Still in the PICU

July 12



Today there has been no change except the BI PAP has been off since 8:00pm last night with Kiera's Oxygen holding at 94% on room air but she is still in need of frequent deep suctioning. Kiera is starting to come out of the sedation a bit more today and she was very itchy because of the mask.

It looks like things are starting to turn the corner some today!





July 13

There were no great changes for the better today but, then again there has not been any huge set backs either.

Today Kiera was transferred out of the PICU to a Gellette Childrens floor. There is a huge difference from being on a regular adult floor in any hospital and staying on the Gillette Childrens floor. Gillette is a specialty hospital for Children who have special needs, the hospital staff are so used to working with children/adults like my daughter and don't get rattled when she has a seizure.

Gillette just changed their age limit from 18 years old to 45 years old, that means that Kiera can always go to this hospital until she turns 45, for now this is great news, if and when that day comes that she can no longer use Gillette, we will cross that bridge then.

Kiera's O2 was still up and down throughout the day and night. She was still in some pain but she did just just have major abdominal surgery... she was very agitated and doing lots of gaging despite the pain meds.

Moving into a regular room, there is usually just a hospital bed, but Kiera has a way to wriggle herself out of any hospital bed. Two to three days before her transfer, I spoke to many staff members about the need of a enclosed bed system while she is hospitalized. The last thing Kiera needs it to fall out of the bed after such a horrible surgery.

We got to her room and no enclosed bed, just a regular hospital bed, this is so frustrating just because nothing moves fast in a hospital.

A bed was finally delivered the next day.

July 14th

Again, no change, O2 up and down, suctioning often, temp still present, now concerns about the incision, it was a bit red down below the brief area. Dr. was consulted and he then consulted the GI Doctors. Late this night the Surgeon came in to inspect Kiera's incision and stated that it was healing well.

Kiera continued to wretch and show signs of nausea, so the RN wanted to reduce the feeding rate to see that would elevate the gaging. Dr agreed to try this for the night hours.

What a horrible July!!!

July 3rd and 4th


I was so excited to go to our cabin for the 4th of July holiday, my sister and her husband were going to join us and we always have so much fun together. They drove up late on the 3rd.


It was about midnight on the 3rd when we started to get phone calls from Kiera's home. The staff told us that Kiera had vomited a few times that day, and that her feeding tube (j mic-key) was giving them problems. The staff tried to change it out thinking that maybe it was just a bad tube. When they took it out, 3 cups of Kiera's formula came poring out. The staff then noticed that Kiera was cold to the touch, she also begain showing a temp of 95.1, her pulse was at 157, resp 54, blood pressure 98/70, and O2 sats at 99%.

The staff attempted to give her a water flush and her scheduled medications, this was unsucsessful, the feeding kept backing up into the syringe. Kiera was pale by then and her blood glucose read 136.

911 was called, she was taken to the hospital around 1:45 AM.


Kiera arrived at the hospital around 2:45AM with a LPN from her home. Her vitals upon arrival were; Temp-99.3, pulse-165, resp- 32, blood pressure-126/63, and still her O2 was at 100%.

Kiera was breathing rapidly.


It was the 3rd of July and that is always a very busy time for hospitals. The medical staff did make a comment that they were getting tons of casualties coming in at that time. Kiera must have not looked critical to the Doctors so they just had a iv for fluids placed, hooked her up to a heart monitor and had labs drawn.


It was about 5:00 AM when they started to prep her for a cat scan and abdominal x-rays. It is still not clear to me as to what really happened (all the notes say something a bit different) but apparently they gave Kiera the contrast that you get before a scan on the intestines. I still don't know what type of contrast that they used either, one is water souluble and the other would be toxic if it got into your lungs. They must have given it to her through her G mic-key, this is the feeding tube that goes right into the stomach, the J mic-key goes directly into the lower (small) intestines, so right after getting the contrast, Kiera vomited and then aspirated contrast right into her lungs!


Kiera's O2 droped to the low 70's, the Doctors tried suctioning and nothing was helping. Kiera was then placed on 10 Lt of oxygen and started her on a non-rebreather mask, O2 sats remained in the upper 70's.

Doctor stated that her blood gasses were all out of normal range. Kiera was then moved to a critical care room, sedated, intubated and placed on a ventilator.

All of this happened before she even had the cat scan.



