Hannah grabbing the toy Ethan is holding out for her -- see that, she is GRABBING it! She couldn't even control her hand movements a few weeks ago.

We have always been told that Keppra was a very safe drug, “benign” was even one of the words used to describe it by one of Hannah’s former neurologists.   She was put on it just days after her stay began at TCH well over a year ago after her initial tonic-clonic seizure brought on by her breath-holding spell.

When she was discharged last July, she was at 1.0 mL three times a day.   After her “seizure-filled” EEG last November, it was upped to 2.0 mL three times a day.   Hannah’s fine motor and balance problems started the end of December and spiraled from there.

It took a specialist who wasn’t familiar with Gaucher’s to make the connection for us.  Had Dr. Sanger not told us that he doesn’t like to use Keppra and that it shouldn’t be used for kids who are neuro-compromised…along with the fact that he didn’t believe what was seen on the EEG were actual seizures but corticol myoclonus….

It angers me that we didn’t make the connection sooner.

Here we are less than two weeks after started our wean off from Keppra.  We are now at 1.0 mL three times a day after a week of being at 1.5 mL three times a day.   Next Sunday, we will be down to 0.5 mL three times a day.   Our goal is to have her completely off Keppra by her next EEG at the end of September.   (Obviously if we see seizure activity, we will call her neuro immediately and look into another seizure-med).

Ethan and Abby are LOVING the fact that Hannah is starting to be able to interactively play with them again!

SO…..We have seen some amazing changes in Hannah in just the past week or so.

One month ago, Hannah couldn’t balance at all when sitting on her knees (her W-sit).   Her balance was just bad that she would wobble and eventually fall over if we weren’t holding her.

NOW…She is able to sit on her knees BY HERSELF for almost 30 seconds!  Her balance has improved so well already!   Confidence wise, she needs to have us right there to fall on or lean on, but it is so wonderful seeing her sit on my lap without me having to hold her entire back and neck.  I just have to hold her around her waist, and she can sit up and support herself.

One month ago, Hannah’s fine motor skills were almost non-existant.   She could no longer grasp toys, reach out to touch anything, or even play the special needs apps on her iPad.   Because her movements and control were so bad, she was constantly holding her arms and couldn’t do anything else.

NOW…She can reach up and grab her new favorite toy, a ball with lots of grabbing areas, that is lifted a foot above her head!  She actually can locate it and put her arm, albeit slowly and with a lot of effort, up and open her fingers to grab at all!   She lays on her side and grabs it all the time as well.

Today she also was sitting her in Pink Rider (wheelchair stroller) and was able to touch the keys on her lighted musical piano for the first time in many MONTHS!   Again, it was kinda of sloppy and not a direct easy touch, but she worked hard to get her hand there and move it!

So my question is — WHAT THE HELL DID THE KEPPRA DO TO HER?!

If we are seeing this kind of improvement in less than just two weeks, what are we going to see in the next two months?!   Does she even have a seizure disorder at all (did the EEG tech that did that fateful November EEG know what cortical myoclonus even was and how it is NOT a seizure?)?

We haven’t even restarted the Klonopin wean yet!    We had seen a huge personality change when we dropped from 3.0 mL three times a day to 2.0 mL three times a day.   She was no longer lethargic, became her happy charming self again, and showed, most importantly, INTENT to want to do things.

Now with this Keppra and all these changes….UGH!!!!!     What if some of her current issues were just bad med choices and not all Gaucher progression?   Everyone just assumed it was progression (even us), but now seeing this dramatic (to us) improvement over just the past two weeks….UGH!!!!!

A bit less than a week ago, Hannah started developing this red rash on the outside of her left elbow.   It wasn’t hot to touch, inflamed, or pussy.   It didn’t even seem to affect her much.  It was just a rash.

Going back in our heads as to “what has changed” in the past week or so, Daddy and I thought it could have been the addition of the chamomile tea.

The tea has been a godsend in terms of letting her sleep through the night!   She had gone from averaging about 5 hours or so of sleep a night to 8 or 9!

