The FDA denied Protalix’s new Gaucher drug this past week.   I have received a number of emails from people asking me how this affects Hannah.

This has no affect on Hannah at all.  This drug is designed only for type 1 and would not even be an option for Hannah.

Unfortunately, no one is working on a drug for type 2 or type 3.

Hannah and I did our interview this morning on Fox 5 morning news with Jason at 7:45 am.

I am nervous about seeing the playback because I was beyond exhausted since I haven’t been to sleep since I woke up at 1 pm yesterday since I still had to do Hannah’s night nursing last night.  Also, Hannah was still fighting her stomach bug and was had a restless night so by the time the interview came, she was trying so dang hard to snuggle in my arms so she could get to sleep.  I don’t think the camera even caught a shot of her face!

Before the segment, Monica (the anchor) came up to meet Hannah.  She was so sweet.  Hannah actually reached out to give her a hug!

It was almost surreal because before the segment I was sitting in the tall chair looking at the teleprompter which was stopped on the intro to our segment.   Talking about how a valley girl with a rare disease is no longer alone and about to mention the school event.

I think I was able to get everything in that I wanted.   I was able to talk about the huge school event this afternoon, the clinic fundraiser on Monday, the Children’s Rare Disease Network, the 7000 Bracelets of Hope campaign, the lack of Gaucher resources and research, etc.  I was also able to mention Hannah’s blog if people were interested in learning more about Hannah.

As soon as the link goes up on the website, I will be sure to post it on here.   It was a great, great experience.   I felt really nervous, and I hope I didn’t come across that way during the interview, but I felt I did the best I could, especially not being a professional speaker.

We didn’t get a chance to take any pictures with Jason or Monica because they were still on air, but we took a family picture which I will post later.

Off to sleep now.  Got to be ready for the big school event in a few hours!

And if anyone from the Fox 5 news crew, especially the sweet lady who was our organizer, reads this — THANK YOU so much for the opportunity to share our story!  It means so much to know that people really care about Hannah and want to help us help her.

I got a call this morning from Fox 5 Las Vegas.   They want me to come in tomorrow morning for an in-studio interview!

Of course, I was half-awake when she was giving me the information, but all I know is that we have to be there at 7 am for an 8:45 am taping.    Not sure if it is live or to be pre-recorded.  I do believe it has to do with the big school event tomorrow.

I have to thank my friend, Jen, for this one.  She contacted a friend of hers who used to work there and got the ball rolling.  I talked to their assignment desk manager yesterday and shared the information, but I wasn’t sure if it would go anywhere beyond that.  It did!

Unfortunately Hannah still has the stomach bug, likely a virus, and has not been herself today.  I’m hoping that she is well enough to go tomorrow.  After all, this is because of her and her beautiful smile!

I am so thrilled to get the opportunity to share why World Rare Disease Day is so important to our family.   We get a chance to share Hannah’s story in hopes that the public realizes that she deserves as much chance for a treatment or cure as any other child fighting a common disease.   In the rare disease community, hope is so much harder to have because there is very little “on the horizon,”  particularly with neuronopathic Gaucher’s disease.

Ethan and Abby are excited to be able to go to the news station in the morning.  They will be missing a couple of hours of school at the most, but I don’t want them sheltered from this either.

Later on that day – the big school beading and ribbon coloring event!   Huge, huge day tomorrow — all inspired by my amazing little girl and that gorgeous smile.

We need help to try and save her life.  Hopefully this will a huge step forward in trying to find that needle in the haystack that will keep Hannah with us longer and keep our family together.

Midnight on the dot.

I have spent the last 20 minutes or so cleaning smelly diarrhea off Hannah and after that, my mattress.  It was time to change the sheets on our bed anyway, and I wasn’t a huge fan of the pajamas she was wearing.    This is the second time in the past 2 hours Hannah has had smelly, explosive diarrhea.

Hannah obviously isn’t feeling well.    We had the vomiting episode yesterday, only once.  But today, her nurse says that she just wasn’t her playful self and wanted just to be held all morning.  It wasn’t until her second nap in the afternoon that she started feeling better and being a bit more playful.

Tonight, after her first evening feed at around 10 pm of about 60 mL,  she started getting really uncomfortable.  Tossing and turning, her face was starting to be very stressed.  I noticed her stomach really big and firm.   About 20 minutes after that feed, she had her first explosive let-go.

I hadn’t fed her anymore this evening.  Usually, I feed her about 2 oz every hour at night for 4 hours (plus about 4 oz water over the course of 8 hours overnight).

About 11:30 pm, she was still awake and obviously very uncomfortable.   She was throwing herself from side to side, and the tears started to well up in her eyes.  Her stomach was still very round and rigid, so I thought that maybe she was bloated with gas.   So I took her feeding tube with an empty syringe to try and vent her out, which is letting out the gas via the tube while I rubbed her stomach.   Some gas came out (sounds like a bubbling geyser), but her stomach was still taut.  I tried massaging her stomach, bicycling her legs, whatever I thought would help relieve her uncomfortableness.

Then I heard the huge gurgles in her abdomen, and well, you can imagine the rest.

She is asleep now, 12:15 am, finally.  Her stomach is much softer now but still looks a bit bloated.  She seems much more comfortable.

I’m worried about what is causing this.  Being that she is on the same formula g-tube diet, I’m leaning towards some type of virus.  But what is most concerning is the disgusting, unique smell… I can’t remember what her rotovirus smelled like (that put her in the hospital for 4 days this summer), but it definitely has an nasty odor.

I think I am going to call her pediatrician tomorrow and see if they can do a blood test for rotovirus, c-diff, and whatever other severe viruses she could have caught.  If anything, just to rule them out.

Tonight, no more food.  Just lots of water.   Also going to pick up some probiotic yogurt to feed her via gtube or maybe get a prescription for FloraStor again just to protect her stomach.   I’ll talk to her pediatrician about this tomorrow.

EDITED TO ADD: 12:30 am, and she has started squirming around in pain while she is sleeping.   My poor thing… I’ll go get another change of clothes, diaper, and protective sheet just in case!