share:

Hello?

New teacher, or therapist, or doctor? Is that you?

Oh hello…

I just wanted to chat with you a second. To caution you. Or warn you.

Please, tread carefully.

You see, what you might not realize as you look at me, talk to me, tell me your opinions, our options, our lack of options, and your predictions of our outcomes is that; well… you see that heart?

The slightly broken, definitely bruised one?

Yeah, that’s my heart.

My slightly-broken, definitely-bruised heart.

Now, I realize that as you look at me you might see…a confident parent… or an angry parent…or a happy-go-lucky parent…

You might think that I understand everything… or nothing…… or that I have all the experience in the world because I have done this for awhile… or that I know the rules… or that I don’t know the rules and that is for the best….

You might believe… that I am high maintenance… or overreacting… or maybe neurotic… or disengaged and uninterested… or that I don’t really care… or maybe I care too much…

But regardless of what you see, what you think, or what you believe, this is what you should know:

I am broken-hearted. And it doesn’t matter if it is the first day or a century later. It doesn’t matter where in the “grief cycle” I might be. It doesn’t matter if the wounds are healed, or healing, or fresh and new. This heart is bruised. Slightly broken. Different than it once was and will ever be again. And when you speak, or don’t speak, in judgment or not, my heart is out there.

Some of “us” parents… the ‘special’ ones… can be a pain in the ass. I know that. WE know that. But we are fighting a fight we never planned to fight, and it doesn’t end. We don’t get to clock out at the end of the day. We don’t get a vacation from it. We live it, everyday. We are fighting without knowing how to fight it, and we depend so much on you to help us. We have been disappointed, by you or others like you. And we are disappointed in ourselves. We are your harshest critics. We are our own harshest critics too. We are genuinely fearful, and driven, and absolutely devoted. And we also know, we need you. So please, be careful with us. Because as hard and tough as we may look outwardly, our hearts are fragile things.

G-tube

The surgery to place the G-tube  was scheduled for Monday morning it was Friday morning.  That meant the whole weekend in the hospital.  We were in a tiny like 8×9 space I swear.  Now I know I shouldn’t complain about the accommodations, my baby was doing well and I should be thankful, but it was awful to stay there and we couldn’t wait to go home.  I give credit to all the nurses who work with these sick kids.  At night we would hear babies crying, it was awful.  I vowed I would never leave Braylon alone not for one second.  It made me want to vomit thinking of him alone in the hospital room crying or needing something and thinking of not being there…yuck.. some of the kids are alone and it broke my heart being there, another reason home was too far away.   The thought of the tube and freedom from suffering with the meds by mouth kept us going.  In the meantime, an NG tube was placed.  So over the weekend we gave the meds through the tube in his nose, so sad to see him with a tube in his nose, and he had trouble with his bottle at times, probably felt weird like something was in his throat probably because there was, at any rate, way better than giving the meds by mouth, it was the less of two evils I guess.  The G-tube would give him freedom from this as well.  For those who aren’t familiar the g-tube is a tube that is put into the stomach to usually allow feedings.  Not in our case, we had what they call the mickey button put in to allow administration of meds only.  We have to give Braylon meds four times a day..uugghh..at first… but now just a part of our day. At any rate, I told the doctors I need this tube, I was desperate to eliminate his suffering with these meds, especially given the frequent schedule and I told them it will also give us freedom if he gets sick to administer meds and any nutrition if needed and that it would ensure that we would know that he got all the meds, instead of the parts he was spitting out previously..they smiled at me and said that my speech was the one they usually had to give parents to convince them of the g-tube surgery and its benefits.  That is good, I just didn’t want them to say no, I suppose that was the doctor in me talking.  The mom in me was still fearful and feeling helpless, still knowing they had to put my baby under anesthesia to do this and what if he didn’t wake up, did we make the right decision….so many thoughts and nothing but time to sit and think and cry some more but yet be thankful because pretty soon it would all be over and we would be going home!

