Things are going okay right now, but Kiera's seizures are still up and we are choosing to ride them out...of course we have the rescue medication and all, but we have learned over the years to not jump every time she goes through these cycles.

last week we had to put into play her new protocol for aspiration episodes while at home. She had a emisis last week but her 02 sats did not drop this time ( they don't drop every time when she throws up) but the plan was put into play and Kiera did well.

I have always said that if I ever needed to go through any type of surgery, I will not complain what-so-ever only because Kiera has gone through SO much in her last 23 years and all the surgeries were because my husband and I made the decision to go ahead with each and every one of them. Kiera had no say in the matter. Two weeks ago I had to have something done and I have never been put under in my whole life. I needed to have an IV and it was placed in the bend of my elbow... wow, did that hurt! I started to think about every time Kiera has had to have IV's and she can have one in for quite some time, and I was feeling the pain....? what does Kiera go through every time?

On a more positive note, our organization had just received our 501(c)3 status!!! We are now listed as a registered non-profit organization. Our name is 'International Foundation for CDKL5 research' (IFCR) Our web site will be launched very soon. We have a lot of work to do this year and our hope is to find more ways to fund the research that so desperately needs to continue so our dream of finding a cure can be more attainable.

There comes a time where you just have to let go, or at least accept how things are and where they might be heading.

About 1 1/2 weeks ago Kiera ended up in the hospital again because prior to going in, she was having issues with gagging that finally turned into vomiting. Kiera's O2 dropped again and with suctioning and oxygen, it just would not go back up. 911 was called for transport.
I was with a friend while he was shopping for new flooring, the store that we happened to be at, at the moment was between Kiera's home and the hospital. About 20 minutes after I got the call that she was heading into the hospital, I saw an ambulance driving about 70 miles per hour on the freeway. I just knew that Kiera was in that vehicle.... the timing was right and I just felt sick.

The chest x-ray did show aspiration, so Kiera remained on oxygen and started on an iv antibiotic. On day 2 she was back to herself!
The hospitalization just prior to this one was identical to this one.... but this is not typical if you have read my earlier notes.

Kiera was out of the hospital within 4 days! The hospital proposed writing a protocol that might keep Kiera at home (in situations like the last two) and try to clear her up at home. My fear...or one of my fears is the fact that while at home, we have never been successfully getting her O2 even up into the low 90's after an episode and the response time for the EMT's is around 10 minutes.

Her Doctor is working on the first draft and I hope that by this Friday I will be able to read what he has so far. I am worried for the first time that we will have to put this protocol into play.

I do however understand partly where the hospital is coming from. They say that if these kind of situations come up and we can take care of this at home, that would be best for Kiera... I agree with this wholeheartedly, but Kiera can turn very quickly so it feels like this could be a ticking time bomb.

Over the Memorial weekend my husband and I were able to get away to my in laws home up north. It was a nice long weekend and much needed. Before leaving for out of town I always tell Kiera's home that we are not going to be in town, well now it's a joke with us all. We always say "Don't tell Kiera" I can count on both hands how many times we raced back home because Kiera was in trouble.... you have to laugh about things sometimes!

We were planing on staying up north through Tuesday, but after looking at my calendar I saw that Kiera had an appointment that day :( so at first I tried to reschedule but the next available appointment was not for another 6 months. This was with her spine Doctor and we had some issues that I did not feel we could wait for another 6 months, so we could not cancel.

In earlier notes I talked about Kiera's TLSO (back brace) and how she has not been able to wear it. I have also talked about the placement of Kiera's new J tube and my frustrations with the whole thing.

Let me back up just a bit, we see this Doctor every 6 months so our last visit was last December. Kiera had a spine x-ray while in her TLSO and in her stander. Kiera did not like this at all because she had not worn the brace or stood in her stander for over one year. While taking this x-ray in December Kiera started to cry and then went into a seizure, doing this calmed her body enough to get a good x-ray.
That x-ray showed her curve at 49 degrees.

This time we took her out of her wheelchair and she was placed on a small bench (this is always so hard to do and Kiera can not sit without total support) she looked awful and again, she was very uncomfortable. This x-ray showed a 75 degree curve. Surgery is usually recommended at 50 degrees.

When we were called back to talk with her Doctor, the x-rays were already up on the lighted screen and I remember thinking to myself 'that has got to be about 75 degrees'.
Kiera's Doctor came in and I started with the problem with her back brace. Since Kiera's twisted bowel surgery last July that caused the Doctors to have to relocate her J tube, bracing Kiera's back has become impossible...for a few reasons. One huge issue is that there needs to be so much pressure applied to Kiera's hips and the new J is right above the bend of her waist. placing the brace then causes stomach acids to leak out and it burns her skin immediately. The burn is very bad and hurts Kiera, understandably so. The burn itself is very difficult to heal after it happens. SO.... the brace is out....unless we can find a GI doctor that will agree to do another surgery on her J tube, that is highly doubtful at this point.

I then asked about the progression of a nueromuscular curve if we can't use the brace and how that would effect her lung function. He said that her curve will most likely continue to get worse and in turn so could her lung function. Kiera's right lung is already compromised because of the direction of the curve on the top part of her back.

We then talked about pain with this degree of Kyphosis. He said that some people that are more' with it' with in their days, will say that it is painful, others might say that they are not in pain. We have been seeing more signs and symptoms of pain with Kiera.

We also talked about the true benefits of continuing the use of her stander (Kiera can not use her stander without her back brace, she just does not have the trunk control) He told me that if at all possible Kiera should get back into it. We will have to see how we can adapt it so she will have the support she needs to be safe while using it. Then there is always 'will she be comfortable in the stander after not using it for so long?'
At the end of this appointment I asked what her x-ray read and he said that it was at a 75 degree curve... but we are comparing apples to oranges, so next time the x-ray will be done in her wheelchair so it will be more comparable to the x-ray from last December.

I feel sometimes I can never get any traction in life.