Archives for July 2010

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!

Seriously, Hannah’s body needs a break!

About 2 days ago, Hannah’s day nurse had mentioned upon leaving that evening (7 pm) that her G-tube area was looking a bit red, but it wasn’t warm and it wasn’t really swollen or anything.  Just looked irritated.  She told us to keep an eye on it.  About 90 minutes later, the redness was spreading, it was very warm, swollen, and was getting really hard just to the left of the Mic-Key button.  Daddy and I looked at each other — it was a Saturday night, there was no clinic on Sundays…we had no choice, back to the ER.

We were both concerned about her G-tube being infected, but it turned out that she had cellulitis around the G-tube area.  They did an ultrasound of the area to make sure there was no abscess, and they did the typical bloodwork.  Her white blood cells were elevated at around 15,000, but considering how serious cellulitis could get, this count showed that they caught it very early. 

In the ER room, they gave her a dose of clindamycin.  Because of Hannah’s very recent history with rotavirus and adenovirus/pneumonia, we were told that they wanted to admit her to the hospital just to make sure the antibiotic would work and just to keep an eye on her.  As soon as they said that to us, Daddy and I looked at each other like “are you friggin’ kidding me?”   If it was most any other child, they would have sent them home with antibitiocs because it was in the early stages.  But because it was “Hannah,” they wanted to keep an eye on her.

We got to the ER around 9 pm that night.  Got a room at around 3 am that night.  Got discharged and left the hospital around 1 pm the next day.   She had three doses of the clindamycin, and they really worked great.  Her cellulitis was already significantly improving by that morning, so the attending (the same one we had all three times at Sunrise) said we could spring her out and take care of her at home.

You know when you have been in the hospital too long when the nurse in the ER is the same nurse you had when you were transferred to the PICU from Texas (and remembered us), the night resident was our new pediatrician’s resident whom we had met the day earlier (and remembered us), the charge nurse remembered us, and the attending was the same doctor for all three hospitalizations at Sunrise Children’s hospital.  We haven’t even been in Las Vegas for a month (transferred 6/25), yet Hannah is already getting well known there.

Hopefully, hopefully, hopefully that will be it for a long while!