By this time it was about 8:45 in the morning. The Doctors then took her in for the X-ray. The next thing I know is i'm getting a call on my cell, the Doctor on the other end was asking for my verbal permision to do emergency surgery. The Abdominal CT scan revealed a vuvulus, a twisting in the intestinal tract. She told me what had happened with the contrast and after that I dont think I heard much more of what she was saying. I wanted to just blink my eyes and then be with my daughter at this point.


The Doctor said that they saw fluid in the stomach cavity as well, I know that that that alone can be a very bad situation. The scan also showed a section of the intestines was noted to have been herniated.

I must have not said much to the Doctor on the phone because as she was explaning this surgery and all the risks to me, she paused and then said "...or we could do nothing, and that would end in death" I was taken back a bit from this comment and it probably got my attention then, so I said "Yes you have my consent, of course you do, Kiera is full code"

Kiera was a very sick girl and now was critical. I thought the first time she was intabated 2+ years ago was horrible, this was much worse.



Our three hour drive home was the longest and quietist drive I have had. We were both very scared that Kiera was not going to make it through this, and we were not going to get there in time.


By the time we arrived at the hospital it was 3 1/2 hour later, we had our dog with us and had to drop him off at home first. Right when we pulled up at home, I got an update call from the staff that was with Kiera, this was nice to get because I was so worried about her. The staff said that she did well with the surgery, she was still in recovery and the staff had not seen her yet.

One of the risk of after this kind of surgery is some people end up needing a colosomy bag, thank God Kiera didn't.

There was one foot of damaged bowel that the Doctors had to resection (remove). The twist was located right at the J mic-key site, so they had to stitch it closed and make a NEW J feeding site.

Kiera was then brought to the PICU. The nurses told us that we could go see her. I was stoped at the door to the PICU by a nurse who also works at the clinic that we use. She was working the PICU nursing station for the weekend and must have heard that Kiera came in and what for.

As she was talking to me, she gave me a hug and I could then see into the PICU. This hospital only has curtains around the beds, and most the time they are left open. So this is kind of funny... as I was done talking with this nurse, I was the first one to go through the door, then my husband and after him was the house staff. Well, in the 3rd bed from the door was a girl with dark curly hair, on a vent and asleep. I started to walk up to her and then see a man sitting in a chair that was facing the bed, this man was asleep and obviously a visitor... he has a plaid shirt on and a grimmy old hat. My first thought was 'Who is this man? why is he that close to my daughter?' I even, actually took one step around his chair to get to my daughter! This is when I see the nurses a few beds down waving their hands and pointing to the bed that Kiera WAS in! I just hope that man did not wake up and see what I did!!


I finally got to Kiera, she was so out of it. She was still on the ventilator because her lungs were so comprimized at this point. She was on continous NG suctioning for increased gastric secretions. Before surgery, they could not find a good vein to place a central line so they ended up using the femral vein, not a typical 1st chioce of the Doctors. Kiera was placed on Propofol for pain, antibiotics for aspiration and sepsis. There was an order for TPN feedings to be started due to her intolerance to feeding.



The Doctors that assisted with the surgery came up to talk with us, they told us that the fluid that they saw on the scan was just her formula, that was good news. They told us how the surgery went and said that it went beter than even they expected. This a bit strange to hear a Doctor say.



After talking with the Doctors we all realized now with the new J-tube, there is now no way to give Kiera her medication untel the new J was healed more. Kiera, by now has not recieved any of her seizure meds. All the rest of her medication could wait but the seizure meds were importaint to get as soon as possible. One of her seizure meds does come in IV form (lyrica) so we could give her that one saftley. later that day we gave her the other 2 seizure medications via her G mic-key, this was a bit scary because we don't give Kiera anything in her G, because of the risk of aspiration, we mainly use it for venting. But things did go well, and given the fact that she was on the ventilator she was not able to throw up, so we were okay doing this.

later that day, Kiera started to get very puffy, mostly in her upper exremities, so much so that her ID and Allergy wristbands were constricting at her wrists.

July 5th

Respitory Therapy was orderd every 4 hours around the clock. Kiera's lungs sounded coarse bilaterally. Kiera was still having problems with tachycardia (high heart rate). Her temp was still up despite the acetaminphen, so ice packs were used to help cool her down. Kiera had more labs drawn and results showed low K+ levels (2.6), the day before that level was 3.4, so it continued to drop. The Doctors then started an IV medication to suppliment the low levels. Kiera remained on the ventilator with 30% oxygen via vent.

