These past three days have been really tough and emotionally draining.

It started a few days ago.   Since about Sunday, Hannah had just not been acting like herself.  Very clingy, not wanting to play, just not feeling great.   She was a bit more gookier than usual in her trach, but it was still white and not yellow/green/brown (which would lead us to believe it was trachiitis).

Thursday was more of the same.  But then in the afternoon, her nurse came in to tell me that Hannah was running a fever of 102.7 on top of everything else.  Immediately we gave her Tylenol.   Even after an hour, it only went down to 102.3.   Then she started going into respiratory distress.  She was starting to have problems getting good breaths, so I made the decision to take her down to the ER.

Oh, and did I mention that Daddy just happened to be out of town for work that night?

My inlaws came to the rescue and picked up Ethan and Abby, and then Hannah’s nurse and I headed down to the ER.  Her oxygenation was decent at that point, still feverish, so we packed her up in the car and took off.

It was the ER visit from HELL.  Same hospital we have been going to since we moved here over a year ago.  Long story short, I could tell that the physician on call had no clue what to do with Hannah after all her bloodwork and chest x-ray came back normal (ruling out bacterial trachiitis and/or pneumonia).

Also, Hannah was poked and prodded so many times I was ready to tell them to back off!   First Hannah’s IV came out, blood pouring everywhere.   Then they got the second IV in, and then respiratory had to do some additional bloodwork to check her oxygenation efficiency — it took then THREE times with three separate finger/toe pricks and two different respiratory therapists to get blood that would work to be tested!

Finally, the ER doc was ready to discharge us, although I could see that she wanted to admit Hannah just because she didn’t know what else to do because she was thinking it was a virus.    But as I told her, unless you have something you can look for, observe for, or have a gut feeling as to what is going on, there is nothing that they can do with an admittance for observation that we can’t do at home where she is comfortable (as we do have a mini hospital here!).

Then when they took the IV out, we noticed that Hannah’s hand was completely puffed up and turning purple!   The vein had blown and the IV saline (fluids) that were being given had infiltrated!   Hannah’s left hand was four times bigger than her right and very scary looking!!   I even took a picture of it there because I was so worried about permanent damage (but Daddy won’t let me post it because he doesn’t want to see how bad it was because he is afraid of being more even more stressed out).

All the ER doc did  was continue to apologize, but I was expecting more.   Tell me how to make it better.  I kept offering ideas like should we put cold on it?   Should we elevate it?   “Oh, yes, you should do that.”   UGH!

We left the ER (5 hours later), and I felt horrible for Hannah because we left with her in worse condition than when we got there!

Fortunately, after 24 hours later (and keeping her arm above her body while she slept), her hand was back to normal.

Yesterday and today, she was still feeling not so great, fevers in the 101 range, etc., but manageable with Tylenol and Motrin overnight and then she was pretty mellow during the day.

Then around 4 pm today, Hannah’s nurse woke me up (I had crashed because I was exhausted) because Hannah’s temp had hit 104.5!   Immediately we started with the Tylenol, cold shower, cold washcloths, fluids, etc.  It was a constant race to work to get her fever down as fast as possible.

It took us a good hour before her temp went down to 102.3 (all her temps are rectally taken for a core temp – not fun!)   But that entire time, I was debating whether or not to take Hannah to the ER or call the paramedics to help us better access her (our pulse ox was acting weird!)

The scary part was that she would desat quite a number of times into the low 80’s.   We had broken out the oxygen and that kept her in the upper 90s.   Once we got her temp down into the high 101s and lower, her oxygenation started to go back to normal without the need for oxygen.

Finally, a few hours ago, her fever broke and she has been sleeping comfortable for about 4 hours now.  I’m still monitoring her oxygen saturations and breathing, but for now we are under control.  Although I just have a feeling we are not out of the woods with this virus or whatever it is.

NOW for my vent…  It is so friggin’ frustrating not being able to have someone who can help us figure out what is going on with her!   The entire time, I sit their weighing whether or not to take her to the hospital by saying “what would they do that we can’t?”  I feel like no one ever knows what to do with Hannah. and *I* have to be the one to tell them what to look for or explain in great detail to docs who sometimes don’t completely listen.

It sucks that we always have to be the ones “in the know” as to what to do and never get a chance to rely on the expertise of someone else because Hannah is always “so different” than what they are used to seeing.  I feel like if I go back to the ER (since we got all the bloodwork and chest xray done already, results not back on the viral panel), *I* would have to give them other things to look for or test for.

