It is almost 10 pm here, and Hannah has been asleep for 4 hours – hallelujah!    That has been her average amount of sleep for the past few nights, even with the addition of benadryl (which we are stopping because apparently that causes urinary retention when used with other certain meds).

It is such a beautiful thing watching her sleep.   With the exception of the few hours after she wakes up where she starts showing signs of personality and happiness, the rest of the day she is incredibly miserable — crying, agitated, wrestling around, bored, half-awake, and exhausted.

There was a bit of mini-drama yesterday when the attending on call (not our normal attending) saw an abnormal T-wave on Hannah’s monitor, so he ordered an EKG.  With Hannah’s lack of urine output from dehydration, her tachycardia, and her other withdrawal-related issues, he was concerned that things were becoming more complicated with cardiac involvement.  Fortunately, it all turned out to be a false alarm — at least we know her cardiac issues are stable now that she has had an echocardiogram and EKG this hospitalization.

Things seem to be a go for our transfer to Sunrise Children’s Hospital this week.  Our discharge planner told us that we were approved and will be taken via Kangaroo Crew fixed-wing transport (air ambulance) “towards the end of this week.”

We are at the point now that Hannah just has to be weaned off these horrible sedatives that are still in her body.   We have a plan from her intensivists here at TCH as well as her neurology team so that the new team at Sunrise Children’s Hospital can continue on this plan and modify it if needed.   Our main goal is to get her through the withdrawals and then once those are more stable, then we will tackle the remaining choreoathetoid movements (if they still are present) as well as the seizure issue (which everyone thinks was due to hypoxia from her breath-holding spell and not a seizure disorder).

Our fellow feels that Hannah will likely be in the Vegas hospital for another two weeks once we are there before they can continue the weaning as an outpatient.   I can handle two more weeks in a hospital as long as I get to see Ethan and Abigail every day, all the time, as well as be able to take a break every once in a while — Daddy can spend the night alternatively so I can actually get some sleep in our new home — the home that I have yet to see in person in the neighborhood we are living that I have yet to see!

Time passes so slowly here.   Hannah is so miserable when she is awake, and there is very little we can do with her.  She has PT and OT, which have been a great distraction, but she has a hard time watching TV, and she just can’t play with any toys yet (not even hold them) because of her withdrawal and choreoathetoid movements.  She is just frustrated, bored, and exhausted.

But the good thing is she is still sleeping as I post this.  Thank goodness!   Every hour of sleep she gets these days is a huge step towards progress of getting her through the next few weeks.  The more sleep she gets, the stronger her body gets to fight.

To end on a humorous note…. the nurses have basically forbid anyone to come in here unless absolutely necessary because she is asleep and has been asking people not to come in!   The housekeeping guy came in here and started trying to clean up in here a bit ago, and they basically scooted him out the door!

Because Hannah was able to get some rest last night, the docs decided to give her one more day off on weaning any meds.  She spent the entire day agitated, uncomfortable, and just not happy for the most part.  One of us (me or Daddy) needs to be right at her side all day long because she is just so miserable and withdrawing so bad.  I’m so relieved that Daddy was able to extend his visit here until Monday night, because I don’t think I would be able to make it all by myself.

They also let us transfer Hannah to a adult-sized bed instead of the cage (I mean, the crib).  We were hoping that cuddling her would make her more comfortable, and we had a sign a waiver since it is against their policy.  The irony is our nurse suggested it!  Our plan was that one of us would sleep with her in the bed, and the other one would sleep in the couch bed.  True to Hannah form, when she fell asleep, she was right in the middle and completely stretched out!  Like we are going to move her now that we got her to sleep!  So Daddy and I are tag-teaming sleeping (I slept from 10 pm until 1 am).

The PT came by to work with Hannah today.  She worked on trying to get Hannah to sit up and to stand (for weightbearing purposes).  Hannah worked so dang hard, but her body is just so thrashed.  Her neck muscles are so weak that she flops like a newborn.  It made me very sad.   All that work over the past year plus with all her therapies have been wiped away with a single hospitalization.