July 6th

Kiera was getting the Propofol throughout the night to keep her sedated, she was also taking morphine for pain. Her temp was still up but what I found strange was a comment from one of the nurses, she said that the sudation medications will be weaned as they may be taking out the vent today... The IV in her arm was removed, the Propofol was also stoped. Kiera slowly wakes from sudation a bit before noon. The Doctor came by and said that she will be moved to the adolescent floor soon.

I could not even imagine that she was ready to move from the PICU, her vitals were not stable and she wa a very sick girl.

She continued to need Lasix for the edema. She was still getting the Neb treatments along with a vest treatment every 4 hours, and now she started to have seizures. Kiera was not ready to be moved!

Another Doctor came by and told us that they will not attempt to take the vent out today due to the exsessive sleepiness. The Respitory staff reported lower left lobe was still diminished.

July 7th

At 1:15 AM, Kiera was still getting Morphine for pain and her temp is up even higher that the day prior (101.2) Ice packs and cold washclothes were again used in attempt to cool her body down. At 5:00AM her temp was still going up (101.3) and again cold packs were being used.

The Respitory staff came in at 9:00AM and turned vent to C-PAP, Kiera was now breathing on her own. The IV fluid rate was decreased as the tube feeding rate increased.

The vent tube was pulled and the NG tube discontinued. Her O2 was at 98% while on 4 LT of Oxygen via naisal cannual.

The Doctor reported that the latest chest x-ray looked a "touch worse than yesterday" so he suggested more vest treatments may be helpful. Kiera was still on IV antibiotics as well as Lasix. The blood labs again were drawn and things were out of range still, her K+ was now at 2.1.

July 8th

Kiera was still in pain so she was getting Morphine and over night her temp started to rise again (100.3) and her lung sounds still diminished. Labs now show low HGB of 7.4, and all other labs abnormal. The Chest x-ray showed fliud in both lungs and her respirations are increasing. The attending Doctor was conserned with this because of the risk of her hyperventilating. She has been breathing so fast for so long now and was still kind of out of it.

Kiera needed 2 bags of blood transfutions due to the low HGB and her respirations still remained elevated. Kiera was still requiering 4 LT of Oxygen. The central line that was placed before surgery was now looking like it was getting infected so that was taken out and a PICC line was then inserted. Her temp was still up there and her breathing was still elevated, staff turned up O2 to 6 LT and had to use a AMBI bag to force O2 due to her oxygen droping down to the mid 80's. Her oxygen then went to 96%, but then dropped again to the mid 80's. The AMBI bag was used for the second time. A bit later a Hi-flow naisal cannual was then started due to Kiera's increased breathing and low oxygen readings. Around 11:00pm, Kiera was assesed and found to be slightly hypoactive, they did deep suctioning 2 times.

July 9th

Respitory treatments with vest treatments continue to be needed every 4 hours along with oral and nasal suctioning. Temp is being monitored and cool washcloths placed on her forehead and under her arms. She still reamins hypoactive. Today the chest x-ray shows the left lobe was looking beter but her right is is looking worse now. Kiera will remain in the PICU.

The Doctor wanted to remove the O2 to see how fast Kiera's oxygen levels would drop, they remained around 94-96 on room air, then later that morning they dropped to the low 90's so O2 was used again starting at 1 Lt. throughout the day. The O2 was continuously adjusted according to O2 sats and oral/nasal suctioning as needed to increase saturations.

The Doctor discussed the need for CPAP/BI PAP or possibly re-intabating Kiera for the fluctuating O2 sats. What a horrible thing to hear but I was not surprised at all to hear this. We all talked about how the tube may have been taken out too soon, we agreed that it was.

We then got the results from some cultures that were done eairler within the stay, it showed E coli. At 2:00pm a BI PAP was needed for increased resps and continued drops in Oxygen levels. Later that night the Doctor was paged due to continued increase with her respitory and after talking with the Doctor it ws thought that Kiera's blood PH might be low therefore causing the increase. More blood labs were drawn.

July 10

Oral/nasal suctioning as needed for drops in oxygen levels and to produce a productive cough. Kiera is still on the BI PAP at 3 Lt.O2. Kiera is now being tested for C-diff because of the antibiotics she has been getting and there is a concern over the high heart rate especially during suctioning.

More blood labs were done, all were within normal limits except for CO2. Kiera recieved a IV medication to help with this.

July 11

Kiera was still having trouble with her oxygen levels even on the BI PAP, (83-90%) deep suctioning was again needed. It was noted that Kiera was having several periods of apnea, they lasted anywhere from 4 seconds to 20 seconds in duration.

