Chiari Malformation

It's been quite some time since I've posted. Life got hectic with Zach in school and the twins growing up. I've been through a couple of jobs and finally landed where I think I was always supposed to - in a classroom of my own. The last semester was a difficult trial because I stepped into a classroom halfway through the year, but even on my toughest day, I was still happier at work than I had been for as long as I can remember. I've been offered a position back at the school and can't wait to have my class for the whole year.

Nicholas has been overall stable in his health and has been improving so much and so quickly. He went from a walker to walking to working on going up and down stairs. His vocabulary has increased significantly and he's holding full, really hilarious. conversations. We went through a fairly painless, though time consuming, process to get Nicholas transitioned from the Early Steps program to Orange County Schools when he aged out of Early Steps. He was evaluated and an IEP was drafted. He now goes to a pre-school program in a school near us for a full day program, and even rides the bus from daycare to school and back daily! He loves the bus, the driver, and the bus aide. He is completely enamored with his teacher.

Emily and Zachary both continue to grow into really great humans. Zachary is finished with first grade and chomping at the bit to start second! Emily had her first dance recital and absolutely loved being on stage. Zero stage fright from her at all.

In May, we were in Boston for my sister's graduation from her graduate school program. While there, Nicholas scared us with what we thought may be a shunt malfunction, so we got to visit Boston Children's Hospital. We had heard nothing but amazing things about this facility and can back that up with our own experience. During Nicholas's brief stay (which ended up being a stomach bug that he shared with all of us, not a shunt malfunction), we had to get a copy of a recent MRI for BCH to compare to. Luckily, he had had an MRI in March for a totally unrelated issue. While in BCH, Mike got a copy of the MRI and we were able to read it. We don't typically get copies of his images because 100% of his care is through the same hospital system and all his providers can access his records at any time, so this was the first time we had read over the radiology report from the MRI. On that report, we saw the words Chiari Malformation Type 2. We had only recently been made aware of Chiari Malformation because Nicholas's neurosurgeon's contract was not renewed (much to the angst and chagrin of his entire patient population), and the neurosurgeon is one of the top physicians with knowledge of Chiari Malformation in the Southeast. We had joined forced with the Chiari population to protest the release of this amazing neurosurgeon and thus had learned of this condition.

Chiari Malformation Type 2 most frequently occurs with a type of Spina Bifida. The cerebellum and brain stem extend through the base of the skull and cause severe issues like debilitating headaches, problems in gross and fine motor skills, problems with speech, and feeding issues. We had never heard of Nicholas being diagnosed with Chiari and certainly hadn't seen the majority of the issues that occur with Chiari Malformation, but the feeding issues piqued our interest. We obtained a copy of the radiology report from BCH and Chiari Malformation was listed on that as well.

Nicholas will be having a fairly standard surgery later this month - getting tubes, getting his tonsils and adenoids removed, and getting a cyst in his nose removed. After the ENT appointment where it was decided to go forward with the surgery, we were contacted by the surgeon who, because of Chiari being listed on the MRI, needs neurosurgery's approval to go forward with surgery. The neck has to be hyperextended to remove the adenoids and this could be devestating for a patient with Chiari, so we scheduled an appointment with the partner of our former neurosurgeon to get approval for the surgery. During that appointment, the neurosurgeon confirmed the Chiari diagnosis, though he doesn't seem to have the more severe Type 2. We were told that lots of people have Chiari, it usually doesn't matter until the symptoms start causing problems with the quality of life. Neurosurgery didn't think his Chiari would be problematic during a tonsil/adenoid removal and granted approval for the surgery.

Part of the reason (among others) that Nicholas's tonsils are being removed is to see if it will help with his feeding. Apparently Nicholas has enormous tonsils and the speech therapist thinks that Nicholas may be instinctively protecting himself by not eating due to the lack of space in his throat. The neurosurgeon warned me that with a Chiari diagnosis, the reality may be that he has a symptom of his Chiari that may need to be addressed. We've decided to go ahead with the ENT surgery (since there are other issues that removing the tonsils will resolve) and if his feeding doesn't improve then we need to regroup with neurosurgery to determine how much of a factor the Chiari might be in his failure to feed by mouth.

