Hannah is still well on her way on the road to recovery.

Feeding-wise, we have finally got her feeds up to 170 mL/hour (a bit less than 6 ounces).   She was at 200 mL/hour before her bout with the rotavirus, but we ended up having to go back to 40 mL/hour continuous feeds just to get her stomach used to food again.

We are hoping to be able to get her to bolus feeding in the next few weeks which would cut down each meal (5 a day) to 20 minutes instead of 90 minutes or so!   Before this hospitalization, she would down an 8- to 10-ounce bottle of formula/babyfood in 5 to 10 minutes!   So if we could get 8 ounces down in about 20 minutes, wow, would that be wonderful 🙂

Before we left Texas, after her trach was put in, she failed her swallow study.   We were told it was likely due to the fact she had no coordinated swallow or suck reflex because of the choreathetoid movements.  In the notes of the swallow study results, I found that the speech pathologist recommended that we can try thicker foods like pudding and baby foods, those with a distinct color, and see how she tolerates that once we felt she was ready to try.  The reason for the distinct color was so that we could see if she aspirated it, as she would likely cough it back up into her HME, and we would be able to see the coloring as we did when OT first tried it back in Texas.

Since her swallow and suck reflex has tremendously improved in the past month, we decided to give baby food a shot today.  We bought a two-pack of Stage 2 peaches (orange) and a 2-pack of berry banana (purple).

We were confronted with the pre-hospitalization problem that we had where she would not take food by spoon (except for gerber yogurt).  But we decided to try anyway.     Our nurse was here when we tried it as an expert eye looking for signs that she may aspirate the food.

It took just a couple of little spoonfuls, but she LOVED it.  Amazingly, she was opening her mouth for each bite!  We fed her a whole entire plastic tub of baby food BY SPOON without argument!  TWICE!  We tried the berry banana around lunch time and the peaches around dinner time.  Both times, she took it by mouth.

Even more amazingly… she put her hand out to ask for more!   She never communicated “more” to us for anything, but after almost every bite, she would put her arm out as if she was trying to grab the spoon for more.    As soon as we put another bite in her mouth with the spoon, she sucked it all down.

We checked for aspiration in her HME dozens of times, just out of paranoia.  But there was not one instance of aspiration today!  Of course, this does not mean we are out of the woods with this aspiration issue, but I am feeling so much more confident now that we can likely continue with thicker foods.  We don’t plan to try juice or formula again until we get another swallow study at the NIH in Sept or Oct, but thicker foods is a great start — especially is she is willing to take it by spoon!

Makes me wonder — did she forget she had an oral aversion to being fed by spoon before all of this happened?  Or perhaps she just loved the taste in her mouth so much, after having nothing in there for almost 3 months, that she was willing to go for it?

Doesn’t matter the reason — she ate baby food today.  By mouth.  Yeah, baby!

That was the term Hannah’s neurologist used when saw what a change Hannah has had in her neurologic issues, chorea movements, and physical demeanor in the two weeks since we had seen her.   We had thought she had improved quite a bit in the past couple of weeks, but hearing a medical professional, especially one who is following her for her neurologic issues (especially the chorea movements), put the biggest grin on my face for the rest of the day.

So the plan with Hannah from the neuro standpoint now is to start weaning off the Keppra.  Since no one believes she has a seizure disorder (since she only had one at the beginning because of hypoxia due to a breath holding spell), it is time to try and get her off her seizure med.  Also, since Keppra is a third-line of a defense drug for chorea movements, this will give us a chance to see if her chorea movements continue to improve.  We should be off that in 4 weeks.

She will remain on her Klonopin and haloperidol for now, both 3 times a day, for her movement disorder.   She also wants us to use melatonin with Hannah to try and keep her sleeping through the night.  She shared that kids who spend a lot of time in the PICU lose their normal sleep cycle, and this is likely the reason why Hannah does not do well through the night.  Admittedly, she is doing better through the night — anywhere from 5 to 7 hours of sleep now, which is so much better than the 2 to 3 hours of sleep a couple of weeks ago (ironically while she was on the sedatives!).

She also made the comment “When is she getting the trach out?   She doesn’t seem to need it.”   I loved hearing that.  After talking to Hannah’s nurse, most kids on trachs have a distinct type of breathing sound and pattern.  Hannah breathes through the trach, but she also breathes through her nose and mouth.  When she pulled out her trach, she was breathing fine — I only noticed because I heard her breath sounding a bit different, but not labored.  So I am keeping my fingers crossed that taking out her trach (that was only put in because she could not be extubated because of oversedation and polypharmacy) in a few months or so!

