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!