Monday, June 30, 2014

VBS!

Last week was vbs at our church!  It was a fun (but exhausting!) week of exploring other countries and learning about the missionaries that work there.  We traveled to Chad, Indonesia, Japan, the Ukraine, and then discussed how we could be missionaries in our hometown.  It was a pretty cool idea and a great way to connect the kids with the missionaries our church supports.  

Caleb loved the music and learning about the culture of each country.  


Abby's favorite part was dancing to the music we sang!

I was a "mission mom" (group leader) along with my sister, and we had a good time...but like I said, it's exhausting! :)


Saturday, June 28, 2014

Look Who Abby Met?!

Elsa came to visit our local Walmart as part of a fundraiser for Children's Hospital.  For $5, Abby could get her picture taken with Elsa!!!  That's a whole lot cheaper and easier than trying to so it in Disney World, so we went for it!  

Abby was soooooo excited...until it was her turn.  So I got a picture with Elsa too! She is wearing an Elsa costume, but you really can't see it.  Either way, I know she will enjoy having the picture of her with Elsa for years to come! 

Wednesday, June 18, 2014

Tuesday, June 17, 2014

Plan M?!

I don't even know what plan this is at this point...M?

I called the jaw surgeon's office this morning, and he called me back within just a few hours!!  Wow!  I love that.  I shared with him that Abby will now have two surgeries three months apart with a jaw surgery in between.  He agreed that was a lot! 

I asked if we could possibly push the jaw surgery back to spring or summer, and he thought that would be fine.  He said if she has trouble recovering from this first surgery, then we will need to do the jaw surgery before the second one.  But if she's fine, we should be able to wait until after she gets through all of this.  We will keep the jaw surgery date that we have already scheduled, just in case we do still need to do it in November.

So...plan M.  How many more will we make?!

In other news, I made Pulmonology and ENT appointments for October and December, respectively.  It's so hard to think past August 6th!!  ENT was really tricky because I had to guess when she would be home from the second surgery! That one may need to be moved.

In other other news, our air conditioning is broken.  Here's hoping it is covered under our service contract, because the guy just came out the service the AC two weeks ago!  I don't mind being warm, but I do mind hearing my husband constantly complain!

Monday, June 16, 2014

A First

Wishing for a little good news?  Me too.  And I've got some!

Abby has been really struggling with eating...and that means we have too.  There have been lots of behaviors and drama surrounding any meals.  We decided to regroup and roll with Dr. C.'s declaration that "We done good" with the weight gain.  While we are still making sure she drinks Pediasure 1.5 and gets at least one good high calorie meal a day, we have decided to give her more autonomy in what she eats.

Enter IHOP, where kids eat free with each paying adult!  We ordered Abs silver dollar pancakes, eggs, and sausage!  Matt ate the sausage, but we were pretty impressed with how much she ate!  This was the first time we've ordered her her own meal, and something we can now cross off of her 2014 goals list!  :)


Saturday, June 14, 2014

Twice the Worry

We had a long meeting with the orthopedic surgeon on Friday who will do the VEPTR. I am very, very glad that we met with him.  It was worth the fourth whirlwind trip in five weeks. Bottom line: we can't wait on the VEPTR.  It needs to be done now.  So, our decision is made!

He thinks Abby will benefit most from attaching a permanent curved VEPTR after breaking and grafting the ribs to make them expand. (if it sounds painful, that's because it is!). This needs to be done on both sides, but is too risky to do all at once. She will have the right side done August 6th, then have an identical surgery on the left side 3 months later. This is not the "typical" VEPTR, but he feels it will be our best bet for pulling out her ribs and getting them to grow properly.

These are Abby's ribs now (images taken in February--the disc is a little whacky and I couldn't open the most recent CT.  They look the same though!).  There are 4 solid ribs at the bottom on her right side and 5 solid ribs on her left.  Remember that the image is backwards.  You can also see the scoliosis here.


This is what Abby's VEPTRs will look like.  She will also have one on each side going from the rib to the pelvis to keep her scoliosis and kyphosis under control, so she will have 4 appliances total.  They will be rib to pelvis for added stability.

This is what the rib to pelvis VEPTRS will look like.  
  
There's also a chance that the ribs she does have aren't strong enough to hold the VEPTR.  He said that xrays have lied to him and he won't really know until he gets in there.  With her rib gaps, she has a good possibility of having flimsy ribs.  If this happens, he will scrap the VEPTR for now, do bone grafts to make the ribs stronger, and wait 3 months before implanting the VEPTR on the right side.  Then she will have to wait 3 more months before getting the left VEPTR.

We've been told that after the first surgery, Abby might have some trouble walking.  The pulling that the VEPTR will do may make her off balance.  Dr. C. thinks that she will walk, but she will have to take some time to figure it out.  If you know me, you know that I will not let Abby get away with not walking!  We will work really hard at it until she figures it out!

