Hannah’s spleen and liver seemed to be getting larger again.   We made huge progress in shrinking them down when she started her Cerezyme treatments back in February 2009, but I’m a bit concerned as to why they are starting to grow again.

Dr. Bernstein, Hannah’s local specialist, scheduled Hannah to get a CT of her abdomen today at 11 am so we can see exactly where we are at.  We can compare this to the CT done back at the National Institutes of Health back in March of last year.

What makes me really nervous is that she has to be sedated for this CT.  After all the problems with the sedatives this summer when Hannah was hospitalized, even using any amount of a sedative is making my stomach anxious.

But I know this is important.  We need to have this information.

Remember that “rush” neuro appointment I set up a couple of weeks ago?   We had our appointment today at 3:30 pm.  We didn’t get into a room until 4:30, and the doctor didn’t come into the room until 5:05 pm.  We left the office at 5:17 pm.   See where this is headed?

We told her everything that has been going on over the past weeks from us noticing Hannah’s movement disorder getting worse around the holidays to her being really sick the past few weeks and the double-antibiotics, etc.   I told her about my concerns her movement disorder and how I wanted to work on getting a better handle on it.

Her reply?  “Let’s sit on it a month and see where we are at.”    Huh?!

It wasn’t just that part that was a disappointment.  Hannah’s nurse and I were rehashing the visit and conversation, and the more we talked about what was said, the more angry I got.

When we told her how sick she was and that we “almost went to the ER,” her reply was, “You should have gone to the ER immediately.  If you see any decline in Hannah, you should just go straight there.”   Um, no.  You don’t talk an already immuno-compromised child with strep and trachiitis to the ER and expose her to other things unless you have to — as per her pediatrician (which I agree).  There was nothing the ER would have done that we didn’t do — Motrin and keep close tabs on her.  Had things turned, we would have been to the ER in a heartbeat.

She also made the comment, “Whenever Hannah gets sick with something like this, there will be skills lost and a tougher recovery.”

When I asked about medication adjustments, she said “Well, we don’t have a lot of options.  You don’t want to put her back on Haldol, do you?”  Again, huh?!  Of course not!  This decreased her seizure threshold and was a total nightmare to wean off of.

I asked about the Klonopin — could she have become so used to it that it doesn’t work anymore, hence the increased movements?  Could it be that her dose is too low, and she needs more?   “Yes, that could be.”   That was the answer to both of those questions — either the Klonopin is completely ineffective or it is just too low of a dose?

We felt completely rushed during the visit.  She made the comment that she was overbooked that day and that she lives 80 miles away and has to commute.  We were obviously once of her last patients of the day.  She examined Hannah for less than a minute.

So, she wants to see where we are in a month and then start increasing the Klonopin.  That is “the plan.”   (Along with getting a Keppra level to see if Hannah is therapeutic yet).   A month can be a huge amount of time for Hannah, especially if her movement disorder continues to increase.

My plan?   Call the third (and last) pediatric neuro in Vegas and set up a consultation.  I don’t expect someone to have a complete understand of what is going on with Hannah, but I want a doctor who will at least look at Hannah as a patient and really consider what is best for her.

I wish, wish, wish we could get Hannah to see one of the pediatric movement disorder specialists in Cali.  But getting Hannah there is going to be so difficult, especially since we can’t drive and can only fly.   But we may just have to bite the bullet and do it.

Today was just a messed up day.

We found out Friday night from our nursing agency that our Monday nurse is no longer coming on Mondays, “Oh by the way…”.   So that means we have to train another nurse for Mondays, but since today was a Cerezyme day, I wasn’t going to have a new nurse start during her Cerezyme infusion.  Just would not be helpful at all.

So the plan was that Daddy was going to drive Hannah and I to the infusion center (because it is just not safe to drive distances, even 30 minutes, alone with Hannah).  He brought his laptop so he could work there while Hannah and I sat in the infusion chair for her treatment.   Also, being that I only had a couple of hours of sleep, it just wasn’t a good idea for me to drive anyway.

Our regular nurse was there, and for whatever reason, she could not get a good stick.   Three attempts, and she could not get a good vein.  Poor Hannah was just so upset by this point.   Never once, in the 2+ years that Hannah has been going for her bi-weekly infusion treatments, has Hannah ever had to miss a treatment because they couldn’t get an IV.  So, we had to reschedule her treatment for this coming Thursday — which screws up our Thursday plans and no future infusions, which we are no longer going to do on Mondays because we have the new nurse.

We came home, and our new nurse showed up.  Very nice lady.  Hannah tolerated her well, which is a good change from the time that she disliked a new nurse so badly she wouldn’t even go near her.  So I spent the day orientating the new nurse to our routine, Hannah’s toys and apps, and everything else.  About 5:30 pm, I was so exhausted from lack of sleep, Daddy came down to finish the day while I crashed.