I’m so proud of my little Hannah.  Everyone seems to be amazed with her.   I know I am.

Today was a good day.

Not only was it Hannah’s birthday, but it was also the birthday party of her cousin who lives here in Las Vegas.  Originally the plan was to just let my inlaws take the kids to my nephew’s party, and we would stay home with Hannah and the nurse.

Hannah had been doing so well these past couple of days.  We thought that maybe today was the day to get out of the house and do something fun.  Since she was discharged, the only places Hannah has gone outside of the house are doctor’s offices and therapy sessions.

But today was going to be different.  We were going to go to our nephew’s party with Hannah (and her nurse).  It was at a bowling alley, so it was air conditioned, so we did not have to worry about the heat.  We figured Hannah would probably get cranky or overstimulated with seeing all the lights, people, and activity, so we anticipated only being at the party for about 20 minutes or so.  But at least it would be a first step to getting back to a normal life.

Around 3:30 pm, Daddy, Hannah, her nurse, and I headed to my nephew’s party.  After taking about 10 minutes to adjust to all the noise and people in the bowling alley, Hannah just lit up and became very social, playful, and had such a great time.   Abby and Ethan loved having their sister there and would often come and play with her, even if it was just to give her a glow-in-the-dark necklace (that she played with most of the time!).  Hannah spent time with her aunt and her grandparents as well, and she soaked up every minute of it.  We spent over 2 hours there!

It was wonderful having the nurse with us because she would take care of Hannah at the times when Daddy and I wanted to spend time with Ethan and Abigail (especially before the grandparents showed up).  Our nurse really cares about Hannah so much so we knew she was in great hands.   Also, when Hannah’s grandmother swooped her up and wanted to take her up to their hotel room with the other two younger cousins (the bowling alley was in a casino), our nurse was able to go with her.  This way Nan could spend time with Hannah, yet feel comfortable that if there was an emergency or incident (like her needing suctioning or pulling out her trach), she had help.

She was such a happy girl today.  Loving life, that is my girl.

My dearest Hannah,

Today, July 25th, we celebrate an amazing milestone, your second birthday.

From the moment we got your diagnosis at 5 months old, we were told our time with you was going to be very limited.  We were told that you possibly would never see your second birthday because rarely does a child with type 2 (which was the type you were assumed to have) live past the age of two.

But here you are.  Full of life, smiling, and still fighting.

Your presence in this world has brought so much good.  It has brought some amazing people into our lives, both personally and medically.   It has rekindled the interest of certain Gaucher researchers and supporters to become more proactive in the search for a treatment and creating awareness for the neuronopathic form of Gaucher’s disease.  It has brought families of other Gaucher children together as a support system and friends.    The list of people who have been touched by your existence is quite long…and continues to grow.

All because of you — my beautiful sweet girl.

Most of all, it has brought me  happiness.  A special type of happiness that I have never thought possible.  One where just a smile or a small achievement could light up my whole world and make my day feel bright.   A type of pure joy that I get when I see you laugh and interact with your brother and sister who just love making you laugh.

Your fight to survive during these past two months was awe-inspiring.  I don’t know if I could have ever fought as hard as you did.   You truly are a miracle child.  You continue to defy the odds, not just during these past couple of months, but your whole life.   You continue to amaze and surprise everyone that gets to know you.

Keep smiling my beautiful girl.  Happy, happy birthday.

Love,

Mommy

Hannah has been a much different baby in the past few days.

Maybe it is because she is finally off all the sedatives and only on her movement disorder and seizure meds (Keppra, Klonopin, and Haloperidol). Maybe it is because she is finally feeling better overall. Maybe it is because her movement disorder seems to be even lessening more to where we really only see slight-to-moderate movements in her hands and face instead of her whole body.

Who knows for sure, but I am just so glad to see it!