It was scary and frustrating at first to receive yet another diagnosis, but as family, medical staff, and Nicholas himself have reminded us, a diagnosis is just something written on a piece of paper and doesn't tell any part of the story of this little boy. He's strong, and joyful, and funny, and amazing and if we need to figure out how to address the Chiari then we know that he will face this challenge with the same upbeat, cheerful, and determined attitude that he's faced all other challenges thrown in his path thus far.

Las Vegas

Just about a year and a half ago, I wrote this post about the shooting at Pulse here in Orlando. We got to hold the honor of being the "deadliest mass shooting in modern U.S. history." Lucky us. The eyes of the world zoned in on us, people offered prayers, Facebook pictures showed #prayfororlando, world media descended upon downtown, and we were changed. When the national and global media pulled out, when the eyes of the world looked elsewhere, we were left to deal with the aftermath. People at the nightclub had to figure out what to do with survivor's guilt, what to do with their fear of being out in the world, what to do now. Victims stayed in hospitals for far longer than the world watched. To this day, if you drive by Pulse, you will see the fence surrounding it, messages of love and hope and peace adorning the fence, flowers left for the victims and the survivors.

On the one year anniversary, the city came together again. Tears flowed, the names of those lost were read, people visited Pulse and left gifts of remembrance. Four months later, we sit watching in horror as Las Vegas takes the title no city wants of deadliest mass shooting in modern U.S. history. Before we even know the final count of dead, the final count of the injured, the why behind the shooting, the title has already been claimed.

When does this stop? When do we reach the point where enough lives are enough? High schoolers weren't enough, first graders losing their lives wasn't enough. 49 people celebrating life at a nightclub wasn't enough. Current count in Las Vegas is 58 dead and 515 injured. I'm terrified that number will rise, but is that enough? How many lives are enough that we try to figure out how to stop this? How to fix this?

Las Vegas, from someone who has now watched this happen in two of my beloved places - Littleton and Orlando - I'm so sorry. I'm so sad that you have to become a hash tag. That you will have to figure out how to move forward. For the victims, I'm so terribly sorry that our tragedy wasn't enough to stop this madness. That your lives had to be taken too. That you are sitting in a hospital, injured and shocked and scared. I'm so sorry.

Baby Steps

Nicholas originally had a goal with his physical therapists that he would be independently walking by age 2. Obviously that hasn't happened, but he's still working very hard on getting that goal. His physical therapist at his school suggested that we get him orthotics to give him more support while we build up his leg strength to support standing and walking independently.

We ordered the orthotics (not much insurance drama this time!) and were lucky to have the same person and company who made his helmet make the orthotics. He is what is known as SMOs (Supra-Malleolar Orthoses). They encompass his whole foot and rise to just above where his shoe stops. He took to wearing them very quickly and seems to be doing well with them.

His physical therapist at school has been trying different ways for him to walk and has found that he liked walking with a gait trainer best and really took to it. She had tried using the push walkers that many babies use to support walking but found that he was too tall for most of those, and that they didn't have the strength to hold the full weight of a two year old. The gait trainer supported him and also gave him the confidence to walk on his own.

We were very lucky that the physical therapist was able to borrow a "floor model" of the gait trainer from the manufacturer before we had to involve insurance and authorizations to see if it would work. Once it was determined that he was doing very well with that, we decided to order one of our own. It took about two months total between placing the order, getting the documentation for medical necessity together, the measuring, the build, and the delivery, but Nicholas finally got his own walker last week and he is loving it. Suddenly, there are places viewable in the house that he's never noticed before. Suddenly, he can keep up with Emily and Zachary when they take off to a new game. Suddenly, he can control his own direction. He's so excited and I think with the additional assistance of the walker, he'll gain the strength and confidence he's currently lacking to walk on his own.

It's amazing to watch him get stronger and stronger - this past weekend he walked for significant periods of time through Disney! He's such an amazing little warrior!

Two Years

Two years ago today, our crazy twins surprised us by jumping the gun on their delivery. They were originally scheduled as a c-section on December 31st. I was to be at the hospital at 5 a.m. and they were scheduled for a calm, 7:30 a.m. delivery. We had everything planned. My sister was going to fly in on December 24th and help us through the Christmas season and then she would stay home with Zachary until the twins were here. Once we were sure that everything was okay with the twins, she was going to bring Zachary to the hospital, let him come in and meet his siblings and then come in and meet them once Zachary was comfortable. The best laid plans....

