What a difference one year can make

June 27^th, 2013

One year ago today we headed into our 20 week ultra sound. We walked out numb, scared and downright nauseous. "There is fluid on his brain. It could be spina bifida, it could be nothing." No real answers, no real plan, just those words. Fluid and brain. By all accounts, joy was replaced by fear, excitement was replaced with anxiety and every bit of hope I had was something I had to fight daily to keep a grasp on.

Before the ultrasound, we had made plans to go to dinner. So, we did. I ate 3 bites and cried the entire time. Thinking "what in the world are we doing here. Why did we come here." Looking back now I think that was an indicator of how we would handle this situation and everything else that was thrown at us over the course of the past 365 days. We kept going. I went to work the next day, and cheer camp every night that week. Mark went to work, and continued on with his grad classes. We had no idea what the future would have in store, but we knew the best way to deal with all was to keep on chugging. 

Evan arrived, and all the doom and gloom went out the window. He was strong, he was healthy, and aside from the pesky fluid and a little unknown syndrome called OAV, or Goldenhar, the kid ROCKED. APGAR with scores of 9 & 10, no need for oxygen. He would need a feeding tube, but in the realm of what could have gone wrong, that was nothing.

We went on to spend 29 days in the NICU. During this time, we celebrated Thanksgiving and our one year wedding anniversary.  We got to spend Christmas at home. We would head back for a one night stay in mid January when his ETV failed, and his shunt was placed. Since then, we only head to Glennon for his appointments, which are getting fewer and farther apart. What was once 2 appointments a week, is now closer to 3 a month. Each appointment gets Evan that much closer to being the best dude he can be J

Here is an update of how things have changed over the past 8 months.

Evan began seeing his ocularist when he was 30 days old. He is currently on his 7^th spacer. These spacers increase the ocular orbit (eye socket) and are increased in size each month. Hopefully by his first birthday, Evan will have a beautiful blue eye on the left side to match the one on the right.

Evan’s early ABR’s (hearing screening) showed moderate to severe loss in his “good ear.”  Hearing in his ear that has microtia (under developed ear) could not be assessed in those early stages. Since there is no opening, there is no hearing on that side at this time. St. Louis is blessed to have 3 major schools for hearing impaired children. Given the choice, we went with the Moog Center and could NOT be happier with the care, education and assistance we have received. A hearing aid was fitted when Evan was 4 months old. We drive to Moog once a month where we spend an hour in the audiology booth, and 45 minutes with our parent educator. His “Moog girls” as I like to call them are also amazing. Over the course of the past 3 months, Evan has shown his loss to be more in the moderate range. (amazing news people J  He also has echo’s on his OAE’s (Children with hearing loss are not supposed to have echos) But he does. We have no idea what that means, but for whatever reason, it makes me happy.

Our ENT may just be my favorite doc. Dr. Do is extremely gentle and talks very slow. He explains everything 3 times and I always leave feeling like he could have talked for 30 more minutes. When Evan was born, we were told he need jaw reconstruction when he was 7 or 8. Through CT scans we now know he has 90% of his mandible and won’t need the surgery. When Evan was born, he had facial palsy on the left side of his face. I noticed a change in his smile at month 5. The upper left corner had a crease that wasn’t there before. Dr. Do said sometimes the nerves come back. I am now starting to notice the lower left corner is moving some too. More and more good news J 

Evan’s shunt was placed on January 17^th. Every day I check his head and once a week I do a measurement. I check his shunt and the area where the tubing is. Every day when I see that bump, I am reminded that a manmade device is keeping him alive. Shunts can malfunction at anytime. I believe the stat is that 50% malfunction in the first two years. At our most recent visit, our CT scan showed “Happy brain” as our neurosurgeon so eloquently explained it. There is still fluid, but in the areas where the fluid has drained, Evan’s once scrunched up brain is fluffing out. So far Mr. Shunt is doing its job. MORE VERY GOOD NEWS J Because of the shape changes in Evan’s head, he needed a helmet. We are currently on a “helmet break” to see if it is impacting his ridges. We meet with the craniofacial team on July 2^nd to see where we go next in getting his noggin to look “normal” Evan's monthly visit to the "helmet dude" have shown steady progress prior to our break.