Woke up at 8 pm tonight, and the new nurse was just getting ready to finish her shift.

Hannah’s naps were off also all day, so she didn’t take her second nap until around 5:30 pm.

Guess what time she fell asleep tonight.  12:15 am!!!   Yep, I just put her down a little bit ago.   Daddy fell asleep at 10:00 pm, and from that time until after midnight, she just sat cuddled next to me, smiling, grabbing my hands, and just being so dang adorable.

Hannah seems to be doing even better today than yesterday.   Even though her movement disorder is still pretty significant when compared to a few weeks ago, her personality is definitely coming back.   Today she was playing with her iPad, reading books with her nurse and I at her little desk, and she actually crawled four or five feet to play with her toys.  She is a lot more engaging today than she was just a few days ago.

Such a huge relief!

Hannah always keeps us on our toes.  There is rarely any relax time around her, at least for me.  I’m always mentally prepared for something to happen unexpectedly… she pulls her trach out or she falls backwards or she throws up.  Something.  When I am away from Hannah in another room, I always am on edge waiting for Daddy to yell, “Honey, get in here.  She pulled her trach out!”   Even now that she has decannulated herself twice with both her nurses to the point of turning blue, I also have that in my head “just in case.”

Maybe that is why I always feel the most comfortable when I am lying or sitting right next to her.  Even if she is just playing on the floor, being right there were I can see her hands or keep tabs on her balance actually makes me relax more.  Weird, I know.

I’ve said it a hundred times already, but you know what makes it all worth it?  Her smile.  That gorgeous, infectious, heart melting smile.

I will do whatever it takes to keep her safe.  So if it means more mental preparation and being a little on the edge all the time instead of total shock and fear when something happens, so be it!  Maybe I’m a freak in that respect, I don’t know how other parents of medically fragile kids do it.   But it works for me.  I always would rather be somewhat prepared than be totally taken off guard.

I don’t even know if “spatial security” is a real term or not, but it is the best way to describe Hannah’s actions right now.

Hannah was a bit better today.  She was more willing to play with her toys, and she would even crawl/scoot a couple of feet at times.   She seemed to both me and her nurse to be in much better spirits.   I can’t tell you how great it was to go downstairs this afternoon and see Hannah smile as soon as I said her name!

Today’s nurse is the one we have had since we first brought Hannah home from the hospital back in June.  She and Hannah are a perfect match for each other, and I know how much Hannah adores her and how much she adores Hannah.    She remembers when she first met Hannah, and Hannah was so much like a newborn that she couldn’t even roll over or suck her pacifier.

We talked a lot about Hannah’s condition today.  The marked increase in her movement disorder.   Her increase in stiffening up her entire body when you go to move her or pick her up.  Her even stronger need for physical support, etc.

She believes that Hannah has lost a lot of spacial security.   After researching it online tonight, it seems a medical term similar to this is called “Developmental Dyspraxia.”  It fits her situation perfectly.

She always wants to be physically….enveloped, I guess you can say.  She always had some sense of it, but it seems to have increased 100-fold in the past week or so.  She stiffens up if she doesn’t feel that security to the point where she becomes like a dead-weight board.    We have even had to put a pillow behind her little wooden chair this week because of it — she was using it without a problem last week.

When we go to pick Hannah up, she will stiffen up so tightly until she feels that complete security where it is okay to release.   There were times today that even though I held her tightly and securely, she still wouldn’t relax.   Before, she will relax once she is in your arms.

Her nurses and I are doing a lot more PT type of exercises and massages with her to try and loosen those muscles.  I can’t imagine how much it would hurt having your whole body clenched up like that so often.

I noticed tonight, as she was lying in bed with me (as she still will only go to sleep as long as she is cuddled with someone), I noticed that she would not let me cuddle her leaning against the pillow.  She pulled me down so that she was lying completely on the pillow, and I had her wrapped up in my arms.  Almost like a swaddle.

Perhaps this markedly increased spatial insecurity is brought on by her increased movement disorder.  She is trying to hard to control her body now, especially her hands and face, that she is just getting wiped out.  It has even reached the point where she can’t grab her paci and put it in her mouth anymore.  (Even if we put it in her hands, she can’t get it in her mouth.)  At least when she is “enveloped,” she doesn’t have to work as hard at obtaining that security because we are providing it to her.

Tuesday’s appointment with the neuro cannot come soon enough.  I hate the fact there are no pediatric movement disorder specialists her in Vegas!  I tried the single adult specialist, but he will not see Hannah.

Sucks, sucks, sucks.