Instead, I spent the majority of the day on December 20th feeling terrible. I laid on the couch the vast majority of the day. We had been scheduled to go to a friend's Christmas party and I ended up letting her know we wouldn't make it since I felt so terrible. I went to bed but was woken up at around 1 a.m. with contractions. I'd been having Braxton Hicks for several weeks at that point, so I got up and tried walking around and drinking some water to see if I could make them stop. Instead of slowing down or stopping, they got more intense. By 1:45 a.m., we figured out I was contracting every three minutes for 60+ seconds. Time for the hospital! Our backup emergency contact didn't hear her phone when we called, so Zachary ended up making the trip with us.

An emergency c-section with the anesthesiologist as my support person (since Mike had to stay outside with Zachary) and the twins were born three minutes apart. Both came out screaming, which was a relief, and I was shocked when I heard that Emily weighed 6 lbs 1 oz. That was a good healthy weight, considering they were born four weeks early.

Two intense years have passed since that day. It's been amazing and stressful to watch these two grow. Emily has turned into an outgoing and friendly toddler. She laughs easily, has no issue whatsoever holding her own against two, and loves giving hugs and kisses. Six months ago we were worried at her lack of speech, but she's recently blossomed and has begun speaking many more words and even trying to put sentences together. She adores Minnie Mouse, baby dolls, anything resembling a telephone, and puzzles.

Nicholas loves cars and books and electronics. He is happy the vast majority of the time and is desperate to keep up with his brother and sister. He likes to wrestle with them and loves to laugh. Bubbles are amazing to him - he will try to pop them and says "pop pop pop." He's recently begun speaking more words. He has amazed us with the amount of fight he has. This child simply knows no boundaries. As you all know from the many many stories I've posted throughout the past two years, we have been warned multiple times from multiple physicians that they don't know what to expect from Nicholas. That he very well may have devastating brain damage and that he may never walk or talk or eat. You also know that we found out that brain damage did cause Nicholas to have some issues with his suck/swallow reflex and that it was ultimately decided a g-tube would be the best way to feed him. And that he's continually working on improving that through speech therapy. And that he passed a puree swallow study but we're still working on liquids. And that he has moved from being able to sit up on his own, to a modified scoot, to a modified crawl. And that we fought against physicians to get him a helmet and shocked them completely by having the helmet work and fix his noggin. Last week, his physical therapist tried using a gait trainer with him and Nicholas used it to stand all on his own (with support). And then, he took two steps.

Two years ago, we were scared of having two babies at one time. We were worried about Zachary going from the only to the older brother of two. We didn't know what to expect with Nicholas. And while we've had some of the most difficult times we've ever experienced (we know far more about the inner workings of an intensive care unit that we've ever wanted to), we've also experienced some of the best.

Happy birthday Emily and Nicholas!

Whoops I Did it Again

Things have been absolutely insane around our household and I have been terribly terribly remiss in updating. I apologize and promise that it's been mostly good news that kept me away!

In mid-August, I started my new job. While it's a big change from what I'm used to, it's really a fantastic company to work for and it's great to feel challenged to learn something new. With the new job came many new things for our family - for the first time the twins are in daycare. We found a school that specializes in special needs children but also accepts "typical" children, and luckily they had space for both Emily and Nicholas! We've seen leaps and bounds of development from Emily who is now trying very hard to talk. She's doing very well, and is even attempting sentences now! Nicholas is trying to emulate the older kids (they are in a classroom for 18 months to 3 years old and are the youngest of the group), and we've noticed him verbalizing a significant amount also, along with working on ways to get from point A to point B more efficiently and voluntarily going to a standing position.

Also with the change, Mike was able to move back to a day shift at work which has been amazing for our family. We can actually eat dinner together most evenings (he still works news - breaking stories still happen), he gets to be more involved in Zachary's taekwondo, and we actually feel like a family again.

