I feel like I am living a nightmare right now.

It has come to the point now that I dread seeing Hannah start to wake up because she most likely will wake up uncomfortable, her movements going uncontrollably, and most of all, seeing her writhe in pain from whatever is causing the pain.

We have made a lot of changes to her med regimen in the past few days including phasing out the scheduled phenobarb, adding valium and morphine, and increasing her klonopin, benedryl, and baclofen among more than I can’t think of off the top of my head.

When we added the valium and increased morphine yesterday, she actually fell asleep for 13 hours!  Her average over the past few days before that had been about 2 hours at a time with very, very little calm and quiet time in between (although she seemed to have some calm awake time for about 2 hours a day a few days before that but she quickly gained a tolerance to the meds that allowed that).   It had become a race to get to that 4-hour mark when we could give her the chloral hydrate again and knock her out.

Oh, and have I mentioned that the chloral hydrate is starting to not be as effective anymore?   Twice now in the past 36 hours we have used it and it did NOT even work.  Hell, a living hell.

She is so miserable, drugged up, and I’m not even sure how much she is even aware anymore.  We aren’t sure if she can see us anymore, we know she can hear some things but what she can understand is unclear.

What kills me the most is that cuddling her now gives her more discomfort, and she would rather be lying down untouched.  Her hospice nurse thinks that she has developed some pain to the tactile stimulation that holding her brings.  KILLS me.  At least she will let us lie next to her, caress her, and massage her.

All I want to do is comfort her.  That is my role as a mom.  At this point in her disease, I feel like all I can offer her is showing her comfort and love.  Yet, holding her makes her uncomfortable.

I look at the pharmacy we have accumulated over the past two weeks.  Massive doses that would knock me on the floor, they barely touch her.  And yet, I am so damn afraid of even going one-tenth of a milligram more than that doctors prescribed for fear that I may cause more harm to her.

Then this afternoon, she started having problems with her oxygen saturation.  She started dipping into the mid 80s.  Obviously a result of all the tranquilizing meds trying to calm her comfortable.  But we broke out the oxygen this afternoon for the first time in a LONG time.    Even on a 10L oxygen level (not direct, just into the area around her trach), she only got up to 95 or 96 (100 is best).

I was exhausted when Daddy got home from work in the afternoon, and I was ready to crash since I hadn’t had much sleep in the past three days because of his work schedule.  I was lying in bed with the door closed, and then I kept hearing the pulse ox alarm keep going off because her O2 was dropping under 92 a lot…with the oxygen.

I just started crying.  I heard Daddy, Hannah’s nurse, and the hospice massage therapist out there, and I just walked out of my bedroom and turned off the pulse ox.  I said to them, “why are we doing this?  We already know what is going on.  Just keep the oxygen on her, and leave the machine off.”   I didn’t even allow for a discussion of it.  I just turned it off and walked back into my bedroom, closed the door, and I start bawling.

This is a nightmare.  Hannah is caught in this horrible nightmare.   This is hell.

There have been many times that I think that maybe someone has made a mistake.  That maybe if we stop all the meds that she would go back to where she was two months ago and that this is all a bad dream.  That maybe we are causing more harm than good with these meds at this point?

But then I go back to the brain MRI.   The one with the “tremendous” amount of white matter loss.  I go back to what her nurses have said since we went into hospice, how they had seen a lot more uncomfortableness and agitation over the two months before we started this last round of hospitalization.

Right now, Hannah is asleep.  Knocked out with a boatload of meds.  Yet, I find myself relieved because her body is at peace right now.

But I am so sad for her.   She deserves peace, cuddles, and oh so much love.  Yet, this disease does nothing but destroy all of that.

And guess who is already staring to walk up … just 2 hours after a crapload of tranquilizers…damn!

I couldn’t handle Halloween this year, apparently.

I didn’t realize this until two days ago when we took Ethan, Abby, and my nephew and two nieces to a big Halloween event here in town.  When Daddy and I were walking, keeping tabs on Ethan and Abby, it hit me that this is our first event that Hannah hasn’t joined us on.  And then I found my thoughts drifting to the fact that Hannah will likely never be able to go to another event like this…ever.

It made me feel so uncomfortable.  I put on a good face for Ethan and Abby because I really wanted them to have a good time, which they did.  I had to step back for a bit towards the end just because my heart was hurting so bad, and I didn’t want them to see it.  It worked out because they shopped with their grandparents and cousins while I stood on the other side of the store.

All these thoughts just came rushing to me at that point… losing Hannah, realizing that from now on that most events will only be Abby and Ethan and no more Hannah with us (even if she is still with us, it is just not feasible to do events like that one with her).   Even if we did take her out in her current condition, she would not get anything out of it and would likely be very uncomfortable and would need to be resedated at some point since we are still not under control fully yet.