While Kiera was moved into her wheelchair the BI PAP was removed for a short time. The respitory thearpist stated that they wanted to begin the weaning process of 4 hours off and 2 hours on through the night

More seizures again.... I should have not said anything

Last night around midnight Kiera had a 13 minute seizure. She was agitated right before the seizure so it makes me feel like she knew one was coming.
Today at 11:45 she had another 13 minute seizure so she needed more rescue meds.
She is zonked out now because of the darn meds.

No sezures and no appointments!!!!!

I know that it's only Wed, but so far no seizures this week!!!!
I should not even have typed this because we know how that goes! But I am happy about this for today!

What a Tangled Web we Weave

Boy it's hard when your a girl and you have epilepsy.

There just is so much that you need to know when you have uncontrolled epilepsy and in your late teens and early 20's.

I have been having some conversations with some ladies that I know, who also have epilepsy. We were talking about birth control medications and epilepsy.
There are some BC medications that can exacerbate seizures, and the 'Dot' that Kiera is on is one of the medications that can do this.

There is also a natural BC medication in the form of a cream, I would wonder how many carbohydrates are in this medication? Kiera is on the ketogenic diet, so carbs have to be limited, and any creams should be patted on, not rubbed in.

long term use of any BC medication has a negative side affect on the bones too, and we are already doing very pore in this area.

I need to plot her seizures again to see if I notice an increase in her seizure activity since starting the 'dot' if that is the case I will need to go down a new route.

Kiera is limited with what she can take in a few ways, first the Red dye allergy, next the keto diet, and lastly being on the keto diet, oh, and not being able to take anything by mouth.

The sad thing is that the current BC med that she is on, is not doing the trick. Kiera is still having 2+ period's per month and the reason why we have her on any BC medications is not to prevent a pregnancy, but to help keep the seizures down around her period and take away the horrible pain that she has with them. Right now we have made her life a bit more uncomfortable by adding this med that results in more period's rather than none!

I really thought this would get easier as she got older, it hasn't yet.

It's just one big circle!

We had Kiera's Doctors appointment on Monday.
Just love this Doctor, he is so smart and seems to know just about everything!
He always takes ample time with us as I am sure he does with all his clients, but you leave there knowing that you have covered everything that needed to be covered.

Bottom line is that we are going to increase the dose of Kiera's birth control patch to try to get better control over her constant (or close to...) periods. We also hope that by controlling the periods better, we can control the seizures better too.
We talked about the edema and our appointment with her epileptologist and dietitian.
The epi told us that girls that take phenobarb and or a few other epilepsy drugs, might need a higher dose of any BC meds. also during the epi appointment we talked about stopping the lyrica wean for now to help to get Kiera more stabilized with her seizures, so this plan has then involved a conversation that we had with Kiera's dietitian about the extra weight gain and/or edema. Am I talking in circles yet??
So we are changing her diet in hopes to loose some of the weight.

After the period issue gets under control, we will again start the titration down from the lyrica.

Boy it's hard being a girl with epilepsy.

The Study is done!!!!!

I just got an email from the doctor at BCM, she said that the paper is done and sent a copy to us!
A lot of it is hard to understand, but it's so nice to have something like this to give to Kiera's Doctors.

last Monday, Kiera had her appointment for her TLSO, thankfully it was just getting adjustments for now.
Tuesday Kiera saw a Stoma nurse, she has a rash around both the G and the J, the nurse thought it looked like yeast, just like the GI Doctor did a week ago, so she is on another med for this.
Today (Thursday) we saw a new epileptologist, she seems to be a great fit for us. she was very interested in Kiera's diagnosis of CDKL5 deletion and will learn anything she can about it. We gave her all the information we had and a copy of the BCM study.

She is willing to take Kiera off her seizure meds (yeah!) We will start that as soon as we get her other issues figured out, ie: menses every 2 weeks, increased weight over the last few months.
It's good to be back with this epilepsy group! (MEG)
Kiera slept all through this appointment because of getting her rescue meds time 2 today, but in a way it was okay, if she was not sleeping, she would of had a lot to say!!! At least we did not have to yell back and forth because Kiera just doesn't seem to take her turn! Haha!