The fluid on Evan’s brain impacted the way his brain formed. His pituitary gland is smaller than it should be and sits at a slight angle. We were told it could impact a number of things, and Evan is closely monitored. Thus far, only his hyrocortisol numbers are “abnormal.” In the event of moderate illness, or surgery, Evan simply takes a pill. During those freakishly scary first few days in the NICU, many doctors trotted in and out. Dr. De came by at 10pm on day 3 and gave us his concerns and simply said “I’ll be your favorite doctor because if anything is wrong, I can simply treat it with a pill.”

Evan has two butterfly vertabrae near the top of spine, and his spine has a slight curve. We have no idea how this will impact him, this is the one area that isn’t being addressed by any doctor or department.

Evan has hit some of his milestones exactly when he should. Some are running 2-3 months behind. He is crossing his mid line and from day ONE has brought his hands together. Remember, way back when I wrote my first blog after he was born I talked about the absence of his corpus callosum. This is a big word for the fibers that connect your right brain to your left brain. At first we were told Evan didn’t have one (which isn’t uncommon in kiddos with hydro, but would make the task of bringing ones hands together, or crossing the mid line when you use your right hand to grab your left foot would be difficult) We have now been told it is there, but it’s very thin. His “praying hands” as we call them have always made me smile. Further proof that progress is being made. Evan currently has PAT (parents as teachers) once a month with fantastically wonderful woman. A woman I got to share an office with during my first 5 years teaching. (Funny how things come full circle.) We also have First steps. Evan gets weekly visits with a hearing specialist and monthly visits with the vision specialist and a physical therapist.

Well, I believe that sums it up. In a nut shell I could have said “We were told a bunch of really scary stuff. Some of that stuff happened. Some of it didn’t. Some pretty crappy stuff that we didnt know to worry about knocked the wind out of us. Some of the bad stuff, improved and became good stuff. Whew, it’s been a wild 365 days!

One last thing…Evan’s ocularist has a glass eye. Evan’s Audiologist at Moog, and our parent educator both have profound hearing loss in one ear. Evan’s endocrinologist also has glandular deficiencies. Our ENT has a son who is Deaf. When we were first told that horrible news one year ago, I was afraid Evan wouldn’t even make it to today. I had no idea what his quality of life would be. Now, seeing everything he has overcome, accomplished and surpassed…I can’t help but wonder what Evan will be one day J

Warrior spirit

Before Evan was born, the docs gave us the worst case scenario  We will take him early. We will airlift him. You may only have a few seconds to see him. We don't know if his lungs will be developed  We don't know if he will breath on his own. We will stabilize him and prep him for shunt surgery as soon as he is strong enough. We will monitor him for seizure activity..blah blah blah. I prepped for the worst and prayed for the best.
Did the fluid go away? No. BUT the BEST that could have happened, did. Our prayers were answered in the God intended them to be.
He was born. He breathed on his own, he cried and he was strong. He came into this world and surprised us all. He came into this world without his left eye, his left ear, with a slightly curved spine, a small pituitary gland and an extra rib.  He came into this world with a small, pea sized tumor in his noggin that I despise. I HATE THAT TUMOR. IT caused the blockage that caused the fluid. Having Goldenhar is a bad enough....and in RARE cases, Goldenhar presents with hydrocephalus. Stupid Tumor.
That "stupid" tumor also gave us a GREAT opportunity.  We could avoid the shunt. We had the chance to give Evan a shot at a shunt free life. Soon after I got to leave St. Mary's and join Evan in the NICU, we were told that Evan was a candidate for the ETV (endoscopic third ventriculostomy) Our neurosurgeon would go in and place a small hole in the floor of his ventricle. Very few surgeons perform this brain surgery on children under the age of 2. Under the age of 2, children have only a 40% success rate. To be given a shot at this was a blessing. Mark and I spend 2 days in the NICU discussing the options. A shunt can fail at any time. 50% of shunts fail in the first year. . Poor kid had been dealt quite a hand....this was his big break :) I truly believed in my heart that the ETV was going to work.
Seeing him in that crib, prepped for ETV surgery was my lowest point. NO CHILD should have to go thru this. No mother should have to sit in a hospital recovering from delivery without a baby. No father should have to pack a car and drive his wife home with a empty car seat in the back. IT WASN'T FAIR. But the ETV..this was going to make up for all of that.
Evan went in for surgery. He came out with a big bandage  Our neurosurgeon said it was one of the most successful surgeries he had ever preformed on a neonate. This was day 12 of Evans of life.
Day 13, day 14 day 15....everything looked good. Day 16, 17...18.19....
During rounds on day 20 (8 days after surgery) his head circumference jumped. They attributed it to the plates "leveling out"...
we left the nicu on day 29. Evans head circumference never stopped growing.
On January 17th at 11 weeks old Evan got his shunt. The ETV had failed. We got to cardinal Glennon and checked in. I was handed the smallest hospital gown you've ever seen and THAT broke my heart. The fact that so many children go through illnesses & accidents that these little gowns are common place just sucks. No eloquent way to say it.
This time I got to carry Evan from the pre-op room to the surgery floor. He was as happy as a clam and looked adorable in his gown, I might add. We sat and waited for anesthesiology to come get him. 4 hours later he was done,  He got another super cool bandage and another battle scar. A one night stay is WAY better than 29 :)
When he woke up the next morning, he was his old self. He smiled at his dad and kicked his legs. He was 11 weeks old and had just survived his 2nd brain surgery. and he was a happy baby :)  Once again, he pulled thru and was no worse for the wear. I hate that the ETV failed, but I am still thankful that God gave us a shot at it. We have no guarantees that his shunt will function properly, it could be 6 days or 6 years before his next surgery....but what I do know is this. Evan is going to take each challenge head on. He will overcome it, and we will make the best of it. And so will we. After all, with a warrior spirit like his, how can that NOT be contagious?

