Roller Coaster

They finished up with Ava's PT and OT. She really seemed to enjoy it. Then came her one hour trial for the CPAP, during which I got in touch with our genetic counselor. Ava did great with her trial. When she was put on the vent for a 3 hour respite she continued to look comfortable and breath over it. She was also awake for an hour after the initial trial. During this time she moved more, sneezed, coughed better. Of course she sucked her pacifier. Her suck is getting louder and more coordinated. She had her eyes more open and was trying to focus. Her pupils are still dilated some and her irises are not all there. But the color has improved and her eyes seem a little more sparkly. (Just a bit, but I noticed). At one point Ava was trying to focus and made eye contact with me. She then moved her whole body when she could see me. It was wonderful!
She then took a nap, sucked on her tube while sleeping.

The conversation with the genetic counselor was pretty encouraging. I was reviewing Ava's labs with her. I was also asking her about respiratory failure and a Complex IV deficiency. I could not find anything about it. She said because respiratory failure is usually a secondary cause for death with Complex IV kids, not primary. Even in Eric's case, all other systems were failing first. She said that is not the case with Ava (I have to qualify with a for now, just in case). I told her all her labs are good, except for ones that point to muscle breakdown. So I asked her is it mitochondrial or is it nutritional? She actually told me that these values sound nutritional. Not a definitive diagnosis, but what I am cautiously taking away from this conversation is that it is not a slam-dunk mito disease. (Then again, what mito disease is slam dunk?) Anyway at least I know that I am not off my rocker thinking about such things.

Honestly, we have no new information. But Ava having a good day and being a little stronger are building blocks I'd like to continue. She has a great doctor on tonight and she will be back fro 7 day shifts in a row on Wed. She is open to my thoughts and ideas. She has a reasonable and conservative plan of care for Ava. She is also a Mom, which helps. She is an answer to prayer. I feel that she understands Ava better than anyone has tried. She is the only doctor in 12 days who came into our room and got the full story from us. From her birth on through. She, too, is concerned that Ava is not sedated. But then saw that she was not liking her chest PT. She made the comment that Ava is more awake. Then she thought that if she were to grow and get stronger she may have some fight about the vent. This doctor listened to me tell her how I observed Ava's personality as well.

She also thinks Ava looks better than she did last week. She also thinks her tone has improved some. She is going to get her into a different crib so we can hang toys for her to look at when she is awake. Her goal is that she is a feeder grower and to get the tube out. She thinks it's too fast to try and pull it because she is not strong enough. WHERE WAS SHE LAST WEEK!!!!!!!!!! At least we are on the same page.

So I like the feelings we have leaving the hospital tonight. I really like what Ava was doing too. I have never been so bold to think that we will outlive our children. I make a point to enjoy them while I have them and while I hope to have them for "longs" I know we may get them for "littles". It doesn't matter I'll take what I can take for as long as they are dishing it out. Tonight Ava looked like she was trying to wake up and show us what she is made of. I am laying my request before God: for us to take home a healthy Ava who needs no oxygen support, feeding tubes and will be on earth with us.