Going HOME!

Sorry I didn’t update sooner! When you’ve got a grumpy, recovering 2 year old who wants the iPad, blogging is not really an option!

Evie’s nose looked PERFECT! It was beautifully open and had no scar tissue growth. We are so incredibly relieved. We’re definitely not out of the woods yet, though, and they want to see her back in 6 weeks to check it again. If it has stayed open, then we may be able to move on to tonsil removal and jaw distraction.

We also got some great news from the audiologist after she did the sedated hearing test (an ABR). Evie’s hearing on her right side is good enough that she feels she’s getting language very well! Evie just needs just a bit of amplification and she’ll be good to go. She thinks it’s probably not worth it to try aiding the left ear, though. That ear is bad enough that if we amplify, it’ll be difficult for Evie to understand speech. The audiologist said most adults with that level of difference typically don’t like aiding the bad ear as it makes it difficult to listen with the good ear. She also saw no signs of auditory neuropathy and thinks we can just take that off the table!

Our plan of action then is to continue pursuing a BAHA for Evie because it’s possible her fluid issues in her ears will return, but we can start wearing her regular hearing aids again. She just stressed that we should only leave them in for short periods of time so we don’t close off that ear and make fluid return MORE likely. She thinks Evie will have little need for Cued Speech since her hearing really is that great and suggested we just focus on sign language to give Evie the expressive language she needs while she’s unable to vocalize. With how Evie is already communicating with blowing raspberries, she was very optimistic that once the trach comes out, Evie should be able to talk someday!

We’re heading home this weekend and cannot wait to be back in our own space. Three weeks is just too long to be away!

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The Journey to Trach Free Has Started (and I’m Not Ready)!

We’re still here at my parents’ house resting and waiting, but leave tomorrow for Cincinnati again. It’s been so nice to see our other daughter and relax a bit. I wish we were home, though.

When we left for Cincinnati, we thought this would just be a “discovery” trip. They’d do some tests, figure out when they would want to do her tracheal reconstructive surgery and then head home.  Some of the tests required her nose to be open, so we thought they would open it, place stents, send us home, and then we’d come back in a few weeks for the testing. It honestly never occurred to me that they would attempt to open her nose once and for all. We feel like we jumped onto a moving train. Or maybe we thought we were touring the train briefly and it took off for its destination instead.

Don’t get me wrong.  There is a part of me that is excited that we’re starting the process to hopefully get the trach out, but I just wasn’t quite ready to start yet.  We’re out of practice! We’ve been spoiled by the slower pace of the last year.  Before this trip, Evie’s last surgery was June of 2012!  We’ve had fewer medical appointments, but increased therapy. It’s been such a nice year for Evie’s progress. I just really don’t want to go back to constant appointments and surgeries.  Wow, that’s pretty whiney of me.

So that’s where we’re at. We’re still just stunned that the journey has started.

Wednesday’s scope will hopefully give us a good idea of what the process to keep her nose open will look like. If she already has extensive scar tissue growth, we may be staying longer to have it opened up again. If it’s looking pretty good we may be able to head home for a few weeks before they need to look at her nose again. We’ll just have to wait and see.

Quick Update

Sorry I haven’t updated you all! Evie’s surgery went very very well. They ended up just keeping her overnight! They did decide they want to scope her nose next Wednesday to check for scar tissue growth, though, so our trip just got longer. I have to check with a few doctors, but I think we’re probably going to stay at my parents’ house in Indiana so we can be with Aleyna.

I’ll try to post more once we’re settled in at their house!

Thank you for the prayers. It really was so much better than we expected it to be! So grateful.

Here’s Evie enjoying the beautiful balloon our church sent to her. When we got discharged, she insisted on holding into it the whole time.

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Extra Info and Tomorrow

There were a few things I forgot to mention with the information overload of Wednesday.