The unknown

"We may never know what caused this."

One of the doctors said these words to Mark and I as we were leaving the fetal care institute in early August. By that point, I knew there was major problems, and I'd been watching the fluid build up week after week. My concern wasn't what caused it, my concern was how it was going to effect my baby. Would Evan make it to our due date? Would Evan be able to breath on his own? Would Evan make it home? This is what I worried about. The unknown. Most days I was faithful that he would be okay, but those fears crept in at times, I'm not going to lie. Knowing what caused the blockage just didn't matter to me. I am sure some parents want to know, but I wasn't one of them....so guess what? Soon after Evan's delivery, I would get to find out EXACTLY what caused the aquaductal stenosis. And what caused that is something that happens randomly, with little explanation in every one in 5600 births. 

I am going to go off on a side note for just a second. I never intended to go this long between posts. I cant even chalk it up to being a busy new mom. The first 29 days of Evan's life we spent the vast majority of our time in room 1823 at the NICU at Cardinal Glennon. I was never so happy as the day we went home, but I was also sad. That place was Evan's first home. Those nurses will forever hold a special place in my heart. In those 29 days I spent a LOT of time sitting and watching Evan. I had more than enough time to blog, but I didn't know what to say, or how to say it. Things changed daily, minute by minute in fact at times. I also wasn't ready to tell everyone that Evan's Hydrocephalus wasn't a random isolated occurrence  It was a bi-product of a much bigger issue. A syndrome that even as a special education teacher, I have never heard of. All of the things I worried about (Him breathing, the rush to cardinal glennon...none of that was worth my worry. He had apgar scores of 9/10. But when Mark carried him over to me, minutes after being born, I knew, I knew something else was wrong. 

Evan has Goldenhar syndrome, also called OAV ( Oculo-Auriculo-Vertebral) For Evan, that means that his left eye did not form. His left ear is very small and there is no opening. Two of his vertebrata are butterflied and he has a 13th rib. Evan's left jaw is underdeveloped, and he has palsy on the left side of his face. Evan has focal alopecia on the left side of his head, yet right above that, he has a tear shaped purple mark, where the hair has grown out 2-3 inches. It sticks straight up and makes me smile every time I see it. Evan is also the cutest little boy I know. Evan has a button nose and a cute little baby mullet  He is laid back and sweet. and only cries when he needs to tell us something. Evan has done better than anyone thought he would. Evan has a mom and a dad who will move mountains to give him what he needs. Evan also has a lipid tumor, just between his third and forth ventricle in his brain. THIS is what cause the fluid to build up. The fluid would keep his brain from forming the way it should. It caused Agnes of the corpus collosum. Evan's pituitary gland is smaller, and sits at an angle. Evan's sweet demeanor and warrior spirit won the hearts of his resident doctor and the greatest primary nurse in the NICU. There are many things "wrong" with Evan, but there are so many things that are RIGHT.

