Back Up and Running

Sorry it has been SO long since I last posted.  As always, it has been busy on so many different levels!  Here is kind of a "run down" on how things have been going for Emmalin so I can catch everyone up :)
 Since my last post in March, I am happy to report that things have been a little calmer for us (at least I think they have).

~Emmalin made it through the summer with minor ear aches and colds (which is fantastic).  The only thing that isn't that great is at some point during all of this Emmalin started consistently having distressed breathing with every single cold now :(  We just watch her pulse ox and make sure it stays at an appropriate level.

~On July 4th, Emmalin got to do sparklers for the first time.  Honestly, I don't know why I didn't think about them before, but Emmalin seemed to really like to look at them and so we will be doing sparklers from now on :)

~Some point in the middle of August Emmalin was diagnosed with pneumonia again.  To be honest the doctor said he wasn't completely sure if the sounds were all coming from upper respiratory or if it was in her lungs and so she was treated for pneumonia (and an ear infection).  Emmalin ended up getting sick again with a different round of viral stuff about 9 days later and so this was the cold that I felt was NEVER GOING TO END (but of course it did finally).

Our Sleeping Beauty

~September 11th, Emmalin had another cold (with distressed breathing) and her pulse ox dropped again (the only other time this has happened was back in March).  She was admitted back in the hospital (this time at Cox South) because she needed oxygen.  Emmalin ended up being in the hospital for 5 days this time.

Em the day she was admitted :(

~September 12th, while we were in the hospital, Emmalin had a huge coughing spell and ended up aspirating for the first time ever :(  So from this moment in the hospital Emmalin was then treated for aspirating pneumonia (it went in her lower right lung).  This was a huge blow because they say in the CDKL5 world that once they aspirate, it makes it easier for it to happen again (big sigh).  Emmalin is doing better now, but it took her body about 2 weeks to recover after being released from the hospital.  Honestly, there are still some small ways that her body is still weak, but all in all Emmalin is back to her "norm".


~When Emmalin was released from the hospital, we then got oxygen for the house.  No, Emmalin did NOT go home on oxygen!  But with winter coming on they (Emmalin's pediatrician) just thought it would be a good idea for us to have some at the house in case Emmalin's pulse ox drops quickly again (like last time).  As much as we hope not to have to use it, if Emmalin does get in situation where she is sick and her pulse ox drops again, by having oxygen at the house our hope would be that we could keep Emmalin at home instead of having to take her to the hospital.  Emmalin gets sick SO easily and

Our New Portable O2 for the House

quickly!  We just figured that (as I said before) with the winter coming on (and the fact that Emmalin had some type of pneumonia TWICE within about a month and a half)  we figured this was probably a smart move.  If I'm being honest with you, I would have to say that getting oxygen at the house was a very bitter sweet thing for me.  As grateful as I am to now have the oxygen at our house in case Emmalin needs it, it was a hard blow for me.  I know that sounds ridiculous, but it's just another piece of equipment that will forever now live at our household.  For me oxygen was just something that I was hoping wouldn't be "living" at our house for a VERY long time... but as I said, I am VERY grateful that we have it now for if Emmalin's pulse ox drops again!


~On a very positive note, the Clever City Park got a special needs swing!!  This may not seem like a very big deal, but it is HUGE for us!!  Emmalin loves to swing :) This means I don't have to drive into Springfield anymore so Emmalin can go swing at the park.  We are SO grateful for the City of Clever and for them seeing the need for a special needs park in our little city!!

Emmalin LOVING her time swinging
at the Clever City Park :)

Therapy-Wise:
Emmalin has been working very hard to start gaining ground physically and cognitively.