We also didn’t get Hannah a costume this year either.  I just didn’t feel right dressing her up just for my sake.  She loved her Minnie Mouse costume last year, and she had so many smiles while she was wearing it.  She knew she was Minnie Mouse.  This year, I just felt horrible because it was a tough fight in my head deciding whether or not to do it.  I just don’t want to go through those motions with her – I think it would make me more depressed knowing she wouldn’t be enjoying it or even aware of it.

So it was easier to just boycott the holiday.

Ethan and Abby had a great time with Daddy and their cousins, so they really had no idea how I was feeling.  Someone had to stay home with Hannah, which they knew, so it made sense that it was me.

It just hit me that we have the even bigger holidays coming up – Thanksgiving, Hannukah, Christmas….  how am I going to get through these holidays?!

Then last night, I just had no desire to go trick or treating with the kids.   Daddy took the kids out while I stayed home with Hannah.  I didn’t even answer the doorbell when the trick or treaters came to the door.

I just had no desire whatsoever to participate this year.  None.

We have been on the new regimen with the addition of the increased Klonopin and Tegretol for a couple of days now, and we have definitely seen some improvement in her movement disorder and agitation.  I would say we are about 50% better than we were just a week or so ago.

We are using the chloral hydrate less, maybe 3 times a day or so.  Today we went 8 hours and 13 hours in between needing the chloral.  When we do use it now, it isn’t so much the brainstem agitation, it is the agitation because she is just so exhausted and wants to try and sleep.  We have been using Benadryl a bit more often to help with this, but it has been hit and miss with its effectiveness.

On a positive note, she has fallen asleep on her own three of four times now.  Granted, the sleep only lasts between 30 minutes to an hour, but it must be a good step.

She is a bit more comfortable, her movement disorder still bothers her.  It is not anywhere near as violent as it had been, but hopefully there is still room for improvement.

Even so, she is still not really aware.  The only smile I have seen in the last 48 hours is one single group of smiles she had while she was sleeping.  It only lasted a few seconds, but I wish I could have captured it.  But other than that, not much interaction with us.

I think I am going to see if we can get her eye doctor to make a house call.  I just need to know if she really can’t see us anymore or if her muscles controlling her eye movements are basically shot.  It kills me when she just stares straight out without any purpose.

Also, I’m going to talk to the doctors about perhaps decreasing one of the earlier sedatives (phenobarb perhaps) to see if we can get more awareness out of her without sacrificing the comfort we have achieved so far.  We still have a way to go for full comfort, but it is less stressful for us now that she is not as stress out all the time.

Hannah still is not comfortable.  It sucks.

It is painful to watch when she gets really uncomfortable and agitated,  Our hospice team is trying so hard to get her comfort manageable, but it seems none of the normal avenues work for her.   Usually they use a lot of narcotics like Ativan, Versed, etc., but these have no effect on her.  They have been reworking her regimen daily, and we have enough meds here to start our own pharmacy even though we only are using about 20% of them now!

Yesterday afternoon, out of desperation, I sent an email to Dr. Sanger at CHLA (the movement disorder specialist we saw in August) begging him for help in trying to get Hannah’s movements under control because they seem to be getting worse now.  Yes, I begged.  That is how desperate I was feeling.

Within 3 minutes, yes THREE minutes, I had an email response back from him saying he would contact Hannah’s hospice doc immediately to try and help.  He also asked me to take some videos of Hannah’s movements so he could get an idea of what was going on.   I was SO dang relieved!

This morning I got a text from Hannah’s hospice doc saying that she had discussions with both Dr. Sanger and Dr. Goker-Alpan from the NIH (who has been available 24/7 since Hannah has been hospitalized), and they came up with a brand new regimen of meds to try.

Apparently, one of the meds that we were using (Haldol) actually may increase her movement disorder even though it did seem to help cut down the agitation but made her more uncomfortable.   They discontinued that and added Tegretol in place of the Haldol and increased her Klonopin.  They also increased the morphine because we have yet to see any effect with it on her.

Hannah is on adult doses of everything.  We are talking major doses here that could wipe you and me out.  Her body is just sucking up all the meds and getting very little effect from it.

Since removing the Haldol today, Hannah actually slept 10 hours straight — she hasn’t done that in days!  But when she did wake up, she was completely agitated and miserable.  Daddy, who was up with her, said that there wasn’t even a moment of calm and he had to use the chloral within about 30 minutes of her waking up.

The pharmacy didn’t drop off the Tegretol until 10:30 pm tonight, so we will have to wait until tomorrow to see how this new regimen affects her.

There has got to be a way to give her some peace.  There just has to be.

Thank goodness we have Ethan and Abby.  Seriously, if Hannah was an only child, I think I would have fallen into a major depression by now and would never leave our house.

