Growing and Getting Better

Big news (at least for us it is) !  Now that Gabe is a year old, he needs a formula with more calories that will provide him with all the nutrients that a growing boy needs.  So today we began the process of switching to Elecare Jr.  The formula he was on before provided 24 calories per ounce and the junior formula will be 30 calories per ounce.  That’s a big jump in calorie intake!  We tried switching him to a 30 calorie formula when he was 4 months old, but his body couldn’t handle it and he started dumping (food passes through the digestive system without being absorbed) .  He’s older now, so we’re hoping that his digestive system has matured enough for him to handle the increased calorie intake.  IF he can handle the new formula and continues to gain weight, the surgeon will pull the Broviac!  WHAT?!  I never thought I would hear those words.  The central line being gone will make mine and Josh’s lives so much easier and Gabe’s life far less dangerous.  No more sterile line changes, no more chance of line infections (thus no more freaking out everytime he has fever) , no more weekly blood draws.  CAN YOU TELL THAT I’M EXCITED?!  …..Of course there’s always the worry that he won’t continue gaining weight once off the TPN and lipids and then he will have to have a new Broviac placed.  But we are trying to focus on the positive. 

Gabe, of course, is doing well.  We did tummy time today and he only cried for a few minutes.  After he finished his little fit, he laid there sulking until I turned him over on his back.  But at least he didn’t cry the entire time.  He’s getting better!  We’ve been working on him supporting his weight on his legs lately and he’s doing AWESOME with that.  He can support almost all of his weight!  Just today we met the occupational therapist that will be working with him.  Some of the goals she set for Gabe are: improving his torticollis and practicing head control by making him do more tummy time.  We are also working on getting Gabe a speech therapist.  The speech therapy will focus more on feeding and improving any oral aversion that he may have.  I am so excited to see the improvements that are right around the corner now that we have a more aggressive therapy in place!

September is a super busy month for us (8 appointments scheduled, all on different days) but I will try to keep y’all updated! 

1st Birthday Party

Gabe had an AWESOME first birthday party!  We rented a cabin at our local state park for the party and then spent the weekend there with Josh’s mom and stepdad.  Little Mr. Attention Hog loved seeing all the family at his party.  We sang and signed the birthday song and he was mesmerized with that.  He loves watching sign language.  For party fun, Josh made a banana piñata to match the Curious George theme; which was a success!  Josh is definitely the artsy one of the family.  Gabe had lots of fun the rest of the weekend spending time with his Yaya and Popi and playing with all of his new toys!  Besides it being his first birthday party, last weekend was also another milestone.  It was Gabe’s first time spending the night away from home other than the hospital!  Wooo!  He did great, and amazingly, with all the supplies we had to bring, we only forgot a couple things, haha.  Attached are some fun pictures from the birthday party!

The epic birthday cake

Gabe with his Yaya

The birthday boy!

Signing Happy Birthday!

Tasting chocolate ice cream!

Chocolate boy.

Opening presents

Gabe with his Aunt Misty and cousin Maggie.

Gabe with Aunt Adrienne

Gabe and Vic

Our little family

Gabe with his Papaw and Mimi.

Gabe and his cousin, Chloe.

Gabe and his cousin, Lilly.

Gabe and his Aunt Danielle

Gabe and Popi

Relief

IT’S OVER!  Woooooooo!  Words cannot describe the relief I felt when I saw the neurosurgeon walking toward us, smiling.  She was very happy with how the procedure went, and now it’s just a matter of time to see if the ETV actually fixed the problem.  During the surgery, a lot of fluid drained out so obviously the fluid level is better now.  In about a month, Gabe will have another MRI to see if the fluid is holding at the correct measurement.  Fingers are crossed!

Of course, Gabe wouldn’t be Gabe if his anatomy weren’t completely abnormal so it wasn’t surprising that the surgeon was thrown for a loop when she got into his brain.  In her words, “everything was a little off “.  Haha, sounds like him.  His ventricles are a different texture than normal, and he’s got some kind of flap partially covering the foramen.  Those little abnormalities aren’t going to affect him so we’re not too worried.

Beforehand, we were warned that about 10% of kids will have to stay on the ventilator for awhile after brain surgery.  But not Gabe!  He was breathing over the vent during surgery and came right off of it afterwards!  That’s our boy!

After he was settled in and awake in his PICU room, we were finally able to see him.  He was super mad when we walked in but after giving him his paci, he was asleep in just a couple seconds.  He slept really well for a few hours and when I saw him stirring later that evening, I went to his bedside and started talking to him.  The first thing he did was give me his heart-melting sheepish little grin where he smiles with his whole body and then covers his face with his hands.  It was so awesome to see him smiling just a few hours after surgery.  Within 10 minutes of being awake, he was waving his arms and legs, playing with toys, smiling and doing all his normal activities.  Man, what a trooper.  The doctor had prescribed him 3 different kinds of pain medications, including morphine, and Gabe didn’t use any of it.

