All We Can Do Is Try...

Reality is setting in. Noah is 2. He is not a baby. He is a toddler. There is a mile long list of things he should be doing at 2 years old. But he's not. And it sucks. It flat out sucks and I will say that without any shame or guilt. Before now, it was easy to turn a blind eye to the fact that he was disabled. Sure, I had to carry him everywhere, change his diapers, bathe him, etc. but that seemed like normal "baby" stuff. But now he's 2. Not so much a baby. He's almost 32lbs and about the same size as his brother. I am having a really hard time with the fact that this is how the rest of his life is going to be. Of course I knew his prognosis for someone with his disease. I know the clinical manifestations of PMD. But I so badly did not want that to be my child. I still don't. All of this is coming at a time when Noah is not doing so hot. He's been sick with fevers a few times over the past few months. He's been vomiting almost every day for the past 9 months. He's getting to a point where he mostly just wants to hang out in his bed a majority of the day. I do not want that to be my child. I want him to interact and play and smile and be happy. But I feel like he is having more bad days than good ones. And it's hard to cope with emotionally. I do not ever want to see him suffer or hurt or be unhappy. I have tried a million and one things to help with his vomiting and at this point it is out of my hands. We are being referred to Dell Children's to have another procedure to place a different type of feeding tube. This new tube will go all the way into his small intestine where he will be fed on a slow, continuous drip for about 22 hours per day. It will be a big change for us but I am so hopeful that this will stop the vomiting. The bad news is that this is yet another intervention. It upsets me to think about this as being another decline in his health. He is no longer tolerating his full feedings like he used to. This new feeding tube is not the end of the world. It's not even that big of a deal in itself. But symbolically, for me anyway, it just means another step backwards. And it's so hard for me to make this decision after I have fought so hard and tried so many things to not let it get to this point. But like I said this is now out of my hands. And maybe this will end up being the greatest thing for him. Who knows? All we can do is try.

Birthday Letter...

To my precious Noah,

As I sit here on the eve of your 2nd birthday I am overcome with emotions. Too many to even count. I think back to that day 2 years ago and I just remember being so excited to finally meet you. I couldn't wait to see if you looked exactly like your big brother. I wanted to kiss your beautiful face and hold your tiny fingers in my hand. I wanted to breathe in that heavenly newborn smell.

You were born into this world at 5:33pm and that's the moment my life changed forever. You were placed on my chest for a brief moment before you were taken from me. A whole 9 months of you and I being "us" ended just like that. You were silent and having trouble breathing. I was in bed taking in the moment and thinking that the hardest part was over. After a few minutes I turned to my Mom who was at my bedside and I asked if you were okay. She said you were fine and then I heard your first noise. A tiny little squeak. Then the nurse rolled the incubator over to me and you had an oxygen mask covering up your precious little face. She moved the mask away, only for a moment, so I could see you for the first time. You were so beautiful. She asked if you had a name and I said Noah.

And then you were gone.

I will never forget seeing you in the NICU for the first time. You looked so tiny to me although you were a good 3lbs bigger than all the other babies there. I could only stand to be in there for about 10 minutes before I had, had enough. This was not supposed to happen to me was all I kept thinking. I'm supposed to be holding and cuddling and nursing you, my hours old newborn. Not staring at you in an incubator while you had machines keeping you alive.

It was all such a blur at the time. But now I can remember everything in vivid detail and I can't decide whether that is a good or bad thing. I suppose one day I will be grateful to remember everything about you, including the day you were born. But sometimes it feels like I am trying so hard to forget.

These past 2 years have seen a lot of joy and a lot of pain. Many struggles but also great achievements. I don't know of anyone that I could possibly be more proud of besides you, my sweet angel. I know we are right on the edge of doing some big things with you. You are showing us more and more every day about how smart you are and how much you know and how much you can do. You are truly the most amazing person I know.

Thank you for letting me love you imperfectly. Thank you for showing me how to be the person I am supposed to be for you and for your siblings, too. But most of all, thank you for being you. My sweet miracle, Noah Jack. I hope you have the best day followed by the most awesome 2nd year. Happy birthday to you, baby boy.

Love,
Mommy

Dropping By...