Things have been pretty quiet here, albeit incredibly busy with life, but nothing out of the ordinary until recently. We had noticed that Nicholas's g-tube was leaking a little. We mentioned it to his pediatrician at his 18 month check up and they told us that the button may be sized a tad small since they've never resized since he had his g-tube placed at 3 weeks old, but that we would keep an eye on it. A brief lesson in g-tube sizing: Nicholas was wearing a 14 french 0.8. That means the part of the tube that actually goes through his abdominal wall into his stomach was 14 french (a form of measurement) around and 0.8 mm long. We noticed the leaking getting worse, so we went ahead and changed the tube. It continued to get worse until almost every feed, he would leak so significantly that his entire shirt would be soaked and smell of stomach acid.

On a Sunday morning, we noticed that his belly around his g-tube site was red and raw. He was screaming in pain every time we fed him. We called the on-call pediatrician who told us that the children's hospital emergency physicians are all trained on g-tube resizing so we should take him to the emergency room to have it looked at. He also mentioned that if we didn't feel comfortable with the emergency room physicians resizing the button, the general surgeon who placed Nicholas's tube originally was actually on call and in the hospital that day. Mike whisked off to the emergency room for what we figured would be an easy fix. After several hours (things do not move quickly in hospitals), they were home. Nicholas had a freshly placed 14 french 1.2 button. He was past due for a feed, so we set him up and started the feed. Nicholas immediately began screaming in pain and the hole where the g-tube button is began leaking so badly that we were actually unsure if any of the feed was even making it into his stomach. Mike packed Nicholas up and took him back to the ER. The surgical resident came down and looked at his g-tube. He decided that Nicholas appeared to be between sizes - he thought a 14 french 1.0 would be too small but a 14 french 1.2 was just slightly too big. Unfortunately, there is no such thing as a 14 french 1.1. He left the decision up to us, but suggested we stay with the 1.2 because scar tissue would form and until then, we could pack the site with gauze to kind of build up the distance and hopefully stem the leaking. He was wary of switching the tube again simply because Nicholas was already in so much pain that he didn't want to add to it.

We took his suggestion and called the surgeon's office for a follow up visit. We were able to sneak into an appointment on Wednesday and the surgeon ended up changing the tube at that visit back down to a 14 french 1.0. At that time, Nicholas was diagnosed with a chemical burn from the stomach acid leaking out of the g-tube site. He was prescribed a topical ointment and we were told to attempt to let the site air dry as much as possible.

By Friday afternoon, we thought things were getting better. As I sat in the chairs at Zach's taekwondo class, Nicholas became very agitated. I was holding him, trying to calm him down, and I realized his onesie was soaked through again. I took him to the bathroom to try and get some cool water on his belly until we could get home and I could change him and fix the dressings and that's when I realized that blood was pouring out of his g-tube site from beneath the button. I asked the director of the school if Zach could stay there (he had demo team practice later that evening) until Mike could get off work and meet him there, grabbed Emily and Nicholas and headed to the hospital. Zach was treated very well while I was off - hanging out with his favorite instructors and fed delicious food from a mom of his friend who graciously took it upon herself to run to Publix and pick up some food for him.

The ER doctors consulted in the surgical team again who decided they would like to see an X-ray just to make sure that at some point during all the button changes over the past week that his stomach hadn't disconnected from the abdominal wall and that we had just been feeding his abdominal cavity for a week. This can happen early in the g-tube placement process as scar tissue hasn't formed to adhere the stomach to the abdominal wall yet, but is a highly unlikely possibility after having had the tube for almost 2 years, however, we agreed with the surgical team - better safe than sorry.

The X-ray found that there to be no problem with the placement of the tube and it was decided that the bleeding was from irritation of the chemical burn. I had made my way back to taekwondo to pick Zachary up from demo team practice (and brought Emily back with me), so Mike was sent home with wound care instructions. The tube is still leaking somewhat, thought it's not nearly as bad as it was during this time. We've gotten the worst of the chemical burn under control, though it's still healing. The best conclusion we can come to is that it was a combination of things - the initial tube too small, transferred into a tube that was two big and allowed for significant leakage over three days before it was switched back out. Beyond that, he's currently getting his molars, so he's had more spit to swallow and because molars really suck to get, he's been crying more frequently than he normally does. Crying causes him to bear down, and may be contributing to the leaking.

Hopefully this will eventually just be a blip on the radar pretty soon. I feel like we're getting close to the end, but it's been a frustrating process.