But Ethan and Abby force us to continue to live our lives, even if it is not at full throttle.  Because we have about 70 of our 80 nursing hours covered (51 by our favorite nurse – 5 days a week), we have felt comfortable leaving Hannah at home while we allow the kids to continue their extracurricular activities.

Saturday, Daddy and I were out of the house for about 4 hours (my first time getting out in over a month for an activity other than a quick meal).  Because Abby was at a harvest festival with one of the girls on her volleyball team, Daddy and I took Ethan out to lunch.  I can’t remember the last time it was just the three of us for a meal!

After that, we went directly to Ethan’s flag football game.   It was the first game I was able to go to, and it was really exciting to watch my baby boy so intently passionate and into a sport that he loved.   Immediately after, we rushed over to where Abby’s volleyball game was.  I was clapping and cheering so loudly for them, especially Abby when she scored a point on her serve!   When the game was over, we all went back to our house (including my in-law family, nieces, and nephew) and had a BBQ.  Even though Hannah was out most of the time, she was still right in the room with us (we have her twin bed in the living room now).

We had kind of forced the kids to take a year off of sports since we moved here from Texas, so we had promised to let them do it this year — not realizing that we would be in this position so soon.   But it is so good for them.  So Daddy and I promised ourselves that we would work hard to make sure they made all their practices and games if they were up for it.  Fortunately, some wonderful moms on Abby’s volleyball team have offered to take Abby to practices so if we don’t have a nurse that evening, Abby can still get there.

Sunday, Daddy and I decided to take Abby out for a few hours.  Ethan spent the night at his grandparents house (Abby is in this “need to be near mommy phase” and didn’t go), so the three of us headed to our local mall.  I can’t remember the last time we actually went shopping in the mall!

After lunch there, Abby asked if she could get her ears pierced.  She has wanted to do it for over a year, but we were going to wait until she was 10 or so.  You know, a right of passage like I went through when I was younger.   But Daddy and I both felt that she needed this now, and if she wanted to do it, let’s do it.    And she did.  She did great!   She got a Hello Kitty starter earring set (she is in love with Hello Kitty stuff now — if they had American Girl doll earrings, it would have been a toss up!), and she is just so proud!   She shows them off to everyone.

Then yesterday evening, after Daddy got home from his conference, I took Abby and Ethan to her Brownie Halloween party.  At first I didn’t really wanted to go because I was just so tired and not in the mood to socialize, but I am so glad I went.  Both kids had a fantastic time, and I really enjoyed just hanging out with other families.  Even though many of them knew what was going on with Hannah, I was able to keep my composure and share what was going on with her when I was asked.

But the good thing was that it wasn’t the main topic of conversation, which I appreciated.   It’s like I want people to ask about Hannah, care about how she is doing.  But I also want people to realize that we can talk about other stuff as well.

Even though it was just a few hours a day for the past few days, I really needed it.  There were many times during these past few days that I went through major guilt pangs, thinking that I should be at home with her, but I was also brought back to reality because Ethan and Abby needed me too.

I had to remind myself on MANY occasions that Hannah was being cared for and loved at home (which is why we aren’t training any new nurses), so it was almost like I had to give myself permission to laugh, enjoy myself, and cheer on my kids.

For the past year, Hannah has had strabismus (her eyes turned inward).   We see it in all her pictures, and I got used to looking into the beautiful brown eyes and seeing them crossed, but she still would be able to gaze back into my eyes.  Classic Gaucher symptom that you just eventually learn to overlook and assume as normal.

Hannah and I would cuddle for hours and just look at each other.  We always said that for a nonverbal child, she is incredible expressive and you know what she is feeling because of her eyes.

That is completely gone now.  All of it.

The strabismus is gone.  Not even a sign of it anymore.  Hannah’s eyes are now both straight ahead in the middle.  She can’t move them left to right anymore or even up and down.   The nurses say they still react to light, but what does that really mean in terms of what is going on?

We also wonder if she can still see us.   Could she have lost part of her vision or even all of it?

Even if I put my face strategically right in front of hers so she can make eye contact, it just doesn’t happen.  She has the actions of how a blind person uses their eyes, but whether or not that is the case, we just don’t know.   But then there are those rare times where we will lay on her side and let her watch her iPad (Wiggles or Elmo) for about 5 to 10 minutes (before it is obvious she isn’t interested) – but unlike two weeks ago, she really doesn’t seem to pay attention to the screen and just seems to listen to it for a few minutes.

Could one of the meds be causing this?  Is it progression of her disease?  Why two weeks ago, even in the hospice, she was able to make eye contact with us but now it is almost if she looks right through us, never making that connection.  I think it is time to send off an email to her NIH pediatrician and see if she has any insight.

I feel like my only way of having two-way communication with my baby girl is gone.  We couldn’t have conversations with words or signs.  Losing that bond with her now, not being able to have her really see in my eyes how much I love her and how much I hurt for her…just so mean.  This disease is so damn mean and cruel.