Once again it has been awhile since my last post. Sometimes I just get so busy that my blog is really the last thing on my mind at the end of the day. But right now Savannah is sleeping, Oliver is in bed watching cartoons, and Noah is being taken care of by his nurse. I have a minute to myself to think and breathe and concentrate. It's nice, however fleeting this time may be. I have a few ideas for new blog entries in my head. Big ideas requiring more brain power than I think I can muster this evening. So I will save those for another day. Tonight will just be about me dropping by to say hello and reassure you that I haven't abandoned this blog project. Noah's 2nd birthday is quickly approaching so this weekend I will be going out to get some party supplies. The theme is sort of "Frozen" inspired with the tag line It's Cool To Be 2. Frozen is one of Noah's favorite movies at the moment and it's such a popular movie that Pinterest is just filled to the brim with cute little party ideas, although I am disappointed by the gender stereotyping going on. Frozen is for boys, too. Both of mine love the movie and I'm sure many other little guys love it just as much as all the little girls. Surprisingly though there were several Frozen character t-shirts online for boys which I added to Noah's Amazon birthday wish list. Anyway, I'm off track, if I was even on a track to begin with. Well it's getting to be about my bed time and I need to take advantage of having the bed to myself before Little Miss Thing wakes up and wants to hog the whole bed. It really is amazing how a tiny little 11.8lb baby can take up so much room....

What I Gained That Day

I feel weird writing this as if I lost a baby. I know I didn't lose anyone. He is still here with me physically. I have never buried a child or mourned over a tiny casket. But I did have a loss. And it is one that needs to be acknowledged. It is a loss that needs to be grieved so that the hole in my heart can heal.

I lost a son the day that Noah was born. I lost my ability to mother and nurture him the way I was meant to. I lost my power and control. But most importantly, I lost the child that I dreamed about in my womb from the moment I knew he existed. And instead I gained so much more than I ever could have imagined.

Two years ago at this time I was heavily pregnant with Noah and chasing a crazy 18 month old. If you would've showed me a picture of what my life is like now I probably would've freaked out and curled up in a ball in my bed and never came out again. I was not strong. I did not know I could be strong. In fact, I didn't know much of anything compared to what I know now.

In the last two years I have learned many things. Some things I have learned from books or from reading things on the Internet. Some things I have learned from other people. But a majority of things I have learned from my son. Not the son I dreamed about that would grow up and play with his brother and graduate from college and get married and have children of his own. Not him. I learned my most valuable lessons from my beautiful son that has never even said a word.

He has taught me to be an advocate for my children and I have found my voice for them when they cannot speak. I have learned how to be a better mother by growing my patience to wait and let them do things when they are ready. I have learned to appreciate the intricacies of pregnancy and the miracle of life because if one little, tiny thing is off it can change the whole outcome.

I have been broken for a long time from this loss I have felt. I haven't appropriately dealt with these feelings. And I haven't really spoken about them until recently. My other self, the self from two years ago, feels like a lifetime away.  I honestly feel like a whole different person and someone I never expected I would or could be. I have gained a whole new sense of who I am supposed to be, a new purpose, and a new life.

And it doesn't matter why this happened. And it doesn't matter why I feel this way. All that matters is that I feel it. And I'm dealing with it. In my own way. One way I'm dealing is to recognize exactly what I gained that day.

sMiles 4 Sammy

Saturday morning we loaded up the whole family and headed to a high school just down the road to join in the sMiles 4 Sammy Family Fun Run.

 Let me just preface this post with a few things.... Noah is not an easy kid to take anywhere. Noah usually does not like to go anywhere. He spends a lot of time at home which makes me sad that he "misses" a lot of fun things but I know that he would not really, truly be having fun anyway. He likes to be at home where things, people, sounds, and smells are familiar. He has terrible anxiety which manifests itself as gagging, retching, and vomiting.

That being said, we were very prepared for the worst. We put extra blankets in the car because the car is his favorite place to throw up. We didn't give him his morning feeding so that his belly wouldn't be too full in case he did throw up. We were anticipating it and ready!

I am so proud to announce that Noah DID NOT throw up at all! He did awesome in the car and listened to his music. And he absolutely loved all the noise and excitement at the race. There was a DJ playing really loud music with lots of bass which Noah got a kick out of. Even though we were late for the actual race and it was really hot (and Mommy forgot sunscreen, shame shame) we walked as a family around the track and Noah was smiling and laughing the whole time.

They had a table set up with pictures and stories of all of the sMiles 4 Sammy Families. Here is the story that I wrote to go with this lovely picture.

This is the story of a little boy that we call a Miracle. Noah Jack was born four weeks premature on July 11^th, 2012. He was in respiratory distress and immediately taken to the NICU where he would stay for the next eight weeks. During that time it was determined that he needed a tracheostomy to breathe and a g-tube to eat. Then we were sent home with 24/7 nursing care and referrals to therapy services.