First, Dr. Cotton wants to be more aggressive in figuring out Evie’s ears. The constant drainage is just unacceptable to him and we’re hoping we’ll find a solution. We had always been told it was just a CHARGE issue and that someday, hopefully, she would grow out of it. We’re really excited that maybe there are some other directions to try since it is either uncomfortable or downright painful for Evie all.the.time.  They cleaned out her ears and put in her 4th set of ear tubes and now we’re on antibiotic/steroid ear drops plus an oral antibiotic.  He’s going to take another look tomorrow in the OR and see if there is any progress.

I also forgot to mention some scarring that was discovered below her trach site. We’re not sure if this scarring is just something that happened awhile ago or if it’s something that’s still happening.  The scarring indicates that the tip of the trach has been rubbing against the inside of her trachea and irritating it. They will look again today to see if there is any change. If it is still a problem, we’ll need to go to a custom trach to get a better length. She’s still too tiny for the pediatric size (she’s in the neonatal length still) and it would likely hit right at the point where the trachea splits in two to go down into the lungs, so we’ll have to figure out a custom length. I’d really prefer that not be necessary as it increases the costs of her trach and makes it harder to get them. We already struggle with her trachs being constantly backordered which gives me a nice panic every.single.month that we won’t have enough.  And the few extra trachs I’ve hoarded away will become useless. So basically, if you could pray this is just an old problem so we can stay with her current trach size, that would be great. 🙂

I don’t think I said that Evie’s esophagus looked fine, either.  I was pretty scared that we would find her esophagus inflamed and scarred up from refluxing/vomiting so much, but the GI thought it looked fine. Very good news.  He’s waiting for the results from some biopsies he took, but he may just take her off reflux meds since it appears her vomiting issues are just related to the size of her stomach rather than reflux. We’ll just have to wait and see and probably have a little trial and error in approaching the problem.

So tomorrow is Evie’s surgery to repair her choanal atresia again. It does look like her nose is blocked by just tissue and that the bone itself has not grown back. Evie had 4mm of bone blocking both sides of her nose originally which is pretty severe (Evie doesn’t like to do things halfway in airway issues, I guess).  I had said that they were going to remove part of her septum that divides the nostrils. I asked around on the different Facebook groups I’m a part of it and it sounds like most people saw a huge success once part of that bone was removed.  That is incredibly encouraging.  Even though Evie’s body does love to grow scar tissue, she does seem to eventually accept whatever we’re doing and stop the growth.  We had horrible issues with her trach and gtube sites at the beginning, but after awhile they both calmed down and we had no problems again until just recently (she’s had some issues with her gtube site again, but it’s nowhere near as bad as it was at the beginning).

I don’t really know how tomorrow will go. She’s going to be hurting from the surgery and we’ll have to be doing flushes and drops in her nose which will not be pleasant for anyone.  I’m going to be having flashbacks to her NICU days. Ugh.  As hard as it was to hand over my 12 day old baby to a surgical team, it is WAY harder now that Evie is almost two.  She’s aware. She remembers. Once she was fully awake this past Wednesday and realized where she was and that she was hurting, she went into a full panic, clinging onto me for dear life. It was heartbreaking.  Tomorrow, we won’t be able to just quickly dress her and get out of recovery so she can calm down.  I’m not even sure I’ll be able to hold her at first.  I’m concerned about how much drainage there will be from her nose. Since her stomach is so small, whenever she has drainage, she can’t handle much food at all.  She’ll probably lose a significant amount of weight over the next few days.

Please just pray that it goes better than what we’re expecting.  Sometimes I wonder if we’ve become pessimists over the last two years, but I think we’re just extremely realistic when it comes to Evie. We’re hopeful and we know that she continues to surprise and amaze us, but we also know that she doesn’t just surprise us in good ways.  She’s a unique and complicated little girl, that’s for sure.

