Good news!

The test that Kiera had to see if she had a Protein losing Disease came back negative! That is great news, and with starting Beneprotein, her levels are back in normal range. It is still not clear as to why she was so low for so long but sometimes you just have to be happy with what's in front of you at that moment.
The edema that Kiera had is also gone now and she is down 7 LBS. Most of that weight loss might be from the edema but some might be from a vomiting protocol. It turns out that Kiera is not always getting her full tube feedings and is losing more fluid than we are replacing at times. This is a tough time of the year for her with spring around the corner. We now have to keep our eyes on the potassium level since we are venting her more often again.

We had a great appointment with one of Kiera's Doctors last Friday (I feel bad for him sometimes because it's never just one thing, and this was a follow up from her hospitalization, and we never talked about that, ran out of time) I pointed out that when Kiera needs to be vented she can lose 500cc or more and also during the time that she is vented she is missing some of the feeding. But in the end, we really have no other choice but to follow this protocol otherwise Kiera is at more risk for aspirations.

Her Doctor has ordered a few more tests to see where Kiera is at and her RN wanted another EKG done because of the tachycardia that developed about four years ago and suddenly. That was the same time that the low blood pressures started too. We are also going to be doing a post-void residual volume to make sure that she is emptying her bladder because there is a concern that if we are adding more water to help her void, and she is not emptying her bladder, that could then back up into the kidneys and cause other issues.

We are going to see if Kiera can have another VEEG to see if the vomiting is seizure related. If it turn out to be, there is really nothing we can do about it, but if they are not then we have other things to look into. The Diet has been questioned because it's not healthy (high fat, low carb, low protein) she has been on this for 7 years and when she was not on it, she was having more trouble with vomiting and she just hung her head all the time. We have been tossing around the VNS (vagus nerve stimulator) I need to talk with her epileptologist about the other benefits the VNS might do to help Kiera, not only for better seizure control but for digestive and heart issues.

Her Doctor said that Kiera is a rule breaker and things are a double edge sword with her. I think that is so true. things were so much easier when she was younger when all we really had to deal with was the epilepsy and the vomiting. Yes there was the hours on end crying and then laughing and the not sleeping for days then sleeping for a few more right after. These issues are a bit more complicated and seem to have many levels to them. I am so glad that we have some very good Doctors that don't just blow us off. We have a wonderful team and all but her primary specializes in the special needs population, we are very luck!

It's strange how some things, and or days hit you. People will ask about Kiera, its usually "How is she doing?" I know they really don't want to know, they are just being polite. There are days that I give a simple answer as well like, "She is doing fine" now I say "She is doing well today" the topic usually ends there. People that ask really don't want to know the details, is a matter of fact, more times than not my friends don't even know when she is in the hospital any more. The friends I used to have when my children were young are not around or at least not much anymore. Don't get me wrong, I am not by any means blaming this on Kiera, it's this life I was dealt and people just don't know what to say or do. The friends that I have made after my children were born are mostly because of Kiera, but most I have not met face to face. The contact list in my phone has more Doctors names than the average person I am sure. My medical vocabulary is boring to most maybe because people don't know what the heck I'm talking about. Of course Kiera has a few constant cheerleaders that in return are my support as well, I cant thank them enough.

My son sat in the living room about a year ago and asked me why I am not upset that he is not more involved in Kiera's life, I can't understand why he would ask this, I don't expect anyone to be involved in her life but my husband and me. It's been 24 years and as the years go by our life gets more and more isolated and I suppose it might be because Kiera's life is getting increasingly complicated that I am more absorbed with all that goes on, so that in itself is very isolating. I am not writing this so people will feel sad or bad about this life I live, but I know the ones that do live this life will and can relate. As sad as this is going to sound I am happy that we have found more families that are effected by CDKL-5 even though there are not many, but together we support each other and do really know what it's like day to day. There are good days and there are not so good days and I know those families are always going to be there.
Many people call these families their CDKL-5 family, I understand that so well.

April 5, 2011

We are so happy that Kiera's Doctor came back and called to talk about taking Kiera off the coumidan, it's one of those meds that the side effects would be more risky than the condition itself.



Kiera's seizures are up again and she is requiring more rescue medications over the last few weeks, even today she has used up her allowed dosage for 24 hours and is still having a lot of cluster seizures. We are calling her Epileptologist to see her soon. I think we may have to rewrite the already comlicated seizure protocol in a few ways. Kiera gets a Diastat pill that is crushed and put down the J-tube for one type of seizure we have targeted, then she gets a liquid medication called Diastat Intensol, this is given bucally (in the side of her cheek). This was prescribed because it is the most direct and effective way to get this med into her so the seizure will stop. There was a very scary situation that happened last Friday while Kiera was taking her shower. Kiera sits in a special needs bath chair to take a bath, unfortunately Kiera has not been able to take a real bath for more than a year because of her yeast infection that has really not gone away, so instead she gets a shower but still needs the bath chair to support her. While in te shower Kiera had a 10 minute seizure that she needed the Diastat Intensol. While in the bath chair, Kiera is reclined a bit but while giving her the rescue med and at the same time trying to hold her head up, Kiera slipped her head out of the LPN's hand and by doing that the Diastat went right down into her lungs. She then started coughing and it sounded wet. Kiera then was moved to her bed and per protocol after getting a rescue med, Kiera is monitored with the ox monitor.

Her O2 was dropping... 89%...... 78%. We started her on 5lt of oxygen and did some pummeling. Her lungs sounded diminished. Kiera started to get pale and then became unresponsive.