Em working hard at Vision Therapy

Cognitively we feel Emmalin is starting to show us that she understands more (which is AMAZING).  Physically, I feel like Emmalin  has lost some ground in her shoulder/arm strength, but we are working on it!  Of course we are still working with Emmalin on head and trunk control.  Emmalin actually had the opportunity to start back up Hippotherapy (where she rides the horse) again last week and she gets to start back up water therapy on Halloween.  We are SO excited for Emmalin to have the opportunity to do these 2 therapies!! I have been told that Hippotherapy and water therapy are the best 2 therapies that you can do for a child who has CDKL5.  Altogether Emmalin (at the moment) therapy-wise does PT, OT, and Speech at the Meyer Center and also at her school.  Emmalin also does Vision therapy 3-4 days a week.  Since all of Emmalin's major surgeries are done for a couple of years, me and Jarrad truly feel that this is Emmalin's time to really have the opportunity to gain some ground strength-wise (and cognitively), and so we are trying to give Emmalin every opportunity that we can think of to help her.

Em and the gang at Hippotherapy

Well...all of this pretty much gets us back up to date on everything.  I will try not to wait as long before I make my next post.

                                                                   God is Good!

1 Year Since Emmalin's Corpus Callosotomy

One year ago today, Jarrad and I had one of the HARDEST days of our life!  It was on May 2nd, 2012 that Emmalin had her Corpus Callosoty (brain surgery) to try to help with seizure control.  Between the length of the surgery and ALL of the risks that were involved with it, I would have to say that not only was it the hardest day, but it was also the longest and scariest day I've ever had in my life!  Of course Emmalin did great through it (as she always seems to do), but I can honestly say that we NEVER expected this past year to be as "eventful" as it turned out to be!  To say the least, this past year has been a really hard one!! If I am going to be completely honest I would have to say that in some ways this past year has really seemed like it has been the length of 10 years.  Emmalin has had some highs and A LOT of lows.  Saying that, of course I ALWAYS know that it could have been SO much worse, but what I was meaning was out of Emmalin's 4.5 years of being on this Earth, this has BY FAR been her hardest!

Most of the major things that have happened in the past year:
~Emmalin had her Corpus Callosotomy Surgery
~About a month later her incision got infected and Emmalin was in the hospital an additional 6 days (plus send home with a PICC line for antibiotics - she was on atleast 2 different antibiotics for a little over a month).
~In August because Emmalin was having SUCH difficulties swallowing (she was aspirating foods), Emmalin officially had to COMPLETELY stop eating by mouth.
~Throughout the next couple of months Emmalin had numerous ear infections.
~In November Emmalin was admitted back in the St. Louis Children's Hospital for another 24 hour EEG because we (me, Jarrad, and Dr. Weisenberg) had decided Emmalin was at the point that she needed to have the Vagus Nerve Stimulator (VNS) placed to also help with seizure control because she was still having SO many seizures.
~We also found out that Emmalin was going to need ear tubes to be placed because she was getting SO many ear infections.
~Emmalin was sick on and off from Thanksgiving on and her VNS surgery ended up having to be rescheduled 3 times before she was well enough to have the surgery.
~New Years Day Emmalin was diagnosed with pneumonia for the first time (right lung only).
~Emmain FINALLY had her VNS surgery on January 23rd.  
~The beginning of March Emmalin we thought her neck incision from her VNS surgery got infected and so we made an emergency trip to the St. Louis Children's Hospital.  While we were there, Emmalin had to be given oxygen for the first time because she had a virus and got distressed breathing (so was in the hospital for 3 days)
~ April 25th Emmalin had Strabismus surgery to try to have her eyes realigned and she also got her ear tubes placed (finally)

As I was saying, you could DEFINITELY say that this has been a big year for Emmalin!  And although we are incredibly grateful that things were not worse than they were (because we realize they could ALWAYS be worse).  As you read above, Emmalin's year didn't really start out on the best note on January 1st because that's when we found out she had pneumonia.  So I am going to start a "new year" now, 1 year after Emmalin's brain surgery.  We are REALLY  hoping this next year will be less "eventful".  We plan on pushing forward with therapies and doing everything we can to help Emmalin make great strides in the right direction not only strength wise but also cognitively.

                                                                  God is Good!
                                                       

Emmalin resting peacefully :)

First Time Getting Oxygen :(

Although Emmalin didn't not feel that well while in the hospital, I was able
to catch this small smile while she was awake for about 15 minutes.