Dr. C. is going to speak with our ENT and jaw surgeon at Hopkins to determine exactly what safety measures need to be put in place.  He also mentioned the possible need for a "short term" trach.  All three of the surgeons seem to be pretty off-handed about it...we aren't thrilled, to say the least.  But, I'd rather take a trach than the alternative.  We aren't planning on a trach, but I don't think it will be a surprise either.

This is even more involved than what we were already expecting. It's heartbreaking to have to put her through this big surgery twice. She is also scheduled for her jaw distraction in November, so she would have 3 major surgeries in 4 months.  Matt and I are considering postponing the jaw distraction until next spring, if the doctors are comfortable with waiting.  We have determined that the VEPTR needs to come first and if the reason for doing the jaw surgery first was to make it easier to recover from the VEPTR, then it shouldn't matter if we wait another 6 months.  Doing so many major surgeries in such a short amount of time is a lot on a little body.  I'm planning to call on Monday to see if I can chat with the jaw surgeon.

Please, please pray for Abby. She is such a special little girl. We love her so much and will do anything to keep her with us!!

Wednesday, June 11, 2014

Tuesday, June 10, 2014

CHOP Friday

After multiple phone calls to our orthopedic surgeon's office today to discuss the latest, Dr. C. wants to meet with us in person.  He said it is really hard to do all of this over the phone and wants to look at scans and reports with us. We're thankful for this because it really wasn't sitting well with us that we wouldn't talk to him about the specifics of the surgery until 2 days before it happened!!

So...of the dates we were given, the only one that was really feasible is this Friday.  A bit last minute, but I'd rather do it sooner than later.  It gives him more time to talk to the team and plan.  We will go up Thursday and hopefully be back home in time for our local Relay for Life!

Sadly, we will miss Caleb's last day of school for the second year in a row (last year we were at the feeding program).  It is what it is.

In other news, our garage door broke today and stopped a few inches from the top when I opened it.  I pushed the button and got in the car, backing out when it stopped going up (not knowing it had broken!).  It was just far enough down to take off the top spoiler part of the van that has a light on it.  It was still danging by the electric cords that run to the light, so we left it.  Matt took it to an auto body and is waiting to hear the estimate.  I called a garage door company to see about getting an estimate for the door.  They are coming tomorrow.  I will now have to look to make sure the garage door actually goes up the entire way before backing out...never had to do that before.  Ugh.  

When my sister heard the story, she asked if that pushed me over the edge.  Matt quipped that I went over the edge a long time ago.  He was joking.  I think.  

Karsyn's Karnival

Abby has a sweet little friend named Karsyn.  They are in the Young Athletes program together and have really hit it off.  She recently came over to play, and both girls had the best time!!   

Her parents run a fabulous carnival every year to help raise awareness and funds for kids with Downs Syndrome.  The money they raise from the carnival is donated to various organizations that support children and adults with Downs Syndrome.  It is getting very well known in our area, and I can't wait to see it grow even more!  It's such a fun CELEBRATION of kids with Downs Syndrome!!

Abby and I enjoyed watching the puppet show put on by high school students. Caleb and Matt played some games while we watched the show.

A petting zoo comes with goats, bunnies, and a turtle. They have pony rides, and an adoption organization brought kittens!  I never touched it, so my allergies stayed at bay.  Abby was so thrilled though!!


This year, they added a trackless train that was a HUGE hit!!  The kids loved riding it. I saw one little boy ride it at least 5 times.  So cute!  Abby enjoyed herself on it!


If you haven't been to the carnival before, I would highly suggest going next year.  It is so much fun and I love the energy and excitement there.  You should definitely go!


Monday, June 9, 2014

No Easy Decision

We're facing big decisions, folks. Ones that the doctors don't want to make for us because, as our ENT put it, "It's anyone's guess.  Don't believe any doctor who says he knows the right answer, because it's just not true."

We met with the new jaw surgeon today at Hopkins.  He is extremely knowledgeable and has a lot of experience with jaw distractions, which is why they brought him on.  We were really impressed with him and the before and after pictures he showed us of his past patients.

During the distraction, an L-shaped cut is made on both sides of the jaw and metal bars are put in.  The bars are attached to external screws that go behind the ear, which we will turn for 14 days.  The external screws will be removed after that, but the bars will stay in her jaw for several months.  Because of Abby's airway issues and the swelling around her face due to the surgery, she will be intubated and sedated for about 5 days before they attempt to extubate.  We should expect for her to be in the hospital for about 10 days.

We had a long conversation with the jaw doctor.  Long story short, he is concerned that Abby's airway is going to cause problems for her during/after the VEPTR surgery.  He was encouraged that she was successfully intubated, but he is fairly concerned that the swelling from surgery will cause her problems when her airway is already so tiny.  We don't want an emergency.