His lungs, heart and kidneys are healthy. He never needed oxygen and breathed on his own from the word go. He is strong and has been through more in 8 weeks of life than most kids go through in 20. He is HERE, and every morning when I wake up, panic hits, just for a moment. Then,  I lean in....and when I hear it, my heart skips a beat and a rush of joy fills my heart. I hear his steady breaths going in and out and I thank God for getting my baby this far.

Staying busy

Disclaimer: This post isn't so much about Evan. This post is mainly written for myself, and some very special young ladies, who will always hold a very special place in my heart. If you are not a member of the MCHS cheer squad, you may want to just skip this entry.

 The night before our scheduled C-section was spent in downtown St. Louis. We went out to dinner at our favorite restaurant (Charlie Gitto's) and then headed over to the hotel. In those hours, I received numerous text messages, Facebook posts and emails. These were filled with love, support and encouragement. Several from my cheerleaders left me in tears. To have these young women fill my heart with such love was a moving experience. When I held tryouts this year, I was 3 months pregnant. I had no idea what was in store for me, but I knew this season would be unlike any other. As we moved from tryouts to summer camp, Evan's medical issues came to light. There was a point over the summer where I wondered if it would be best for me to turn the squad over to someone else. Would I be able to give them the time and focus they needed? Would the drama and stress that comes from coaching high school girls be to much to deal with? I wasn't sure, but what I did know was that this group of girls is unlike any I've coached before. First of all, there was 23 of them, and that in itself is a lot for one person to take on. I thought about it, and I decided that I had worked to hard and put up with way to much over the past 5 years to walk away from THIS group. This is the group that, along with a wonderful middle school coach, I've gotten to work with since they were 7th and 8th graders. In addition to these ladies, came girls who transferred in, girls who had never cheered before, and girls that left volleyball to give cheer a chance. I decided that this maybe exactly what I needed. It would be a challenge, something to keep me busy, and hopefully, keep my spirits up. I can say without a doubt, that this is exactly what happened. (Thank goodness too, because this decision could have gone the other way and ended in disaster!)

 They are young, and as young ladies often do, they worry about things like who is sitting with who on the bus, how their jeans fit that day and if their makeup looks just right. These are the type of things they SHOULD be worried about. Sadly, they also have bigger issues. Things kids shouldn't have to deal with or worry about. I normally keep my life private (as much as you can in a small town ;) but I felt that maybe if I shared what I was going through, it would help them see that you can be faced with a less than ideal situation and still find the good. To stay positive and focus on the good things in your life, not just the bad. To get up and go on with your daily life, even when all you want to do is lay in bed and cry. That we are ALL stronger than we know.

 To say that we had a wonderful season would be an understatement. (I could talk about the reduction of drama, the fact that they rarely missed practice/games/community events, or their squad GPA of 3.6...but I think this story sums up the season better.)Early on (week 2 or 3) They cheered for 2 hours in the POURING rain (thank you, hurricane Issac!) with only 20-30 people sitting in the stands. Did I see a frown or two at first? Sure, but by game time, they were dancing and singing and having FUN. As I stood below the bleachers watching them the cheesy cliche was almost enough to make me laugh out loud. Here were these girls, dancing like complete goofballs in the pouring rain, soaked to the bone with mascara streaming down their faces, and they didn't care. They were dancing the rain with not a single care as to how they looked, or how uncomfortable they felt. They were making the best of a bad situation. I had started this season thinking maybe my positive outlook would rub off on them. And there they were, dancing in the rain. Showing me that even when life hands you lemons, you make lemonade.

 As the season drew to a close, I cherished each game. In years passed, as the season wore on, Id be frustrated by the cold weather, the drama, cheer moms...but this year none of that was an issue. Every game I made it to was one more I got to spend with "my girls." As I boarded that bus for our 1st, and then 2nd post season district game, I thanked God. Not only because of the extra time I got to spend with these wonderful young women, but because it was one more week of Evan's lungs developing. One more week of him growing strong. At our last game, these ladies surprised me with two framed letters, one to me, and one to Baby Evan. Again, I was amazed by their spirit and dedication to this squad, to each other and to me. The letter to Evan sat on a shelf in his room at the NICU, a constant reminder of some pretty special young ladies. I am so thankful I didn't hand this group over to someone else. They brought a great deal sunshine into my life and when I look back at that summer and fall, yes, there was heartbreak, sadness and worry...but there was also laughter, joy and many MANY memories.

