Archives for February 2011

I am really starting to dread our weekly trach change for Hannah.   We never seemed to have a real problem until the last few months where, for one reason or another, she panics when we take out the old trach and try to put in the new one.

It happened again tonight.   I was holding her hand and singing to her while Daddy was getting ready to make the switch.   He took out the old trach, and she just started getting fussy and did not like me holding her down.  Daddy wasn’t able to get the new trach in because she panicked, tightened up her throat, and started having a breath holding spell because she started getting upset.   We immediately had to put in the smaller size trach, which thankfully is small enough to get into the hole even when she clenches, but it doesn’t go in easy.

Hannah started having difficulty breathing as Daddy was trying to get the smaller trach in and started turning blue.   I told Abby to get me the oxygen, and when she turned it on, it didn’t go on.   Ethan came over to help because he has done it before (Abby hadn’t).   He still couldn’t get it working.   Turns out that it became unplugged!

Finally, we got the oxygen going while Daddy was able to get the smaller-sized trach back in.   We let her get a continuous oxygen push for about 4 or 5 minutes until her color came back and her lips became pink again.   She also started calming down even though it took a while for her to catch her breath and start breathing regularly.

About 10 minutes later, we turned on the iPad and put on the Mickey Mouse Clubhouse theme song, and while she watched the iPad, Daddy was able to change out the small trach with the regular size one without any problems.  She didn’t even notice.

When it was all over, Daddy and I both breathed a huge sigh of relief.   I hate this trach, hate it.  But on the other hand, if we didn’t have the trach during this breath-holding spell, we couldn’t pour oxygen into her lungs as easily as we did once we got the small trach in and she was still involuntarily breath holding.

Not only do we have the big fundraiser on Monday afternoon, but Hannah is also getting her Cerezyme treatment at the same time.  She made a huge impression at the school last week with the kids, but I think we are going to let her be more low-key on Monday, as her treatments seem to wear her down afterwards.

After the fundraiser, we have her OT assessment with Early Intervention.  We need to get this done in order to get ready for our IEP meeting in May.   Reminds me we need to find an OT that can work with Hannah at home.

Tuesday, we go back to physical therapy and get some more work on the stander.  She was only able to handle a few minutes last week, but we need to keep getting her in there so she can get used to it.

Wednesday, we are having our consultation with Dr. Raja, the new neuro.  I have heard good things about him, so I hope it goes well.  We really need to get a better handle on her seizures and movement disorder.  I also think we may have to bite the bullet and get the 24-hour EEG and/or the sleep study to see where we are with the seizures.  My gut tells me that if we can get a better handle on these two issues that she would be much more comfortable.

Hannah then gets to end the day on Wednesday with her developmental therapist at home.  She loves having play time with her.

Friday evening is our Make-A-Wish meeting.  Hannah has been granted the opportunity to make a wish, and as I have said before, we really would love to take the kids to Disney World/Give Kids The World.  Just seeing Hannah’s face during the parades with the lights and music — Wow!   A friend told me to make a list of anything else we really would love to have happen, and I am thinking some quiet one-on-one time with Mickey and Minnie with some of their songs.  I know if there are too many distractions, she will get overstimulated and not pay attention.   So some one-on-one time in a quieter area would be amazing.

I need to also have her CT of the abdomen rescheduled as well because I think her spleen and liver are starting to get even more enlarged now instead of shrinking.  She may need her Cerezyme dosing upp’d.

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.