A few quick stories of Evie’s awesomeness:  before surgery/scopes on Wednesday Evie was playing on the iPad.  We gave her the stylus (pen) and let her play on a drawing app. Ty showed her ONE TIME how to drag up from the bottom to bring up the color selection and then drag down to put it away again. She’s drawing away and then drags up, picks a new color, drags down, and goes back to drawing! Ty and I just looked at each other, stunned!

Then yesterday, we were just hanging out at the hotel and every time I would pick up the iPad, she would crawl over and sign “share”! hahahaha.  She’s just amazing.

Thank you again for all the prayers and encouragement. It’s wonderful to have such support!

The Scoop

First of all, thank you all for your prayers. Evie did great with the procedures and anesthesia and is just a bit grumpy from it all.

Waking up from anesthesia.

Waking up from anesthesia.

GI and pulmonologist both had good news. GI didn’t find any of the problems he suspected except one: she has a tiny stomach that doesn’t have the ability to expand very much. Most stomachs have lots of folds in them so they can expand with a meal, but Evie’s has very few folds. This explains why we’ve never been able to do very large volumes (she can only take about 3 ounces in a feeding).

There should be way more folds in her tiny stomach.

There should be way more folds in her tiny stomach.

In case you ever wondered what her gtube looks like from the inside of her stomach.

In case you ever wondered what her gtube looks like from the inside of her stomach.

He also noted that while her Nissen fundoplication is still intact, it is so small that it is basically doing nothing for her, so that explains why she still throws up quite a bit. So I guess the downside of these two tidbits is that we’re still pretty stuck with her feeding issues. There’s not really much we can do except wait for her to grow. Overall we’re very relieved that she doesn’t have any serious GI issues that will require surgery, though. The pulmonologist said Evie’s lungs looked great and are very normal. We’re still waiting back on some cultures, but anatomically, she was very pleased.

Now the more complicated stuff.  First the good news: the ENT feels that someday Evie will be able to get the trach out! Yay! Unfortunately, she has quite a few airway obstructions that we didn’t know about, so it will be a long process of taking care of that list.

Here’s the current plan.

  1. Open up her nose this coming Monday. Her nasal passages were completely blocked off with scar tissue.  Her CT scan showed that she had a tiny hole, but they couldn’t find it during the scopes.
    Can you tell what it is??? That is looking from the back of her nose towards the nostrils. Notice anything missing? Yeah, that would be two holes.

    Can you tell what it is??? That is looking from the back of her nose towards the nostrils. Notice anything missing? Yeah, that would be two holes.

    With her first two repairs, both sides of her nose were opened separately and this next time they are actually going to take out some of her septum that divides the sides and create one big hole in the back. Since the hole starts off bigger, it will hopefully take longer to close up again. It doesn’t sound like they’re going to do stents to keep it open like I was originally told since the data doesn’t really show better results with stents. Once we’re pretty sure her nose is going to stay open (which could take 1 surgery or 15 surgeries, no way to know) then…

  2. We’ll get her tonsils removed. Apparently her tonsils are extremely large and block off a good portion of her airway. This is a pretty common procedure for CHARGE kids. After that…
  3. We will get a consult with a plastic surgeon to address her small jaw. I think we’d been told before that her jaw was small, but no one ever mentioned that it could be an issue.  Unfortunately because her jaw is so small, her tongue sits back further in her mouth and falls back into her throat a little, blocking off her airway. This is a little bit of an intense procedure involving cutting both sides of the jaw bone, attaching metal brackets and slowly turning screws to lengthen the jaw. The new bone grows in and then is given 6 weeks to harden before the pins and brackets are removed. It sounds like kids do very well with the procedure and it’s usually harder on the parents. Here’s a good link with some slideshows explaining the procedure more. Then finally after that…
  4. We will do her tracheal reconstruction surgery and hopefully remove the trach right away. Evie’s tracheal scarring is bad.  They said it’s not the worst they’ve ever had, but it is very severe. The opening into her trachea right now is only 3mm wide. That’s about the size of coffee stir straw.

