12/9 - Update - Doing Great!

Wow! Emily is just doing great. :) We have been working on getting her hydrated just right and I think we are getting it done. Too much hydration and these SMA kids have too many secretions. Not enough and they dry up which is bad for a number of reasons. Right now dry is not good b/c she still has some "junk" in that right lung. Dry junk is sticky and heavy and causes atalectasis, which is like you running out into traffic for an SMAer... not good. In case you didn't click on the link, atalectasis is basically some level of lung collapse. Severe atalectasis would be a complete lung collapse, which was the issue with Emily when we went to the PICU.

I'll just put a plug in here for one option SMA families have for pulmonary care. It is not hard, but intimidating -- but the results can be phenomenal. Proper use of the Cough Assist, BiPAP, CPT, Positioning, and suction can GREATLY increase your child's quality of life and their length of life. I'll be honest and tell you that we are not interested in putting Emily through Hell on Earth to keep her alive, but we are interested in treating her pulmonary condition just as we would our own. Heck, I have to use an inhaler at times and if I had sleep apnea or snoring problems I'm sure I'd use a BiPAP or CPAP as well. Anyway, Emily is our second SMA daughter and in comparison we have a better understanding of how the proper use of these machines can vastly improve your child's life. (We used them with Linnea as well, but not as aggressively for various reasons.) After a 3.5 week hospital stay and a completely collapsed right lung we are able to keep Emily's O2 sats in the upper 90's quite easily. She tolerates the Cough Assist treatments and all b/c it is a normal part of her life now and she even sleeps through the Cough Assist sometimes! The BiPAP is a welcome part of the day for an SMA child too, after some getting used to, b/c it allows them to really relax and rejuvenate. If you are an SMA parent considering the options for your child the NIV protocol is a viable option for your family and you can take it only as far as you want.

OK - so right now Emily has been at 99% or 100% O2 saturation for HOURS and she is as happy as can be playing with all her things. Since we've been recovering she has spent most of her time in bed, but we are making the most of this time and getting things situated. So, today we moved our entire third floor around to better accommodate Emily. We have moved the Toddler Medical Bed (looks pretty cool and is EXTREMELY helpful when doing her treatments) into the house and Emily has since taken over our Master Bedroom her stuff. We have subsequently moved into Annabelle's room (much smaller than the MB), and Annabelle has moved into Emily's old room (the smallest of all). I think it will work though. There was some disagreement about the practicality of the whole ordeal, but the point was to make Emily's care and comfort more appropriate for her needs and that is what we have accomplished. If we are fortunate enough to get a night nurse they will have space to work and there is plenty of space to hang out with Emily if she is not up to venturing around the house or elsewhere even if the nursing doesn't work out. That is the big news for the day, besides Emily doing incredibly well, of course.

Thanks to the families that have brought meals and for the families that have been spending time with Annabelle. You are a tremendous help and essential to our lives. I hope you know how much we appreciate what you do for us! :)