• She has gotten much of her personality back!
• She smiles all the time. She wants to grab for her toys like she used to (even if she can’t keep a grasp on them, but even that is getting better).
• She can balance while sitting on my lap with me just holding her waist instead of having to steady her whole body.
• She is starting to reach out and grab for things, even if it means scooting on her back and rolling to her side to get something.
• She has decided she does not want the trach anymore and has been trying a lot to pull it out (and succeeded once!) .
• With the help of a Boppy pillow, she can go on her knees again, for almost a minute now, and you can see she is thinking about crawling because she is trying to get her knees to move.
• Even though her fine motor skills are still very poor because of weakness and the movement disorder, it has not stopped her at all from playing with her favorite toys — piano, rolling wheel, and she even really got into her grandma’s iPad (the baby piano app that her friend, Bertrand, plays with).

Even her nurse who was here today, who had not seen her in a week, was excitedly surprised at how much progress she had made in a week.  When I was in my office working, I heard her yelling to me “Carrie, look!”   Hannah was on all fours, rocking back and forth, trying to crawl.  She was so excited because when she last saw Hannah, just a week ago, Hannah was barely doing anything.

She started occupational therapy last week, and next week we start our physical therapy (Mondays) and continue our occupational therapy (Tuesdays).

I just love seeing her come to life now.   I am finally seeing that sparkle in her eye, that smile that makes me just want to kiss her constantly, and even hearing her soft-sounding giggle sneaking past the trach.

She turns 2 on Sunday.   What a momentous birthday this will be.  Hitting that “2-year-old” mark for  a neuronopathic Gaucher baby is a huge, huge deal, as most of you who have been along this journey with me know.   Even though Dr. Goker-Alpan clearly said to us, “she is not a type 2 baby,” she still is on the type 2/3 spectrum.   Even though we may not be having a huge party this year to celebrate like we did last year, in my heart, I will be celebrating with a huge smile on my face and a beautiful baby girl cuddled in my arms.

Hannah still does not have a normal night schedule yet.  It is getting better now that she is weaned off her sedations (as of a few days now), but she still wakes up around 2 am to 3 am every night and is awake for a couple of hours before one of us bring her into bed with us and try to get her back to sleep.  Of course, one of us has to end up awake during that time.  Needless to say, we are both pretty tired these days.

This morning, I was the one who kept Hannah from around 3 am to 5 am until she fell back to sleep.  When she did, I put her back in her crib and hooked her back up to her overnight feeds which I had stopped while she was in bed with me.

She woke up again around 7:30 am, and Daddy picked her up and brought her into bed with us.   She was thrashing around, and she apparently pulled out her Gtube connector that was connected to her feeds.  No big deal, just messy.

About 30 minutes later, Daddy woke me up saying “her Gtube is gone!”  Sure enough, there was just an empty hole in her stomach, and her Mic-Key button was no where in site.  We were racing against the “you have just an hour or two before the hole starts closing up” and needing to go to the ER.   We realized then that we never received our backup Mic-Key from our Home Health company.   We both looked at each other again like, “ARE YOU FRIGGIN’ KIDDING ME?”

Daddy found the Mic-Key in the crib.  It took a few tries to get it back into her stomach, and it broke my heart seeing that it was causing her pain as we were doing it.  But he finally got it in.  Fortunately, Daddy found the box from the smaller size Mic-Key that TCH had given us and was able to get the balloon inside the Mic-Key inflated again.

Feeds went well that morning.  Then around 3:30 pm, our day nurse and I both noticed that Hannah’s Mic-Key was starting to hang out of her stomach, definitely seeming loose.  We got the syringe to measure how much water was still in the balloon (Daddy had put in 5 mL earlier that morning), and she was down to less than 3 mL.

We had a leak.

Daddy grabbed the smaller-sized Mic-Key that we had, and at the suggestion of our GI doc’s nurse practitioner, we inserted that one and inflated it to 4 mL.  Worked like a charm.

After checking out the old Mic-Key, there was definitely a leak in the balloon.  Glad we caught it when we did.  The thought of having to go back to the ER because we couldn’t get something in there and having the hole close up (requiring new surgery) just made us both ill.  We so don’t want to have to go back to the ER unless absolutely necessary — and hopefully not for another year!

Our GI’s nurse practitioner made some more recommendations for backup Mic-Keys, and their office is going to put in an order for multiple back-up options (an extra Mic-Key and a PEG-type tube kit) for “when” this happens again (it turns out it is more common than we thought!).

Later in the evening, our home health company’s driver showed up with a backup Mic-Key of the right size.  We are keeping it safe.  Just in case Hannah decides she wants to pull out her Mic-Key again…

This is not Hannah in the video (obviously), but it gives you the idea of what a normal Mic-Key button change is like!