    This is the 3mm hole left after the scarring occurred. You can see the slightly darker tan area that is supposed to be open.

    This is the 3mm hole left after the scarring occurred. You can see the slightly darker tan area that is supposed to be open.

We don’t really have a timeline because Evie will need to heal fully from each procedure before moving to the next one, but it will be at least a few years. The ENT said most CHARGErs have their trachs 5-10 years.

We’re both happy and sad right now. We’re so glad that they think someday she’ll be trach free. That’s wonderful. But it will take us awhile to adjust to the knowledge that she will be having many more surgeries than we had anticipated. We just wish things were easier for her.

We have a few days off now and plan to just rest and enjoy some time as a family. The weather is beautiful here and we’re hoping to get outside a bit with Evie. Next step will be her surgery on Monday with a few days inpatient.

Thank you again everyone. We could feel your prayers keeping us calm and focused this morning.

Day Two Complete. Day Three Prayer Requests.

Evie, Evie, Evie. Why? Why do you insist on throwing curve balls???

First the non-curve balls.

CT scan this morning went smoothly. Child Life put on an episode of Yo Gabba Gabba while they started anesthesia and she barely even noticed people were around her! All went fine during the test and we got to be with her both as she fell asleep and woke up. The only issue they had was getting a good stick for injecting the necessary contrast dye into her veins. I counted at least 10 jabs. So so glad she was asleep for that! She decided to go straight from anesthesia into a nap and we spent an hour or so in recovery while she slept.

Later in the afternoon we had our consults with GI and pulmonary.  I was always glad that they had all three specialties work together, but I really only thought of them in regards to Evie’s trachea.

There’s a look that we’ve seen time and time again from doctors in regards to Evie. It’s a bewildered look that says “Whhhhaaaat?” and we saw that look A LOT in the consult with GI. Now this is not someone who just doesn’t have enough experience. This is a doctor who has seen hundreds of CHARGErs plus tons of other complex kiddos.  Every new bit of information we gave him made him pause and you could tell he was just trying to figure out how all the pieces fit together.  We’ve always had issues with Evie vomiting, but had always attributed them to the trach. He has some other theories and possibilities.  He explained quite a few, but the two that stood out were the possibility of a bad placement of her gtube in her stomach or that she’s not actually physically able to get food/secretions into her stomach due to esophageal problems or issues with her fundoplication (which was put in place to help with reflux).

Further explanation: Evie had issues with her gtube getting sucked down into the stomach exit (the duodenum) back when she had a gtube with a balloon on the interior.  Her surgeon mentioned that her stomach was shaped a bit oddly, but no one ever mentioned it again since switching to a different gtube style solved her problems. The GI here thinks it’s possible the new gtube is still blocking and/or hitting the duodenum which would slow her stomach emptying and possibly make her vomit.  The solution to this could be as “simple” (*not actually simple) as redoing her gtube placement with another surgery.

Further further explanation: Evie has what’s called a Nissen fundoplication where the top part of the stomach is wrapped around the eseophagus to prevent refluxing. This was done when Evie got her gtube as testing showed she both refluxed and aspirated and they worried about her getting pneumonia or being unable to breathe.  If this is too tight, she might not be able to swallow her secretions so they just sit in her espohagus until they gag her and then she throws them up. The force of that brings up some of her food as well.  It’s also possible that this could be happening, but because of issues with her esophagus rather than the fundoplication.

Too much info? Yeah, we thought so, too. It was a great appointment and we learned a lot, but boy, was I exhausted afterwards. I’m trying to adjust to the possibility of finding more issues and dealing with yet another diagnosis.  Overall, he said he just needs more data and he’ll be able to see quite a lot in the scopes tomorrow. It’s very possible he’ll want to do some other testing while we’re here as well.

Pulmonary didn’t really have any surprises, which was nice.  Evie’s lungs sound great and looked very normal on the CT scan, so all good news, but it doesn’t mean we won’t find something in the scopes.