He also reminded us of a hard truth that we know too well:  with her airway and lung functioning being what they are, Abby is only a common cold away from the PICU.  We refuse to live in fear, so we don't isolate her from others.  We use a lot of hand sanitizer and try to avoid sick people, but now that she doesn't have a trach, we have eased up quite a bit.  But that fear is always there in the back of our minds.  Today, it was pushed to the forefront again.

His initial recommendation was to do the jaw surgery before the VEPTR, but that would mean pushing the VEPTR back.  Because the VEPTR will address the lung functioning, it is important to do soon too.  If we keep the VEPTR when it is, the jaw surgery would be in November.  We are tentatively scheduling it to get it on the calendar while we make our decision.  If we do the jaw surgery before the VEPTR, the earliest it could be done is September, pushing the VEPTR back until at least November.  But then that could mean she could lose more lung functioning between now and then.

We've got two big surgeries that absolutely need to be done. We know that. The question is, which one needs to be done first?

We have already consulted with our awesome pediatrician and ENT, our top two doctors.  Our pediatrician is calling our ENT to discuss it, and our ENT had a lengthy conversation with us tonight.  He agreed that the jaw surgeon had very valid concerns, and that Abby's surgery will be complicated.  No doubt.  He feels like in order to get the full benefit of the jaw distraction, we would have to wait 6-12 months after that before we did the VEPTR.  We can't wait that long and he knows that.  He also said that Abby's airway is tiny and the distraction won't "cure" that.  It will help and will get rid of the obstructive sleep apnea, but she will always have a difficult airway. (yay)  He encouraged me to continue to be vocal about Abby's airway with the doctors during her recovery from the VEPTR and not to let them attempt to extubate her until they are absolutely certain she will be okay.  We talked candidly about the possibility of Abby needing a trach again, but he assured me that it would be short term (less than a year) because she has proven that she can do it on her own.

The jaw doctor discussed the trach with us as well, and he told us that he gave Abby a 1 in 10 chance of needing it.  And I burst into tears.  And now he thinks I'm mentally unstable.  Add that to the chart.

I'll be honest.  The T-word makes me shudder.  It's not trach care or suctioning; that stuff is fine.  Been there, done that.  It's the nursing drama, the having to get a doctor's order for every single little thing (ie: do NOT wake my baby up to change her diaper!  True story!), having people in your house all of the time, the grand event it is to go anywhere at all because of all of the stuff you have to take with you, living in isolation because of germs, having to send a nurse with her to school and keeping her from being independent, not being able to drive by myself with her...ugh.  As our ENT said, he can't try to downplay trach life like he does for some of his other patients, because we know the truth.  We know how much of a life change it is!

But it's not about me.   It's about a little girl that I love to pieces and want to watch grow up.  So if a trach will keep her with us, then so be it.  But I will be praying fervently that she will be extubated perfectly with no complications.  

So new prayer requests:

*  That we will have clarity in our decision.  I am terrified that I will choose wrong.

*  That our doctors will all be in agreement.  This would make the decision-making process a whole lot easier!!!

*  That we will get to speak with our orthopedic directly.  We have some big things to discuss with him and don't want to have to go through the nurse practitioner.  I am hoping I won't have to get pushy.

*  That NO trach will be necessary at any point!!!!

*  That Abby will be successfully intubated and extubated with no concerns and all of the worrying and sleepless nights will be for no reason at all.

God got us this jaw appointment so quickly for a reason.  I know it.  I will not make light of the fact that this jaw surgeon had some very valid concerns.  I am praying that we will do what is best for Abby.  I just wish I knew the answer.







Sunday, June 8, 2014

BIG Medical Update Because I'm Big Time Behind...

I never want blogging to become a chore.  When I don't have time or don't feel like writing, I don't.  That's why I'm not a money-making blogger; I can't commit to blogging that much.  I like to write and it's therapeutic for me, but it is also put to the side when other things take precedence.

That being said, I've been short on time the last few weeks during our travels to and from Philadelphia and I have lots of medical stuff to share!

*  Abby had a dynamic MRI done on May 29th.  Our concern wasn't the MRI itself, but the intubation that needed to happen.  She was successfully intubated on the first try in just 15 minutes!!  We had anesthesiologists from the "Difficult Airway Team" (that in itself makes me happy!!) who were excellent.  They took detailed notes of what they did and what they saw the whole way down, and I couldn't have been more pleased.  Abby came out of anesthesia just fine too.  Once she woke up, she was in a great mood.  It's different than your typical post-anesthesia wake up when nothing hurts!  She didn't love the mask while she was being put under, but it helped that I was there to sing to her.  I think there's something to be said for the last face you see being your mom's.

