Addison Bower Walk

Outside Hannah’s vocal cord weakness and laryngomalacia, our biggest issue for her is feeding-related issues.

I have to tell you.  Trying to feed Hannah by spoon each day, few times a day, is my biggest disappointment.  It is still an incredible fight to get her to eat by spoon, and she will still only eat stage 2 fruits by spoon.  We still feed the majority of her food through her Avent bottles, even pureed stage 3 foods.  She is now on whole milk and no formula.

HUGE accomplishment coming…

As for feeding by hand, she now will eat air-puffed foods.  Cheetos are her absolute favorite.   She will eat four or five of these at one sitting.  She actually will dig her two bottom teeth into it.  Thank goodness for Cheetos!  She also is eating the air-puffed Gerber-like air puffed foods like the wagon wheels, air tubes, and the little air-puffed stars.   She will also eat Vanilla wafers and gerber-like cookies! 

So, we are definitely making some strides.  We are dealing with two completely separate, yet both totally frustrating issues.  First one is that she will not be spoon-fed.  She also won’t eat it if we leave it on her plate, toys, or a spoon next to her.  Yet she will put EVERYTHING in her mouth that is not food, and she will chew like there is no tomorrow on those.  Yet, when she is willing to be spoon fed, we have to slip the spoon in her mouth, between her lips, at the right angle between her gums and teeth.  She does NOT open her mouth for it.  It will take 30 minutes for one stage-2 jar of food, IF we are lucky.

The other issue is that she doesn’t like chunks in her mouth.  We haven’t been able to get into real stage-3 type of foods yet because of this.  We are slowly making progress, as she will take thicker with micro-chunk stage-3 foods, like a blueberry-banana type that had tiny pieces of blueberries in them.  Anything else, she will either spit out or attempt to gag out any little chunks.

It is almost as if she doesn’t put “chewing” and “food” together.  She chews on toys.  She chews on non-breakable food (like beef jerky, carrots, etc).  But if it will be something that breaks off in her mouth.  No chewing.  That’s why the recent advancement of her eating Cheetos air-puffs and other air-puffed baby foods is such a big deal.  She doesn’t chew on them, but she does work them in her mouth and swallow them.

Anyway, why are these a problem, outside of the obvious?

Hannah now isn’t getting enough calories.  She has been around 20 lbs for almost 4 months now.  It isn’t that she isn’t eating, the problem is that what she is willing to eat is just not caloric enough.  In one day, she will eat about 7 to 8 jars of stage 2/3 foods plus about 18 oz or more of whole milk.  Plus a few air-puff sweets every now and then.  Five full meals a day.  She just doesn’t have the stomach space to add more.

She is just so active now, especially in her little walker.  She just zooms all over the house. 

I talked to Dr. Vartabedian about it, Hannah’s GI doc, and he recommended started to add Pediasure and Boost Essentials to her bottles in addition to the milk.  

Thankfully, we are meeting with a pediatric nutritionalist on Tuesday so we can come up with a real plan on feeding so that we can increase the calories, make sure she is getting the correct amount of protein, etc.  We have been just “winging it” for so long, and since she isn’t making as much progress as we had hoped by this point at 14 months, we have to work with what we are given.

For anyone around the Fisherville, Virginia, area, please consider participating in this event.  This event will DIRECTLY help children like Addison (for whom the walk is named), Hannah, and all the other children diagnosed with Gaucher’s disease, types 2 and 3.

You can find complete details here, but are some of the details…

Amanda Bower Walk
Sunday, September 20th

Wilson Memorial High School
189 Hornet Road
Fisherville, Virginia

Registration, 1 to 2 pm
Start time:  2 pm

Registration fee (day of event):  $35
Early online registration:  $30
Children 2 – 12:  $15
Registration Fees include a Tshirt

I ended up spending the night in Hannah’s room last night.   I wanted to be closer to her if she had any more choking spasms in the night plus I wanted to give my husband a break and get some sleep.   Little did I know that he kept the monitor on anyway just in case I didn’t wake up.  I was sleeping two feet away from her — just shows what a loving spirit and dad he is.

She did end up having about six choking episodes last night.  One really bad one that lasted about a minute, two medium ones, and three smaller ones (fortunately, she was able to resolve that within just a few seconds in her bed). 

Right now, she is in her crib sleeping, and I am watching her on the monitor.  Wondering what tonight brings.  Will tonight be the night she is able to sleep without a choking episode again, and we can get back to “normal?”

We have a busy day tomorrow.  She has physical therapy tomorrow morning, only half of a session because she has a meeting with the GI doctor to work out these reflux issues after that.  Fortunately they are in the same floor.  Right after that, we have to immediately go downtown to TCH to see the Rehabilitation doctor.  This is an important meeting because therapy for Hannah is her biggest tool for survival right now.  The more she can learn now, the longer we can keep her with us, and the more time we have to find a treatment or cure for her.

I wish these two important doctor visits weren’t happening while she is really congested and not feeling well.  But it isn’t something that we can really postpone because, especially with the Rehab doc, it could take months for another consultation with him.  And we really need to get the reflux understood and under control — we don’t know how much this is affected her breathing, feeding, and talking issues.

Here to hoping we have a good night tonight!

We saw Dr. B. this afternoon.

After checking out her lungs, throat, ears, etc., he found nothing more than a dull ear infection.  However, she did have enough nasal drainage to replenish a river! 

Since she never turned blue during these night episodes, it is very likely it is her choking on the drainage (being that she is lying down) and having weak throat muscles due to the laryngomalacia.  Also, since she was acting normally (outside of normal having-a-cold crankiness) and her O2 sats were at 100%, this also played into his theory.

It is what I had hoped for!

So we are to raise her head 30 degrees at night (hard to do with a restless child), use the humidifier (vapor rubs do no good), and give her the steam from hot showers 3 times a day.  We basically need to help her drain her discharge.  Even though we both feel it is likely viral in nature (since she gave it to me), because of her ear infection, he gave her amoxcillin, which would also help if it was a bacterial infection.

We will see how tonight goes.  We all need some sleep. 

I really, really, really hope this is the reason for her choking spells at night.  It makes perfect sense, and in light of the other possibilities, this is the best case scenario.  Still dangerous because we have to deal with her choking, but it is better than what could have possibly been the cause.

Hannah is finally napping right now. 

She is so terribly congested that consistent sleep has been elusive these past few nights.  After her first rough night, we have had two more of them.  So we have an appointment to see our pediatrician this afternoon. 

I am so fearful that this is the beginning of something terrible, like needing to go ahead with a trach, or laryngospasms, which are pretty common in nGD children, especially type 2 kids.   I keep hoping that it is something simple like just really bad reflux mixing with the congestion or something. 

I honestly have no idea.  All I do know is that she will wake up suddenly, panicked, as if she can’t breathe.  I hear her upper stomach or lower chest grumbling a bit on occasion when it happens.  The episodes only last anywhere from a few seconds to last night’s episode at 2 am that lasted almost a minute and freaked the hell out of my husband and I.  As I was holding Hannah in her room during that episode, I was thinking “do we call 911 now?” 

We are all exhausted.  I could have crashed at 6:30 pm last night, but with three kids, that didn’t happen.  I’m on incredible slow motion today.  I can’t even imagine how my husband is functioning at work right now, as he has been up all night too.

I have no idea what to expect after this afternoon’s appt with Dr. B, our pediatrician.  Hopefully, it will just be something simple and “normal” (in terms of baby stuff).  But then there is another part of me that says, “this disease is progressive, it is going to happen at some point, this may be the point.”  I just don’t know.