The beginning of March of this year (2013), we ended up having to make an emergency trip back up to the St. Louis Children's Hospital.  The first reason we headed that direction was because we thought Emmalin's incision on her neck from her VNS surgery had got infected.   Of course we didn't discover all of this until around 5:00pm on Saturday.  After talking with the doctors we had decided that since it was already so late in the day, that we were going to head that direction at 5:00am on Sunday to put us at the St. Louis Children's Hospital ER around 9:00am (we had to go through the ER because the normal "office" was closed).  To our surprise, at 3:30am on Sunday, Emmalin started having distressed breathing.  I guess I should mention that Friday evening Emmalin had started to get a cold, but when she typically gets colds she doesn't get distressed breathing.  Anyway... so even though we were originally going to the hospital to get her incision checked out, Emmalin also ended up being treated for something completely different, distressed breathing.
Back to that day... by the time we get to the St. Louis Children's ER, Emmalin's breathing was not that great and they immediately put her on oxygen.  I thought that she had just developed pneumonia because she had just got over pneumonia (having it for the first time ever) the beginning of January.  I just assumed that since she had just had pneumonia SO recently that Emmalin just got it a lot quicker this time.  So once they got the oxygen on Emmalin, they did a lung x-ray to check her lungs, to our surprise they were clear.  What we did find was that she had the Rhinovirus and Branchiolitis.
The Rhinovirus is literally just a common cold.  But what we didn't know is that it is VERY common for the Rhinovirus to cause distressed breathing in kids with neurological issues.
The Bronchiolitis diagnosis meant that even though the lungs were just fine, the areas above them were inflamed (which was helping cause the distress).  Emmalin started out on 4 Liters of Oxygen and was then weaned down to 2 Liters of Oxygen by the time she got to her room on the 12th floor (that's the neuro floor).  Emmalin was off of oxygen by 6pm on Monday night and so she was released on Tuesday afternoon (you are supposed to be off oxygen for 24 hrs before you can be released from the hospital).
As I had said in the post title, this was Emmalin's first time to have to be put on Oxygen.  To be honest, it was a sad moment because I have been told that once CDKL5 kiddos have to be put on it the first time, it seems to be easier for them to need it when they get sick after that... I guess we will see (but I sure hope they are wrong).  On the up side, Emmalin did great weaning off of the O2 and for that we are SO grateful!!!

As far as the infection goes that was on her neck, it ended up just being stitches that had come through the skin.  When the stitches came through it created some puss around the stitch and that is what we saw... but it was with the same doctor that Emmalin's brain surgery incision got infected and so this time the doctor wasn't taking ANY chances.  So even though it ended up  being nothing, while we were in the ER the doctor took a sample of the puss and took it to grow in a lab for a couple of days.  While she was being treated for the Rhinovirus, they went ahead and gave Emmalin 2 IV antibiotics JUST in case something grew in the lab that they were not anticipating.  Emmalin was given Vancomycin and Cefepime (which is what she was put on when her brain surgery incision got infected).

So Emmalin was admitted on Sunday AM and got released that following Tuesday afternoon.  As I said before, it was a VERY unexpected trip, but we are SO grateful that everything went as well as it did.

This has been an increablibly hard winter for Emmalin as far as sickness goes, to be honest this has been her worst winter so far with sickness.   To say the least, we are REALLY looking forward to warmer weather!!

God is good!

Our sleeping beauty :)

Emmalin Playing Her "Piano"

One of our amazing friends is letting us use their Kindle Fire.  We found an app that looks like a little piano.  This is Emmalin playing with it.  It's SO amazing to see her open her hands up as she hears the sounds as she touches the "keys".

This may not seem like a big deal to some people, but to us this was a HUGE step (or maybe even leap) forward for Emmalin and learning to use technology :)

2 Week Follow Up From VNS Surgery

Emmalin went back last Tuesday, February 5th for her two week follow- up from her VNS surgery.  Dr. Limbrick (Emmalin's neurosurgeon) said she was doing great.  Emmalin's incisions were just a tiny bit red in a couple of places so they decided to put her on a 10 day antibiotic to be safe. 