Even though Hannah was awake all day, she actually fell asleep around 9 pm after Daddy and the nurse gave her a bath, and I was combing her hair.  Even though we come it out every night, today she developed a huge mat in the back of her head, and I was able to comb out most of it, but I did ending having to cut out a little chunk of her hair because it was just beyond tangled.  Unfortunately, they don’t have good hairpieces here because everything we use falls out pretty easily.

Hannah spent the day on the HME (humidifier nose) attachment on her trach and did well.  Tonight, she is sleeping on CPAP set also at room air.  Our goal for nighttime is to get her to only a humidified vent and not CPAP.  But I don’t think they are going to touch that until our regular attending comes back on Tuesday.

The resident came in about an hour ago (midnight) and was worried because Hannah has not peed in over 11 hours, and she barely peed this morning.  She has had no issues with her kidneys since we got here, so now I’m nervous about why she is experiencing urinary retention now.   They want to come back in about another hour and do an ultrasound on her bladder to see if there are any signs of anything.

Please, please Hannah pee.  We want to get you well and at home and don’t want you to have to go through any more complications like acute kidney failure or something.

Yesterday was a tough, tough day.

Good news is that Hannah’s choreathetoid movements are now finally starting to show signs of lessening. This gives us hope that these neurologic movements are only temporary, most likely drug induced.

Bad news is that now we are starting to wean the final sedatives (pentobarb, methadone, and ativan), the withdrawal is really starting to take its toll on Hannah’s body.   With the exception of two 30-minute naps, Hannah was awake, thrashing, and completely inconsolable for 36 hours straight!  It was exhausting and horrible to watch.  Even our night nurses were trying to do everything they could to help coerce her to sleep.  Daddy and I ended up taking naps at the hotel across the sleep on shifts just so we could each get some sleep (uninterrupted by alarms, monitors, and people) just because Hannah needs one of us at her bedside at all times.

And the irony of this is that she can’t get a sedative to put her to sleep because that is the problem that got us here in the first place!  We are no longer dealing with the adenovirus or pneumonia at this point, it is just the dang polypharmacy that was required to keep her sedated during intubation.

That is the reason I didn’t post an update last night.  By the time I left the hospital at 9:30 pm and walked to our hotel room, I think I stayed awake for an entire 10 minutes of television before I crashed.  Then, I got up at 4:30 am to come back to the hospital to relieve Daddy so he can go back to the hospital and get some sleep.

I was able to get her to sleep for about 20 minutes yesterday, about 30 minutes after her pentobarb and methadone, and she became truly apneic (forgot to breathe) for more than 30 seconds until the nurse came in and we aroused her.  Of course, I was fearing that she has now developed sleep apnea (a symptom of Gaucher’s), but the fellow on her case is not convinced and wants us to “wait it out” because it could likely have to do with the sedatives she had just gotten.

HOWEVER…there is good news….

Hannah stayed on her HME (little humidifier on her trach) and off the vent for over 12 hours yesterday, and she did great.  This means that she can handle being on room air, and she does not have to be on a vent during the day.

This morning, I came in at 4:30 am to relieve Daddy, and Hannah finally got some sleep!    She finally crashed at around 10 pm last night and slept until 2:15 am.  Then, thanks to a suggestion we received, we asked the docs to give her good old-fashioned benadryl.  They gave it to her around 2:30 am, and she went right back to sleep!  Even though she has had some moments of restless wakeup and dozing, she has technically been asleep for about 7 hours (off and on!)   Even better, she did it overnight, which will help her day-night cycle get into rhythm.

So yes, the old family favorite, Benadryl, did what the other tons of sedatives could not do.  Got Hannah to sleep.  And yes, there is an order for Benadryl every night now if needed.

Hannah’s resident is here to do an evaluation.  Good thing is she is starting to wake up now, at 6 am.

What a morning, literally!

Hannah fell asleep last night and 9:30 pm and woke up at 2:30 am.  She was just not a happy camper and could not get comfortable because her movements started up again and kept her awake.  Finally at around 4:00 am, the nurse put Hannah into my lap on the rocking chair to see if I could cuddle her to get her more comfortable.  I tried for about 30 minutes, but no matter what position we tried, she just was not comfortable.

