We are in Vegas, baby!!  When the TCH transport crew came to pick us up at 7:30 am, I had the biggest grin on my face!   I was so excited to see them that I think I was talking much faster than normal and could not stop smiling.  They loaded Hannah up on the stretcher, grabbed our suitcase and bags, and we were off!

After a quick 30 minute ambulance ride to the airport, I saw the Cessna Kangaroo Crew jet.  It was awesome!  If I could find my camera adapter, I would upload the pics, but those will have to wait.  I will admit, it was pretty cool flying in a personal jet to Vegas.  The nurse and the respiratory therapist on board were fantastic and very engaging with Hannah.  She needed a bit more suctioning than usual, and they ended up giving her just a bit of oxygen support because the air pressure changes brought her O2 sats to where they hovered in the low 90s. 

I saw the most incredible view of the Grand Canyon as well.  It was so much more massive than I had ever realized!

Once we touched down in Vegas, we were met by a local EMT ambulance who drove us to Sunrise Children’s Hospital.  The “rule” is that they have to transfer ICU to ICU, so since Sunrise did not have a PCU step down unit, we were back in the PICU.   We got settled in fairly quickly!  

The rules were different than at TCH so that Ethan and Abigail could actually come into the PICU and spend time with us in Hannah’s room.  I was thrilled because they had not seen Hannah in almost 6 weeks!   We also got to put Hannah in her regular clothes instead of naked/diaper or hospital gown. 

As soon as Hannah saw Ethan (he ran to her bedside), she lit up like I have not seen since she was hospitalized!  He was making her laugh, making her want to move, and just so happy to see him!   When she saw Abby, she even tried rolling over on her side towards her!    It was beautiful, beautiful to see her not only recognize her brother and sister, but obviously she missed them too!

I also could not stop hugging Ethan and Abigail.  I had not seen them in almost a month!  Abby was attached to me like crazy, but I did not mind one bit. 

We met with the ICU attending, and he felt there was no reason to keep her in the ICU because she was doing “much better than he was told.”  His logic was that most of weaning can be down as a closely-monitored outpatient, because she would likely recover better being at home than sitting in a hospital bed all day.  At first I was very nervous about this because I was told by TCH that it could be a couple of weeks in this hospital, but then I realized that we basically just spent the days in the hospital room, never really needing any medical support or assistance other than just regular care help like getting Tylenol, getting a bath, or trying to silence the heart rate monitors that would not stop giving errors because of Hannah’s movements. 

And after seeing how motivated Hannah was after seeing her brother and sister, I know that she will do much better with a normal home life than lying in a hospital bed all day long.

So the tentative plan is to start the weaning process again at Sunrise tomorrow and use them to help us come up with a lifestyle plan for us to settle in here.   This includes a G-tube class, nutritional guidance to figure out a feeding plan, setting up PT and OT, coming up with a med schedule, setting up home medical supply services, setting up home health services, finding follow up physicians, etc.   Fortunately, we took all our trach care and change classes at TCH, so that is off the list!

If all goes as hoped, we could get Hannah home in less than a week!!  

As for me, I am now sitting in my new home in my new office on my computer I have not touched in a month and a half.   My father-in-law came to the hospital to sit with Hannah so that Daddy could drive me to our new house, give me a quick tour, and then he was off to spend the night with Hannah at the hospital.  My inlaws took my kids for the night so that I could just crash and relax.

And I did.  About 5 minutes after Daddy left, I went upstairs to my new bedroom, crawled into bed, and went right to sleep.  I just woke up a bit ago after a 7-hour “nap.”  Of course, my days and nights are still off too, but hopefully I can get some more sleep later today.   So for now, I am just vegging at my new house, having quiet “not having to worry about beeps and monitors” time, and trying to decompress.  After 6 weeks in a hospital and coming to a new home, I feel a bit out of place, but I will get over it.

We are “home” now.  In Las Vegas.  Our family is back together in the same city.  If all goes as hoped, we will all be back under the same roof soon.  Hannah has a long, long recovery ahead of her to get her back where she was, but if her attitude she had when she saw Ethan today is a sign of anything, she will get there!

