The clock just changed to midnight.  It is now Christmas Eve.

We spent the day today (day before Christmas Eve) doing a major winter-cleaning of Ethan and Abigail’s rooms.  We have this “tradition” that the kids rooms have to all be cleaned and really organized or Santa won’t bring them any new presents.   You know, throw out the old, broken, and no longer played with (those get donated).

This year, the rooms were disaster areas, moreso Abby than Ethan.   In all honesty, we had not spent any time really organizing it properly since we moved here this summer.  Abby’s room alone ~ 2 black trash bags full of junk!    Ethan was very proud and did most of his room himself, but he did let Daddy help after a couple of hours.

It was a great feeling getting that accomplished!

We have two more rooms to tackle before “Santa will come” later on today, which include Hannah’s area downstairs.  Ethan and I started going through Hannah’s toy area this evening.

Hannah has had many of the same toys she has had for the past 2 years.   Since her development has not changed much in the past 18 months, many of the toys she has area still at her level.  Those toys that we were being ambitious with many months ago in hopes she would gain certain skills were put in the ‘garage sale’ box.  Daddy and I both came to the realization that there is just no reason keeping around certain toys for many, many months at a time in hopes that she will get the hang of it.

It is just not in the cards for her right now, and if in the future she does gain those certain skills, then we will rush out to the store and buy them again.   I’d like to say I’m okay with this, but truth is, it is not like I have any choice other than to accept it.   As I keep saying with regards to this disease, “it is what it is.”

Santa has more presents for Hannah on Christmas, as well as gifts from friends and family.  Some her age level, some being ambitious again.   We have to keep trying to be ambitious because we never know what may click with her.   I guess that will never change with us.  We will never give up on her.  And if she doesn’t play with them for the next 12 months, there will always be next year’s winter cleaning.

Hannah had her Keppra level drawn at her Cerezyme treatment on Monday.  It was at 9.0.   Dr. Brown, Hannah’s neuro, wants it to be therapeutic at 15.0, so she is raising her Keppra dosing to 2.0 mL 3 times a day from 1.5 mL three times a day.  The Klonopin will stay the same for now.

I will have to admit that Hannah is sleeping more soundly now that she is off the Haldol (completely as of Friday).  Even though she is still tired almost all the time, it is not as bad as it used to be.  She still fights sleep INCREDIBLY, but for the past few nights, she has not gone to bed until 11 pm instead of 8 pm!  She still is taking her two naps a day, but instead of her second nap being at 3 pm, it is more like 5 or 6 pm now.

We don’t get another EEG for a few months, but I keep hoping that with the removal of the Haldol and the increased Keppra that her seizures are under better control.

On a separate note, we finally received Hannah’s “smart vest.”  It is a vest that she wears that pounds her chest for a period of time to loosen the junk in her lungs.  It is used a lot for cystic fibrosis patients.  Her vest is “yellow.”  Initially, I thought she would hate it because she does not like being restrained at all, but the three treatments we have done with it so far she has actually liked it!  Weird, huh?    I’ll take a video of Hannah with it, but to get an idea of how it works on another baby, click here.

Because the kids are home from school for the next two weeks, we have cancelled most of Hannah’s therapies and appointments with the exception of her Early Intervention service coordinator visits, her cardiology followup for her enlarged aorta, and her upcoming Cerezyme treatment.

Our 12-1/2-year old beagle’s body is failing her.   Molly lies on her dog bed all day long, and when she does get up to walk to the food or water, it is a very slow, uneven, and limpy type of walk.  Going through the doggie door takes her quite a bit of time, as she is slowgoing.  Also, Daddy had to make a path around the deck because she can no longer go up the stairs.

Instead of getting up to bark, she lies there on her bed yelping when someone comes in the door these days.

But she does not seem like she is in pain.

Daddy and I are faced with the decision now as to “when” it is okay to let her go.  This decision seems so much harder these days, especially in light of all of those children that are fighting so desperately for life right now.   I feel like this decision is now so much bigger than just one dog, you know?  It is almost like this decision of “when or wait” is going to dictate so much more of our life than we expected.   Quality of life versus quantity?

My dilemma stems from having Ethan and Abby find her either not breathing or suffering if we don’t do something.  I feel it would be more devastating for them seeing Molly no longer alive than if it happens when they are away at school or something.

For me, I think it is almost time.  Daddy is not so sure.  Neither of us know when the right time is.  We are both afraid to make that determination.  Shouldn’t it be an easier decision?

Obviously, I know there is a bigger picture here with this decision that is making it so much more difficult than it should be.  I can’t even put it into words, but I really don’t need to.  I know there is only so much protecting of Ethan and Abigail I can do…I just don’t know what to do.

We finally got Hannah’s nursing figured out with our new insurance company.  We get 56 hours a week, broken down however we want to use them.   Previously, we had 48 hours a week, no more than 8 hours a day (currently M – Sa, 9 am to 5 pm).  So this is a pleasant change.

I just don’t know what to do with them.  I have asked Daddy for advice, and he keeps saying “whatever YOU want, as this helps you more than anyone.”

My dilemma….as you know, I do the “night shift” every night with Hannah until about 4 am or 5 am.  I get about 4 to 6 hours of sleep at that point (usually broken down into two “naps”), depending on if Daddy has an appointment in the morning or if Hannah has an appointment (though I have been making a point not to schedule anything before 11 am these days).   The days Daddy is out of town or can’t do the mornings with all three kids are a nightmare for me (like this morning).