 Love you ladies :) Mrs. Kim

Hope, Faith and Cardinal Glennon

Soon after finding out Evan had fluid on his brain ,we were referred to the Fetal Care Institute at Cardinal Glennon Children's Hospital in St. Louis. Growing up in the suburbs of St. Louis, I knew what an amazing hospital it was. The mix of emotions I felt pulling up outside before our first appointment were unbelievable  I was heart broken and thankful, all at the same time. When we walked into the office, the receptionist greeted  us by name and lead us to a room with a couch, a recliner, a flat screen tv and a computer. We met with our nurse who explained what the fetal care institute was, what they did and how they were going to guide us through the rest of this pregnancy. I had an MRI done and then a ultrasound. We met with neurosurgeons, a fetal care specialist, nurses, a nun, the geneticist, a social worker, our neonatologist and a doctor who's title and name I cant pronounce. Each doctor came into our little "living room" and spoke with us one on one. We were spoken to in terms we could understand, and the doctors never ONCE made us feel like there was no hope. I have heard horror stories from other hydro mommies. Tales of doom and gloom. My heart breaks for them because for us, we made it clear early on we were keeping this baby. We had faith and by the grace of God, we were paired with doctors who also chose to focus on the positive.

The MRI  showed exactly what we expected, and from this, our team of doctors gave us the diagnosis of Aqueductal Stenosis. For the next 3 months, we would come here monthly and meet with our team. Soon after Evan is born, he will be airlifted by helicopter to Cardinal Glennon. This is where his brain surgery will take place.  A shunt will be placed in his brain to drain the fluid. It is our hope that the brain will bounce right back into the space where the fluid once sat. It is our hope that he comes through the surgery like the champ I believe him to be. I hope he flirts with his NICU nurses and that his stay is short. I hope we get him home and his shunt functions just as it should, with no malfunctions or revisions needed. Hope. When dealing with a situation such as this, you can either crumble or you can lean on your faith and be hopeful. We choose to stay strong in our faith. Cardinal Glennon is amazing, it gave us hope that while Evan had a very scary and threatening issue, that he is in the best hands possible  This isn't a place you WANT your baby...but your heart is so thankful to have a team like this, in a hospital like this. Although I have a feeling our journey is just getting started I will never forget what this hospital has done for us. And really, they haven't even gotten started yet.

This will be my last blog post until after Evan's delivery (Tuesday, Nov 6th.)  I will keep you all posted by Facebook and when things calm down, Ill do my best to update this blog.

Much love to you all, and please keep Evan in your prayers :)

The diagnosis

Disclaimer: I am not a doctor. This is simply my way of explaining what is going on with EVAN. Evan's Hydrocephalus is caused by Aqueductal Stenosis.

We all have a brain. (Well, most of us do) Our brains produce cerebrospinal fluid .  This fluid flows through our ventricles (vents) and in normal cases, it carries nutrients and proteins to the brain while carrying waste products away from surrounding brain tissue. It is created, it flows down, and out of the brain. No harm, no foul. BUT, for some children (Evan for example) this fluid is blocked by an obstruction in the vent. The fluid cant drain, so it builds up, the vents expand, and this expansion pushes the surrounding brain tissue to the edges of the skull. This fluid is produced daily, so everyday Evan's vents are getting bigger and bigger. The fluid has no where to go, but over. Over into the area where Evan's nice and spongy brain matter should be. At our first ultrasound on July 9th, his vents were 18mm on the left and 21mm on the right. By August 11th they were 23mm on the left and 28mm on the right. I cant recall September's measurements but at last measurement, on October 11th, the left was 28mm and the right was in the 40's. To put this into perspective, his vents should measure 10mm on both sides. Evan's are nearly 3 times larger than they should be on the left, and 4 times bigger on the right. Did I mention this is the good news???? Let me back track a little bit and explain how we got to the "good news" :)

