Today has been a good, good day.

Hannah went 6-1/2 hours on her CPAP trial, and she did great.  The docs decided to stop it because it was her first trial post trache about 10 days ago, and they did not want her to overdo it her first time.  But she handled it great!   This is one step closer to getting her off the vent completely, hopefully!

They did the echo today, and unofficially, her echo came back normal.  Meaning, no endocarditis, no strep infection.  ASO blood titers still pending.  It was a longshot, but it would also explain her choreathetoid movements.

They are upping her feeds more aggressively now that she has been off IV sedation for more than 24 hours.  She is at 30 mL/hour right now with a goal to get her to 38 mL/hour.   The PICC line is now on hold because if she can tolerate full feeds, we don’t need to get her one for TPN because she won’t need TPN anymore.

She is finally getting more sleep!  She is still getting oral sedatives like pentobarb, ativan, and methadone through her G-tube because we are still in the weaning process (plus chloral hydrate every 6 hours if she hasn’t slept), but it seems like the more sedations they remove, the better she is sleeping.  She has slept more today than she has slept in a LONG time!  So far right now, she has slept for 4-1/2 hours — this is AMAZING!   They are talking about weaning her methadone (I think) starting tomorrow — well, if not methadone, one of the ones remaining.

Her movements have definitely improved since yesterday, hence the reason she is also able to get more sleep.  I would say that it was a 10 out of 10 at her worst, and now I would rate them as a 5 out of 10 when she is awake and a 4 out of 10 when she is tired.   They decided to increase her Klonopin to three times a day.  I found out that she is at a low dose as it is, so we can even increase the dosing plus go 4 times a day if need be.  They are still trying to figure out if this is a temporary thing or a permanent thing requiring lifelong medication, but the fact that they are markedly decreased over a week ago is a big deal, regardless of the reason.

The best part…

They are moving us up to the PCU tonight.  It is their progressive care unit, which is for kids who are not in need of critical care (PICU) but need more care than the average patient on a regular floor.   This is HUGE!!!!   This means my beautiful baby girl is NO LONGER CRITICAL!   28 days in the PICU, and we are finally graduating to the PCU.  AWESOME!!

We will still be working with all the same issues (neuro, feeds, etc.) up there, but they feel that now that she has passed all her PCU transfer goals with the completion of her CPAP trial, which went better than they expected, she is ready to move up.   We thought it may be a couple more days down here after, but the attending who we like (different than the guy this morning) is moving to the PCU floor, and he will be the one leading her care.  That is awesome because he knows everything about her, and we don’t have to start over with a new attending!   He also made sure that we are put in a room in his “station.”

Of course, as any mom who has been in the PICU before knows, transfer times could be anywhere from 10 minutes to 10+ hours.  We got the news about 3 hours ago, and we are still hanging out down here in the PICU.  But at least the transfer plan is coming together.  Once they can get a bed up there for her, we are MOVING ON UP!

The bestest part… (Yes, I know it is not a real word)

We get to start coming up with a plan to transfer us to Sunrise Children’s Hospital in Vegas once we are up there!  It is VERY conceivable that if all goes as hoped, we could be in Vegas within the next two weeks to finish her recovery there!

A beautiful, beautiful light is becoming brighter at the end of the tunnel…. I am still cautiously optimistic because I don’t want to get too excited, but things are finally looking up, finally!

It is only 10:30 am in the morning, and things are really busy around here!

At rounds this morning, there was a new attending that we had never seen before, an older man.  I really liked him because he really shook things up with Hannah’s care and got the fellow and residents to think more about Hannah as a patient than as a puzzle that needed figuring out.  He also has been the first attending to really get me involved, asked me a lot of questions about her needs, and I really appreciated that.

