I have never been so relieved and excited as I did when the medical transport team carrying Hannah and I drove up to our house to take us home.  I couldn’t get out of the van fast enough to get the door open.

I needed Hannah to be home, and now she is here.  Finally.

But it comes with so much guilt.  Hannah had a great morning, and I had a good 2-1/2 hours of quality time with her before the agitation started up again.  The hospice had a spiritual massage therapist and a traditional massage therapist working on Hannah at that point, and we were able to get her another good hour before we had to give her the chloral to sedate her.  The smell of lavendar-chamomile massage lotion permeating her body was just wonderful.

We had a 4-hour wait at that point until the medical transport team was to come and take us home.

An hour before that point, Hannah woke up again.  As much as I was thrilled she was up, I was nervous too.  We were supposed to leave in an hour…her seatbelted to a gurney.  My child who does not do well with car rides in the first place, then add the possible agitation from her disease as well as the agitation from mommy not picking her up when she is tied down…it really stressed me out.

We had the authorization to give Hannah the chloral every 4 hours, but really we don’t want to ever have to use it that quick because it just isn’t really safe, although it is do-able  (usually, every 6 hours is our max).

But as the med transport team showed up (4-1/2 hours after her last chloral dose), the nurse could tell I was stressing out about it.  How the hell am I going to get her home safely?  I was so worried about her getting agitated in the van because she had already been up an hour plus having the transport team need to pull over every time I would need to suction her (their rules) which would be quite often since when she does have an agitation episode, her secretions go on overload because of her swallowing difficulty.

Feeling horribly guilty but torn, I went ahead and gave her another chloral dose to get her home safely.  It killed me to make that decision.  I kept thinking, what if this is the dose that just shuts her down?!

We did get home safely…8 hours ago.  She is still completely knocked out and has to wake up or even move her body from when we put her down.

I just need her to wake up soon so I know that the decision I made to sedate her to get her home safely was the right one.  I’m really wracking myself with guilt right now for doing that to her.

Even though I know our overall decision to keep Hannah ‘comfortable’  is the right one, I find myself constantly actively reminding myself of this when she is asleep most of the day under sedation.   She is so peaceful, so angelic looking.  I love to snuggle with her, caress her face, brush her hair with my hands.  Her smell.  I am constantly in love every time I look at her.

It kills me that we are running out of time.

I had about 30 minutes of awake time today, earlier this morning.  I was cuddling and singing to her, and she smiled back at me like she usually did when I sang those songs!  It was awesome.   It gave me hope that we have the ability to have more good times with her ahead.

I thought, maybe she has more fight in her and we shouldn’t give up on her?

But that was short lived.  The agitation started again, just two hours after her chloral hydrate dose, our magic drug, because of a previous uncontrollable agitation episode.  It didn’t make any sense since we have been able to get at least 4 to 7 hours in between these doses.   We were working on building up the phenobarb in her system, but it wasn’t there just yet.  We couldn’t give her the chloral again until two hours later.

It was hell.  Pure hell.  She was in so much agony, you could see it in her face.  Watching her writhe back and forth, seriously as if she was trying to crawl out of her skin.  It killed her daddy and I watching her.

After talking to Hannah’s two soul-sister mommies (Addy and Olivia) the night before on what meds they were on, I saw the common denominator between the three girls – phenobarb, which Hannah was currently on, and baclofen (for muscle spasms).  We had tried a quick dose of baclofen the day before, but didn’t see much results initially – but in fairness, we didn’t really give it enough time because we had to give her the chloral to sedate her.

The big question was — is she getting muscle spasms which are leading to agitation or is the agitation so great that her muscles are starting to spasm?  With the baclofen, if we could decrease the muscle tightness/spasms, would it help the agitation or even the muscle tightness?

Our amazing nurse coordinator talked to the doctor, and he agreed to let us try baclofen again but on a 3 times a day basis instead of a quick shot dose.   Also, our doctor wanted to try haldol on her, the same drug we tried almost a year ago for her movement disorder with no luck, to see if it would help the agitation.