So of course, because it had been working so well, it probably was the tea giving her the rash, because let’s face it — nothing is ever simple with Hannah.

These past three nights we decide against giving her tea to see if the rash would go away.   Sure enough, it definitely has started to dissipate…along with Hannah’s good night sleep.   Each night, without the tea, she only slept 4 or 5 hours.

Heartbreaking.

So we are trying something tonight.   Instead of giving her the 5 or 6 ounces of tea at night, we are going to limit it to just 3 ounces at around 2 am and see if that will allow her to sleep a bit longer without the rash coming back.

If the rash comes back, it is on to plan B…  whatever that may be.

Gosh, I hope we can keep her on the tea and that the rash was just a fluke.   It has worked so well for her sleep!   Keeping my fingers crossed this works.

Two days ago, Hannah’s nurse noticed a little bump under her left armpit when she was giving her a bath.    You have to feel for it, but you can touch it, and it seems to move a bit.   It doesn’t hurt her when you touch it either.

Yesterday morning, this bump was clearly visible without having to look for it.   Still didn’t seem to hurt her, but it was concerning enough to me that I brought her in to our pediatrician in the afternoon.

She commented how it seemed “cystic” in nature, and how it wasn’t inflamed, red, or hot to the touch.  It actually was almost slightly bluish-greyish.   It was about 2 inches lower than where the lymph nodes are located.

All I kept thinking was “when does Hannah get a break?”

Because our pediatrician wasn’t exactly sure what it was, Hannah is scheduled for a left breast ultrasound on Monday to have it checked out.   I’m hoping, hoping, hoping it is something completely benign like a cyst or a blood vessel.  It just has to be!

Monday just can’t get here soon enough!

One of the other issues that was brought up with Dr. Sanger a few days ago was that of Hannah’s lack of sleep.   Including naps, she averages about 8 hours of sleep a day.   Rarely more, sometimes less.

We explained to him that sometimes when she is sleeping and wakes up in the night (just as many people do for a few seconds or so), her movement disorder kicks in and she can’t fall back to sleep.    Also, her movement disorder is so constant that it is also hard for her to initially calm down enough to go to sleep, and it can sometimes take hours (no joke!) before she finally crashes.

Dr. Sanger believes that Hannah’s condition also could start to improve with just the addition of getting more and better sleep.   When a body is in constant motion like hers, it is constantly wearing her out.   Without good sleep, she doesn’t let her body get a chance to rejuvenate energy and muscle.

He wants to consider putting her on a sleep aide such as Lunesta or something.   Even though it isn’t prescribed for kids, he believes it would be safe enough (another thing he is checking with his Pharmacology group).   We have been so afraid of sedatives because we thought her movement disorder was caused by oversedation, but if he is right and it isn’t, this may be just the thing for her!

When we told my parents (whom we were staying with in LA) about this, they suggested why not try chamomile tea.

So that night, we got out my parents tea and gave her some instead of some of her nighttime water.   She was out in less than 60 minutes!   However, she still woke up about 5 hours later.

The next night, she happen to fall asleep on her own at 10 pm, but I gave her some tea instead of her nighttime water at around 1:30 am.   She slept until 6 am!!   She was a little restless at around 1 am before I gave her the tea, but all in all, that was almost an 8-hour stretch of sleep for her!

So tonight, we are going for three nights in a row.   I gave her some tea (2 oz) around 10 pm, and she fell asleep around 11 pm.    I’m going to give her some more at around 2 am (instead of her 2 am water, 3 oz), and I can only hope that she sleeps in again.

I’m not getting my hopes up just yet, but it would be wonderful for her (and me) to not wake up at 3 am in the morning anymore and let her get a REAL sleep!!

Could it really be as simple as chamomile tea?

We met with Dr. Terence Sanger down at Children’s Hospital Los Angeles a few days ago.  We is one of the leading pediatric movement disorder specialists in the country, and we went with an open mind, not expect answers, just hoping for some, well, hope!