Tomorrow (friday) we see her dietitian, she wanted us to try other things to help Kiera loose the extra weight before she would tweek the diet, but we are in a catch 22 right now because Kiera is having her menses every 2 weeks and because of that she is having more seizures, so we put the lyrica ween on hold to try to stabilize Kiera a bit, but Kiera is continuing to gain weight, soooo, we need to do something and soon, her afo's and TLSO have already been adjusted to accommodate the extra weight, but when you gain weight, your seizure meds need to be adjusted because the dose has to keep up with the weight gain, this is just crazy! So to me it makes since to decrease the calories in the diet, but it is the keto diet that is used for seizure control, this can get so confusing, UUURRRGGG!

Kiera can be so funny!

Every since we took Kiera off the Topamax she has really been using her eyes much more and seems to smile and laugh more appropriately!

We were waiting in the waiting room at the orthopedics clinic and as one family was walking past us, Kiera looked right at them and and made a sigh sound, but sounded like a "H' sound, the mom looked at Kiera and responded to her sigh with a "Hi"
That to me, as Kiera's mother, was the best feeling, someone responded to Kiera as a person, not a wheelchair with a body in it.

Yesterday we had the final fitting of Kiera's left foot AFO (leg brace) after the edema has gotten so bad and we are not seeing any changes with it so far, thus making it impossible to wear her old one because it is just too tight.
So now she has both braces adjusted, so she is back in them.
Her right foot has turned in some since she was not been able to be braced for so long, her Orthopedic Spec. told me what could happen if that continued and it was not good, basically the calf muscles could contract so that it would turn her foot in even more and her heal cord would also shorten. This foot, Kiera has never needed surgery because it has always been flexible. I am glad that she is back in her braces.
While she gets up into her stander we will have to place a thin book or something like that under her brace at her toes, so this will flex her foot to give her a stretch that will correct this issue.

We are not ready to go into the stander quite yet though...on Monday we go to see the spec. that works with Kiera's TLSO, this too needs adjustments to accommodate the edema, I only hope that we don't have to start from scratch, UHHGGG! If that would be the case, it will be another month before she will be able to get back into her stander.
And we have another issue going on, her right hip is now 50% out of it's socket, we just noticed this about 1 month ago.

When we finally can get back into the stander, I am going to go to our clinic with Kiera and her stander, I want to get her up in it and have the PT evaluate how we are going to introduce this activity back into Kiera's routine, so she will be safe and without pain. I will ask for a stander schedule to work her back up to where she was before with this.
I also want to get an x-ray of Kiera while she is in the stander to see what her hip is doing.
I can not stand the thought of Kiera going through hip surgery again. the pain level of this surgery is right behind back surgery.

Last week we went to see Kiera's PMD (Physical Medicine Doctor) this Doctor is the one that gives Kiera the Botox shots in her left calf muscle, she noticed that Kiera's left foot was turning in much more again (due to not wearing her leg braces) so we used a bit more of the Botox in hopes to get better results.
We also, at this time talked about Botox in her jaw muscles to help with her teeth grinding. this Doctor does do this, she said that it can be very helpful, but was cautious because of Kiera's issue with secretions. She felt Kiera's jaw muscles and commented that they felt like a rubberband, they were very tight, and given the fact that her teeth are now like pancakes in the back, she explained to me that if this got worse, Kiera's back teeth would wear down so far to where she would, or could loose them so if that were to happen and Kiera is still grinding her teeth, she would eventually be grinding on her jaw bone, YIKES!!
So we did it, Kiera HATED the first shot, but I think that was because I was holding her still, and you know, most girls with CDKL5 are in constaint motion with her head.
But the second shot she handled just fine.
So we are keeping our fingers crossed in hopes that this works.

As far as seizures go, last month she went for 9 days without seizure activity!! yeah!!! that was between the 17-27th, today she is on day 3 without seizure activity.
We are again titrating down some with the Lyrica in hopes to lessen the edema. I think that the edema is still progressing so I have a feeling that she might have to come completely off to correct this problem.
We are going to see her new Epileptologist this coming Thursday, I am going to bring information about CDKL5 because I think that the more she understands about this disorder the better we can work together and start to take Kiera off more of her medications.
The keto diet is showing better results than medication with a small population of girls affected with CDKL5, this gives us hope.

Next week we are again, full of appointments. Kiera has appointments every day next week besides on Wednesday. Monday morning is going to be a very early one for Kiera, she will have to get up very early witch she does not like and then be fussed with, again is not a fan of when she does not want to be up! and then drive in morning rush hour, Yuck!
This will be her appointment for her TLSO, this is always a full day and is hard on Kiera, not looking forward to this day.

lets see what next week will bring.

