We made it about 45 hours post discharge until we were back in the ER.  Her agitation was excruciating to watch because she was so extremely uncomfortable.  It was almost like she was constantly trying to crawl out of her skin.    We were admitted after a quick stint in the ER, and we have been here since (this is our fourth day).  Again, my facebook posts…

Monday, 5:21 pm:  Hannah has been readmitted to the hospital. Incredibly agitated and in pain, thinking severe klonopin withdrawal, as Ativan isn’t helping much. Same room, same passcode.

Monday, 7:35 pm:  Hannah is finally sedated and sleeping and considered PICU status – on a Versed and Precedex drip. Plan is to reintroduce the Klonopin, which takes 48 hours + to take affect in her system they hope. Hopefully will only be here a few days.

Tuesday, 9:11 am:  Hannah had bouts of agitation last night, but was sedated through most of it. Not feeling confident at all about the attending we have this visit (will explain later), so I have started the process in getting rid of him and getting her neuro to be in charge (or another attending who we trust).

Tuesday, 2:15 pm:  Hannah’s neuro came by today, and he is going to stay on top of all this. Learning politics of hospital…suck! He told us to plan to be here about 3 more days just to make sure she gets on the Klonopin safely. Brain MRI tomorrow just to rule out any other issues. Going to start decreasing sedatives tonight and restart feeds. Tired of seeing her sedated all the time! Missing my smiles…

Wednesday, 9:57 am:  After a really rough day worth both a crappy doctor, hospital politics, and a horribly agitated Hannah al l day long, Hannah is finally on the road to recovery and feeling better thanks to amazing nurses who fought like hell for her and her wonderfully compassionate neuro who got caught up in the politics but still got things accomplished!

Wednesday, 9:41 pm:  Rough afternoon. Agitation returned this afternoon big time. Meds aren’t helping except chloral hydrate. Withdrawal no longer the likely cause. Lots of consults tomorrow including neurosurgery, gi surgery, NIH conference call, etc. Hate seeing her so miserable.

I’m very nervous about what tomorrow will bring…all I know is that it is almost unbearable watching her constantly uncomfortable and agitated to the point she doesn’t want to be held for any length of time.  We need to find a way to give her some relief.

Neuro gave her phenobarbital tonight, and that finally gave her some brief relief.  She has been out for about an hour now.  We also have chloral hydrate, the only thing that has worked that gives us a few hours, if she wakes up from the phenobarbital.

Will know more tomorrow.

We brought Hannah home on Saturday afternoon.  With the exception of anxiety and problems sleeping, she was doing really well.

Or so we thought.   She is so uncomfortable to the point where she can’t get comfortable, cries out constantly, and just can’t be still.  The only relief she has is when we give her chloral hydrate at night, and that is only because it knocks her out completely.

Is it the Klonopin/Ativan wean causing withdrawals?  Could it be too much for her body right now?   Maybe the docs didn’t really think this one through because hindsight tells me that we probably should have waited until her body had recovered from everything else going on.

Or is she is in real pain again?  This time, not caused by constipation because she pooped twice today and has a soft belly.  Maybe caused by something else?

It has been so horrible to see her in so much agony.  She is still on the Ativan 0.5 mL four times a day, but I don’t think that is either enough or just bad timing because something else is going on.

We have to work on figuring this out tomorrow.  I need to find out what harm there would be with putting her back on her normal dose of Klonopin at this point and holding off on any weaning so we can see where this pain and agitation is coming from.  But is it too late at this point since she has been off Klonopin for a week and only on the ativan?!

We sprung her from the hospital because we assumed all of this agitation was from being cooped up in the hospital bed and unable to communicate with us.  But since she has been home, she has become more agitated and uncomfortable.

I have yet to see any smiles since she has been home, and I miss that.

We need to figure this out ASAP, or I’m afraid we will be back in the ER again.

Our neuro and the hospital attending have both decided to take Hannah off of Klonopin effective now.   We have been kind of unintentionally weaning her off it this past week with the use of Versed (which is in the same family) and now Ativan, which is another benzodiazipine but has a shorter half-life and doesn’t last as long.