At first we thought his delays stemmed from a combination of being premature, going through multiple procedures and surgeries, and staying in the NICU with minimal stimulation. He continued to receive physical, speech, and occupational therapies but by six months of age it was apparent that he was very, very behind developmentally.

We took Noah back to his neurologist that did his initial brain MRI when he was in the NICU. The doctor ordered another MRI and some genetic blood work. The genetic testing came back negative for everything but the MRI showed something very interesting. It revealed that Noah had an abnormal amount of white matter, or myelin, in his brain.

Myelin is a fatty substance that coats the nerves in the brain. Without it, it is very much like an electrical wire without proper insulation. The messages to and from the brain sometimes “leak out” and don’t reach their proper destination. This affects everything from his head control and vision all the way to his vocal cord muscles, which is why he needs the trach to breathe. This lack of myelin fits under the umbrella of a group of diseases called Leukodystrophy, and the specific one indicated by the radiologist is called Pelizaeus-Merzbacher Disease (PMD).

This diagnosis needed to be confirmed with a very specific (read: “expensive”) type of genetic blood test. The results came back that Noah has a point mutation on the PLP1 gene which is associated with PMD. But because Noah’s specific mutation is the only one reported in the whole entire world they can only be 99% sure that he does in fact have PMD.

After his final diagnosis we felt a mix of emotions. We felt relieved to know finally after 10 months of being in the dark. We felt hopeful that now we would know how to better help him once we understood. We felt sad that this was not something he would “grow out of” as he will most likely need his trach and g-tube for his whole life. We felt scared to see what the future holds for Noah and how hard this disease has and will affect his body.

Noah is now 22 months old and as we approach his 2^nd birthday we are so grateful that he is still here with us and staying healthy. He works hard every day to do things that everyone else in the world takes for granted. He is learning to roll on the floor as well as play with some toys and his iPad. Last week he started splashing in the bath tub. These are all things, along with many others, that doctors will tell you that children with PMD cannot or will not be able to do. But Noah is here showing everyone the things he CAN do and that is our Miracle.

I  also got to see a family that I met over a year ago at the Dell Children's NICU when I went to meet with them before their baby had a trach surgery. They are also a part of the sMiles 4 Sammy Family. And here is a picture of Noah with Sherlynn Scott who is Sammy's mom and founder of sMiles 4 Sammy.

We had a great time as a family and it definitely opened my eyes to the possibility of being able to take Noah more places because he did so great!

Ready For The Weekend!

Sorry it's been a few days between posts. Not much has gone on around here. We are just spending the week relaxing to gear up for our super busy weekend ahead. Nathan went back to work last night after spending 6 weeks off for his paternity leave. Things went fairly smoothly with the kids so I'm hoping the trend will continue.

I got a call yesterday from Noah's pediatrician. She had spoken with Noah's neurologist about the seizure he had a few weeks ago and he decided to increase Noah's medication. Hopefully that will keep Noah seizure-free like he had been for the last year.

This Saturday we will be taking the whole family, Noah included, to a 1K walk, run, jog, and roll hosted by sMiles 4 Sammy. We got hooked up with this wonderful organization through the Palliative Care team at Dell Children's last year. They were so wonderful and made us a gift basket with some awesome gifts for our whole family. They are just the sweetest, loving, Christian family and the story of their sweet Samantha touched my heart. You can click here to visit the sMiles 4 Sammy website. I will write another entry about the race on Saturday. I am hoping that the rain will steer clear of our area until the afternoon so we can enjoy the morning outside!

Then on Sunday, we will be celebrating my Great Grandfather's 91st birthday. Yes, 91! We are having an early dinner at The Salt Lick restaurant followed by a Round Rock Express baseball game. I am so excited for Oliver to see his first baseball game! We have a few lawn tickets so he will have plenty of room to run around.

Today I have the fun task of heading to the courthouse to pick up Savannah's birth certificate. We ordered it online shortly after she was born but for some reason we have yet to receive it and it needs to be turned in by June 13th in order to get her added to our insurance. The deadline is approaching quickly so it needs to get done before we forget and run out of time!

That's all for now. See y'all tomorrow!

Mr. Noah

I think it's about time for an update on Mr. Noah.

We made it through the winter with ZERO hospitalizations or illnesses! That is a major accomplishment for a kid with a trach. In fact, it's been over a year since Noah was in the hospital at all, minus when we went for his surgery over Thanksgiving break, which I now realize I never even wrote about. Oops!