Everything went really well for the 2 days we were in the hospital.  The recovery from his brain surgery went great.  He also got another IV iron infusion while we were there and he had no problems with that.  For a month or so, he’s at risk for infection from the surgery so we are watching him closely for those signs.  He seems completely back to normal, besides his hair…and that’s gonna take weeks to get back to normal.  The surgical gel/betadine look is not is best look.

For the time being, we are assuming the surgery was a success unless we see otherwise.

Thanks to everyone for all the thoughts, prayers, texts, phone calls, visits, and hugs.  Each one was very much appreciated!

Now that that’s over, it’s back to birthday party planning mode!  Less than two weeks!

Truth

I’m trying not to think about it.  I’m trying not to think about the fact that my baby is going for brain surgery next week.  I’m trying not to think about part of his head being shaved or the incision the surgeons are going to make in MY BABY’S HEAD.  I’m trying not to think about the chance that this procedure might not work and that he will have to have a shunt placed.  I’m trying not to think about the big artery they are going to be working beside.  I’m trying not to think about Gabe having a massive hemorrhage and becoming a vegetable or dying.  But that’s all I can think about.  On the surface I’m great.  I can talk about my confidence in the doctors and the high success rate of this procedure and the low risk of infection and blah…blah…blah, but deep down inside it’s killing me.  And I just want July 24 to be here and be over and the surgery to go great and us to get back to our normal life.

New Territory

We are venturing into new territory this month.  On July 24, Gabe will go in to the hospital for his first, and hopefully only, brain surgery.

We’ve known that Gabriel had ventriculomegaly (a milder version of hydrocephalus) since before he was born.  In all his months in the NICU it never grew and didn’t cause a problem.  When we went for his genetics appointment, the doctor noticed his full fontanel.  During an ultrasound that day, his hydrocephalus was discovered.  We saw the neurosurgeon a few weeks later and after looking at him she suspected that he would need surgery a few months later to relieve the buildup of fluid.  Gabe’s two options for relieving the fluid were a shunt or an Endoscopic Third Ventriculostomy (A procedure where they put a camera down into his brain and use a blunt object to make a hold in his third ventricle.  The fluid is then able to drain properly and is reabsorbed into the lining of the brain.) .  Because of his brain anatomy and his age, Gabe is a good candidate for the ETV.  There’s about a 5% chance of the hole closing up and then the surgeon would have to go back in and place a shunt but we will cross that bridge if, and when, we come to it.

Last week we went for a checkup with the neurosurgeon, Dr. Notarianni, and she noticed that the veins in his head were popping out a little bit more than before and his fontanel was bulging more than last time so she recommended we do the surgery sooner rather than later.  She believes the reason his hydrocephalus is increasing now is because he lays on the back of his head so much.  Lying flat the majority of the time is causing crowding in the back of his brain and thus, making it difficult for the fluid to drain in a timely fashion.  Dr. Notarianni also recommended Gabe be fitted for a helmet which will over time correct the shape of his head and hopefully help relieve the hydrocephalus along with the ETV.  So Gabe will be getting another accessory soon!

Great news on the esophageal dilation front!  When we went for Gabe’s dilation last week the surgeon saw that he had held the measurement from 3 weeks ago!  We will have to come back at the end of August to check and see if Gabe is still holding at 14.5 and if he is then we will try even more time in between each procedure.  Hopefully soon we can stop the dilations all together!

On Friday, I was bragging to Dr. Boykin (Gabe’s surgeon) about making it 3 months with no line infection.  I was so proud of us!  Then on Friday night Gabe ran a fever.  We ended up taking him to the ER that night because we couldn’t get his fever down.  The doctor put him on antibiotics and sent us home.  On Saturday afternoon, the home health nurse came out and did a peripheral draw for a blood culture.  Then on Sunday evening we ended up taking him to the ER in Shreveport because he had started having bad diarrhea.  They discovered that Gabe had a UTI so they gave him a shot of antibiotics and told us to keep him on the antibiotics he was already getting from the ER visit on Friday night.  We got home about 2:30 Monday morning and a couple hours later I got a call from our home health agency saying that Gabe’s blood culture had come back positive.  Great.  Since he was already acting like he was feeling better and wasn’t having fever anymore, the doctor decided to let us stay home but she switched him to IV antibiotics.  We’ll have to stay on the antibiotics for the full 10 days but so far all of his other blood cultures haven’t come back.  No news is good news when it comes to blood cultures.  Busy busy weekend but we are glad Gabe is feeling great again!  Restart the clock on no line infections, dadgummit!

