Thank You!

For all of you that have made comments on this blog, thank you! I have to translate your comments most of the time, but your messages are very nice! :)

Wow, it's already been one year since Kiera's surgery

It's hard to believe that a year has gone by already since Kiera had her emergency surgery last July 4th.
I still remember how I felt when I was asked for my verbal consent to go ahead with emergency surgery. I also can remember what the drive home from up north was like and having the fear that we were not going to get there before something horrible would happen.
Her scar is still there, and issues around the surgery, but unrelated are still present....and always will be now. she was critical and had to be placed on a ventilator even before the surgery due to aspirating on the contrast the Doctors gave her. But Kiera's here, and has proven to be a very strong young woman.

Over the last few day's Kiera has been in one of her giggly moods which makes everyone around her very happy, love that!!!

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.

How time can get away from me...

It's been so long since I have blogged anything here.

July is past us now and Kiera's surgery site is looking good. We are still having issues with the J tube site, it's been a constant battle with leaking that leads to a big rash around her stomach.
Her Doctors keep telling us that J's are notorious for this, but I tend to disagree only for the fact that the old j did not do this, it was up a bit higher than the new one so I think that might be part of the issue.

We have tried everything we can think of and nothing seems to help the rash, but then why would anything help when there is a constant issue with leaking?

I hope to one day find the answer but I hope that would not involve another surgery.

Kiera has been doing okay since July although a few times in and out of the hospital, but mostly related to the surgery.
She was just in for 3 days for what we think may have been a intestinal bug, but with Kiera nothing is ever simple.
Kiera seemed to have had a cold the day prior and the next morning we had an early appointment. While getting ready Kiera vomited, the house called me to see what we should do about the appointment and it was decided that we cancel because this was going to be an all day appointment and if Kiera was not feeling well, I did not want to put her through this.

The house called the clinic to cancel, then with-in a half an hour they called me back to say that Kiera had thrown up 2 more times and that her Oxygen was down to the mid to low 60's. They tried suctioning and gave 10LT of O2, that only brought her Oxygen up to the mid 80's. 911 was called and the EMT's made the decision to take Kiera to a local hospital rather than the one that we use in the city to get her stabilized.

We met her at the hospital and she did not look great, she was pale and she continued to vomit. Her heart rate was higher than her normal (she has tachycardia) and her blood pressure was at her normal low, but the Doctors were worried about this until we told them that this was where Kiera's numbers are most of the time.
At one point her fingers and toes got very gray and the nail beds were purple, then she started to just shiver, she would do that and then quit, then do it again... very scary to see as her mom.

She did need to be on oxygen up until the day she left the hospital but did not need to remain on it.
So in the end it seemed to be a 24 hour bug with the added aspiration episode to complicate things for a bit.

Kiera just had a nice break from her seizures since the hospital stay, she went 13 days without any seizures!!
But now they are back with a vengance, it's strange how things can change so quickly in just a day.
Yesterday she had 5 seizures ranging between 7 to 13 minutes, two of them required rescue meds and today 3 seizures, so just to try to stop them from continuing today, she received another rescue med.

We will see what tomorrow will bring.

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