It has now been almost two weeks since we finished the Haldol taper, and she has been Haldol free.

We have noticed some definite changes in Hannah, mostly positive.

The good is that she hardly twitches when she sleeps now, so we are hoping that means we are getting her subclinical seizures under control with the removal of the Haldol and the upping of her Keppra.  Haldol is known to lessen seizure thresholds, so we are hoping that is the case and now that it is gone, whatever seizure activity she has been having is under control.

The bad is that her movement disorder has worsened to some degree.  Where once she used to hold on to toys and keep them in her grasp, she does not seem to be able to do that as well anymore.  I feel like we are having to put things in her hand and help her get the grasp going now.  She still loves to play with her toys, but it is more of a press-button cause-effect type of thing.  We got her Megablocks for the holidays, and she loves knocking them down.  But I think we are going to have to focus on her grasping and using her hands for fine motor toys again.

You can see this movement disorder most prominently in her face and her hand/arm movements.

Another good is that she is no longer taking two 2-1/2 to 3 hour naps each day.  Even better, she seems to be trending towards sleeping through the night again!  She still takes 1 long nap each day and maybe a second  power nap, but she definitely seems much less sleepy during the day (even though she still seems exhausted much of the time).

But the plan stays the same for now.  We wait until late January to start working on fine tuning her meds for her movement disorder.  We need to get the keppra therapeutic as well as make sure the Haldol is completely out of her system.

I know they say that this movement disorder is not a Gaucher thing.  But I can’t help but think that the oversedation and polypharmacy during her hospitalization this summer created a firestorm neurologically that may not be able to be reversed.

We had a fantastic Christmas, but I want to wait until my pics are done uploading before I share all about it.

Tonight Daddy changed out Hannah’s trach like we do every Sunday night.  Out with the old, in with the new.  Trying to keep things as clean and sanitary as possible.

I was with Ethan in my office when I heard Daddy yelling for me to get over there quick.  Ethan panicked (as he usually does at first) and I ran to the family room.

Hannah was visibly upset and panicked, and she was trachless.   For whatever reason, this time she got very upset when Daddy changed out her trach to the point where she clenched up, started breath holding, and completely freaking out.  When this happens, it is much difficult to get the trach in because it is tighter and she is freaking out.  Abby even started noticing that “Hannah’s face is changing colors.”

She started turning blue around the mouth.  She was trachless for about a minute at this point, and I just sat there holding her hand trying to talk calmly to her to get her to release the tension in her throat.  Daddy got the smaller size trach, and with that combination (her calming down a bit, the smaller trach, her becoming less tight), we were able to get her trach back in.

The color around her mouth started to come back from the bluish-grey.   Daddy was able to change the trach back out from the smaller one to her normal size without any problem.  She was really junky afterwards with all the manipulation, so we had to suction quite a bit of blood-tinged secretion to get her clear.

She is fine right now.

But the one thing that really freaks me out, after the fact, is that Hannah did not try to breath through her mouth and was having a breath-holding spell during this.   She can breathe fine through her mouth when she is capped, but when she panics, she breath-holds.  It is a neurologic response, not something we can tell her not to do.   This is what sent us to the hospital back in May — the tantrum, the breath-holding, the hypoxia-induced seizure, and the possible anoxia-induced stroke.

Daddy and I talked about it tonight, and we both think that maybe getting rid of the trach in the spring may not be in her best interest.  Not because of swallowing difficulties, not because of laryngospasms (the typical Gaucher reasons to get trached)… but because of these damn breath-holding spells.  It is disheartening to come to this conclusion, and we aren’t making any decisions about it right now, but NOW we are not expecting to get rid of the trach in the spring like we have been.   It is now something we will have to think more seriously about.

So many things go through your mind when your little baby is turning blue and your two other children are right there watching it…sucks.

It is 2 am. I just finished Hannah’s overnight feeding and put on her humidified trach collar. Then I went into the hallway and looked over our ledge upstairs to our family room below. It is overflowing with Santa presents. We really overdid it this year with the gifts, especially for Ethan and Abigail. Let me rephrase that…SANTA overdid it this year. (Yes, Ethan and Abby still both believe in Santa still!)

A lot of these gifts are ‘guilt’ gifts.

I had a very fun and  enjoyable Christmas Eve with Daddy and all three kids tonight. We baked brownies, had a delicious Christmas dinner, and spent time all together in the family room.

Even so, I couldn’t help snuggling closer to Hannah as much as I could. I kept having these horrible thoughts in my head about wondering what next Christmas may look like for us? How much I wish I could change Hannah’s prognosis.

I don’t want Hannah taken away from me. I don’t want to spend next Christmas without her or the Christmas after that or the ones after those. I don’t want Hannah’s condition to worsen any more than it already has.

I know Christmas is a tell of celebration and happiness. But as happy as I should be because she is still here this Christmas, I am scared to death about what next Christmas may hold for us. I know how horrible it would be for me and Daddy, but I don’t even want to think about how it would scar Ethan and Abigail.

So I the ‘guilt’ presents are just that. I want Ethan and Abigail to remember this Christmas with lots of love, baking, playtime as a family of 5. I want to have them spoiled rotten today because Hannah is as good as she will ever be (unless the miracle we pray for comes through), and they love playing and interacting with her, and she loves smiling and playing with them.

In just a few hours, Ethan and Abigail will be running to the ledge and see the presents and be horribly anxious to get downstairs. We will all wake up, and then the present unwrapping will begin. What will make it even more special is that, like last year, they will be even more excited opening up presents for Hannah and sharing them with her, because they love her just so much.

Dear God, please give us another Christmas together. I beg of you…

Dear Santa,

All I want for Christmas this year is a cure for Neuronopathic Gaucher’s Disease.

Love,

Ethan, Abigail, and Hannah’s Mommy

I was watching Ethan start playing with the iPad.  Hannah heard it turn on, and she crawled over as quick as she could to see what Ethan was doing with it.   It was really funny because she basically threw herself over his legs to see what was on the screen.   Then, she proceeded to “turn the page” of the iPad so that Ethan could not play anymore.    (“Turn the page” is what Hannah does when she is done with something — when she is done with the iPad, she will turn it over as if it was a page in a book.)

She didn’t want her brother playing with the iPad anymore because SHE wanted to play with it!  I WISH I had my camera with me for that one, it was so cute!

The iPad has been a godsend of a tool for her.   We have about 5 or 6 apps that we have found that really have made Hannah focus on her cause/effect and especially her fine motor control.

We have Talking Tom Cat to thank for really getting Hannah excited about interacting.  This one took awhile for her to get and understand, but now it is by far her favorite.  I have to admit that her nurses and our family all like it too because we get to say things, and Talking Tom repeats them in his higher cat voice.  Hannah does not seem to put two-and-two together and realize it is us saying these things.  She just loves it when Talking Tom says her name and talks to her!

She has finally learned how to ‘pet’ him and tries to get him to fall down.   There are white buttons on the sides that you can press to have him do certain things (play cymbals, drink milk, catch a bird, etc.).  Hannah has not quite figured out which one to press to get him to do certain things, but she definitely loves when she does get him to do it.  She thinks it is hysterical when Talking Tom toots!

I can now see why so many people recommend this app for kids like Hannah.  It is fantastic!

It is really awesome to see Hannah WANTING to be interactive with her iPad.   We probably let her play with it about an hour during the day (broken down into anywhere from 10 minute to 15 minute sessions).   It also has been a godsend when having to deal with doctor appointments because we have her Wiggles and Mickey Mouse movies on there.

And the real reason Hannah loves her iPad?  Ice, Ice, Baby!