Less than one month till Gabe’s first birthday!  We are so excited!  I’ve always said that I wouldn’t be one of those moms that had the huge birthday parties.  But when you’re baby struggles to breathe for the first few months of his life, you feel like he deserves to celebrate his first birthday in a BIG way.  I’ve promised myself that the next one will be smaller….maybe.

Busyness

It seems like life has been especially busy the last couple weeks.  Between doctor appointments and visits with family we’ve been away from home more than we’ve been at home.  Gabe is handling it so well though!  He used to be so tired after a day out of the house but he seems to enjoy it now.  He likes seeing new places and loves the attention at family gatherings.  Speaking of family get-togethers…Gabriel’s 1st birthday is next month!  After talking with his surgeon we decided that Gabe is well enough to have his little cousins at his party!  I’m pretty sure I embarrassed myself in the doctor’s office jumping up and down and clapping when the surgeon gave us the okay for that.  Keeping with the good news, we took Gabe for his first grocery shopping adventure this week!  He did absolutely marvelous!  He was very interested in all the new sights, and just talked and played in his stroller the entire time.  He’s growing up so fast and learning and enjoying new things.

On June 24, Gabe went to the hospital for a 3 in 1 procedure; tubes placed in his ears, ABR (sedated hearing test) , and CT scan of his ears.  Tube placement went well.  In the recovery room, Gabe was dropping his oxygen saturation into the 80’s.  After watching him for awhile, just to be safe, the doctor admitted him for observation that night.  He was super fussy and nothing was soothing him so I didn’t really want to make the drive home anyway.  The next morning he was doing great so we packed up and headed home.  We haven’t found out the results of the CT scan yet.  The ABR didn’t go was well as we had hoped.  He does have profound hearing loss in both ears.  I expected it, but it’s still disappointing.  We have an appointment with the cochlear implant doctor this month to just talk about that option.  We really don’t know what we are going to do.  It’s not a decision that has to be made now so we’re not rushing anything.

This weekend, Gabe’s night nurse said that his lungs sounded really bad and she was afraid that he aspirated after the swallow study.  He was happy and playing, no fever, o2 sat was fine…just the bad sounding lungs.  So we headed to our local ER for a chest xray.   The chest xray looked good.  An hour later, we were headed home.  First ER visit without an hospital admit.  SCORE for the Stapletons!

Gabriel went for a swallow study last week.  I had brought a bag full of different flavors of yogurt and baby food hoping that he would be able to swallow something.  The radiologist was going to let me in the room to feed him so I was very hopeful.  We got all settled in and Gabe was very happy lying on the xray table.  I started feeding him but as fast as I could shovel in the food he was spitting it out.  We couldn’t get him to swallow any of it.  Spoon feeding was a no go.  On to the bottle.  I tried to get him to suck and swallow but I don’t think he even sucked hard enough to get anything out.  When the speech therapist started working with him, he got mad.  As a last resort, we squirted a syringe full of milk into his mouth just to see if we could trigger a swallow.  By this time he was super upset and just spit all the milk right back out.  So this swallow study didn’t go as hoped but there’s always next time!

It’s been a busy few weeks but thankfully we’ve only got 4 doctors appointments coming up in July!  Yay for more down time at home!

Updates

First and foremost, we are home again!  We are so excited to be back together as a family and in a normal routine again.

In other news…

Gabe was scheduled for a swallow study on Monday but it got cancelled and he had another esophageal dilation instead.  Gabriel’s esophagus had held the stretch from the last dilation, 13.5 mm, and the surgeon was able to dilate it to a 14.5 mm.  The surgeon told us that 14.5 mm is the right size for Gabe and since he’s holding the measurement between dilations, we are moving the dilation out to every 3 weeks!  Twelve down, hopefully less to go!  Progress, wooo!

I was finally able to talk to the neurosurgeon on Tuesday and find out the results of Gabe’s MRI.  The MRI showed a few different brain abnormalities, all are associated with CHARGE.  He has some crowding in the back of the brain which makes it difficult for the fluid to drain properly, resulting in the moderate hydrocephalus.  The neurosurgeon predicts that eventually he will need a procedure to correct the drainage problem.  There are 2 different procedures that would alleviate this problem.  The first option is a shunt that would empty the excess fluid from the brain into the abdominal cavity.  The second is an Endoscopic Third Ventriculostomy (ETV), which is procedure where they use a camera and go into the 3rd ventricle of the brain and poke a small hole that would enable to fluid to drain properly.  The surgeon thinks that an ETV will work well for Gabe because he doesn’t need another foreign object in his body, like a shunt.  The MRI showed that his optic nerve is very small, but since his vision is fine that shouldn’t be a problem now.  Also, his corpus callosum isn’t fully formed which could affect his speech development.  So, no real good news but nothing emergent or terrible.  We’ve learned that life is easier if you take the punches and roll with them, and thankfully this punch didn’t hurt too bad.