So….yeah.  It seems like it’s always the appointments that I think are just routine where we get hit with new things.  We’re doing okay, but now we’re nervous about what will be found in the scopes tomorrow in regards to her trachea AND GI system.

So prayer requests:

We have another rough night ahead of us with feedings at the last moment before she has to stop all feedings for surgery. Pray we don’t get too run down and can actually sleep in between those feedings (10:30pm, 2:30am, 4:30am).

Pray for Evie tomorrow and her reaction to anesthesia. She’s never had anesthesia two days in a row before and anything new makes me nervous.

Pray that any issues found tomorrow are FIXABLE! Pray that they see hope in what they discover.

Pray for us to be able to clearly understand and remember what they share with us after the scopes.

And pray that we can get back to the hotel quickly after surgery and rest.

Thank you again for all of your prayers!

Day One Complete. Day Two Plans.

It was a long day, but overall, I think a good one.

Her cardiology appointment went well and the doctor didn’t make me feel like I was a completely crazy person for wanting another opinion.  She noted the issues in the Evie’s EKG (lag in communication between the two sides of her heart and an abnormal electrical direction) and she didn’t feel they were hugely concerning. She did decide to go ahead and piggyback a sedated echo and angiogram onto some of Evie’s other procedures, though.

We had a little time to kill in between appointments, so we went and got Evie’s walker out of the van. She had a blast! Usually in hospitals, Evie just clings to me like a little koala baby, eyeballing everyone, thinking they are going to hurt her.  But today, she was independent! She was free! And she loved every minute of it. She walked all over, smiling at everyone, getting in the way, and following people. She’d walk straight up to groups of people, wait for them to look at her, babble away, and then move on to the next crowd. It was so neat to see this social side of her personality finally get a chance to shine!  And it was so fun for us to show her off. We really never get a chance to be out with her, so it was pretty nice to just soak up the exclamations of how cute she was!

 

We then headed over to her swallow study which was a total bust. We tried to get her to take a nap before the test, but she wouldn’t hear of it! But then in that dimly lit radiology room, a nap started sounding pretty good and soon enough she was sitting up in Daddy’s arms, completely unable to keep her eyes open. We were able to wake her up, but she was pretty uninterested in swallowing. The speech pathologist (wisely) decided that we should back off and not risk setting her back in her therapy. She’ll be having another swallow study called a FEES, but that requires going through the nose (which is one of the main reasons we’re opening her nose up next week).  She zonked out about five minutes after that and slept hard for the next few hours.

We were then able to meet up with a CHARGE mama that I’ve “met” online. Their little guy is six months and is scheduled to go home next week. It was so nice talking with another mom that “gets it”!  We had such a good time just sharing our stories.

Then we had to rush up to our next appointment because I had it in my head that it was at 2:30 and not 2:00. Oops. This was a consult with anesthesia, a chance for them to ask question about Evie and for us to ask questions about their procedures. It went well and Evie loved showing off in her walker up there, too.

We were done for the day with appointments, but ran up to meet the little six month old CHARGEr! Wow! Talk about blown away. He is just so interactive! He makes eye contact, grabs toys, laughs at his daddy, smiles at his mama. Just amazing. Evie didn’t have nearly the time in the hospital that he’s had, but she took a long time to warm up to us and start interacting like that. I’m just so excited for them to get him home! So please be praying that he stays healthy and doesn’t decide to throw any more curve balls at them! Oh, and I should note that the whole time we were up there, Evie just toodled around in her walker having the time of her life!

Today Evie has two appointments. First at 8:15am she has a CT scan of her chest to look at her airway anatomy.  They like to do this the day before the scopes so they know what to look for the next day.  She will be under general anesthesia and I’m not thrilled about anesthesia two days in a row, but the anesthesiologist said the time under is a more important factor than the frequency (not that you’d want to do it every day!). Both procedures are fairly short, so she shouldn’t be under very long total time. We got up a few times in the night to give her feedings so that she’s not running on an empty stomach today, so we’re pretty worn out (and we’ll have to do this again for tomorrow’s procedures as well).