Today Emmalin is actually 3 weeks out from having surgery and she is doing wonderful!!  She was able to start up therapies yesterday and she is doing really well with it all (she hadn't been to therapy since the first week of December).  I think we are both glad to be getting back into the swing of things!!

                                                                    God Is Good!

Vagus Nerve Stimulator (VNS)

Jarrad, Me, and Emmalin before her surgery.

January 23rd, 2013, Emmalin had a device called a Vagus Nerve Stimulator placed into her chest to try to help with some seizure control. This device was placed in Emmalin's upper left chest and then a wire was attached to the device. Then Dr. Limbrick (Emmalin's neurosurgeon) made an additional incision on her neck so he could "run" that wire up Emmalin's neck and attached to the Vagus Nerve.

The blue is actually pen marks,
but this is what it looked like right
after surgery.

Although it has been expalined to us that the VNS will not help with any seizure activity going on in Emmalin's brain, the whole idea of this device is that it will act as a medication but without all the side effects. Of couse our first hope with this is that it will help control Emmalin's seizures better by making the seizures less frequent or by decreasing the lenght of each seizure (or both). If we can get what we (our epiliepsy specialist and me and Jarrad) would consider to be a "good handle" on Emmalin's seizures, our next hope would be to one day be able to get Emmalin off of some of her seizure medications. The VNS device responds to a certain type of magent and the device is "adjusted" by a type of wand (that's what they call it).  They (the doctors) stick the wand in front of Emmalin's VNS device and from there they are able to adjust the settings to make the device "fire" a stronger current (for lack of a better term) or to adjust how long it will "fire" each time.  At the moment Emmalin's device "fires" every 5 minutes for 30 seconds.  As far as if the VNS has helped Emmalin so far... to be honest, Emmalin has been ALL OVER the place seizure-wise!  Some days Emmalin will only have a couple seizures, a couple days she has had none (that I know of anyway), and other days she has still had SEVERAL!!  The "bonus" thing with the VNS is that we were given a magnet (yes it's literally a magnet) for when Emmalin has seizures.  The idea of the magnet is that you can swipe the magnet across the device in Emmalin's chest and it will make the VNS device "fire" an additional "round".  This "round" will last for 1 minute (instead of 30 seconds).  The idea behind swiping the magnet and making the VNS do an additional "fire" it will hopfully make she seziure not as intense or possibly even stop it altogether.  It is still REALLY early on as far as being able to report "offical" results. The VNS can take up to 2 years to get up to reach it's "full potential" of what it may be able to do to help Emmalin and the quickest you can get it adjusted is every 2 weeks. So I'll keep you posted! :) 
Isn't there a saying about "slow and steady wins the race"? In this instant, we hope that this is the case for Emmalin and her "win" will be fewer or NO SEIZURES! :)

As you can see, it didn't keep
Emmalin down for long, this
is her literally a couple
 hours after surgery.

 Emmalin's biggest complaint
was that we wouldn't
let her roll around - lol

I knew that I wouldn't be the best at describing all of this VNS info/lingo.  So I went to the company's website that made the device (called Cyberonics) that Dr. Limbrink placed in Emmalin to try to see if I could find anything that would be worth posting on here. Although the pictures wouldn't post, I got a lot of the information. If you want to read more about the VNS, click on the word "Cyberonics" (below) and it will send you to that company's website. But just so you know, the device that it pictures is not the device that Emmalin had placed, Emmalin's is smaller.

                                                Cyberonics

Here's the info I got from the Cyberonics Website:

What is VNS Therapy for Refractory Epilepsy?

VNS Therapy is a non-drug treatment option that could help provide a new sense of control and independence to people living with seizure disorders. VNS Therapy is indicated for use as an adjunctive therapy in reducing the frequency of seizures in adults and adolescents over 12 years of age with partial onset seizures not controlled by medication or who experience intolerable side effects. More than 60,000 people worldwide have been treated with VNS Therapy for epilepsy.