About an hour before we were able to give the chloral again, they gave Hannah the baclofen and an addition shot of phenobarb about 15 minutes later.  Within about 30 minutes, we noticed that Hannah was still really agitated BUT, and this is a big BUT, her muscles were no longer tight and clenching!    She was clearly still upset and agitation, but it was more of a relaxed agitation.   We were thrilled!

About 15 minutes before we were able to give our magic chloral dose, our fantastic nurse grabbed the chloral so as soon as that minute hit, we could give it.

But then 10 minutes before we were able to give it, we noticed Hannah trying to fall asleep.  Daddy, the nurse, and I were like, “Is this really happening?  Is she really calming down on her own?”

You have to understand, not once has Hannah EVER been able to get out of an agitation episode without chloral hydrate.   And we have had dozens of these horrific episodes.

Two minutes before we were able to give Hannah the chloral hydrate, the nurse walked in the room with the syringe.  Hannah fell asleep.

Did you hear me?  Hannah fell asleep!  On her own!   Without chloral hydrate!

What does this mean?   Perhaps the additional of the baclofen, which clearly caused her to relax her muscles during these episodes, as well as the additional shot dose of the phenobarb are working their way up to place where they are useful.   As a result, it was decided to definitely keep the baclofen 3 times a day as well as bump the phenobarb from 2 times a day to 3 times a day.

But we are still trying to figure out what works best.  As a result of all of this, she has been asleep the 6 hours since.

I miss having my awake time.  But I have to keep reminding myself that this is where we are.  I would rather have her sleeping and comfortable 24 hours a day than have her experience even 1 minute of those agitation spells.

But I miss my smiles.   I miss seeing her eyes gazing at me, knowing how much I love her.  Argh!   It just destroys me that there is a chance I may not get to see those beautiful big brown eyes lovingly looking at me again.

After Daddy and I made our decision on how to treat Hannah’s care from this point out since she is now considered “end stage,” we decided to just bring her home and spend the rest of her time with us.

It was really important to me to have her home so we can just give her love all the time.

But our biggest concern was how to tell the kids.  I mean, huge stress.  I mean, they are going to live with this situation for the rest of their lives.  It is going to affect them terribly because they just love her so much.  I mean, they are going to lose their little sister!  So not fair!

I ended up calling a pediatric hospice group that listed pediatric counseling on their website.  Just for guidance on how to handle telling the kids — at what point?  where?  how?  I ended up breaking down talking to the nurse coordinator, and she dropped everything and came to the hospital to talk to us.

I knew of hospice because of the families I know who have been in this situation, but I really didn’t understand what it entailed.  I just thought it was like extra nursing care, but it is so much more comprehensive than that.

Later that afternoon, we asked for their team to come talk to us to learn more.  It helped tremendously that our friends’ mom was part of that team and worked at this hospice.  The counseling services for not only the kids but us, having a team take over coordinating all of Hannah’s care (nursing, appts, meds, etc.), and having someone who will work to do whatever it takes med-wise to make Hannah comfortable at all times.

Once we decided to forgo the brain coma and the neurosurgery consult, it seemed like a no-brainer at that point.

So from that point on, our main goal for Hannah was “comfort care.”

We transferred her to the Nathan Adelson Hospice on Friday, which has been an amazing blessing in disguise.  Since they focus on quality of life and not recovery and ‘fixing things,’ it has been a 24/7 process coming up with the right med combination to keep Hannah’s agitation under control but also try and give us some quality time where she is awake and aware but not in any pain or uncomfortable.   They are also working with us to change her feeds over to the 1.5 cal/mL (instead of the regular 1 cal/mL) so that she can have less volume and more calories.

The problem we are realizing is that Hannah is metabolizing these drugs so fast that they are not lasting as long as they did just a day or two before so she is requiring more.   Right now, we are working with the phenobarb and the chloral hydrate (her miracle drug, works so quick, but is not lasting as long because we are using it more).  The goal is to get the phenobarb to a good level so that we don’t require as much chloral but can use it when we need to.  We know that versed, ativan, and fentanyl don’t work for her – very little affect – so those are out of the equation.

The hospice group is also going to work to put everything in place so we can just go home and ‘be.’   They are coordinating all her meds, supplies, and nursing assistance and even working on getting Hannah’s cerezyme treatments done at home.   Also, our nursing company is working to increase our hours to 80 a week (from 56), which will definitely help.

