Hannah is definitely on the mend.   She only has 2 more days of Cipro left for her trachiitis (pseudomonas), and we are finally starting to see her old personality come back.  She is not as exhausted all the time (no more 3-long-nap days), and she is willing to be more engaged and is wanting to be more active.  She also had a fantastic therapy session with her Early Intervention specialist today, so I knew my “old Hannah” was coming back to us.

The older kids had their Fall Festival at their school tonight.  It ran from 4 pm until 7 pm.  It was a big event for the school, and my sister-in-law, nephew, and two nieces were going to come join us.

I was getting kind of hesitant about going around 3:30 pm.  Hannah had not taken a second nap yet, which was unusual for her.  She usually crashes after her therapy session and sleeps until around 4 pm.

But the kids were so excited to go.  I decided that since Hannah had such a great day and was obviously feeling better that I would take a chance with her.  So we all piled into the car and headed to the Festival.

She did pretty well in her stroller for the first 20 or 30 minutes or so.  Then I could see her exhaustion setting in… the eyes rolling back, the droopy head, and then the crying.  That silent cry that always makes me nervous because I fear another breath-holding spell (leading to seizure) again if I don’t pick her up and console her.

From that point on, I had to carry her.  She clung on to me but was still awake.  After about 20 minutes or so of holding her, my arms and back were starting to hurt.  Of course, she took that as a cue to cradle down in my arms and get into her “I’m going to sleep” position.  She fell asleep finally, and after about 5 minutes I put her in the stroller.

Of course, she immediately woke up and started crying again.   From that point on, she was inconsolable.  No matter what position I tried or how I held her, she just would continue crying and thrashing.   But the older kids were having such a great time that I felt I had to try everything not to cut the night short and head home.

I thought that maybe if we sat down and let the kids grab a bite that I would be able to get her back to sleep.   But go figure, the food area was right next to the loudspeaker and all the noise and activity.  Hannah went into sensory overload, and I could not get her to calm down at all.

I had to admit defeat.  I told my sister-in-law that I had to take Hannah home.   She offered to keep Abby and Ethan with her and take them home later, but the truth was that I needed to have the kids in the car with me to help me try and get home.  Hannah does not do well in the car as it is, and I honestly don’t know how I would make it home without having to stop 50 times along the way.

I told the kids we were going to have to go.   Ethan and Abby were disappointed.   But Ethan accepted it immediately and worked on helping me get the stroller together for the walk back to the car.  Abby was a bit more upset.   My nephew, who was not used to these “well Hannah is upset so our plans change again” episodes, was so adorable and kept saying “It’s okay Abby.  I’ll come to your house and we can play.”   Even though Abby was upset, it was very short-lived because she knew that we needed to get Hannah home.

As my sister-in-law and I parted ways in the parking lot of the school, my kids and I were trying to get everything in the car.  I tried to get Hannah into her car seat, and she started with her hyperventilating spell and was so horribly upset.  I kept thinking to myself “Please, not here.  Please, calm down.”  It was horrible.   As Abigail got into her seat, I took Hannah from her car seat and sat up in the front passenger seat just trying to rock her.   I started the car and blasted the cold air on her back because she was sweating and very heated from her agitation.

I didn’t realize how amazing this was at the time, but as soon as I started rocking Hannah in the front seat, Abby came over to us behind the seat and started singing “Twinkle, Twinkle, Little Star” to Hannah (this is our “go to” song when we need to try and calm her down).    Then, I heard the back door of my mini-van shut.  Ethan had taken it upon himself to empty out the stroller (huge diaper bag, suction machine, etc.), put the stroller in the trunk, and organize Hannah’s bag and suction machine in their respective places we keep them in the car.

About 10 minutes later of sitting in the car, Hannah finally passed out.  She had worked herself up so much that she was still trying to catch her breath as she slept, but she was calm and comfortable.  I was able to get her into her car seat.  As I got into my driver’s seat, I saw Abigail covering Hannah up with a light blanket.   She held Hannah’s hand the entire way home.

As we all piled back into our house after our Fall Festival adventure, I put Hannah on the living room floor where she slept for over two hours.   The other kids, my sister-in-law, and I shared a quick McDonald’s dinner that my sister-in-law picked up on the way over.  Then the other kids went off and played — my nephew and Ethan on the Wii, and Abby and my older niece (almost 2) in my office.

I am so proud of Ethan and Abigail for their sacrifices they make for Hannah.  They are so damn good with her.  Even though they may initially complain, it never lasts long, and they never blame her.  Even my nephew surprised me tonight because he was also having such a good time at the Festival because I thought he would not understand why we had to just “up and leave.”   But he did.  He was awesome about it.

I know that Hannah has her limitations, and I know that I still have to keep trying to push her a little bit each time so that the older kids can still have their fun and activities.  I just feel so lucky that the kids accept that their little sister just can’t handle it all the time and love her unconditionally even if it interrupts our plans.

I’ve decided to make some changes to Hannah’s therapy routine.  Before this week, she was having PT and OT across town weekly (Mon & Wed) and developmental therapy here at home.   The drive was really to and from the therapy clinic (30 minutes each way) was really getting to be too much for Hannah, especially since she has been so tired lately.  It is almost like by the time we get there, she is so tired and does not want to participate.

We found a therapy clinic that is just 5 minutes from our house.    We had our initial OT consultation today.  It is a smaller office than the one we went to before.  Even though Hannah was completely exhausted (for whatever reason she did not take a morning nap), she still was able to work with the new OT for about 20 minutes before falling asleep.  She did not even complain!

It was a good consultation, and this new OT got Hannah to do something I have never been able to do.  Get her to stack a block on top of another one (with some assistance)!   Hannah stacked two blocks!  The OT helped guide her hand, but it was just beautiful.  Her nurse and I were so excited to see that.

The OT asked me the standard question “What do you hope to get out of occupational therapy?  What are your goals for Hannah?”   My answer sort of surprised her.  I explained that I am very realistic as to the fact that Hannah has a very difficult time learning new things.  Her skills are very scattered — most are at a 12-month level, but there are a very few that sneak in that are more age appropriate!   I told her my goal is to keep trying to teach Hannah new things because the more neurons we create in her brain, the longer it will take for the disease to progress.  The regular “goals” for occupational and physical therapy don’t apply to Hannah, at least as far as I am concerned.

Reality is:  Her disease is killing the neurons in her brain for the most part.  So whether or not it is true, logic tells me the more neurons she can make in the brain, the longer we have with her, which in turn, gives us more time for whatever may be on the horizon to help Hannah.

Next Tuesday we have our PT consultation at the same place.  A 5-minute drive each way is so much better than a 30-minute drive each way!

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.

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.