Wow, it's been 3 months sence my last post!

Boy, a lot has been going on over the last few months. There has been so many Doctors appointments but mostly because of odd issues not because Kiera has been ill, thank God!

She did however go through a 7 day illness, vomiting, temps, O2 dropping, but we managed to stay out of the hospital and I have to say I was amazed!

Her seizure activity has been up and down again, mostly up.
We did take her completely off one of her seizure medications, Topamax. She did do well while coming off it, we did expect increased seizure activity while titrating off.
But during the taper, Kiera did start to show more edema, so far she has gained 12 LBS since January, and she is still on the keto diet so her weight should be more stable than this.

During all of this, we lost her epileptologist, that was such a headache trying to find a new one. I was questioned why we thought that Kiera needed a epileptologist rather than a general neurologist, and was told they are really the same... so being who I am, I googled it and did find that there is a difference. Kiera's biggest neurological concern, for us is her epilepsy (of course there are many issues in this area, but the epilepsy is something that we can DO something about, hopefully)
So, anyway we were kind of on our own for a few months as we were titrating off the Topamax which was a very unsettling feeling.

As the edema and the increased seizure activity continued, we became more concerned. Kiera was on a roll with receiving rescue medications on a daily baces again so we had to do something.
I called the clinic where our epileptologist used to work and talked about my concerns (two years ago we were kind of at this same point with seizures and rescue medication, and Kiera ended up in the hospital, on a vent for 14 days and continued to stay there for up to 63 days, I just did not want to get anywhere close to that again) so they directed me to a peds epileptologist in the mean time.

This Doctor is great, right away she said that the edema is most likely caused by another seizure medication that Kiera is on, Lyrica.
I was talking about this for awhile, but none of Kiera's Doctors really looked into it.
So now we have been decreasing the Lyrica some, but there has not been any change in the edema. I then put a call into the dietician to try to adjust Kiera's diet and at this point the dietitian wants us to try other avenues before we play around with her diet. We did just change it because Kiera had a blood draw that showed low protein levels, and as a result we increased her protein in her diet but by doing this it increased her seizure activity, so we backed off the protein.
So we are going to try to decrease the Lyrica again.

I also had to get Kiera's AFO's adjusted too so she can get back up in her stander. She has not been able to stand since January. However, she can not stand without her TLSO (body brace) so that too will have to be adjusted or remade, but if we can get her weight down then we will have to redo all of the bracing, what a pain!

Currently Kiera's seizures are down again even though we are messing with the seizure medication, so it makes you wonder what the meds were doing for her anyway.

We had our first appointment with our geneticist since Kiera was diagnosed last May, it felt weired just because her job is done, she says that she does not need to see Kiera any more often than one time per year...just kind of bitter sweet.
She did ask me to gather information from all the parents that I know that have a child with cdkl5 deletions, she said the only way to gather needed information that would lead to more children diagnosed and sooner, is by talking with more families that are affected by this disorder, Doctors just don't have that kind of access.

Some exciting news we have is that the Doctor at Baylor College of Medicine that found Kiera's deletion has written a paper about Kiera and cdkl5. Kiera's deletion is very unusual because it is not a mis-coding, but a large deletion (missing chunk) and also involves the gene itself.
The paper is due to come out shortly!

Diastat again, but it's been a while.

This morning Kiera had an 11 minute GTC, she needed diastat.
She is zonked out now.

The last time that she needed a rescue medication was on the 8th of this month, so I still think that we are doing well.

I hate the med, if only we could just let her ride it out, but I know that's not a good idea.
It just knocks her out s much.

But I did get a great video of her a few days ago while she was being loud and smiling.
On the video you can see how she is really using her eyes. I have a friend that calls Topamax, Dopamax. that name fits it better.

Coming down off the topamax we are seeing better eye contact and a bit more awareness of her surroundings.

We are so happy that she is coming off this medication!

Epileptologist leaving...

So we need to interview another one.
It does get to be a pain in the neck when you have to retell your story to new Doctors and get them to understand your views about what works and what does not.

The topamax wean is still going well even though Kiera had a cold the other day and was given Benadril. This med lowers the threshold for seizures and so Kiera had 8 smaller seizures in a few hours.
Today she is very vocal (happy) and is not showing any signs of a cold.