The plan is to keep Hannah on the Ativan to help her minimize the withdrawals and then wean her off the Ativan.   She has grown a tolerance to her current dose of Klonopin to the point it doesn’t really affect her movement disorder anymore but cutting cold turkey could prove dangerous.

Once we wean her off the Ativan, we can see where she is at with her movement disorder and then talk to our movement disorder specialist in Los Angeles about using something like tetrabenazine (which apparently isn’t covered by our insurance…great!).

I’m looking forward to seeing what her movement disorder is like once Klonopin is out of her system and since we have stopped the Keppra ( as of last month ).

With the fundoplication out of the way, we have (hopefully) solved her problems with reflux and aspiration.  This is a huge relief because I feel like we have just been so lucky and have dodged the aspiration pneumonia bullet (common with nGD) that she could have so easily gotten at some point.

Even with all that is happening, the big question is what is causing her constipation and stomach pain.  All her GI tests came back normal.  Even the hiatal hernia found on endoscopy turned out to be just excess folded skin which the surgeon stitched.

She is on TPN (IV nutrition) now, and she is tolerating the intake great.  She has been gassy a couple of times, which is a good sign.  That is a good first step.

The next step would be starting g-tube feeds slowly and take it from there.   These are the questions we need to find answers for…

• Was the intense pain she was having from the constipation?  Now that she is cleaned out, will she have pain upon feeds?
• Will she become constipated again?
• Are there slow motility issues in her colon at work here?
• If she has trouble tolerating g-tube bolus feeds, would we have to put her on a pump?  Last ditch resort to TPN?
• Will we need to change formulas?  We have been on the same formula for a year, and it was only selected because that was what the hospital had on hand.  Perhaps something with more fiber?  Different nutrients?  A higher calorie/ounce count so there is less volume?
• We have worked so hard over the past three years keeping her pain-free that maybe there is a chance that she is only having a moderate amount of pain but because she is not used to pain that it is overwhelming for her?

We are just now starting to tackle our original issues for what we came here for.  Hopefully the pain she was having yesterday was mostly post-surgical, and we can start working on these issues.

But what if the pain persists?  Then what?  The hardest part of all this is not being able to tell Hannah what is going on and her not being able to tell us where it hurts.

It has been hard to do updates on here because the hospital doesn’t have internet access, so I can only connect via my phone!   So it is easier for me to share my mobile facebook updates and elaborate…

Tuesday, Oct 4, at 7:30 am:  Hannah was increasingly restless last night, but that was okay because she was becoming more responsive as the sedation wore off. Only had to do a half dose of versed when she was starting to get over agitated. Even got some smiles a bit ago! Surgery at 1 pm today.

Tuesday, October 4, at 1:48 pm: Hannah was taken into surgery. Was told by the attending that they wanted to do a central line because they are going to start her on TPN and aren’t sure how long until she gets up to full feeds. I agreed. Get down to surgery and find out that they want to do a PERMANENT central line (Broviac). That was NOT what was discussed with me! We agreed that it will be done but it can be taken out easily upon discharge.

Yeah, I was really pissed about this at first.  We have worked so hard to keep Hannah port-free because of all the problems with sepsis and infection that we have seen with other kids.  She is a good IV stick, and in the 2-1/2 years of her Cerezyme treatments, she has always gotten an IV really easy.

After I calmed down and asked my friends on facebook about their experiences, I realized that this might not be a bad thing.  It isn’t an actual port, and the risk of infection and sepsis is much less (although not gone).  The surgeon after surgery told me that we could have it removed very easily once we get her feedings under control, but the more Daddy and I thought about it after the fact, it might not be a bad thing to keep.

Tuesday, October 4, at 4:57 pm:  Hannah is out of surgery. Did great in recovery. Back upstairs in her room. Was in lots of pain when she woke up so we are starting the pain meds, and she is out again. Hopefully start TPN nutrition tonight. Not sure when they are going to try and restart G-tube feeds.

Tuesday, October 4, 8:27 pm: Problems with Hannah’s pain management. Even with versed, chloral, and morphine, she hyperventilated again and had to be bagged. Fever rising up to 102.2 after Tylenol an hour ago. Waiting to hear from doc.