It is now 5 am on Saturday morning.  Hannah fell asleep (as did I) at around 9 pm, and she woke up at midnight.   Her nurse and I got her back to sleep a couple of times, but that lasted only 10 to 15 minutes.  I have been up since midnight.

Barring any emergencies (like the Kangaroo Crew needing to transport an emergency patient), they are coming to pick us up at 7:30 am.  2-1/2 hours!!!   They want to be out of the room by 8:05 am.  Why the :05, who knows.  Just get me on the plane and get me to my kiddos!

I spent yesterday saying  ‘goodbye.”   We had good bye visits from Dr. Eng (Hannah’s TCH genetics doc) and Dr. Roy (Hannah’s ENT).   I gave thank-you cards to the PICU nurses, the PICU desk staff (who automatically put my name on the Ronald McDonald house list every morning so I could sleep in and not be down there at 7 am), and to the Ronald McDonald staff as well.

I told Dr. Roy that doctors like him and Dr. Bhakta really have spoiled me, and I have come to expect so much now.  Unfortunately, I am learning that doctors like them are very rare, and we just got very lucky.   It was also great therapy for me to be able to talk to him about what I am truly feeling about this whole situation and experience and get his very honest opinions as well.   At least he can’t get rid of me — we plan to keep in touch by email and phone, and he offered whatever assistance he can give.   We will look into removing the trach in a few months, but I just know in my heart is isn’t going to be a yes/no decision, as we still have the Gaucher type of progression in the back of our minds.

What we have left to deal with is — weaning from the Ativan (supposed nightmare), continue weaning the pentobarb and the chloral hydrate, chorea movements, seizure issue, possible worsening reflux (on Zantac now, but arching her back a lot!), and gas issues (perhaps need new formula?).   Hopefully they will continue daily PT and OT there, and as soon as we get settled there, I want to talk to the social worker to see what programs Hannah qualifies for and hope to get the process started for the Katie Beckett program (similar to Texas’ medically dependent children’s program).

I hope, hope, hope we are headed to the airport in a few hours.   The “plan” is to have us at the new hospital by 11:00 am-ish  Vegas time.   Shortly after that, major hug time for Ethan and Abigail (and Daddy will get a couple too!)

Yes, we are still in Texas.  Why you ask?  Because the plane had to be taken in for maintenance today, so therefore we are “scheduled” for tomorrow (Saturday) to be transferred to Vegas.  I say “scheduled” loosely because I got my hopes up to be leaving this morning, and found out about noon yesterday that we had to be postponed for another day.

I am SOOOOO ready to get to Vegas.  I am sitting here counting down the hours right now until tomorrow comes, and it is not even 10 am right now!!  GET ME OUT OF HERE!!!

Hannah seems to be doing better overall.  Her chorea movements have improved to the point where I would probably rate them a 3 out of 10 now where she was 10 out of 10 at its worst.  She is actually sleeping during the night now, about 6 to 8 hours (interrupted a few times).

Also, for the first time ever, she is actually taking a nap RIGHT NOW!  She has not napped during the day, a good nap, since we moved up here!  She has now been asleep for 45 minutes!!  PT has walked by twice to see if she was ready to play — nope!

Bad news is that Hannah failed the swallow study yesterday.  She aspirated the liquids and sometimes aspirated the thicker, pudding-like foods.  The thought is that because the chorea movements affect her tongue movements, she can’t control her swallowing mechanism that well.   Hopefully this will be temporary — good thing she will get another swallow study at the NIH in September when we go back.  Until then, she will be fed via Gtube primarily.   We were told we can try thicker foods like the pudding and refried beans on a “special times” basis, but for the most part, we are to let her just keep chewing on toys.

That is the one thing I have a hard time wrapping my brain around.  She was such an oral child.  She lived on the pacifier.  She loved her bottles.  She always had a Little People or other toy in her mouth.   Now she can’t even control her hands even to hold a toy, let alone put it in her mouth.  She can’t even suck on a paci right now because she can’t get her tongue to do what it needs to.

