Tonight, we have Hannah hooked up to both her humidified trach collar and her oxygen.   This is a first.

We actually haven’t needed to use the humidified trach collar (keeps her trach moist while she sleeps) in months in the old house.   I don’t know whether it is much dryer in this house or the fact that the air filter was incredibly nasty (which we just changed out this afternoon), but she has woken up horribly dry to the point she has a bit of difficulty getting a good breath.   This has happened all three nights here, even with me giving her saline bullets many times during the night.

Even after a nebulizer treatment in the morning, it takes a while with a lot of saline bullet drops to get her back to normal.  But the good thing is that she does get back to her normal clear trach secretions.   Ironically, after this past bout of trachiitis, she has been more gooky than usual which is common for her, so it was surprising how dry she became overnight these past few nights.

Also, she is still desatting into the upper 80s/low 90s while she is sleeping.   With the added oxygen, she goes back up to the upper 90s where she should be.

Even though the two aren’t meant to work together (at least I don’t have the proper equipment to do so), I have it gerry-rigged to make it work.  I know the oxygen is helping, and I am keeping my fingers crossed she wakes up more comfortable and moist.

We meet with her pulmonologist on June 1st to work on a game plan as to what changes we need to make.  I’m worried about her oxygen levels dropping while she sleeps, but I also am realistic that this very well may be part of her disease process as it has been this way with many Gaucher babies.

It is incredibly loud in our room right now.  I wonder if they have a quieter humidifier compressor because I can’t hear anything, not even the TV which is just about 10 feet or so away let alone if Abby or Ethan wake up and need me.

New reality?  Temporary setback?  Well, either way, hopefully she will wake up more comfortable and not so dry tomorrow morning.

We moved into our new home this weekend.  My husband, father-in-law, and brother-in-law moved all the heavy furniture themselves.  My in-laws and my hubby must have made dozens of trips back and forth between the two houses to bring more and more things over.   Tonight we celebrated with them with a BBQ in our new backyard.

I LOVE this new house.  It is a combination of a fantastic layout, a massive purge of stuff, and getting my ocean blue painted bedroom again.  It is weird because the house is quite a bit smaller (700 sq feet smaller), yet it still feels big, open, and very comfortable.  The backyard is also great (and even has a built-in space for a jacuzzi at some point), and we love sitting out there with Hannah while the kids are playing.  Even better, the way the house is situated, we get shade in the backyard in the afternoons, which definitely makes it cooler and more comfortable!

My first priority was getting Hannah’s area created and her supplies organized.   It was incredibly important to me that we make the living room look less like a hospital and more like a living space (in the old house, there were med supplies everywhere in the living area).  We gave her the big linen closet for the med supplies, and that still is just not enough so I will have to redo that at some point, but at least it is all hidden.  The only things left in the main living area are her oxygen, her chest percussion vest, suction, and a small basket of go-to supplies under the coffee table.

Another big change is that Hannah is now sleeping in a big girl bed by herself and not sleeping with me!   For the past few months, Daddy has been sleeping in the spare bed in the office and Hannah was sleeping with me.  In this new house, we have our king-size bed AND her twin bed in our master bedroom (and it fits great).  It is going to take an adjustment because I used to doze off at night holding her hand or leg so that if she woke up, I would know it (since she doesn’t have a vocal cry).   But I miss having my hubby sleeping next to me.  Now I have my hubby in bed with me, and Hannah just two feet away in her own bed.  She still has her toddler bed in the living room for daytime naps.

Our nursing situation is still a big question mark.   Today, our normal Monday nurse was supposed to work 7 to 5 today because she couldn’t work on Monday.  Yet, she ended up leaving at 11:30 am because of a family crisis and was too upset to finish the day.  She says she will make up the hours tomorrow (Monday, on the day she originally needed off), so I’m keeping my fingers crossed.  We have a backup nurse for Tuesday, but I don’t know what the rest of the week holds for us.  Our Monday nurse (that left early today) is supposed to take 4 days a week starting the week after next, but I don’t know what the nursing agency is going to do for those extra two days.

My hope is to get our house completely unpacked by Hannah’s birthday, July 25th.   There are so many possibilities with this house, and over time, as we have the money and time, we will make it perfect.  I am really, really loving this house!

Our Tuesday thru Thursday nurse was fired today.   I got the call 5 minutes before the end of her shift from the nursing supervisors to let me know that she wasn’t going to be back.  They couldn’t give me a tangible reason why, but obviously it was something serious enough that it had to be done at that moment.

Hannah LOVED her.  She was absolutely wonderful with Hannah.  I could honestly say she was the best nurse we have had since we started with nursing.  You could tell that she just adored Hannah as well.  She would sing to her many times during the day, and Hannah would have a huge grin on her face every single time.   She always was right on top of all our “daily goals” in terms of feedings, therapies, etc.

Most of all, I felt like I could totally trust her to take care of Hannah’s needs when I had to go out of the house.

It sucks.  I can’t blame the nursing company because obviously there were circumstances that warranted this termination because they know how much we liked her and how we are still in need of a normal nursing team.  I could also tell in the nursing supervisor’s voice that there was a sincere disappointment that it had to be done.

But it still sucks.   The timing is absolutely horrible.  Tomorrow I am supposed to get the sutures removed from oral surgery I had done last week.  We move the furniture to the new house on Friday and Saturday.   There will be weeks of unpacking, I’m sure.  Maybe even more.   Ethan and especially Abby are needing more and more attention these days as Hannah’s disease progression is becoming more obvious.