As the nurse and I were putting her back in the crib, her GTube got pulled out…completely!  I started a mini-panic but the nurse just called in another nurse, and they padded it and taped it up.  Pedi Surgery was called, and they came within the hour and put in a temporary tube at her crib.  I was so worried that she would have to go back to surgery, but no, it was all done at the bedside, quick and easy!  Then this afternoon (about 1 pm), Pedi Surgery came back to bring in the permanent tube AND the Gbutton!  I was so excited because we are getting rid of the long tubing and already going for the MicKey button.  One less wire!

Daddy and Hannah are currently waiting to go to Radiology to get a GTube study to make sure everything is in the right place.

On a separate note, Daddy and I have become more hands on with Hannah’s care.  We have bagged and suctioned her ourselves without supervision (with permission) and have done all of the trach care today.  This afternoon we have our Trach CPR class and then tonight we have our Trach change class.  We are feeling more and more confident in being able to care for Hannah and her trach.   Also, the nurses are now letting us administer Hannah’s meds through her Gtube so we can get practice in that as well.

Keeping my fingers crossed that all goes well with the Gtube study so we can “mark that off the list,” one step closer to getting transferred!

Lastly, Hannah is now permanently “off the vent.”   She is on 24-hour CPAP now (which still using all the vent tubing, just does not do the breathing for her), and she will be doing a 5-hour humidified trach collar trial this afternoon (she did 90 minutes yesterday).

Another good day.  I love the fact that these past three days have been wonderful in terms of Hannah making progress, regaining her personality, and beginning the recovery process.

Early this morning, we put my laptop next to Hannah as she laid on her side in her crib, and we turned on Wiggles on my ITunes.  She was babbling and totally interested in watching it for almost 2 hours!   The timing was great because she was just talking away when the neuro resident, the critical care resident, and the critical care fellow came to do their early morning assessment.  I was thrilled because we are starting to see more personality — we know how much she loves the Wiggles and Mickey Mouse!

Humidified trach collar trial — passed!   In the middle of her 12-hour CPAP trial today, they decided to push Hannah a little bit to see if we could get her to breathe off the vent.   So they put on a special mask that went over her trach that delivered just a little bit of oxygen (equivalent to 2 liters) that was humidified.   We had a bit of a scare about 30 minutes into it because her sats started falling into the upper 80s and lower 90s, but we quickly learned (thanks to the respiratory therapist) that Hannah just needed suctioning, and once we did suction her, her sats were between 99 and 100 during the entire trial, all 90 minutes worth!  Hopefully we will get a longer trial tomorrow with the humidified trach collar or maybe decrease the oxygen support (21% oxygen is equivalent to room air).

Why is this huge?  This gives us more hope that she will be able to breathe during the days without any ventilation support.   She needs to be able to do it longer, but they only stopped her because it was her first trial.  She did great.  We’ll see what tomorrow brings!

She had PT and OT today.  Thanks a combination of the “homework” her PT gave us and her movement disorder becoming less intense, Hannah’s feet and arms are much looser than a couple of days ago.  Because of her movement disorder, her muscles seemed to have gotten really right, especially around her ankles and feet.

OT gave her a tumble form chair today, and she absolutely loved sitting up!  She tried to play with this toy (looks like a big one-light traffic light) where she touched the light and it flashed and made a sound.  Unfortunately, she was not able to control her body enough to do it on her own yet, but we will keep working on it.  She was definitely interested in it.

They finally took out Hannah’s central line now.  All she has is a peripheral IV.  Now that her feeds are up to almost the level they want (she is at 32 mL/hour, and their goal is 38 mL/hour) and all of her meds go into her Gtube, we don’t need to get a PICC line.  Yay!  One less invasive procedure.

Weaning Process.  Still working on the weaning process.  Today they are cutting down the methadone to almost  nothing.  Next they are going to work on the one they are most worried about, the pentobarb.  Their tentative plan is to cut it down by 10% each day until it is done.  Then, it will be time to wean the Ativan.  (However, we can do the weaning of the Ativan in Vegas).

They are still trying to figure out her movement disorder.   We still don’t know if it is permanent or temporary, but the Klonopin seems to be helping control the severity of her movements.  Today was just a bit better than yesterday, and that is a good trend.

