This movement disorder she acquired when she was hospitalized this summer is really doing a number on her.  I don’t know if it is her actual choreathetoid movements getting worse or if she is just growing a tolerance to her medications (Keppra, Klonopin, and Haldol).

Her balance is getting worse.   So I ask myself — is it the movement disorder because she is just constantly moving now or is the “ataxia” symptom of Gaucher’s?   She faceplants because of her weakness in her left hand (from using it wrong), and she falls straight backwards at times because of lack of balance.

You can mostly see the movements in her hands and face, which is where it has always predominantly been.  She clasps her hands much of the time now, which we think may be her way of stopping her hands from constantly moving.

Because her body is constantly moving, she is always exhausted.  Moreso than usual these days.  She took 3 naps today!  The first one was 90 minutes in the morning, then she took about a 2-hour nap a few hours after she woke up from the previous nap.  Then, she crashed again just a few hours after waking up from the second nap — right in the middle of Halloween festivities!

You would think with all this exhaustion that she would sleep through the night.  But no.  Around 3 am or 4 am every morning, I start noticing her movements start while she is sleeping.  They eventually wake her up.   Sometimes cranky and still exhausted.  Sometimes “Okay, I’m up, let us play!”

Despite what is listed on the wemove.org website, there are no pediatric movement disorder specialists in Nevada.  I’m trying to reach out to Dr. Sanger over at Childrens Hospital Los Angeles because he has a good reputation when it comes to movement disorders in children.  Unfortunately, he is not accessible by email, so I have been trying to reach him through his office… ugh!

Between her movement disorder and her newly-acquired seasonal allergies (and the throwing up of mucous), she is just getting thrown through the ringer these days!  We are lucky if we get a good hour or so out of her before she just starts getting exhausted again.

We also can’t seem to get her enough calories these days (barely making 1000 a day now) because of the throwing up.  Also, with these increased movements, she needs more calories.  She is starting to lose weight again (about a pound in the past month).  Yet we can’t feed her as much as we would like because her stomach gets full of the mucous she is swallowing from her allergies (we can’t even suction it out because she swallows is before we can — just more proof we don’t need the dang trach right now!).

We have a busy two weeks coming up for her — her flu shots, new PT and OT consultations (closer to home), follow up with her pulmonologist, neurologist, gastro doc, and consultation with her new pediatric orthopedist.  I’m exhausted for her!

Great news: No pneumonia!  Hannah’s chest xray came back clear, so pneumonia has been taken off the table.  We are still waiting on the cultures from her trach aspirate.  Hoping it is not another nasty bug like the pseudomonas or MSSA, but she is already on the Cipro, so if it is, we will already start knocking that out.  Hope to hear about that tomorrow.

Bad news: Because of Hannah’s weakness on her left hand and arm, she has been using it upside down when she crawls and plays.   Her nurses and I constantly are trying to “fix” her hand during play, but she always reverts it back to the upside down (and seemingly painful to us but not her) position.

Crawling like this for the past few months has taken its toll, it seems, on her upper body.  We started noticing her yesterday doing face plants when she is trying to get into a crawling position.  Today, she did it many, many times.  It is usually when she is tired (which is most of her day), and it is so sad to see because she gets so upset because it probably scares her and hurts a bit.

So I set up a consultation with the only pediatric orthopedist in Vegas for 11/11.  Even though her OT was trying to avoid a hand brace (trying redirection and tape), I really believe Hannah needs to have that hand braced so she can get it back working the way it is supposed to be.  She still can use the hand to hold toys and play, but I think she has stretched out that muscle (that is not meant to be stretched out) so much that it finally has “gone too far.”

Birthday news: Today is my birthday.  I am spending the morning getting Hannah’s Cerezyme infusion, and you know what?  That is a-okay with me! Want to see my birthday wish? (And thanks to Julie, Heather, Libby and family, and Chuck for the perfect gifts so far!)

Trachiitis has struck…again.    This is the fourth time in as many months.  We had a great week of very little secretions, starting to eat baby food again, and we were just ready to start the passy muir speaking valve again after dealing with trachiitis for the previous 3 weeks.

