Friday, October 25, 2013

Avacake, VNS Removal, and Her Intractable Epilepsy *Update

So here's the deal, down-low, low-down, scoop, deal-y-o, and you get the point.  If you follow us on youtube (sevenswansaswimming) or Facebook (Avacake), than you know that Avagrace and I just got back from Cook Children's Hospital in Ft. Worth Texas because Avacake had Neuro-Surgery.  A few years ago Avagrace got the Vagus Nerve Stimulator placed.  For those of you that do not know what it is I will describe it in the next paragraph, if you do know what it is feel free to skip to paragraph three.  :-)

A Vagus Nerve Stimulator is a device that has been used to treat seizures that are resistant to medications, diets, and basically any other seizure treatments.  It is a pace-maker-like device that they place under the chest muscle on the left side near your arm pit.  The battery pack/transmitter then has a coiled node that wraps like a spring around the vagus nerve traveling up the left side of your neck.  This delivers electrical stimulation at regular intervals and varying intensities that are set by your doctor.  It is treated like a medication in the sense that it is increased in increments until the right "dose" is found.  It's purpose is to send electrical currents at a regular rate in hopes of interrupting the irregular electrical activity in the brain that is also known as seizure activity.  The device comes with a magnet and works with magnets.  So when a seizure starts you place a magnet on the "battery pack" portion to get it to deliver the currents at that very moment to try to stop the seizure.  This becomes your first line of "rescue medicines."  Rescue medications are what you give to stop seizures.  Most medications are not given for every seizure.  However, the magnet is.

When Avagrace got her VNS it was not an easy recovery.  What is meant to be an outpatient surgery ended up being a three day stay in the hospital because she could not swallow her pills and choked every time the stimulator went off.  It was initially set for every five minutes lasting 30 seconds to one minute (I cannot remember exactly) and it was a nightmare.  Eventually with OT she was able to swallow again but despite getting use to it going off, she never didn't feel it.  We changed the setting frequently and hoped to find one that worked.  Because of how many seizures she has it needed to be on a high setting.  Unfortunately, it just made life a nuisance.  However, for the first year it was exactly that, a nuisance.  The pros still outweighed the cons.  She was able to adapt to it and although she still felt it, she could deal.

The VNS did not stop her seizures.  However, it did work wonders on her absance seizures (petit mal), her complex partial seizures, and her overall focus, memory retention, word finding abilities, and mood.  There was good to the VNS.

Then after about a year her neck started to look very strange around the VNS.  We could always see the battery pack and the line up her neck.  Avagrace is tall and very skinny.  She has a very high metabolism and therefore has to be on extended release on any medicine that has XR/ER as an option.  She drinks pediasure and maintains a healthy weight.  But it is something that we have to be mindful of.  Because she is so skinny, her VNS was obvious.  Slowly the lead seemed to slip down lower and lower on her nerve.  Then a strange spiral was visible on her neck.  It looked as if part of the lead had come off of the nerve and coiled just under the skin.

I immediately brought this up with her neurologist and he said that it was obvious that it was wrong and that we needed to talk to our pediatrician to get a referral back to a neuro-surgeon to fix it.  Well forgive me but I did not want to deal with the same doctor that did the first surgery if this is something that happened because it was placed wrong (which was what our neurologist had also agreed with).  So for nearly a year the office that put the VNS in argued back and forth with my Pediatrician (who NEVER gave up on Gracie) because they didn't want to see a patient of one of their partners, it was too awkward.  On top of that our military insurance would not approve to go to another clinic.

Then on New Years Eve of this year Avagrace had a series of status Abdominal Seizures (not even the "big" grand mal GTC seizures), and her heart stopped!  With a VNS paddles cannot be used so CPR is the only option to help her.  Fortunately it worked with one swift punch to her chest.  Unfortunately, she has needed to have CPR to restart her heart two more times since then.  Her epilepsy doctor here prepped us that this is just par for the course in an intractable epilepsy such as Avagrace's.  It is a progressive degenerative disorder and this is to be expected.  I was devastated that he did not have more fire to fight for her, no plan, no actions, nothing.  Just increase the meds that we are on and see us back in a few weeks.  My beloved pediatrician and I were not going to just sit idly by and not get help.  So even though the epilepsy doc here told us that "if we go to Cook Children's for a second opinion he will not see Avagrace anymore" we went anyway.  And I am so glad I did!  