How does the VNS work?
VNS Therapy is used in addition to medications to help improve seizure control. The vagus nerve is one of the primary communication pathways between the body and the brain. VNSTherapy is delivered by a device (generator) similar to a pacemaker and a thin, flexible wire (lead) that sends mild stimulation to the left vagus nerve. VNS Therapy is not a drug and, therefore, does not have the same side effects and does not interact with other medications. The vagus nerve delivers these periodic stimulations to the brain and helps to prevent electrical irregularities that cause seizures. Treatment is automatically delivered at regular intervals all day, every day so that you do not have to worry about missing a dose.

If I have VNS Therapy, will I still need to take medications?
VNS Therapy is an added treatment to your current medications. It is not a replacement for them. Some people in the clinical trials were able to reduce their medications over time. You and your physician will determine your ongoing treatment regimen. It is important to always follow your physician’s recommendations about your medications.

What does the procedure involve?
VNS Therapy is not brain surgery. VNS Therapy involves a minimally invasive procedure, which is typically performed under general anesthesia by a surgeon. The procedure takes approximately 1 hour and patients usually go home the same day. Typically, two small incisions are made—one in a natural crease on the left side of the neck and one in the left chest area, below the collarbone. The generator is typically placed under the skin in the left chest area. A thin, flexible wire connects the generator to the left vagus nerve in the neck. The small scars from the two incisions typically fade over time and become minimally noticeable for many people. Another procedure is required to replace the generator once the battery is depleted. This procedure requires only one incision and usually takes less than an hour. The VNS Therapy procedure is completely reversible.

What is the function of the VNS Therapy Magnet?
The VNS Therapy magnet may provide additional benefits for some people, but it is not necessary to use it for your regular stimulation. By swiping the magnet over the generator when you feel a seizure coming on or during a seizure, you or your caregiver will be able to stop the seizure, shorten the seizure, decrease the intensity of the seizure, and improve the recovery period following the seizure. You can also fix or hold the magnet over the generator to temporarily stop stimulation during certain situations or activities when even mild side effects are troublesome, for example, public speaking, singing, exercising or other strenuous activity. The magnet can be applied by any person or caregiver trained in how to use it. If you experience troublesome side effects for an extended period of time, contact your physician.

What are the potential side effects of the VNS Therapy?
The most common side effects of VNS Therapy include temporary hoarseness/changes in voice tone, coughing, a tickling sensation in the throat, and shortness of breath. These side effects generally occur during stimulation periods and typically decrease over time.

Catch Up

I realize I haven't posted anything since Thanksgiving, but things were pretty hectic in December and January (I'm sure they were for some of you also). 

To start off Emmalin was sick on and off from about Thanksgiving until about January 23rd.  Then on that day, Emmalin had a surgery (I posted all about it in the post Vagus Nerve Stimulator - VNS), and then from that point we have been focusing on Emmalin's recovery and just getting back into the swing of things.  Emmalin was originally supposed to have the VNS surgery on the 19th of December, but 5 days before she got a cold and a double ear infection.  Surgery was then rescheduled for the 2nd of January, but starting the 27th of December Emmalin got another cold and an ear infection in her right ear.  The difference this time was that by New Years Day (Jan. 1st, 2013) Emmalin's cold had turned into pneumonia in her right lung and at this point she had a double ear infection.  So then surgery was rescheduled (again) for the 23rd of January (and she actually had it on that day).  We had to wait a minimum of 3 weeks each time before we could reschedule the surgery.  Between each of her scheduled surgery dates, we spent our time trying to keep Emmalin sealed in the house and focusing on getting her healthy and keeping her that way (but as you can see keeping her healthy was harder to do than we thought). 

Emmalin with some crazy hair hanging out in her swing
waiting to get released from surgery so she could start backup therapies :)

Anyway, so yesterday, February 11th, 2013 was officially Emmalin's 1st day back to therapies since the first week of December.  She did WAY better than I had anticipated and I am excited to get back into our routine and for Emmalin to start making some progress again (therapy wise). 

This pretty much gets you up to date as far as Emmalin goes, I am going to try not to wait so long until I post next time!

                                                                  God Is Good!