It started a couple of nights ago when I was on my night shift with her.   She started getting really “gooky” with a greenish-tint “gook”.  I was suctioning her constantly throughout the night.   Then the throwing up mucous started up again, which usually happens when she has more secretions than normal and because she doesn’t have swallowing issues like most kids with traches, she swallows it all.  It then builds up in her stomach, and the only way to release it (because it is a lot) is to throw it up.   As hard as we try to get it out by her trach suction, she is so good at cleaning it out herself that we maybe only get about 10% of it through suction.

We missed her Cerezyme treatment on Monday (rescheduled for this Thursday) because I didn’t want to go through a 3-hour treatment session with her throwing up and being uncomfortable on me.

Today, we went to the pulmonologist and saw his nurse practitioner.  We talked about trach care, and since we do trach clean care twice a day, we don’t think the trachiitis is from that.  Then we talked about what she grew out last time — pseudomonas and methicillin-sensitive staph aureus (not to be confused with MRSA).  These are pretty strong bugs.  That’s why we were on the Cipro.

The NP also listened to her chest (she had fallen asleep by this time), and he noticed a lot of junk in her right lower lobe.  Possible aspiration pneumonia.  The possible theory is that because she has so much secretions from the trachiitis that she is starting to aspirate them into her lungs.   If that is the case, that could be a HUGE problem for us.

We talked about the risks of keeping the trach until Spring versus these constant bouts of trachiitis.  Because of the pneumonia issue and the flu/RSV season upcoming, they will not consider taking the trach out.  If she does get hit with pneumonia and has to be hospitalized, the trach will basically save us from some more serious issues.  It makes sense, but it is just such a crappy catch-22.

So the plan of action is this:

• Chest xray tomorrow morning, first thing .  They want us to wait for the films and bring them to the pulmo to take a look at them asap.
• If the chest xray does show aspiration pneumonia, then the conversation starts for fundoplication surgery, which will not only stop her from throwing up her secretions and, in turn, aspirating them into her lungs but will also relieve her reflux symptoms.
• Cipro prophylactically, starting tonight.
• Albuterol treatments every 4 to 6 hours.
• CPT (chest physical therapy) every 4 hours for 10 minutes at a time.

The hope is that the chest xray is actually clear, and she is just junky from the trachiitis.   She has had no fevers, and the only other outward symptom is fatigue (she is tired more than usual for the past few days).  But then again, she is also teething with 4 teeth breaking through, including her canines!    That has not helped her secretions either since drooling plus secretions just adds more junk in her tummy.

Hannah is almost 2-1/2 years old now.  She is still sleeping in her crib.  But she is almost to the point where she has outgrown it, and she crashes into the sides when she moves around at night (we have bumpers around it).

It is time to start researching options as to what is available for that next step, “the big girl bed.”

Because of Hannah’s hypotonia (lack of muscle strength) and her developmental age (12 months), a regular twin bed just would not work.  A mattress on the floor would not work either because if she rolled off it, she would not be able to get back on (nor would she think about getting back on it to go back to sleep).  Railings would not work because she could easily fall over the normal size railing when she goes up on her knees like she does her crib (her crib is still on the middle level).

We need something more secure for her.

This is their medium. They also have one that is double-rail high, which definitely would keep her safe!

I found these SleepSafe beds online.  They would work, but part of me thinks this is just too much overkill in terms of what we need.  But the other part thinks, “Wow, she definitely could not fall out of this bed!”     I also like the fact that the SleepSafe beds come with adjustable mattresses so we could easily lift her head at whatever angle we need to if we have to.

I just don’t know what would be the best thing to do.  Hannah probably has another good 6 months in this crib until it is just way too small for her.   Also, would our insurance or Medicaid (which we hope to get in the next 4 weeks or so) cover something like this?

The main goal is to keep Hannah safe at night.    I need to find out what other options we have.  Perhaps I will ask Dr. Carrie (Hannah’s new pediatrician) what she recommends.  There has got to be something, you know?  We can’t be the first family who has come across this situation.