Eye See - on the other side

Because he had surgery first thing in the morning, Nicholas was not allowed to eat past midnight on Thursday night. I fed him his 9 p.m. feeding and watched him for a few minutes. I knew that this time tomorrow night, we'd be about 12 hours post-surgery. I wondered what that would look like.

We'd been told the risks - they were low and few, but there are always risks with surgery. The biggest risk was that the surgery wouldn't fully work and that the issue would be either over or under corrected.

To be honest, we still don't know if that has happened. We won't know for several weeks once the swelling has gone down completely and the eye has healed from the trauma of surgery if it's worked or not fully.

Friday morning, Mike and Nicholas left crazy early to get to the surgery center by 6:15 a.m. We had decided that it made more sense for Mike to go with Nicholas and me to stay home with Zach and Emily. I got pictures of Nicholas in pre-op, dressed in his tiny hospital gown and happily watching Disney Junior.

I got a call when he was taken back to surgery. I got a call when the doctor came out and said he was done, and I got pictures of a sleepy little man when Mike was allowed into recovery.

When he was discharged and they got home, I got sweet little cuddles from a groggy, red-eye child.

We put him down for a nap and he slept for some time.

Over the holiday weekend, he remained off and on groggy, probably the efforts of his little body to heal itself. But he was never grouchy. He remained the sweet little guy who thought everything his siblings did was hilarious. He continued to work on his modified army crawl to get to the toys that he wanted to see. And every time we turned around, it seemed, he put himself into a sitting position.

It was driving us crazy to know that he could do it but that none of us had seen it happen yet. We really just wanted to know how he was doing it.

Nicholas had his follow up with his ophthalmologist today. His eye is healing quite nicely, the redness has reduced significantly, the crusty yucks that were leaking out have slowed to almost nothing, and as far as we can tell, his eye is in a much better position than it was. He has another followup in 6 weeks and we will have better answers then.

And yesterday afternoon, I finally saw him put himself in a sitting position. He has decided recently that sitting seems cooler than laying down and continues to sit up more and more frequently. He decided to show both Mike and his physical therapist the trick and today in therapy, with some help, he pulled himself to a standing position against the couch and stood very confidently for some time.

Checking Off Milestones

In all of his recent evaluations, Nicholas has been determined to be about eight months behind in his physical development. He's maintained a steady eight months behind in development level, which is great because it means, while he's behind his "peers", he is staying on his own track and isn't falling behind.

He is a few months behind in cognitive development, and is actually age appropriate, or even advanced, in social skills (which anyone who knows him will tell you - our giant flirt).

So, even though he's currently eighteen months old, he's technically considered seventeen months old developmentally since he was born a month early, and he's measuring at nine to ten months in his motor skills development level.

As I've mentioned before, Nicholas isn't crawling or walking yet, and we honestly don't know when that will happen. We're fairly confident that it will - he's proven himself to be a singularly determined individual, but it will be late. Many people with hydrocephalus, especially at the severity that Nicholas had, will never walk, so we're grateful that he likely will, even if late.

He's been sitting up on his own for some time now, as long as someone helps him into a seated position. His core strength has grown that he now sits for the entirety of a taekwondo class (while playing with the other babies), and it doesn't exhaust him. Early Saturday morning, I was up with all three kids. Zachary was watching cartoons, Emily was playing, and Nicholas was practicing getting into his crawl position. Suddenly Zachary says, "Mom! Look at Nicholas!" I looked over and Nicholas was sitting up next to the entertainment center, trying to get the DVD player. I said, "Oh wow, that was nice of you to sit him up, Zach!" Zachary said, "Mom, I didn't!" I asked him if he was being honest, did he really not help Nicholas at all? Zachary swore that he hadn't helped Nicholas up. Which left the conclusion that Nicholas had gotten himself into a sitting position for the first time ever. We watched him throughout the morning and it never happened again.

Until nap. We went to get him after his nap and he was sitting up in the crib. We knew at that point that he had to have done it himself. That night when we went in to feed him his 9 p.m. feed, we found him sitting up in the crib instead of sleeping, laughing and incredibly proud of himself. Since then, we've found him in a sitting position several times, though we've never actually seen him put himself there.

This little guy continues to amaze us, and we are so proud to be able to watch this journey of his!