So emotionally exhausting and friggin’ frustrating!!  Worst of all, I still am not convinced we are dealing with a virus (WBCs are normal) and these high fever spikes a few days later, but I’m not sure what else it could be and really don’t have a physician who is really willing to figure this out.   (Although we have an appointment on Wed with her pediatrician to go over the viral results that should be back by then).

For now, she is fever free and sleeping.  I hope it continues.

Hannah has started to develop some what-we-think-are gastrointestinal issues.   There are really two separate issues, but I digress…

Constipation.   This started a couple of months ago (a few weeks before we started the Keppra wean).   Where once she was somewhat regular, it became more like a few days in between.   It had gotten to the point that after 4 days, she was so backed up and tight that you could see the obvious pain and discomfort it was causing her.

Suppositories do not work on her (well, they do about 1 out of 10 times).   So we started with the Miralax and a lot of tummy massage and leg movements.  After seeing her GI doc when this started, he suggested using 1/2 cap of Miralax once a day, every day just to help her along.   He thought it could be part of her neuromuscular degeneration process in that her motility isn’t as good as it once was.

Then it got to the point recently where after a few days, we had to also give her prune juice, just a few ounces, to help the Miralax along.   That was only twice, but now we have a big ole’ 64 ounce of prune juice in our fridge just in case.

So far, that seems to have help her “go” at least every other day.

The other issue, and this to me is the more serious one, is the spit up issue.  It started with a few times a week (again a few months ago), and now we are at at least a few times a day if not more.

She will be doing whatever, and you will see a lot of secretions and sometimes even formula come pouring out of her mouth.  Not a lot, but enough to soak up part of a washcloth.   The formula is the one that makes me nervous is because she is fed by G-tube, and this “whatever” is causing the formula to come back out of her mouth!

Also, she has been a bit more uncomfortable during her feeds over the past month or so.  Sometimes, even when she is sleeping, she will act agitated when we start a feeding.   Not horribly so, but at those times (about 25% of the time), you can tell it definitely affects her.

So I called the GI doc today.   Hannah’s local GI doc just recently relocated out of state, so I was transferred to the doc who was replacing him.   He asked a lot of questions and I did remind him that even though she is Gtubed, she does not have a fundoplication.  Since he had never met Hannah nor does he realize how truly complicated she is, I took it for what it was.

What *I* think may be happening is that either her reflux has gotten worse (even on 15 mg of Prevacid twice a day) which is causing her to vomit or spit up whatever is sitting on the top of her stomach or perhaps she may have some kind of stomach irritation (ulcer, inflammation, etc.).    On top of that, I think that she then may be aspirating what she is refluxing up, because on the rare occasion, we see formula come out of her trach (which of course wants me to get answers immediately).

He had mentioned considering changing her G-tube out to a GJ tube (goes lower down), but I don’t really think that is the issue.

Perhaps it is time for a fundo (which many Gtube kids have).    But a fundoplication means surgery, and that scares that crap out of me.

So we agreed that she needs some testing done to see what is going on.   He suggested doing a gastric emptying study as well as an endoscopy so they can see the path everything is taking.   Since she has to be put under for these procedures, I asked him if he could coordinate doing a broncoscopy at the same time with Hannah’s pulmonologist since it hasn’t been done since she was trached, and this is something that we definite would like to have done (especially to see if there could be the possibility of getting out the trach).

In the meantime, we are going to slow down her feeds even more.  Even though he was surprised at how little we are giving her at a time as it is.  I hope that they can get these scheduled quickly, but this is Vegas, and I’m not holding my breath.   Although I do think I may call her pediatrician and see if she can help move the process along!   If she is aspirating this stuff, this could be very serious!

I just hate not knowing what is bothering her and why she is spitting up, especially the formula!

We had Hannah’s IEP meeting yesterday.   We were told ahead of time that it would take 2 to 3 hours and there would be quite a few people there.   Sure enough, about 15 people brainstorming together (including us) for about 3 hours is what it took to put together goals and tools for Hannah’s future.

We were a bit apprehensive going into the meeting, mostly because of all the horror stories we had heard from other families and reading online about how they felt they had to really fight to get the services they felt their kids needed.   Many times they shared that they didn’t always get the appropriate services.

But it wasn’t like that at all in this meeting.  We had OT, PT, four nursing specialists (including Ethan and Abby’s local school nurse), speech, vision, psychology, teachers, etc.   Really, every specialist that would be important for her IEP.  Daddy and I really felt that each member of that team put a lot of time, consideration, and effort into their suggestions, reports, and benchmarks for her.

There was a very strong cohesiveness between everyone all geared towards what is best for Hannah, my little girl, instead of just Hannah, ID# 1254324.    They had put together a lot of very appropriate suggestions and benchmarks for her, and they were very open to modifying them when we brought up our concerns and hopes.