Diagnosis

We got some good news this week.

For most of Gabe’s life we’ve researched and read, trying to figure out what caused him to be born with so many problems.  My mom was the first to bring up CHARGE syndrome to the doctors (big thanks to her for pointing me in the direction of the right diagnosis!).  The neonatologist told us that it was a possibility but after two chromosome and DNA tests came back normal, the diagnosis wasn’t pursued any further.  While we were still in the NICU but after things settled down a bit, I began actively researching CHARGE and became convinced that that would eventually be Gabe’s diagnosis.

In more than half the kids with CHARGE syndrome, there is a mutation in the CHD7 gene, which is located on the long arm of chromosome #8.  Finding this mutation requires a specific test.  When we went in for Gabe’s genetics appointment back in May, the geneticist ordered it.  We got the results back this week…

Gabe has CHARGE syndrome.

The news didn’t come as a surprise but definitely came as a relief.  We felt like we needed to celebrate.  After so many months of struggles and questions, CHARGE seemed like the answer.  I’d heard the doctor say on more than one occasion, “he’s one of a kind, not like any other kid” when talking about his complex array of medical issues.  But now we had a reason for all of his rare and complicated conditions.  With this diagnosis, we would know better what to expect long-term and we could understand past issues now that didn’t make sense back then.

Two years ago, if someone had told me that our son was going to be born with a genetic mutation that would cause many birth defects that would, in turn, require multiple surgeries, prolonged hospital stays, and cause developmental delays I would have been crushed.  Maybe, we might have even considered not having a kid.  If I had been told about the CHARGE diagnosis while I was pregnant, I would’ve focused on the bad news.  I would’ve been consumed with the heartache and it would have been hard for me to consider the joy that a child would bring.  If we had known when he was first born, it would have been a huge weight that would’ve had a negative effect on us. But that has all changed now.  I see that smiling face everyday and I forget about the hard times and the lifetime diagnosis.  I forget about the fact that he can’t sit up or crawl yet and remember that he’s starting to hold his head up now.  I forget that we spent the first 7 months in the NICU and remember that we’ve been home for almost 4 months.  I forget about the hearing loss and focus on his great vision.  The hard times are extremely hard but our happy times are extremely happy.  Yes, Gabriel has CHARGE syndrome but it does not define him.  He is medically fragile but so strong.  He has struggled to live but he lives happily. 

We wouldn’t trade our boy for any healthy kid in the world.  He is ours and he is his own kind of perfect.  We are completely fine and happy with the CHARGE diagnosis.  We know that he is so much more than just a diagnosis, and we can’t wait to see what he has in store for us in the years to come.  If anything, we know that because of him, our life will never be boring or “normal”, haha.

Feels Like Home

We’re back.  Back in the hospital, that is…for the fifth time since his discharge from the NICU.

Gabe hasn’t been himself for the past 2 weeks.  At first I thought he was just trying to get over his last sickness , but then we started to wonder if it could be his low hemoglobin and hematocrit or maybe lack of nutrition.  So we went in to see his doctor yesterday to try and figure out what was wrong with him.  After looking at Gabe and talking to me and my mom, the doctor decided to admit him for a few days and do some tweaking.   We were concerned that he’s wasn’t getting adequate nutrition so they changed his TPN recipe;   he’s been having a lot of gas lately so we’re switching formulas;  his H&H has been low for months so they gave him a blood transfusion;  his iron levels are low so they’re moving him to the PICU for 24 hours to give him some IV iron;   and he’s on an antibiotic for possible C-diff.  Lots of little changes, but hopefully it will be what he needs to get back to his crazy self.

These last couple months have been a roller coaster ride with all the re-admissions and a guessing game for us when trying to decide if/when we should make an appointment or take him to the ER.  But there’s a security and familiarity in being back at Willis Knighton Hospital.  I’ve spent the better (and the worst) part of the last year here. Walking through the hallways at Willis Knighton, I see the faces of doctors, nurses, and RTs who have become like family to us.  Most of the staff knows us by name and each asks about Gabe’s progress and are really happy with us when he’s doing well.  I never imagined that a hospital could feel so much like home, but since we’ve spent so much of our time here, I’m glad that it can and does.