We then can just hang out until her next appointment at 2:30pm. This will be her consult with the GI and pulmonologist who will be doing scopes tomorrow. They’ll want to know more of his history and issues so they know better what to look for tomorrow.

Please pray for Evie while she’s under anesthesia. I’ve said before that CHARGE kids can react unpredictably to anesthesia and sedation, so even though Evie has done well thus far, we still don’t like putting her under one bit. Pray the CT scan goes well and they get a good picture. They are also tacking on that angiogram (and possibly the sedated echo) to have a look at her heart so please be praying for that (I’m not sure how I want you to pray there. I do NOT want them to find a problem, but if there is a problem, I want them to find it! We’ve been so uneasy about her heart and I just want things resolved one way or the other. So I guess if they don’t find anything, pray for our peace of mind.). Continue to pray that we don’t get sick, especially with how little sleep we’re getting. And maybe just start praying now for the scopes being done on Wednesday. I’m very nervous about what they’re going to find.

Thank you again everyone!

Day One

Well, we are here and have a busy day ahead of us.

Evie sees cardiology at 7:30 this morning. Please pray that we will either feel comfortable with Evie’s heart just being a bit weird, but fine or we’ll find whatever it is that is causing some of her issues. And if there is a problem, that it is minor and fixable.

Swallow study will be done at 11am. Pray that this isn’t too traumatic for Evie. She can’t actually fail the swallow study (it looks to see if she is allowing food into her lungs) because her trachea is blocked, but pray that it gives them the information they’re looking for.  I’m just dreading them forcing Evie to swallow nasty stuff. She HATES these.

Anesthesia consult at 2:30pm. I suppose just be praying that we feel good about allowing this team to care for Evie while she’s under anesthesia. It’s a hard thing as a parent to pass your child off to a stranger whose sole job if to keep them alive during surgery. And Evie’s complexity makes it a tricky job.

That’s our day. Long and busy. Also, please pray for me. I haven’t been able to sleep well the last few nights and am starting to feel it. Just pray I can stay healthy and focus on what needs to be done during the day.

Thank you everyone! We so appreciate your support and prayers!

Evie needed THREE babies in the car. She's such a good little mommy.

Evie needed THREE babies in the car. She’s such a good little mommy.

And then she told me off for taking her picture.

And then she told me off for taking her picture.

 

Cincinnati Schedule

I just got our schedule for Cincinnati and wanted to share it so you all know how to be praying.  These times are in Eastern time zone.

4/8 Monday: 7:30am Cardiology (we just want a fresh set of eyes on Evie’s heart)
11am Videoswallow (this is a live xray that shows us how Evie swallows)
2pm Anesthesia Consult

4/9 Tuesday: 8:15am CT Scan of her chest under sedation
2:30pm Clinic Appointments with GI and Pulmonary

4/10 Wednesday: 9:30am (arrival 7:30am) Evie will be put under anesthesia for scopes done by GI, Pulmonary, ENT and will also get a 4th set of ear tubes. This should be an outpatient procedure.

 

Then we have a nice break where we’re hoping to do a few fun things as a family.

 

4/15 Monday: 1:30pm Bilateral Choanal Atresia Revision (Evie’s nasal passages will be cleared of scar tissue so that they can do further scoping and testing. This will be the 3rd surgery on her nose and they expect her to be inpatient 2-3 days afterwards)

 

So there it is. A busy few days.

Prayer Requests for the next few days:

  • Smooth travel with nothing forgotten!
  • That Aleyna will do well at my parents’ house while we’re in Cincinnati.
  • Staying healthy!!!!!!

 

I’ll post updates throughout our trip and share more specific prayer requests. Thank you everyone!