Best news: The transfer planner met with us again, and things are definitely in the works to medi-flight Hannah to Vegas!  Our attending is tentatively thinking “towards the end of next week.”   That could be a week from now!   Now I don’t know if the insurance is covering it now that all the “work” was done here in TCH and our new residence is in Vegas, or if she is using a charity flight she found, or if our planner just has not mentioned it to us yet that we will need to cover it.

On a side note:  Just to prove once again what a wonderful, wonderful pediatrician Dr. Bhakta is…. Ethan was running out of his ADHD meds, and for obvious reasons, we have not been able to get him to see the pediatrician we had originally planned on.  Since we had no way of getting to Dr. B’s office to pick up the prescription (and it cannot be called in), Dr. B actually came to us here at TCH to give us the prescription!   It was also a great chance for him to see how well Hannah is doing and for us to give him a goodbye hug.  I am so going to miss that man!

No, that is not a typo — we are in the PCU now!   (Progressive Care Unit).  Room 711.  A huge private room with two TVs, a private bathroom, and a couch that pulls out to a bed.

We got moved up here at 4 am this morning, after the night attending felt that Hannah was ready to be moved up, especially since he was going to be covering the PCU floor, “red station.”   This way, we have the same attending following her and knows exactly what has been going on.

Today was another good, good day.  Hannah was still moving around this morning, but I would still rate it about a 5 out of 10 compared to her worst last week.

It was another very busy morning… rounds with even MORE people, all new, except for our attending.  Today’s plan was to wean the methadone (was cut in half today), do an 8-hour CPAP trial (which she passed), and work with neurology on her movement disorder.

Neurology showed up about 20 minutes later, and their plan was to slightly increase the Klonopin to see how that affects her movement disorder.  The Keppra is staying the same.   They still don’t know why this happening, but the plan is to get her controlled first, and the Klonopin seems to be working.

Occupational therapy showed up, and she worked with Hannah for at least 30 minutes.  Basically working on range of motion exercises, trying to get Hannah to do purposeful movements, and to help her with control.  They are going to put in an order for a tumble form chair so we can work on getting her to be able to sit upright more, especially since she is too weak and not in control enough of her body to do it on her own.

Then, the transfer planning coordinator came to talk to us!   She talked about what our plan was to get to Vegas, and she said that she would start working on it.  Because we don’t have many of the doctors we need there yet, she said “not to worry,” that they will put together a team for us for Hannah for whatever we need.  When she asked me when I wanted her transferred, I told them that as soon as the docs would feel she is stable enough for a regular room is when we would want to be transferred to finish her recovery in Vegas.  We want to get her trach situation stable, her neurology situation under control, and whatever else we need so that all she has to do in Vegas is PT/OT and recover.

Daddy and I already started our trach training today.  The nurse was apparently impressed that we were willing to jump into it so quickly.  Since Daddy has to go back to Vegas on Saturday, he is the one who was trained first and will probably do much of it until he leaves.  But he was guided to do a trach change and bag/suction.  He did great!  We have our trach CPR class on Thursday afternoon and our trach change class on Thursday evening.  I am so glad that Daddy is here for this!

One of the best things is that we got to hold her today, for 4 hours!   She finally fell asleep at around 3 pm this afternoon, and she is still asleep now at 10:30 pm.  Her body is FINALLY letting her rest, and it is a beautiful thing.  Both Daddy and I got to let her nap on us, just like old times.   It was wonderful!

Also, she apparently has a leak in her trach (good thing!) so we are hearing a whole bunch of babbling come from her.  We were prepared not to hear any sounds because of the trach bypassing the vocal cords, but I don’t know if Dr. Roy, Hannah’s ENT intentionally did that, but we are so grateful.  She still has a voice!  It is much softer and a bit mousier than before, but it is so dang cute 🙂

Tomorrow we are hoping to do a humidifed-trach collar trial tomorrow in hopes of getting her off the vent in the next day or so.   I admit that I am nervous about her not getting off the vent and needing it 24-hours a day, but all signs are looking promising that it may not need to be that way.  Especially since she dropped no less than 98% during her CPAP trial today, even when she was sleeping!

As I type right now, in the comfort of our private room, the nurse is giving my sleeping Hannah a bath.  Hannah really is just out!  She moved a bit and complained during the bath, but she fell right back to sleep.  Her body is finally resting!!

I’m looking forward to tomorrow, full of hope 🙂