Hopefully the face she saw wasn't like this scary one!  :)

The magic tutu stayed on until she fell asleep, no questions asked.  She's quite partial to the Elsa tutu!

Waking up and watching cartoons

*  The MRI gave the doctors some good information about her ribs and showed that her spinal cord is normal.  This is something they need to check out before they go operating on all of that stuff! We did find out that some of her vertebrae are fused and missing discs.  I don't think that means anything for the surgery...probably  just that she will have back trouble.  I figured as much!

*  During that same CHOP visit, we met with the general surgeon who will be involved with the surgery.  His job is basically to do the incisions and sew her back up.  He was intrigued by her rib gaps and had never seen anything like that before.  (Dr. C has operated on CCMS kids, but none since being at CHOP!  Apparently, CCMS is the only syndrome that causes ribs to be in pieces like that.  So special...) He said her surgery would surely be "exciting," which is not quite what I was going for...it is what it is!  She will have large C and J-shaped incisions from the shoulder blade to down below the ribs on both sides.  Then she will have an incision down most of her spine.  She may have some incisions at her pelvis if it is determined that they can't attach them anywhere else because her ribs aren't strong enough.  They probably won't know that until they go in.
This is a good look at the scars, except that Abby's will be on both sides.

This is what the VEPTR looks like from an xray.  Abby's will be on both sides like this one.

*  We met with our ENT at Hopkins, who scoped Abby to take a look at her tonsils and adenoids.  That was fairly traumatic for the poor girl, but it needed to be done.  Her tonsils look fine and she doesn't have any adenoids--that solves that problem!  I asked if that was an issue and he said that's just one less thing for him to take out.  Sounds good to me!

*  This past Thursday, we left at 4:30 a.m. for Philadelphia again to meet with the pulmonologist...if I'd known it would be a two hour wait before we actually saw the doctor, I would have left later!!

  She had another pulmonary functioning test that showed her lung functioning to be a little better--50%.  He thinks that's pretty accurate and didn't have much to say other than that.  A lot of the questions about surgery we had for him couldn't be answered because it all depends on her how long she is intubated, whether she will need a ventilator, if she'll need CPAP post-operatively, if she'll need a chest tube, etc.  We will just have to wait and see how she does.  I'm praying that she will get her tube out in the OR and won't need any of that stuff!!

*  We got into the Ronald McDonald House this time, which was great.  After Abby's pulmonology appointment, we were able to go back to RMH and play all afternoon.  It was a beautiful day, so we spent a good amount of time at the playground!  A musician came to do a kids' song singalong in the evening, which was adorable.  Abby had a great time with him.  RMH is awesome.  It's so hard keeping Abby happy in a tiny hotel room!






*  We were supposed to meet with the orthopedic on Friday morning, but he had an urgent medical matter of his own to attend to.  It's no one's fault and we weren't angry, but it is unfortunate that we couldn't get our two pages of questions answered!!  The nurse practitioner did her best and promised to get the answers to me soon.  I know they have a detailed plan for surgery, but this type A girl needs to be informed as well.  I can't let them cut open my girl without knowing some information!!

*  A definite praise is that Abby has met her goal weight and we can cut back a bit on the calories.  There's not quite as much pressure to make sure she eats, which is good considering that her eating behaviors have been terrible this week!!  Feeding is such a struggle.  I'm looking forward to starting feeding therapy again now that we've met the goal.  I need help!!

*  The one thing we were kind of upset about is that Abby's surgery date got changed to a week later.  It's now August 6th. They've known for 3 weeks and forgot to tell us.  If we'd known then, we wouldn't have made some of the plans we did.  I think that everything has been switched now and it turned out okay, but it's frustrating when you plan your summer around a date and you have a husband who has a really tight schedule!

*  Tomorrow we are meeting with the jaw surgeon at Hopkins to discuss the jaw distraction.  It won't happen before the VEPTR, but we are thinking it may need to be done in the fall after she recovers from the summer's surgery.  We know she has trouble breathing and the jaw is what is causing that.  If bringing it forward and opening it up will help her breathe better, we'll do it.  And buy her Frozen memorabilia to make her smile through the pain.  Poor, sweet girl.  I hate that she has to go through all of this.

I think that's it.  Shew.  That's a lot.  It makes my head hurt just thinking about all of this.  When I write, I sometimes almost forget that this is Abby I'm talking about...not some fictitious kid with whom I'm just a third party.  This is my daughter who is going to have to endure all of this.  God has entrusted us to support her and to make the best decisions we can for her.  At the end of the day, I have to know that we are doing what is best for her. I know the VEPTR is the right choice, but it doesn't make it easy.  I hope one day a healthy, middle-aged mom named Abby will look back on all of this and know that we made the right decision to choose the VEPTR when she was three.