He immediately wanted to get Hannah set up on a CPAP trial in order to try and get her closer to a humidified trach collar off the vent.  There was a controversy about how long to have the trial during rounds between the fellow and the resident, and he said “why don’t we just let her decide how long to do the trial.  Don’t set a time limit for the trial or stick to so-many-times per day.  If she can go 20 minutes, then do it 3 or 4 times a day.  If she can go longer, then we will do less trials a day.”  FINALLY, a doctor with logic!

So Hannah is currently on her CPAP trial.  One hour so far, and she has done really well.

There is FINALLY concern about her not getting enough calories thanks to this attending.  I have brought it up numerous times, but everyone felt that the TPN was good enough coverage.  When I said that she used to get between 1200 to 1500 calories a day, and she was basically maintaining her weight.  Come to find out that TPN was less than 700 calories a day PLUS she is burning more by moving around constantly…. well, this became a priority conversation.   So, it was decided that since her central line has to come out anyway (it has been in for a month now), she is going to get a PICC line tomorrow so they can continue TPN plus be able to work on getting her feeds up (she has been at 10 mL/hour but was having trouble progressing to more because of sedation/movement issues).  They are going up 5 mL/hour every 4 hours today up to 30 mL (I believe).  Hopefully now that she is off most of the sedation meds (all her IV drips were finally done as of last night), her bowels can start working better.

Physical and occupational therapy came by today to do their assessment.  They sat her up to see how her tone was, and it was great to finally see her sitting up after a month laying in bed.  She had to be completely held up since her body was still flailing, weak, and she can’t control her neck muscles that well just yet.  They have set her up for PT and OT every day, and they gave us some exercises to do with her as often as possible in order to help her muscles since they contract so tightly during these movements.

She has an echocardiogram set up for some time today to rule out a strep infection and/or endocarditis causing these movements (rheumatic fever type of syndrome).  They also did a blood draw for ASO titers to check for strep as well.

The BEST part is that Hannah now recognizes me and Daddy again.  I didn’t want to post about it yesterday in case it was a fluke, but when we sing her favorite songs to her now, she starts smiling really big, and you can hear a little giggle (for some reason, not all her sounds are muffled with the trach).   OMG, it was awesome.  We had all the nurses come in to see her do it (especially since most of them have had Hannah at some point).  We have done it multiple times now, and we even got a video of it.

I see Neurology outside in the hallway at another bedside, as Hannah’s nurse is starting her Cerezyme.   I hope they will come in here after and give us more idea of their game plan.

28 friggin’ days in the PICU, seriously.  I am so ready to get the heck out of there!!  I can’t believe tomorrow will the beginning of week 5 already.

Daddy took the red-eye here and got here at 6 am yesterday morning.  Having him here has been everything I needed.   Support, friendship, a laugh, a hug, a co-fighter, and just my best friend.

I don’t think I prepared him very well for what Hannah was really like yesterday, as seeing her made him very emotional.  I can count the number of times I have seen him cry in our 14+ years together on one hand.  So it broke my heart when I saw how much it affected him.  But once he realized what was going on, he did what I have been doing — wipe the tears and get on our game face.  Our little girl needs us to fight for her — now she has both of us there for her.

Facebook update, yesterday night:
Hannah still moving constantly. Second neuro team confirmed chorea mopvement disorder. Hannah had MRI at 130pm today, results hopefully tomorrow. Scheduled for echo on Monday to rule out strep infection. Daddy is here…exactly what I needed 🙂

We got the results of Hannah’s brain MRI today.  No focal issues, which is the fantastic news.  However, there was mild-to-moderate volume loss.  It could be progression of her Gaucher’s disease, it could be in relation to being sedated for a month and having fluid build-up around her brain.  It is not known.  I was kind of annoyed when Neurology showed up saying “We should not hang our hat on these results” and to redo the MRI in about a month to see if there are changes.  Daddy and I were both like “What the hell does that mean?”  It obviously means something –whether it is a major, minor, or benign issue.  There is a reason Hannah’s brain is atrophying.