Ugh, last night sucked!   She had a post-surgical fever, thrashed around violently and hyperventilated.  Because she was moving around so much, her new broviac catheter site started bleeding and we were so afraid that she was going to pull it out somehow or dislodge it.  The versed only worked for less than an hour, and the morphine did absolutely nothing for her.  The chloral hydrate, which usually hits at about 30 minutes, didn’t even seem to kick in for 90 minutes.

Tuesday, October 4, 8:35 pm:  Going to versed IV drip for sedation control. Almost ripped out her broviac twice with her episodes. Changing to PICU status for closer monitoring tonight.

Once they got her calm with her third dose of versed in 2 hours (she was only supposed to get 1 every 2 hours), her doc decided to get her on an IV drip overnight to keep her calm.   At first her respirations started getting really low, averaging around 11 but dropping to 8 or 9 per minute, so they did a CO2 blood test to make sure her carbon dioxide wasn’t high.   Fortunately, her CO2 was perfect, so it was okay to have her breathing at that level.    Eventually, they were able to cut the dose rate in half to a very low dose of versed.

Today, October 5, 7:53 am:   Hannah was comfortable last night (sedated). She finally got her Cerezyme that was due last Friday since didn’t make her clinic appt. Tolerating TPN well.

Because we missed her Cerezyme last Friday, we finally got it last night.  We were at 2-1/2 weeks since her last infusion, so the timing was perfect.   It was at the clinic where we usually go for infusions, so it had to be delivered from there to here.  And then there were the policies and paperwork to get it approved…at least it is done!

TPN (IV nutrition) was started after the Cerezyme, and she has tolerated that really well.  Hannah hasn’t had anything in her stomach for a few days, so I was so relieved to see that started.

Tuesday, October 5, 9:03 am:  So the fun begins — they are taking Hannah off the versed drip and other sedatives so we can see where her pain level is this morning. Hoping to be able to control her pain with as-needed sedatives and pain meds instead of a drip. Waiting to see when they are going to start tube feeds.

They stopped the IV versed drip, and they are now giving her low doses of Ativan around the clock with some chloral hydrate on order if needed.

Tuesday, October 5, 1:30 pm:  Having a good mellow day. Sedated, but awake. EEG done, on her way to xray just to rule out any long bone fractures maybe causing pain ( long shot )

She really is doing well today.   She is sedated still, but she has been awake and responding to our voices and our touch.   We aren’t seeing any pain issues right now, which is great, but the question is if the sedation could be masking the pain or is the pain just manageable now.   She had her skeletal survey done, and we are waiting for the results.  Although I don’t think they will find anything, it was great that they were willing to try long-shots to help us figure out this pain.

We also had her EEG done.   It was actually scheduled for this week at her neuro’s office to see where we are with her questionable seizure disorder since we have been a month or so off Keppra.   Since we were already here, her neuro decided to get it done as well as a brain MRI tomorrow that we will send to the movement disorder specialist in Los Angeles who requested an updated MRI.

We were given two options to consider to deal with Hannah’s reflux and, in turn, aspiration issues.

The first was a fundoplication.   The big “con” in this choice is that it is surgery.   It may be a common surgery, but with Hannah, we can’t treat even common things as “common.”   Other cons is that there is a slight chance that it may not work or even loosen over time.

The second option was a J-tube.   Instead of her G-tube which goes directly into her stomach, the J-tube goes into the jejunum (colon).   The cons here are that she would be tied to a feeding tube 24-hours a day.   Also, if she pulled her J-tube out, it requires an immediate trip to the hospital (interventional radiology) to get it put back in unless a G-tube which we can put back in ourselves.

But the J-tube is not surgery.   That is the big plus.

We decided on the surgery.  When all is said and done, it comes down to what is best for Hannah’s quality of life.   If she is tied to a feeding pump all day long, she won’t be able to have the freedom to roll on the floor (her only way of independent moving).  Also, with her movement disorder in her hands, it will be a literal constant stress making sure she doesn’t grab at the tube and pull it out.

The doc made the comment that the fundo may fail “in a couple of years.”  My first reaction was that I HOPE we have to deal with a failed fundo in a couple of years!

So in a few hours, we are taking the riskier route in hopes that it works, in hopes that Hannah does well in surgery, and in hopes to give Hannah the best life we can.