We are still dealing with the edema and I am getting a bit concerned because it's now been about 1 month (I think) that we can not get her AFO's on, so in return, she has not stood in her stander.
I am worried about contractures developing, her left foot turning in more along with other issues like lung and batter problems.
Forget about the bone density problem that we already have.
I can already see some muscle mass loss in her lower legs. It will be a gradual introduction to standing again if we ever get back to it.

We are scheduled to see her Endo Doctor soon and he will want to take her off the BC shot, I sure hope that he has another great option for Kiera because she has so much trouble with her menses, pore girl :(

Kiera's edema is getting worse

I think that I might have figured this out, but we will see.

After we started to take Kiera off the Topamax this is when we really started to see the edema issue, she always had slight swelling in her legs if she was in her chair for to long and especially if she had her afo's on.
But this is different, we can not even get the afo's on now, haven't for about 2 weeks now.
Some of the side effects of Topamax is weight loss, acne and some other more common side effects from seizure drugs.
The Lyrica side effects are weight gain, hair thinning, edema, and a burning feet sensation, kidney stuff and again among other side effects.

Well this is what I am thinking, Kiera was put on the Topamax before she was laced on the Lyrica, so she did loose weight at first and she started to get acne.
Then the Lyrica comes on board.
We did not see any real side effects besides thinning hair, and a bit of swelling, but nothing to be worried about.

Now we are coming off the Topamax and I think what we are seeing is the side effects of Lyrica that might have been masked from already being on the Topamax.

The other day she just kept rubbing her feet together and they were a bit pink, the swelling does not go away at all even after having her legs up. We are seeing hair on her shirt, maybe 10-15 strands. I pick one off and find another.

I think we should look at stopping the Topamax wean and starting the lyrica wean right away.

We are going to see a Adult Doctor next Thursday, he will get to the bottom of this, he always does.

Oh how I hate medication and what it does to Kiera.
I feel sick that we are causing this to her and she just has to deal with it.
Enough already!

Sometimes my head just spins...

Kiera is doing well with the titration of the Topamax, we are going down very slowly though, slower than you would normally go off, but we definitely don't want to throw her into a tailspin.

We do have another issue going on and this is where I get so tired because with Kiera it just never seems to ever be a simple fix, and the issues are not 'normal' issues, uuuggghh!

I like to use the phrase "consistently inconsistent" that's Kiera!

So, she started Omega3 this week.
We have been dealing with some edema, more so than in the past.
Kiera has started to wear T.E.D.D socks to aid in the edema. She hates them, she has been fairly aggravated while they are on and I am thinking that they might be too small.
The other night after getting out of her stander, her afo's were taken off as well as the tedd socks, we noticed that her leg (just one of them) was puffier than her normal puffiness and that leg was cold to the touch, and also it was whiter in color.
We put her legs up and the swelling did go down, her leg warmed up again and the color came back.

She has been having, on average, 3 bm's per day so we still don't have that under control, this can also be contributing to the extra water weight.
She wears afo's and when the edema is bad she can not wear them, but that then does not help with the left foot contracture that she has that requires botox every 3 months so she can still be braced and so wearing the braced will allow her to keep using her stander. With Kiera's porous bones it is very important to keep her Bering weight as much or as long as we can.

She is already taking a medication for acne, and this medication is a diuretic, so I think that we might have to increase that medication to help with the edema.
If that does not work then we will have to get her afo's adjusted, but my thought on this is what happens if we do get the edema figured out after we had adjustments made to her afo's?

Then I was told today that Omega3 can be contributing to the loose stools, we are not sure about that but if that is whats going on with her that would be sad to me because we started her on Omega3 because there are studies out there that say that O3 is as good as carbaziphine, another seizure medication.
We started this in junction with the titration of the Topamax to maybe help keep the seizures down as we go off the topamax.

You can see how my head is spinning!
So where do you start? try a bigger pair of tedd socks? increase the diuretic? Stop the O3? get her afo's adjusted and ignore the rest?

And of course this all comes up on a Friday.
Oh and she has some eye thing going on now too.

We got through the night!

But yesterday was a bit unsettling.
Kiera received the rescue med at 8:30 am, it knocked her out.
She slept all day and finally woke up around 6:15pm.

After getting the medication her O2 dropped down to 84, we shook her to arouse her a bit so the O2 would go back up and in a few minutes it did.

She was so zonked that her O2 was hanging around 94-97, not bad but Kiera normally runs at 100%.
She felt warm to the touch, but this is something that I have seen before after giving her a rescue med.

It is 10:30am and she is still asleep.
I am thinking that the dose that she is on is too high, maybe she would do better with 2cc rather than the 4?