In November Noah had his adenoids removed and Botox injected into his salivary glands by a wonderful ENT doctor at Texas Children's Hospital in Houston. It was a relatively quick out patient procedure so we drove down the day before and spent the night. Then we got up early the next morning to get to the hospital and we were headed back home by around 1pm. The surgery was without complications and he was only back in the OR for maybe an hour. It was so fast! Afterwards he was in a good amount of pain I'd say so he basically cried for the entire 3-4 hour trip home. That part was rough. But he recovered really well in the end.

Then about a week after his surgery he started vomiting which was something very new and different for Noah. Vomiting is very dangerous for someone with an unprotected airway like Noah has. When a healthy person throws up, their vocal cords close off the passage into the lungs. But with Noah who has floppy cords, they don't always shut properly which puts him at a greater risk for aspirating his stomach contents which can lead to an infection in the lungs like pneumonia. Pneumonia is especially dangerous for Noah because he has PMD. PMD causes weak muscle tone so it is harder for the lungs to squeeze out and cough up all the mucus that gets stuck in there from an infection. Respiratory illnesses are one of the leading causes of death in PMD patients. Needless to say I am terrified every time Noah gets sick which is why I work so hard to keep him healthy. Anyway, back to the vomiting... Hooray!

So after the surgery Noah started with this throwing up business. I tried every single thing I could think of to make it stop. I would tweak his feeding times or feeding volumes or whatever else I could think to do. And sometimes it would work for a few days at a time and then he would go right back to throwing up. After a few months of this I finally had to resign myself to the fact that Noah is just going to throw up. I also had to explain to his nurses that this is just his new 'normal.' There is nothing wrong with him, he's not sick, he doesn't have reflux, he just vomits.

Sometimes it is due to anxiety. Every time we get into the car with Noah, he throws up within the first 5 minutes. If he is too over stimulated he will make himself throw up. It is something he can do and something he can control. I relate it to a child throwing a fit that will bang their head on the wall. Yes, it hurts and it's not fun. But that is the only way that child knows how to express himself at that moment when he feels very out of control. The same thing happens for Noah. Throwing up is not fun. It is messy and stinky and occasionally requires us to do trach care AGAIN if he gets throw up on his trach ties or dressing. But that is his way of telling us that he is upset or doesn't like something. We are working very hard on other ways of communicating but it is a slow process.

Some days he does great and he doesn't throw up at all! Other days we are constantly on our toes because he is gagging and retching all day and we are just waiting for the throw up to come. But it's just one of those things that we have to deal with now and we are all getting better at reading him and learning his cues as to what will set him off.

Within the last two weeks Noah has been showing some signs of seizure activity. Noah is on a medication for seizures called Trileptal. He has been taking it for over a year now and we haven't seen anything remotely seizure-like since then. But a little over 2 weeks ago he had a big break through seizure. Our nurse for that night was out sick so it was only Nathan and I here when it happened. And it was scary. Initially I didn't really know it was a seizure and since he doesn't have them like ever it honestly didn't even cross my mind that he was having one until it was already over. Let me just say that it is not a fun experience to see your child with an unresponsive stare and have their face turn purple for 30 seconds because he stopped breathing. We gave him some oxygen and eventually got him to calm down by holding him and rocking and singing his favorite song, "Twinkle, Twinkle, Little Star." Then he fell alseep hard for the next 5 hours. That is called the postictal phase. During a seizure, the brain and every muscle in the body are working in overdrive so it is very normal for the person to fall asleep or be very "out of it" after the seizure is over.

Since then, he hasn't had anymore episodes of that magnitude but we have noticed a few different things that could definitely be associated with seizure-like symptoms. Luckily we already had an appointment with his neurologist scheduled for the beginning of June. I'm not really sure what the doctor will want to do at this point but I'm at least glad for the opportunity to speak with him face-to-face again. I think it's been about a year or close to that since we have been to his office.

Other than those few things, Noah is doing great! He is really coming along with all of his therapies. He is doing really well learning how to roll from his side to his back as well as learning how to activate his toys more consistently. We are also working with him on making choices. When we ask him what movie he wants to watch we will hold up two movies, one in each hand. We show him both movies and which ever one he looks towards is the one that we will put on for him. Eventually I want to have pictures printed out of the things that we do almost every day so we can show him the pictures and let him decide. For example, have a picture of his wheelchair and a picture of the couch and let him decide where he wants to sit. I am really hopeful that this will be a good way to communicate with him or at least be able to give him some control over what he wants to do.

He is also turning out to be an excellent big brother. When we first brought Savannah home, Noah wasn't too sure about her. He usually made what I call his "sour pickle face" whenever she was near him. But now I will lay the baby in bed with him or next to him on the floor and his face just lights up! I can tell that he loves her so much. It really is the sweetest thing to see.

That's all for today. See y'all tomorrow!