12/23: Extubated, but not right

Emily has been extubated. The breathing tube has been taken out and she is now on BiPAP, an external breathing assist device that we use regularly in Emily's care. The problem is that she is very irritated and very cold. If her body does not have nutrition and protein to break down, causing energy (HEAT), then she is going to be cold. If there is no nutrition to break down then there is only her own muscles and fat, which has has very little of - or none at all, depending on how you look at it. Emily has NO reserve for this situation and THAT is why we have advised the docs against it. I still cannot understand why we are even dealing with this when there is documentation clearly defending what we have been talking about regarding nutrition, and everything else SMA related for that matter.

We are praying that Emily will stay strong and fight through this and that the docs are learning something. I will say, with some confidence, that we are done with Hopkins after this. We are going to do everything we can to get Emily somewhere else if she is getting sick, avoiding these situations altogether.


Anonymous said…
Nate and Diana,
I just read your updates and my heart is pounding and I'm so angry that you are having trouble with the hospital and now Emily is having more complications. It is so unfair to your family and especially Emily. Although our children have this terrible disease, there are ways to make things go more smoothly for them and I wish doctors would be more willing to listen to the experts and us parents. We shouldn't have to battle them and the disease.

I will be praying for Emily and both of you to have the strength to get through this rough period. I wish I could give hugs to you all from Ally and I through the computer.

If you need to vent or anything, please do not hesitate to call or email.

Your friend from Chicago,
Anonymous said…
Nate and Diana!

I just wanted to know I am reading your updates and Emily and you all are in my prayers.

Eden's Mom
Anonymous said…
Nate and Diana,

Shannon and I have been praying for your family and you have been in our thoughts all day. Sorry that Hopkins has not come around enough for you. As always, if you do need to move closer to a "SMA Friendly" hospital you are more then welcome to stay with us while you get settled. We have plenty of room. We have a fully finished basement complete with kitchenette and 2 bedrooms and a full bathroom that your family could use :) Please keep us updated!!!

God Bless!
Justin, Shannon, Haley and Addison
Lizzy's Nina said…
Nate and Diana,
I am so sorry you have to deal with ignorance. Its very upsetting that a hospital that is so reknown as John Hopkins still just not "get it".
DR. Kelley also knows that kids with SMA can not fast for very long.
As you said in your post lessoned learned.
Many prayers for all of you.
God Bless,
Anonymous said…

I am so angry reading your blog. I cannot believe this has happened at Hopkins. I really thought Crawford got it and would protect you all. I am willing Emily all my strength and hope.
Rebecca K.