Rounds are hopefully going to start soon, and my little girl is starting to wake up.  Maybe she heard me typing and thought that 45 minutes was long enough to nap?

Hannah got a decent night sleep last night, finally!  She fell asleep at 5:30 pm (as did I), and she slept off and on for about 8 hours within a 10-hour period!  That is the best sleep she has gotten since we have been starting to get her off sedation!   And even right now, it is a bit before 9 pm, and she is finally asleep.  I hope, hope, hope that she does just as well tonight as she did last night.  Since she is not napping, this is the only time her body really gives herself time to recover.

Hannah met with her OT today who worked on spoon and bottle feeding again.  She was working with the butterscotch pudding, swallowing it (when it was put on the side of her mouth), but then she coughed up a bit of it into her trach HME (container that collects secretions and humidifies incoming air).  This is not good.   It means that she aspirated the pudding into her lungs instead of it going down the esophagus.   Needless to say, a swallow study is scheduled for early tomorrow morning to see if we now have an aspiration problem where we did not before this whole mess.

I changed out Hannah’s trach for the first time tonight.  We had done the CPR and trach change classes, practiced on dummies, done the daily trach care (changing out the collar and cleaning her neck).   That is the last thing to check off the list in terms of the things parents must do before they are discharged.  It was funny because I was so nervous because I had never seen Hannah’s stoma (hole) before, but it was really not a big deal.  So now she has a Shiley no-cuff trach, size 3.5 (for other SN moms).

Earlier today, Hannah’s attending came to visit us (which was so unusual since he had never done that before).  He wanted to let me know that Hannah and I “have our plane ticket,” and we are leaving at 8 am on Friday morning for Las Vegas!!!   What makes this cute is the transfer coordinator came by later in the afternoon asking me if the attending came by.  Apparently he was very excited for us, and he wanted to be the one to share the news.   She joked that he “stole her thunder,” as she likes being able to give good news.

But we are ready for transfer on Friday morning.  To say I am excited is an understatement, a huge understatement!!!   All the coordination is being finalized, and there is an attending there who will be there on Friday to take over Hannah’s care.

I am so close to seeing Abigail and Ethan, gosh I miss them so much!  On a separate note, I am so proud of them — they got their report cards… Ethan got all A’s (in the gifted and talented class no less), and Abby got mostly S’s (obviously talking too much and not listening is not her strong suit yet!).

It is 9 pm now.  Hannah is asleep, and soon I will be too!   My schedule is so off too — I have been up since 3 am this morning!!

Lots of phone calls were made back and forth between the Vegas hospital and TCH.  How do I know this?  Because all conversations seem to take place at the nurses desk outside Hannah’s window!  Unfortunately, I couldn’t make out details of what was being said, but it was definitely about the transfer.  I’m hoping Thursday we get to transfer, but no one seems to give me any details.

Hannah had  a rough day Tuesday.  She is just so damn uncomfortable!   There seems to be nothing I can do to comfort her, and that is so hard to admit, let alone watch.

She had a bedside swallow study.  Hannah’s OT, Ashley (who I love!), brought a tray of food to see what Hannah could tolerate eating.  I laughed because I saw the ice chips, ice cream, cheerios, applesauce, muffin, and a few other things, and I thought to myself “there is no way she is going to eat any of that!”   Sure enough, she eventually sucked on the ice chips and enjoyed them.  She reluctantly took the ice cream and applesauce by soon.

Then, we went to the bottle of apple juice.   She would put it in her mouth and chew on the nipple, but apparently the chorea movements also affect her tongue, and she just does not have a useful suck reflex anymore.  No wonder she does not like her paci, she can’t suck on it!   This broke my heart.  She is 99% bottle fed, and now she can’t suck on a bottle right now.  Thank goodness we got her the Gtube!   Otherwise, we would have ended up having to get one at this point.