PICU

The first night in the PICU, pediatric intensive care unit, was actually not that bad, so to speak, we got settled in and we could hold Braylon again.  It was a little tough with all his monitoring equipment and fluid lines, but I couldn’t think of putting him down.  So here I am five days postpartum hormones to boot and this news of Braylon’s disorder, so my eyes swollen shut still couldn’t speak that well.  The nursing staff was so great, they took one look at me and said you should try to get some sleep…yeah right.  but they were right, I was exhausted, not to mention how Braylon must have felt, this morning he was in his comfy swing, waking up to nurse and now he has been poked and prodded all day with tubes every where and every time he sleeps now, time for vitals or time for meds or time for blood draw, the worse one,  so how was I suppose to sleep when he couldn’t.  The worst was that he couldn’t eat, part of the initial treatment for high ammonia is protein restriction, so until we knew if his ammonia was dropping, he couldn’t eat.  I don’t know what was worse hearing him cry for food and not be able to give it to him or all the blood draws??  At any rate, I did lay down and the nurses came in and rocked him and gave him a nuk while I “slept”.  The next morning we got the ammonia results and I believe he dropped to 17, so this was good, he responded to the oral meds, not need for IV drugs, and now we could feed him! Yeah, or not…At this point we had to wait for rounds, where the doctors on the floor, discuss the case, consult with his metabolic doctors and the dietician, to come up with a plan for refeeding, sounds speedy doesn’t it?? Yeah not really, so at this point they did offer him pedilite.  Great for suckling but this stuff shoots out a premie nipple like a fire hose because it is water, so he was drowned in the liquid or suck down 2.5 oz in less than a minute.  Now for the meds, the meds were awful, he was taking buphenyl and arginine.  Buphenyl is the treatment to help scavenger the nitrogen and ammonia from his body and the arginine helps the urea cycle pick up from where his left off or was dysfunctional.  At any rate, the meds are very caustic to take by mouth for an adult, let alone a five-day old.  So he was turn purple and choke and his heart rate would run wild on the monitors and he would scream.  I tasted then and the buphenyl tastes like a salt lick, so for anyone who has every taken down a big swig of ocean water accidentally, not fun, probably what it tastes like.  This was the part that then became most stressful, as we had to give the meds four times daily and I would dread giving his meds and start to hyperventilatemyself and tense up at the thought of giving them.  So there reached a point where he was doing well but I thought I cannot do this four times daily for the rest of my life, there is no way, that is no life for us, let alone him, he hated it.  To make matters worse this is the time in his life where we are trying to build trust with him and here to him we are poisoning him every 6 hours, so he didn’t want his nuk he didn’t want the bottle because everything that hit his lips he thought was going to be the meds…awful…so along came the G-tube….love the g-tube

The Diagnosis

This story does not have an unhappy ending, so it is okay to keep reading, this is rather the story of the start of Braylon’s life and how he became diagnosed and managed with a disorder called Citrullinemia. 

So we arrived at the ER, the drive was a blur, I was numb to any feeling, still not quite sure what to make of the phone call.  Upon arrival they said they were waiting for us and they were waiting for us, the whisked us to the back of the ER and torn off hsi sleeper and his diaper and he immediately started to cry and peed everywhere, he is a boy, there is no controlling that, once the diapers off, game over.  They started poking him and he cried harder, it all happened so fast.  I cried harder.  I thought why so abrupt, again my baby is fine.  The nurses assured me that they were former NICU nurses and could hit a vein they weren’t experimenting on my baby.  They did place a catheter, started him on D-10 fluids, but took about six-seven tries, including his head, yes, his head, to draw blood, then an hour and a half went by and the lab showed up that they had not drawn the right tubes of blood, they needed ammonia levels and they needed more blood for that, so it started over, multiple unsuccessful attempts to draw blood lots of crying.  They finally got the blood but they said that I couldn’t feed him, his disorder required the fluids he was on, an ammonia level and fasting-specifically protein restriction.  I just wanted to nurse him, he was crying and he nursed so well and he was hungry…We had now been in the ER for five hours, my two-year old daughter, Reese, was getting restless, by this point, none of us had eaten, or napped for that matter, in her case, and finally the results.  His ammonia was 132 and that meant he had to stay in the PICU, these values needed to come down, they told us, or they could lead to brain damage or death, more tears…They said they would get his values down by fasting him and starting him orally on two medications that were used to treat this disorder, these meds are given four times daily, and they are lifelong; if he didn’t respond to the meds orally then a line would be placed to give the drugs IV, we were looking at a least five days in the hospital.  We were still trying to process all this at this point, trying to get a hold of my parents, Jeff parents. We had not called anyone at this point because we didn’t know we would  have to stay and did not want to worry anyone.  At this point we were worried and needed some support.  Jeff made all the phone calls, as I still couldn’t speak and we headed up to the PICU, our little baby on a gurney, headed up to start the fight of his life at five days old 😦

Our Story

The story of Braylon’s fight begins on August 18th, 2010.  We started the day like any other day.  I was on maternity leave and my husband was home on leave as well.  We were celebrating the birth of our son, our second child.  He was now five days old.  He was born on August 13th, 2010.  8 lbs, 15 oz, big baby.  Big healthy baby, or so we thought.   It was the morning of the 18th and we were outside talking to a neighbor and decided to come in and get ready for the day as we were going to take our two year old daughter for her first hair cut.  That is when my cell phone rang.  I don’t remember who it was, it is funny when your whole world changes some things are a little blurry.  The call I believe was a Physican from our peditrician’s office, indicating that the MDH (the MN department of health) had contacted them that Braylon had a positive new born screen.  I remember the second night in the hospital, after he was born, they took a drop of blood from his heel, that would be used for the screen, and never gave it a second thought.  The person on the phone said they suspected Citrullinemia.  I had never heard of it, I thought maybe this was good, I listend furthur as she asked me how Bralyon was doing?  I said he’s good, he’s eating, drinking, peeing, pooping, etc.  She asked if he was awake?  I answered, yes, that he was actually looking right at me as I was on the phone.  The woman on the phone, proceeded that she was glad otherwise she would send an ambulance, as Braylon needed to be seen right away, and that the Doctors would be waiting for us at the UMN emergency.  My breath caught back and the tears started to flood my face as I processed what she had said, nothing made sense, he was fine, my baby was fine, I have no idea what this woman is talking about.  I handed the phone to my husband as somewhere in my tears I lost my voice, I couldn’t speak, what was there to say, they are waiting for us at the Emergency room? what did that mean?  And so our story begins.