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!
Kiera's Road with CDKL-5 I am writing this blog as a journal, but also to bring awarness to this rare disorder. Thank you for reading!
Monday, April 25, 2011
Thursday, April 21, 2011
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.
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.
Tuesday, April 5, 2011
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.
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.
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