When we were first told something was wrong, we were urged to do an amniocentesis  We were basically told, you need to decide now, because you are currently at 20 weeks along. If we need to terminate, we need to decide within the next 12-24 hours. TERMINATE?? I couldn't even wrap my head around that word. I was NOT going to terminate my pregnancy  I was living in fear that I would lose this baby due to what ever was causing this issue. How on earth could they think I would end my babies life. And then they explained it to me....
There are many causes of hydrocephalus. Spina bifida, down syndrome, and Dandy Walker Syndrome just to name a few. There are also other causes, and with these, the child is not expected to live outside of the mothers womb. Trisomy 18 is the word that stopped me dead in my tracks. Being a sped teacher is both a blessing and a curse. I knew exactly what this meant and I realized that while I wouldn't actively terminate my pregnancy  I had to be prepared for what ever this birth may bring. That his survival may very well be out of my hands. The doctor left mark and I alone in our room to decide what to do. After 20 minutes of back and forth, we both knew we had to do it. Now, I am the girl who cold cocked her doctor with a right hook at age 5, when he tried to give me my booster shot. Needles are not my friend. The nurse kindly asked that I keep my arms down and asked mark to stand back. The pain wasn't nearly as bad as the worry I had over what that test would tell us. We waited on pins and needles for those results. When the initial set of results came back negative for downs and the two defects that are fatal, I felt as though we had won the lottery. 9 days later we got the news that there were no chromosomal defects. The relief I felt that day was unlike anything I had ever experienced  It was like a ton of bricks had been lifted off of my shoulders. I sat on my mom's couch and cried tears of pure joy. Something was wrong with my baby, but it wasn't nearly as bad as it could be. In that moment, I truly felt blessed.

Working through the guilt with Dr. google

Dr. Y told me NOT to Google this. He said it 3 or 4 times in the span of 5 minutes. The last thing Dr. Y said to mark as we left the office was this "Do not let her go home and Google this." This man obviously doesn't know me. How could I NOT search out as much information as possible???? And anyway, tell me NOT to do something?? You better believe I'm going to do it.

Everyday for the next 2 weeks it consumed me. If you were to sit down at my computer right now, and type in any combination of the words "hydrocephalus" "infant" and "fluid on the brain" I can assure you every single link that comes up, 20-25 pages deep, will be highlighted, showing the link had been read. In most cases, read, re-read and read again. The information varies, it is terrifying and scary. It is also uplifting and inspiring. Basically, in a nut shell-there is no way to know HOW this will affect Evan. It is most often said that you have a 50/50 chance. There is 50% chance that your child will have a mild delay, such as a speech delay or a motor delay.  There is also a 50% chance your child may never walk or talk. The pressure on the optic nerves can cause blindness...the fluid may result in so much brain damage that the child is a vegetable. No way to know, no way to tell.  This was week 20 of our pregancy. I had 20 weeks left of having to WAIT, not having any idea if my child would be one of the lucky ones, or if he would be severely handicapped. And this is when it hit me. THE GUILT. Not because I thought I had done something wrong (That was evident in my Dr. Google research) But because I was sad that I would have a special needs child. How dare I be sad??? The reason for this.......

I am a special education teacher. I LOVE my job, I LOVE my kids. I have worked in Special Ed for the past 10 years. I KNOW the love, joy and laughter that fill these children's hearts. I cried when one of my darling dears toured her new elementary school with sass and attitude, when walking through a door frame a year prior had caused major meltdowns. I've nearly done back flips when a certain little girl walked onto a bus for the first time without my aid. I cried the first time one of my little friends put the beginning AND ending sound on a SINGLE word he had practiced for MONTHS. I KNEW these kids, I LOVED these kids. They were my life's passion, and now here I was devastated that mine would carry that label. So often I looked to these parents with awe, amazed at their strength. I had said, on more than one occasion, "You were given this child for a reason." and here I was, worried about what would become of Evan. These children had been given to accountants, Brain surgeons, fast food workers, the unemployed  Here I was, with a degree in education, 4 praxis exams that basically certify me to teach special education at every single grade level...and I was wondering "why me" I worked through the guilt and it was apparent. The question wasn't "why me"" it was WHY THE HELL NOT ME??!!!!