Oh how I hate seizures!

It's been 5 days sence we started her med wean

Well so far Kiera has been doing very well seizure wise while we are titrating down from the Topamax medication.

There has been a lot of changes that we are making right now and I dont like to make more than one change at a time but sometimes it has to happen.



Kiera is on a lot of medications and the bill from the pharmacy is growing, so today we decided to look at all that she takes and try to eliminate some that might not be helping her.

We are going to stop both her allergy meds and stop the probiotics.

Right now she is not at risk for getting C-diff so I feel okay with discontiuing this and the allergy med, well time will tell.



Her Epileptologist said today that she does not want Kiera to get a second dose of the new rescue medication diazapan in-tensol. If Kiera has a seizure lasting 10 minutes, she will get the medication, if the seizure lasts 5 plus minutes longer after getting the medication, she wants 911 to be called. Also, if after getting the rescue medication, if she were to have another seizure in a 24 hour peroid, she wants us to call 911.

We will see how this goes, my thought is that she will be taking a few trips to the ER.



Kiera has been wearing a pair of T.E.D.D socks because of some idema that she has but it does not seem to be working from what I can tell, and she hates them. I also worry about her bones, it taks about 10 minutes to put theses socks on and Kiera's bones are porus, and very thin. She is at risk for fractures and I get so worried that her ankels are going to break just by putting these on. We will see if we continue to wear these.

Update 2/21
Well what would you know, Kiera had a 14 minute GTC seizure early this morning, it was while she was getting woken up to take her bath, and bamm, she started to seize, she got the rescure medication at 10 minutes then continued to seize for another 4 minutes.
The medication just knocked her out, it is a new medication so we do not know how she was going to respond to it, now we now.
If she has another seizure in the next 24 hours we are told to call 911.
did I say I hate seizures???

We are going to start the Topamax ween!

After the prolonged seizures a few weeks ago, I thought for sure Kiera's Epileptologist was not going to give us the go-ahead to start the ween, but Kiera has been doing very well seizure wise over the last few weeks so it is a go!



We will start next Monday and do the ween very slowly.

Kiera may also start Omega3 supplement which I am very excited about because there are a few studies out, one from MN, that talks about this as being just as good as Carbaziphine.
It has so many other benefits too. The one thing that I do worry about is that it can help with lowering blood pressure, that is one side affect we don't need. Kiera's blood pressure runs very low so that might be the deal breaker if its too much of a risk.
wish us tons of luck as we go through this titration!

We also found out that Kiera's curve on her spine has not gotten worse sense 6 months ago and we are very happy about that, we are hoping to put off back surgery as long as we can and maybe even never do it.

She is now a proud new owner of a pair of T.E.D.D sock which she does not like. I hope that it will help with the edema but I am very concerned about her brittle bones as they are being put on, it takes a good 7 -10 minutes to get them on correctly and safely, Kiera goes through so much I really hate to add things that bother her. Maybe she will get used to them, I guess time will tell.

She is scheduled to get Botox in her jaw muscles to help to stop her teeth grinding, she has worn her back teeth down so far that they are flat and smooth. I have never heard of this before but I guess it can be very helpful for some people. Her Dentist says that he has referred many children for this very same reason, so we will see what this will all entail, if it sounds too horrible then we will not go through with it.

It seems to always be a wild roller coaster ride, you never know how the next day is going to go and what is around the corner.

It seems like I keep posting the same story :(

Well Kiera is still continuing to have the prolonged seizures, Tuesday night at midnight while she was sleeping she had a 16 minute GTC, then about 2 hours later she had a 10 minute cluster seizure.
Nothing has really changed as far as medications or her health so there seems to be no cause for the increase.
Kiera, although the seizures have increased, is still doing well, so that is something that we can hang our hat on.

Last night we watched American Idol (AI) and she was smiling a bit while vocalizing some, that was good to see.

Kiera and all the children that have CDKL5 are tough little cookies, they go through so much but keep bouncing back. Sometimes I think it's us parents that have a harder time with things as far as our children go.

Another long seizure

I am feeling kind of sad today, Kiera had another long seizure on Wednesday, it was a 19 minute seizure.
She is not ill or anything like that, and even if she was sick, she would most likely not have any seizure activity.
Because of that, there is nothing to blame the seizure on. She is on three seizure medications now, and all are at their max doses. What do we have to loose if she comes off some of the medication? on the other hand, what would we have to gain if she was to come off some of them?