Our goal is to get Hannah home on Tuesday where she can spend the rest of the time just being loved and cuddled 24/7.

When we sat down with Hannah’s doctors a couple of days ago after her neuro, intensivist, and NIH pediatrician all concluded this was likely “end stage” of her disease, we were forced to make decisions on what to do next.

It took them 4 days, but they finally got a drug combination that sedated her enough and kept her body calm.  It has been an absolutely stressful nightmare until that point, and when she finally was out for more than an hour, I felt a huge sigh of relief.   They tried Ativan, Fentanyl, Versed, Precedex, and even Morphine to try and calm her agitation.  Even a Versed drip wasn’t any help at all.

Finally, it was a combination of chloral hydrate and phenobarbital that did the trick.

I think the 24 hours that followed that meeting with our hospital intensivist were the worst 24 hours in my life.  I mean we always knew that this time would have to come at some point, but I just thought it was more “in the future.”  To have it staring at us in the face, talking about “end stage” and “Hannah” in the same sentence just kills me.  I don’t think my husband and I have ever cried so much.

We were faced with the only two options of meeting with neurosurgery in hopes that Hannah had a very rare case of hydrocephalus ex vacuo (brain atrophy with spinal fluid filling the space) that caused pressure on her brain (usually they don’t).   In that case, the treatment would be drilling a hole in her brain to let out some of the fluid.

The other option that was available to us was to put her in a medically induced coma with pentobarb.   Basically, flatline her brain for 24-hours in order try and ‘kick start’ her brain in hopes that the agitation, etc., would disappear.

Both of these options had tremendous risks, pain, and possible death involved with them.  Both of these options were very unlikely to help, very slim change for either to work.

We decided to leave Hannah alone and have no more intensive invasive treatments.  That was the first of our many decisions that followed.

Daddy ended up having to take the rest of the week off of work because we were just both such a mess.  It was even harder because we both had to keep a ‘normal face’ in front of Ethan and Abigail because we didn’t have a plan for them yet.

We had to figure out what to do.   We already agreed that we didn’t want to subject Hannah to any further invasive attempts if there wasn’t a reasonable chance of success.

But then we were faced with the advanced directive question.   This was such a horrible thing to have to discuss.  I mean we knew we would have to talk about it at some point but it was so damn hard to start that conversation and really share what each of us thought.

But we were both on the same page.  We decided to put her in DNR code – Do Not Resuscitate.  No CPR, no advanced cardiac life support (such as epinephrine), and no vents.

When Hannah is ready to let go, we are going to let her go in peace.   As much as I want to keep her here with us, it can’t be because I am forcing her.   I don’t want her to be in pain, I don’t want her to suffer, and I don’t want her to be forced on a machine just so I can have another hug.

Happy and pain-free.  That is what we have always wanted for her.  I feel like we are failing in that right now, and I know that keeping her around when her body is ready to let go is just me being selfish.

God, it kills me to even think that we are going to lose her.

I have never hated a word so much in my life.  I never knew I hated this word so much until just two days ago.

This was the word Hannah’s neuro used to describe Hannah’s condition to her NIH pediatrician.

“Tremendous amount” of white matter loss in her brain, according to her latest MRI.   This was compared to her MRI at the NIH two years earlier.  Hannah’s brain has atrophied “tremendously,” and in its empty space is a lot of cerebrospinal fluid.

No matter what we try to do to help “heal” her at this point, it won’t matter.  Reality is, there is nothing to help anymore at this point.  Her brain is continuing to deteriorate.

Things have snowballed this past month starting with that first hospitalization just a few weeks ago.

Hannah’s NIH pediatrician shared that extreme agitation and problems swallowing are some of the signs of end-stage Gaucher’s type 2.  In Hannah’s case, she has lost the ability to control her secretions, trouble swallowing, and is losing control of her tongue.

Friggin’ sucks!!!!!!!!  More later…need a break…

Hannah’s condition is continuing to deteriorate.  We are working towards figuring out what is best for her at this point as well as what is the best way to help Ethan and Abby.

To my family and local friends, please don’t talk to Ethan and Abigail about it – right now, we want to let them keep as normal a life as possible until we know what our plan is.