She had PT later in the afternoon, and she tried so hard.  Jay, our new PT, put her on her tummy to see how she would do with tummy time, and she just could not get the coordination and strength to push herself up.  She wanted to so badly, as you can tell when he held her chest when she was trying to push up from the ground.

I’m angry that her body has become so thrashed because of all the sedatives.   Shouldn’t the doctors have known that throwing that many sedatives to her while she was intubated would have some kind of addiction effect like this?   Plus add in the chorea movements, and she just is miserable, absolutely miserable.

I’m looking forward to going to Vegas to get a new set of eyes on her.  Even though both neurology and the PCU team have submitted their plan for Vegas to continue what we have been doing, I just need to get her out of here.   I know that having her family around her, besides me, will be great for her, as I feel lost as to try and do things with her because there is just nothing she can do — can’t play with toys, can’t stay still enjoy for me to read to her, can’t watch TV for more than a few minutes.  The worst thing is that she just does not like to cuddle that often anymore, so even that does not help as much as it used it.

Hopefully Wednesday will bring some better news…

We met with Dr. Roy yesterday, Hannah’s ENT. He is one of those doctors that you just feel a trust with, someone that is going to tell it to you straight, but also willing to actively “ride the ride” with us. Dr. B., our pediatrician, searched him out for us after our first ENT wanted us to proactively trach Hannah back when she was 11 months old because of her laryngomalacia and her prognosis.

We starting seeing Dr. Roy back in September of 2009, and yesterday was the third visit with him.   He noticed pretty significant laryngomalacia and some vocal cord weakness when he did his first laryngoscope (camera down Hannah’s nose while she was awake) as well as moderate reflux.   Back then, he heard the stridor sounds Hannah made when she was upset and the breathing issues when she was upset.  The trach was always in the picture, but we were able to walk away from the two previous visits with “We will review where we are in two months.”

Then we had Hannah’s multiple gagging/breathing episodes, two of which landed us with 911 at the ER.  I thought for sure we were close to having to make the trach decision.

We stopped solids and put Hannah on a liquid diet.  Not a single gagging episode in the 6 weeks that we started that.  Not even a minor one!

So when Dr. Roy came in yesterday, Hannah yelled at him.  He actually loved hearing that because he had never heard a strong vocal from her before.  We explained that she does now yell and has a stronger cry these days.  He also thought she looked really good too, which she does.

He did another laryngoscope today, and Hannah was a trooper.  This was her 5th time having that dang camera put down her nose into her throat.  But the results were GREAT!   He was so pleasantly shocked and surprised that Hannah’s laryngomalacia has significantly IMPROVED since our last visit plus her vocal cords were much more active — the vocal cord weakness has IMPROVED as well!  Her reflux is still there but definitely looks better.

We never expected an improvement in Hannah’s symptoms, especially the ones that are so closely tied to her Gaucher’s disease.  But it happened.  Her laryngomalacia and her vocal cord weakness have definitely IMPROVED.  Say it with me, IMPROVED!!  I love saying that!!!

We all agreed that we would rather have to deal with a gastric tube in feeding Hannah if it comes to that than having to deal with a tracheotomy.  For whatever reason, a G-tube just does not seem anywhere near as drastic as a trach.  Dr. Roy also agreed that a G-tube is much easier and safer to deal with than a trach.  But we are not at a G-tube yet, as long as she keeps her weight and keeps growing, we are going to stick to bottle feeding.

So all of the breathing/gagging/feeding issues we have been dealing with for the past many months have been all related to her hypersensitive defensive gag reflex, a neuromuscular nGD issue.  But no laryngospasms, no breathing issues, nothing else.  We have dodged this bullet for now (I know it may happen in the future, but for now I’m rejoicing!).

IMPROVEMENT!!  We drove him from this appointment, and I had a HUGE smile on my face the whole way home.  It was just so unexpected.  It wasn’t that I was expecting it to be much worse (though I was expecting some worsening), but to have it significantly improve?  WOW!!!