We had a fantastic conversation with our fellow today.  We haven’t seen him in a few weeks, but he was the one that was on the first week we were in the PICU.   He has a very interesting theory as to why Hannah required so much medication, “enough for an adult,” and nothing really worked.  It also related to her choreoathetoid movement disorder.  What he said made a LOT of sense to us.  However, I don’t feel comfortable sharing it here until we learn more if it is a possibility (I would hate to post it and then someone searching for info come across it and think it is ‘fact’ and then find out it was not really on target.).

As a result of this conversation, he asked if I could give him the contact info for Dr. Sidransky at the NIH to see if she could answer some questions about his theory.  Ironically, she is at an international movement disorder conference outside the country, and she asked us to take a video of Hannah’s movements and send it to her.  She is going to share this with some of her colleagues there.   We sent it just a few hours later after taking it on Daddy’s cell phone.  Hopefully this will either lead to something either leaning towards proof of this theory or refute it.

We are almost done weaning Hannah’s IV sedation meds.  All that is left is 0.25 pentobarb, and that will be discontinued tomorrow.

Tomorrow, they also will be testing Hannah on the CPAP on her vent and eventually get her off the vent and onto a humidified trach collar with oxygen connector.   Also, occupational therapy and physical therapy orders are going to be put in to start getting Hannah back to being Hannah.  Lastly, Wound Care will also be visiting tomorrow to help with Hannah’s diaper rash (from a lot of acidic diarrhea after forgetting to cancel her Colace orders for 24 hours), her Gtube site for regular care, and a bruise/rash under her chin that has developed from the trach/vent holder.

If all goes as hoped, and I am not betting on this because things change constantly with her, but we MAY be able to be transferred out of the PICU to the PCU (step down unit) midweek.   The goal is to get her off the vent and off the IV sedation meds, and we are working on that.

I guess after 26 days of watching my helpless beautiful daughter just continue to get thrown worsening curve balls left and right, I finally hit my breaking point today.   I just can’t do this by myself anymore.   Daddy is flying in on the red-eye tonight to be here with me for a week.  I need him.

Hannah’s brain MRI was ordered as a “stat” today at 3:00 pm, Friday afternoon.  So come 4:00 pm, the nurse anesthesiologist came to do the paperwork.  Apparently, it is very rare to get a “stat” brain MRI done when ordered late on Friday afternoon unless there is an “overwhelming necessity” to get it done that quick.   Hannah was “next” in line to get her MRI, which amazed me and scared the hell out of me at the same time.   Everyone was rushing around trying to get things signed, paperwork in order, and assessments done.

But, like everything else, there was a catch.  Hannah was still on feeds.  Even though they were only at 10 mL/hour, it still mattered.  (She is getting most of her nutrition from TPN right now until her GI system also recovers from the sedation).   She had to be n.p.o. (no food) for at least 6 hours.

So she is scheduled for early morning, Saturday.  Yep, I know what you are thinking — wow, they are doing it on a Saturday?  I am so thankful they are.

On a separate note, they cut down her fentanyl drip this afternoon as well, part of her weaning process.   By early evening, I could see that Hannah’s movements were becoming more jerky, obviously as a result in the drop in medication.  It wasn’t as bad as the first few days, but it definitely worsened since earlier in the day.

She was able to get 1-hour of nap in this afternoon.  That was it.

Around 11 pm tonight, I was exhausted, so I called down to Hannah’s night nurse and asked how Hannah was.  I was going to bypass my late night visit and get some sleep.  Unfortunately, Hannah was still thrashing around.

I could not even think of going to sleep until she was resting.  It just drove me crazy thinking about her down there like that and me all nice and comfy in my RMH bed.

So I went downstairs, and I broke the cardinal rule of weaning.  I asked the fellow and the resident that were on tonight to sedate Hannah enough so that she could sleep for a couple of hours.   She was so exhausted, but her body just would not let her rest.  I could see the resident was nervous because at rounds earlier that day, it was clear that Hannah was not to get anymore p.r.n. (as needed) sedation meds since the night nurses during the last two nights overdid it (in all fairness, they were NICU floaters and getting Hannah was a ‘tough’ assignment).