It went better than we expected!   It was very clear that he went through Hannah’s paperwork very carefully before our meeting because he asked very specific questions that could only be found in the paperwork.   He spent almost 30 minutes with us (along with his fellow, two residents, and a videographer who tapes all of his clinic visits).

It was a very eye opening visit and gave us a LOT of things to look at and to think about.

• He doesn’t believe that Hannah’s movement disorder was caused by oversedation during her hospitalization.   He believes it would more likely have been caused by a hypoxic brain event during that initial seizure.  He brought up how her CO2 was really high during that time and that her O2 sats in the ambulance were in the 50s.     Her movement disorder was clearly apparent just 7 days after the initial event, 5 days after intubation.   After really thinking about it more, Daddy and I both remember that right after she was admitted, she had a difficult time breathing when she was awake (causing more respiratory distress) and when she was sleeping, she was breathing much better.    Coincidence?
• He is a strong believer against using Keppra for seizure control in children who are already developmentally compromised such as Hannah.    He also is not convinced that she is having subclinical seizures while she is sleeping based on our description but may likely be cortical myoclonus (which is a Gaucher symptom).    Again, after thinking about it more, Hannah’s Keppra was upped in November due to the results of that EEG back then…and her fine motor control and balance started getting worse around Christmastime.   Coincidence?

I received an email from another special needs mom a couple of days ago asking me if Hannah was “okay” and if everything was alright because I don’t seem to be posting as much as I used to.   Then as I look back at my posting for the past few months, it does seem as if I post less than I used to.

This blog is like a therapy tool for me.  Yet, I realize I really only like to share when I have good news to share.  Some sort of accomplishment or milestone achieved.  But those are becoming more few and far between.

I feel that we are losing the fight for Hannah’s life.  For whatever reason, her condition is continuing to deteriorate in so many different areas and there is nothing I can do about it.   To list them all right here is just too painful and seeing the list in front of me would just devastate me right now.

I was cleaning out our old computer so I can work to get all the videos off it.  I went through all the videos from the day Hannah was born up until she was about 7 months old and a couple of sporatic ones throughout the past couple of years.

It broke my heart seeing all the skills that she was able to do just 13 or 14 months ago that are so much of a distant memory that a part of me must have blocked it out.   I saw a video of her just sitting there, non W-sitting, at around 14 months old playing with toys in her hands, putting her little people in her mouth, picking up new toys, and just very steady and secure.   There was the video I took of her at around 6 months old eating food for the first time in her high chair, sweet potatoes.   Then there was the video of her at around 15 months or so with her standing against the couch, cruising with her little peoples in her hand.

Then, on top of all of that, I have what Dr. Barranger, a Gaucher specialist who comes out to Vegas every 3 months, ringing through my head at our visit a couple of weeks ago.   “Unfortunately, even if there was something on the horizon, Hannah’s condition is too severe for it to likely affect her.”    At the time I just kind of blew it off without a response, but those words keep coming into my head and heart.

It hurts.  It really hurts to see her losing more and more function and skills.    I’m scared for her.   With these increased laryngospasms or reflux problems (which no one can seem to answer for us definitively), we are seeing a lot more tears and winces of pain and fear from her.  I hate seeing her go through this.

It is all I can do to just be able to cuddle her and give her kisses.  There isn’t much to play with her since she has lost much of her fine motor control and strength.  We still continue to read to her, let her watch her Wiggles and Backyardigans, and let her roll around the floor (since she can no longer sit or crawl).

We have our meeting with the movement disorder specialist next week in California.  I’m not expecting anything, but I am hoping that there is something we can do to improve her quality of life.  I just know that she is just such a unique ‘case’ and not close to textbook that to expect answers is just not realistic.

I guess maybe I am not so much at a loss for words as I thought.  But sometimes these are just not the words I want to have to commit to writing down because it just makes them more real, and even though I know what the reality is, I just really wish it wasn’t the case.