The LPN said that she was shaking Kiera and was talking loud into her ear and Kiera did not respond.



911 was called and off she went to the ER.

Kiera was placed on 2LT of oxygen and after about 1-2 hours, she had a chest x-ray. 4 hours later, Kiera was vocalizing and acting like herself.... very strange! She reacted so bad to this medication although she has received this many times in the past, so it had to be the fact that she aspirated the Diastat. I did look this up on the web and what I found that this route (inhalation) is NOT recommended. That route has caused what is called Pulmonary Toxicity. Kiera just has a tendency to throw her head back and she is so strong that this can happen very easy. I feel that we need to look at what we can do while she is in the bath and we are in that situation again.



While Kiera was at the ER, her Doctor calls me, I thought 'Wow, he already knows she is there?' lol, no, he was calling as a follow up from our conversation the other day. He had talked to a few of Kiera's other Doctors about everything that is going on with her (edema, right side considerably more swollen and at times turns purple, and the hypoproteinemia (low protein))

He did talk with Kiera's dietitian to see what we can do to add more protein with a diet that is already high fat, low protein, low carbohydrate. He also talked with Kiera's endo and together they want to test Kiera for a protein waisting disease. Kiera has been showing low protein levels (from what I cal gather) since December, maybe even longer.



We are now seeing that Kiera will not void for 8-19 hours, so she is on a voiding protocol, but why is this happening now? her kidneys are fine.... always something. her seizures were up for a few weeks, she had 52 seizures last month and her average is about 35-40 per month. Her Epileptologest was called and she gave us two opptions, one, to increase a med that we are working in getting off~ lyrica, or to try a new drug...we thought to do nothing and thank God, today her seizures are down again. We did start Beneprotein for the low protein levels that she has had and two days later the seizure streak broke, coincidence I think.

The veinous doppler ultra sound did show something

Kiera had the VDU done last Thursday and the results are that she has thick blood and has many small blood clots in the small veins. That same nigh she started a medication called Coumadin, it's a blood thinner. It's also a very scary medication to be on. The Thrombosis (Blood clots) are also very scary as well. She has had 2 INR tests done to see how thin her blood is after taking the medication. The first test was 5 days after starting Coumadin and that one read 0.9. The goal is 1-2. I am waiting to hear what it read today and move from there. Prior to the veinous ultra sound, Kiera had a EKG to make sure that the right side of her heart was fine. The tech could not see the right side of the heart for some reason. I don't know why they could not view it but they "assumed' that the right side was normal. There was a note on the report saying (stage 1 distolic dysfunction) of course I looked that up and now have many more questions for her Doctor. How timely, Kiera's regular Doctor just called, he is back from vacation. He is taking Kiera off the Coumadin, he says it is too risky of a medication for Kiera to be on and the small blood clots that are in the small veins are not at risk of moving and getting into the heart, lungs, and brain....whewwww! We told him that the edema was a bit less now, but he said that the Coumadin should not have made any difference with that. So we are kind of back to square one as far as the edema and the more concerning issue with the right sided swelling with her arm turning purple. We were also told that in order to look at the right heart Kiera would have to go through a very invasive procedure that not many Doctors even do any more. I am guessing that the stage 1 distolic dysfunction may be caused from the tachycardia that Kiera has. Next step is to see the endocrinologist. I should have named her Mystery because she sure is...

Tomorrow Kiera is going to have a test called a doppler ultrasound on her vains. We are hoping to find an answer for the extra edema on the right side. She has Genralized edema from an unknown cause but the localized edema is a bit worrisome.

Can she just get a break?

It's again been a while sence my last entry, there just seems to be something always going on.
For some reason Kiera's pain has been increasing over the last year without any answer. It breaks my heart that we can't take that away. She also has been dealing with a bad yeast infection that is also around the g and j sites. We have used everything imaginable to take care of this and some of the times the topicals work for a bit but it always comes back, she really does not go for more than a week without the rash....poor girl!

Kiera has also been having J-tube issues (when has she not!)which has brought her into the hospital only because we have to keep her hydrated and the only way to do that is to do an IV. We really believe that the placement of the new J was a failure and it's caused a lot of problems. We have been told that it would be highly unlikly to find a Doctor to re-do the surgery. That really leaves me wondering about the years to come... what would be the next step if we can no longer feed her via the J?

Latley, Kiera has been having more emesis episodes, they can be bad such as projectile vomiting. Today her tempature is up a bit, so we will see what will happen next.

Kiera has again developed edima again from an unknown cause, the other day as I was visiting her I noticed that her right arm/hand and leg/foot were much more pufffy than the left side. About 3 days prior to this we had an appointment with one of her Doctors who just about covers everything when we see him. At that appointment it was determend that Kiera should have another EKG to look specifically at the right side of her heart, well over the last year Kiera's curve in her spine has gone from 42 degrease to 65 degrease and the curve is to the right, with that I think that made it hard to see the right side of her heart. The left side looked fine so it was "assumed" that the right side was fine as well.... that does not set well with me. I had talked to Kiera's Doctor after the results were sent to him and told him about the new right side puffieness, He has now orderd a Doppler Ultrasound on her vains. That is not untel the 27th of this month so there must not have been a rush to get this done.

She did get her new bed and a fantastic new wheelchair. The bed is a Sleepsafe bed, it's wonderful and I think Kiera is very comfortable in it!
I would love to see her have many more happy, healthy days in the future... still hopping.