The fellow agreed to do whatever it took to get her to rest.  So the nurse tried one dose of fentanyl and versed.  It lasted about 5 minutes, maybe.  Then she got another dose.  Finally, Hannah fell asleep.   I asked the fellow to make sure she sleeps for at least a few hours if not more, and he agreed that she needed it.

When I saw her resting there, peacefully, her body all still …. I just took a deep breath.  It was then I realized how stressful it was just watching her in that state all day long.  I cannot imagine what it must feel like being her.

So it is 1:00 am.  Daddy’s flight comes in at 6:00 am.   But now I feel better getting some sleep.  Hannah is finally resting.   Daddy will be here soon.  I can breathe.

Tomorrow (well, in a few hours) will be a new day.

Seriously, just when we finally get some good news and start to turn a corner, we get slapped with another negative possibility!  It is incredibly frustrating!!

Late, late last night (after I blogged), Dr. Schiffmann and I emailed back and forth regarding his conversation with Hannah’s neuro doc here.  Chorea type of movements have never been documented in Gaucher’s Disease before, and he felt that, after talking with Hannah’s neuro, that it was likely a drug-induced movement disorder.  Something that once she was weaned off the meds, it would hopefully disappear.    He said the attending was likely going to want to do a brain MRI just to rule anything out.

Put me in a good mood.  A glimmer of hope.

I went down this morning, as I always do, right before rounds.  Hannah’s nurse, same one as yesterday, asked me how she thought I looked today.  I took one look at her, and I was so happily surprised.  Her movements were much, much less violent, and even though she was still constantly moving, it did not look as stressful and painful as the previous 3 days.

On rounds, they even talked about the rest of the plan to wean Hannah off the IV sedation drips and get her on only G-tube sedation (much less quantity, much less often) by Monday so that she could be transferred to the PCU by hopefully Tuesday.

Put me in a better mood.  More glimmers of hope.

Dr. Christine Eng, Hannah’s genetics doc, came by to visit and get an idea of what was going on.  She also agreed that these “choreoathetoid movements” were not known in relation to Gaucher’s disease either.  It was concerning to her, but since she had not seen Hannah the past few days, I did not put much into it.  Also thinking possibly drug-induced.

Put me in a great mood.  Lots of hope.   That was my mistake, apparently…

I went up to the RMHouse to take a quick nap.  I got a call about 45 minutes into my nap from the neuro fellow from yesterday asking me to come to Hannah’s bedside.  I rush down there, and there was the attending from yesterday, the fellow, and two neuro docs.

They were asking me about Hannah’s eye problems, how sometimes one eye will get stuck inward temporarily.  I explained that was pre-existing with her Gaucher’s disease and was not something brought on by this event.

Then the fellow asked if they could take a video of Hannah doing her movements to “share with a colleague” to get their input.  Sure, no problem.  Until he made the comment to the attending taking the video, “Make sure you get her left hand in there, see the left hand.”   I looked at her left hand, and I did not notice anything different except the same involuntary movements the rest of her body was making.

Then the attending told me they want to do a brain MRI to rule out a brain injury or “other abnormalities,” perhaps caused by her seizure, hypoxia, drug-induced, etc.  Now, I knew this was coming, because Dr. Schiffmann mentioned it to me.  However, now with this concerned look on all four of their faces, plus needing to take a video to show a colleague, and then all the other questions — I am scared…

What if there is a problem with her brain now?  Is that why she has not yet looked at me or noticed me yet?   Is that why she failed extubation because her brain is no longer communicating with her body the right way anymore?

Argh!!  I don’t know when the MRI is scheduled yet.  Hopefully I will find out today.