Best of all, we got for Hannah everything that we could have wanted.   Really, everything.

She is going to get to go to Abby and Ethan’s school for their morning program.   She is going to have a dedicated nurse in the class.   She is going to get PT, Speech, and OT every week (30 to 40 minutes each).    I will share the goals later on (they are in the other room), but there were a dozen or so that just seemed very “on target” for Hannah.   We shared our concerns about breath-holding spells (tantrums) as well as her heat intolerance issues, and they were all discussed at length.

Daddy and I walked out of the meeting kinda stunned because we were so worried about this meeting.   Yet, we went through everything we wanted to push for Hannah, and well, we were given it.

Hannah starts school on September 26th.  It gives us time to get the nursing situation organized as well as get the school staff trained and prepared for our special girl, especially since she will be the only trached child in the skill.

Daddy talked with the nursing company that works with the school district, and they are very willing to work with us on getting Hannah nurses that she feels comfortable with and who would be motivated on working with her as well.   After all, one of our big points was that Hannah really has to feel comfortable with her surroundings.

Best of all was the excitement Ethan and Abby had when we told them that Hannah was going to be going to their school.   To say they were thrilled is really an understatement!

So, September 26th is the day!

We are just a few days from our complete wean from Keppra, her seizure drug.   We haven’t noticed any increase in her movements or any other activity that would indicate she is experiencing seizures.   She has her EEG on September 29th, so we will see if she is having anything going on.

As a matter of fact, as I have shared over the past three weeks, we have seen an actual improvement in her fine motor skills, gross motor skills, personality, and cognition.

Three weeks ago, when she would cuddle with Daddy when she hears me call her from just a few feet away, she would just sweetly smile.   NOW, she will actually throw her body open, visually connect with me, and grin really wide and lift up her shoulders as if she is trying to tell us that she wants me to pick her up.   It is so awesome!

One of the best gifts we have received from this wean, however, is her ability to give real hugs.   She will actually work really hard to put her arms around someone she cares about (us, her nurse, her grandparents, the kids, etc) and give real hugs.   I mean, arms around the neck and squeezing hugs.  Even before the chaos of last year, she never really gave us hugs.   We always gave them to her, but she never reciprocated.

Now, not only does she reciprocate hugs but she seeks them out!   Abby just melts all the time now because Hannah will reach out and put her arm over Abby’s neck when Abby is laying next to her, face to face.

My absolute favorite thing is she now will caress my face when we are lying together cuddling.   She puts her hand, open palm, on my cheek and leaves it there.  Then she just smiles.   We will just stare at each other, very lovingly, for 10 to 15 minutes.   Just smiling, eye gazing, and caressing.   Weeks ago, it was me always doing the caressing.

Now, she caresses my face.  I really can’t describe how amazing I feel when we have these moments.  These intimate, sensitive, and just pure moments.   It is just pure love.  Sweet, innocent, true love.

She truly is one of the best things that has EVER happened to me.

First off, just wanted to share with everyone that Hannah’s “mysterious lump” was just a cyst!!!!   Talk about relief 🙂   Even better, it is almost gone already.

Next week is a big week for Hannah’s educational, therapeutic, and socializational future.   We have her first IEP planning meeting on September 1st.  You know, I just realized that I don’t even know where the meeting is being held — I better call on Monday to find out!

But before that meeting, Hannah has an audiology appt on Monday and then on Tuesday a pre-IEP meeting with the school district nursing supervisor, Ethan and Abby’s new school nurse, and a school district nurse who specializes in trach kids.   I felt very good that they called and wanted this meeting — tells me that they are really wanting to look at Hannah, the person, not Hannah, the “medically fragile kid on paper.”

I’m not sure what to expect at all for this IEP meeting.

Today we went to Ethan and Abby’s new school open house.   I just LOVED all the people there, the kids’ teachers, the support staff, and we even introduced ourselves to the school nurse who will be out to our house next week for the pre-IEP meeting.    They have a ‘strong’ special early education program, but I don’t know if it will be the right fit in their eyes for Hannah.

If Hannah could go to the kids’ school, OMG, that would be a dream come true!   Just thinking about it is getting me too excited — almost too good to be true.   I keep thinking how I would be able to volunteer at the kids’ classes while Hannah is in class, how if Hannah is needing some loving during the day, Abby and Ethan could be right there (boy were they excited about that idea!  “Mommy, I will come give Hannah kisses every day in class!”

Getting ahead of myself here, I know.   Keeping my fingers crossed that what is best for Hannah will work out to be there!

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).

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!!!!!