I decided to take on this night shift in order to take advantage of having a nurse here for Hannah when the kids get home from school.  They get home around 3 pm and the nurse leaves at 5 pm.  This is my one-on-one time with Abby to get her homework done and whatever time is left over for Ethan and Abby.  Also, it helps tremendously having a nurse along Hannah’s appointments with me, especially in the car because I don’t have to stop everytime she has a tantrum or needs suctioning.  But it also gives me free time during the day to make phone calls, etc., but really not enough time.

We still want our Sundays nurse-free.  That is a given.  It is a nice break to just “be a family,” and we have settled into a good routine with that.

So, do I give up a couple of nights and get night-nursing?   That is a possibility.  But then I wonder if how my body would deal with that?  I mean, I am so used to be up until 4 am or 5 am now.  Would I even be able to sleep drug-free if we had night nursing?  I wouldn’t take any type of sleep aide because I want to be able to wake up in case of an emergency.

Or do I just accept the fact that I am the night-nurse and that is the way it is.  I get caught up on my email communications plus the business I have created (to go live on 1/1/11) was created with that kind of flexibility.   The ones who suffer, besides me, are the kids on the weekends because I usually sleep until noon or take a 2-hour nap during the day.  But again, they are used to that and accept that.

I don’t know what to do… give up some nights or not…  I really just don’t know what would be best.   I have 56 hours to divvy up.  How best to use them?

Hannah has a cold.  A simple, normal child, everyday cold.  She has no fever.  Her white count, as of today, was normal.  Her ears are fine, so we know there is no ear infection.  We know it is not trachiitis because her secretions most of the time are clear.   So why is it when Hannah gets a cold, I am so on edge with every cough, throw up, and gag?

I’m starting to get concerned that Hannah is developing laryngospasms when she coughs.  She gets so gooky in her trach, and it is almost like she goes into these spasms trying to cough it out.  They can last anywhere from a few seconds to maybe 30 seconds.  Rarely is there any more secretions in her trach, but she still is acting as if there is something in there.  Also, her body will go stiff sometimes during these episodes as if she is using all of her power in her body to deal with these spasms or gags.

She has also started throwing up mucous again.  We had a week or so of throwup-free days.  But again, it seems like she is generating so much secretion in her trach because of her cold, swallowing it before we can suction it, and then her stomach fills out, etc.

Also, it is only 1:30 am, and I have already had to suction her about 5 times tonight because she has thick mucousy secretions that are causing her to choke because they are too thick for her to swallow. (I rarely have to suction her on a normal night).

It also doesn’t help that she is still going through the Haldol withdrawals.  Tonight was her last dose…hopefully forever!

Makes me wonder if she will really get the trach out in the spring.  Is it in her best interest to do so?

Of course there is no one to really ask here.  I know more about this disease than any of her doctors.  They will be like “it could be” or even may say it is because it may look like it when it could be some other neuromuscular problem caused by her Gaucher’s.  So I feel like we are on our own to make these decisions for her.   We will cross that bridge when we get to it, I guess.

BTW, this post took me about 30 minutes to write because I ended up suctioning 9 different times.

This was just one of those days I wish I could write off…

It started with me packing Hannah up for her biweekly Cerezyme infusion.  For me, I was running on 3 hours sleep, and Hannah was cranky about 5 minutes into the 30-minute drive requiring a LOT of mommy singing “Wheels on the Bus, Ice Ice Baby, and Old McDonald”… over and over and over again.

While in the waiting room, there were two young kids (about 3 and 2 I guess), and they were fighting at the toy wall while the mother was at the window.  The little girl (about 2) started screaming at the top of her lungs.  Ear piercing, mind numbing screaming at her brother.  The mother did not flinch.  The other family that was waiting and I kept looking at each other like “Come on mom, deal with this” while nurses started pouring out the door to see what that horrific noise was.  It must have lasted at least 3 minutes, no exaggeration, before the mom came back acting like “yeah, whatever.”

It freaked Hannah out after about a minute, and we moved to the other side of the waiting room (which is not that big, mind you).

We finally get into the nursing room, and they start to do Hannah’s BP and temp.  Hannah’s infusion nurse, Mary (whom I adore) comes in and tells us that they don’t have her Cerezyme.

Apparently there was a huge ‘thing’ last week about Cigna, our new insurance company, refusing to cover Hannah’s Cerezyme treatment because she has neuronopathic Gaucher’s disease.   There were a lot of phone calls made on our behalf late last week between the infusion center and Cigna, and they finally got Cigna to agree to cover Hannah’s life-extending treatment.

I could make a whole rant on the insurance industry not wanting to cover type 2 and type 3, but I don’t feel like it right now.  Just know it is in my head.

Hannah’s infusion nurse apologized for forgetting to call me that it wasn’t there.  So we headed back home.  Another 30-minute drive with a toddler who hates being in the car for more than 10 minutes.

THEN, there was the fiasco with trying to get the process started for Medicaid to cover Hannah’s over-the-counter meds.  We have done everything we were told — get the prescription, tell the pharmacy what the plan was, etc.  CVS could not figure it out.  They said they required certain codes to process it, but Medicaid could not give out those codes.  Huh?!?

Finally, I contacted our awesome Katie Beckett case manager who offered to help straighten everything out for us.  I tell ya, she sure makes up for that horrific case manager we had in Texas!

We have to go back on Thursday for Hannah’s Cerezyme treatment now.  Better late than never.  I am so thankful that our infusion nurse didn’t give me a heads up that this was all going on last week — the stress of Hannah losing this treatment would have been a nightmare for us!

This was all done by noon.  Needless to say, I was ready to write the morning off…