I told Daddy that if this brain MRI comes back abnormal, he is hoping on a plane to be here.  I’ve been able to handle all of this by myself up until now.  But if there is something deeper going on, I think I will just lose it.

Yesterday was a nightmare.  Today was even more of a nightmare.

Yesterday’s facebook update (because I don’t feel like rehashing right now):
Having a fantastically crappy day — Hannah is a mess now that she is trying to be weaned off her meds to the point where after 5+ hours of thrashing around and finally crying, we had to give her another heavy dose of sedation meds to knock her out so she could get some sleep. Catch-22 from hell.

Early this morning’s facebook update:
Just had a one-on-one with Hannah’s attending for the first time ever. Unfortunately, there is no better way to wean Hannah off than to “just do it.” After yesterday, not the answer I want to hear. How ironic is it that we are no longer dealing with the virus and the pneumonia is gone — the things she originally was hospitalized for?

This evening…
Today’s nurse was awesome (as most of them have been).   We had never had her before, and I think that may have been a saving grace today.  After talking about it with another nurse who has had Hannah before, neither of them believe that was she is doing, the constant jerking/flailing/agitatedness for many, many hours on end, uncontrollably, was not completely withdrawals but could be neuro related.

After discussing all the withdrawal symptoms with the two nurses, Hannah only has a couple of them.  The flailing/jerking motions, but hers are completely full body from her head to her feet and everything in between, including her eyes.  Also, her pupils are slightly dilated whereas most withdrawal patients have really dilated pupils.  Hannah does not sweat profusely nor does she have loose stools.

After talking about this, they called the resident who came to talk to me, and I asked them to consult Neurology.  They were down within an hour, but they had ordered an EEG in the meantime.  While she was flailing and jerking around, they did an EEG on her, just to rule out seizures (which they did, thank goodness).     When the neuro attending and fellow came around (same fellow I had met when we first got into the PICU for seizures), they both said that it did not look like withdrawal to them either.

They believe Hannah has developed a “movement disorder.”  Of course, my first thought when I heard that was Hannah’s dear friend, Bertrand.

They explained to me that her movements are of a chorea type.   Whether this was brought on by her seizures (doubtful), the sedatives she has been given (likely), or her Gaucher’s disease (also likely), is unclear right now.   However, it is incredibly important to figure this out because if it is not drug-induced, we are in deep trouble, as it may likely be able to be controlled, not for certain.  I cannot imagine Hannah living with this condition, it is just outright cruel.

The attending neuro and Dr. Schiffmann talked via phone later in the afternoon.  I don’t know what was said, but hopefully Dr. Schiffmann was able to give him some insight into the neurologic part of this disease and how if could play into this movement disorder, if indeed this is the case.

What is worse, it could be a combination of this movement disorder and her withdrawal, so we still have to wean her off the sedatives.  But the only time she gets a break from this constant jerky movement is when we can sedate her.

Hannah is so exhausted.  In the past few days, the only sleep she has gotten from this horrible condition is when they load her up on sedatives.  Even then, she only gets between 30 to 90 minutes of sleep.   She is averaging maybe 6 or 7 hours of sleep a day now, and it is not a comfortable sleep as well.

I am pissed off about this whole situation.  My heart really hurts for Hannah.  I sit there with her, at least 10 to 14 hours a day now, just holding her hand and trying to let her know I am there while she is constantly jerking around.  There is nothing I can do to help her except try and hold her arms and legs down so they don’t go flailing all over the place.   At least when she is mommy-swaddled or pillow-swaddled, we are conserving some of her energy — even though her body still tries to throw her muscles all around, we can keep them from doing it.

The downside is that she develops a fever every time she gets more riled up, to the point of 102.4 today.  Then of course, the doctors want to do cultures AGAIN, to see if there is an infection in the line, which there isn’t.

Hannah needs a break.  She needs to be able to start feeling better.  She needs a chance to recover.

I just feel like it keeps getting worse and worse for her, and there is nothing I can do to stop it.