Finally, on Friday October 18, 2013, we go to go to our "second opinion" specialist out of state.  He is a doctor that is part of a team in one of the best Pediatric Epilepsy Monitory Units in our region of the USA.  Because he is both out of network and out of state our insurance would not allow him to be the full time doctor but rather the distant voice of wisdom that we saw only once or twice a year (we have been going to him for the past three years but had to discussed the problems with the VNS yet) .  So this was the first time we had seen him in over a year.  We had been to his ER a few times recently and talked to him over the phone and through one of his fellow docs that was on call, but we had not had an office visit with him.  We went over everything and as soon as he saw the VNS he texted a Neuro-Surgeon from the same hospital and in less than five minutes he was joining our appointment.  He felt it, looked at it, read our history reports and they both came to the conclusion that X-Rays must be done because this could be causing Avagrace's heart to fail.  So, we finished up the appointment with a new plan.  The VNS was turned off and a new Anti-Epileptic Drug would be introduced to help with her still way above baseline seizures (hundreds a week).

We then drove home and had a nice weekend.  On Monday Morning I got a call from the Neuro-Surgeons office.  They got the X-Ray's and wanted to see us immediately and had rearranged schedules so that we could see him at noon the next day to get surgery schedule with emergency/urgent status.  All right!  So I know that when a doctor calls, you jump and run and do whatever they say because 1. they are a specialist for your baby and are rearranging their busy life for you (this must be serious).  2.  They care enough about your baby to rearrange their busy schedule and to call me the very day he got the report (I must trust him).

So despite the lack of baby sitters for four kids while my active duty husband still has to work, despite me taking our one vehicle and leaving him without a working car, despite the ridiculous amount of gas we have spent going to and from Ft Worth, none of that mattered because we knew that beyond a shadow of a doubt that God was orchestrating this.

We got to Texas and checked in to have the appointment and we learn that our suspicions were right.  It looks like the VNS has been put in wrong or that it has slipped on its own and it has to come out immediately.  We could just leave it in and keep it turned off but there is always a risk that a magnet (think theft protection at stores/my husbands air force base/airport security/the science museum/cellular devices, etc...A magnet could turn it back on and we wouldn't know it.  But there is also the huge risk that even if it is not causing the heart to stop we cannot use paddles to save her if CPR does not work.  And that I am not ok with.  So we agree that for so many reasons (some that I do not include here) the VNS and lead need to come out.  The reasons for it coming out right away are because of her heart.  Over the past several weeks her seizures have gotten progressively worse and based on her history we have to be prepared for her heart to stop at any moment. It is also deemed an emergency because the slippage/coiled part is wrong and there is a threat of it causing damage to the nerve or other areas of her neck/throat area.

So, Surgery was scheduled for the next day.  We head to pre-op, get blood drawn, breathing checks, heart checks, and her history evaluated and communicated.  We got our instructions for arrival time, surgery times, and what we needed to do for prep at home prior to surgery.  Fortunately our doctors talked to the social worker who was able to secure a room in an amazing Ronald McDonald House.  So, it is the night before surgery and I am pulling into the RMDH when I get a call stating that Tricare is not going to cover the surgery for at least 6 days.  So I call tricare and 57 minutes later I know what form I have to tell the nurse to call in and complete over the phone.  I call the nurse she says that she will in the morning.  I tell her that she has to do it now.  She gets huffy but does it and finally we get approval for my daughter to have the surgery that her doctor classified as emergency surgery.

The next morning we got the call that Avacake was supposed to arrive several hours earlier because she would be going back with a different surgeon, ahead of planned.  All went smoothly and the new doctor was great.  I mean GREAT!  He even asked to pray with us before taking her back to operate.  It was such a huge blessing and confirmation to me that we were in the right place at the right time. 

After taking her back to the OR I anticipated surgery times of 2-4 hours depending on how grown-in to the nerve the coil node was.  At just shy of 2 hours the nurse called to tell me that the coiling/slippage was a benefit in removing it because it just slipped right off of the nerve in all but an inch of nerve.  The scar tissue that formed, actually formed in between the wire and the nerve rather than fusing them together.  There really could have been no better news in this surgery!  Secondly, the direction of the node was not wrong so it was unlikely that the VNS was causing her heart to stop.  That means two things.

1.  Her heart stopping during status seizures may just be a normal wear and tear on Avagrace's body when it comes to her disorder and how intractable epilepsy and her specific seizures affect her as an individual.

2.  We are so glad that we got the whole system out of her body because now we can do more to get her heart going if it stops again.

So this is why we did what we did.  There was nothing done during this phase to work on decreasing the seizures aside from increasing and adding a fourth medicine.  So Avagrace is still seizing hundreds of times a week and there is nothing more that we can do at this point.  The surgery was for the reasons listed above.

I hope that this sheds some light on the confusion.  If you have any questions please feel free to comment below or contact me, I am glad to answer them or find the answer for them!

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