Most of all, Hannah needs someone she can feel comfortable with and trust.  We have had a parade of nurses since the end of March when we lost our two regular nurses.  I hate doing this to Hannah…all these new faces, new people, and we just leave her alone with them for hours on end (even if we are in the same house).

I have a tremendous, tremendous amount of guilt every time a new nurse starts with Hannah.   It kills me to just leave her alone with a stranger after just sitting with them for about 30 to 45 minutes or so.  They don’t know her cues, they don’t know her wants, they don’t know what makes her happy and comfortable.    And what is worse is that I am usually sleeping the first half of that shift because I was up the night before doing the night nursing stuff.

Right now, we only have one regular nurse, and she works on Mondays.  Thankfully one of our backup nurses was able to cover tomorrow’s shift so I can get my sutures out, do carpool, and get to the new house to pay the painters (I really didn’t want to bring Hannah to the new house with the smell of fresh paint fumes).  Friday and Saturday are covered for this week, but who knows what next week holds.

There is an emotional side of nursing that I don’t think outsiders get.   Yes, we need it to help us try to keep our heads above water each day.  But there is also the guilt and frustration because we are leaving our child to be cared for by someone else, which is a constant reminder that I, as a mom, am not able to get my crap together enough to be able to take care of her and do everything else.

What makes things worse, every single time I leave the house and leave a newer nurse home alone with Hannah, I am scared to death that something is going to happen to Hannah — her trach comes out and the nurse can’t get it back in, she has a tantrum that leads to breath-holding, etc.   It takes me many weeks before I feel comfortable just leaving her with the nurse without feeling that way.

But yet, it isn’t reasonable to be at the house every single minute the nurse is, but I do try.  I just feel like I need to be there for her.

Right now, with the loss of this latest nurse, I feel completely overwhelmed, stressed out, and just very sad.   Sad that Hannah has to deal with the loss of another person that she came to like and trust, and now the roller coaster of new faces, being left alone with strangers, and separation anxiety begins again.

We made it to physical therapy today.   We haven’t been there in 3 weeks because of the Disney trip and then the subsequent trachiitis that knocked her out.

When we got there, Hannah’s PT brought us into a side room and told us that their corporate office is becoming more strict on missed sessions.  Reality is that we only go to PT when Hannah is awake and up for it.  There is no reason to wake her up or go when she isn’t feeling well because she won’t do anything except cry and get upset.

Our PT’s office has been really understanding since day 1, understanding the fragile nature of Hannah’s condition and the reasons why she can’t always make it.   But apparently their corporate office is not so understanding.

Her PT suggested strongly that we go to “on call” status.   Basically, I call on Monday mornings and see what cancellations they have (which seems to be quite a few) and pick a time that would work for Hannah.  This actually would work well for us because it gives us the opportunity to get her speech, OT, and PT if there is availability instead of just PT.

It isn’t the most ideal situation because she doesn’t get a set therapy time, but the reality is that she can’t keep a set therapy time no matter how hard we try.  She will only do well in therapy when she is feeling well.  We can’t tell her to suck it up or make her do anything she doesn’t want to because she just can’t comprehend that.

We still have our twice monthly PT with early intervention which has been wonderful and we are hopefully going to get twice monthly OT through early intervention (now that they have hired more OTs).

It isn’t enough therapy for her.  But we only have so many options because of Hannah’s tolerance.

We have our school district evaluation next week which will include PT, OT, and speech.  It will help the school district, Daddy, and I come up with a good IEP plan for Hannah when the school district takes over her care in August.

Not thrilled with not being able to get her regular therapy services, but we are having an incredibly difficult time finding home therapists who will work with Hannah here.   Because we have primary private insurance and only secondary medicaid, we can’t find anyone who will take private insurance.   If she had primary medicaid, we would have quite a few possibilities.  But for now, it really is up to Daddy, Hannah’s nurses, and I to therapeutically work with her for the most part.

I have been emailing with a few families over the past couple of weeks who have children that have been newly diagnosed with neuronopathic Gaucher’s disease.

One mom asked me if she should “hold out for hope for a treatment to save” her child diagnosed with type 2.    Another dad I emailed with described one of the top experts he had seen as a supposed “savior” for his child when that person said there was really nothing that could be done.

As I watch Hannah’s condition deteriorate over the past few months, I am losing the idea of hope.  I am very realistic to know that it is very unlikely that there will be a treatment found to save Hannah.  As much as I wish I could change that, reality is that there is so little money going into neuronopathic Gaucher research and so few researchers working on this form of Gaucher’s.

What can I say?   How do I try to provide support for a family grasping for hope when I am watching this disease take away my daughter’s mobility, agility, and ability to do even the most basic of infant skills?  I don’t want to take away what hope any family may have but I also feel that families need to be realistic with the diagnosis, especially those with type 2.

This is a cruel disease.   We have to squeeze quality time out every moment that it becomes available because each month that passes we realize that this disease is taking more and more control over Hannah.    She is losing her ability to have fun, to play with her favorite toys, and even to interact with her iPad on special apps (thank goodness she can still watch her Wiggles).

I will not give up on trying to find something, anything to help Hannah and the other nGD kids.  But with the very real possibility that Hannah is going to need supplemental oxygen in the very near future, I honestly feel like we are losing this battle and are heading into even more complicated territory.

I find my heart breaking more and more these days.