Dysautonomia... What Is It, Anyhow?

Many people have asked us exactly what is wrong with Kayleigh. It does have a name, although it is hard to explain.

Dysautonomia is a dysfunction of the autonomic nervous system in the body. The autonomic nervous system regulates the automatic organ functions of the body that occur on their own... things like heart rate, blood pressure, your temperature, your respiration, your digestion, etc. In Dysautonomia, some of these things don't function properly sending the body into chaos. Often, the symptoms are so unrelated to each other that it's tough to diagnose.

There are 4 types of Dysautonomia and Kayleigh has been diagnosed with 3 types as of the time of discharge from Duke Med! Lucky her!

Postural Orthostatic Tachycardia Syndrome - basically it is when the body is unable to adjust blood pressure and heart rate when a person goes from sit to stand. In Kayleigh's case, when she is sitting and stands up, her heartrate goes up by about 50 beats per minute and her blood pressure drops drastically... all within seconds of standing up. Kind of like an orthostatic hypotension to the max! There are also other things involved in POTS that aren't understood that make one fatigued, weak, and unable to function.

Neurocardiogenic Syndrome - This is characterized also by a rapid drop in blood pressure, but also a rapid drop in heart rate causing the person affected to pass out and sometimes even become asystolic (not have a heartbeat at all for a period of time). Asystole is what the doctors believe has happened to Kayleigh on at least 2 occasions - once when she collapsed on April 12th at school and once during a test they performed at Duke Med on July the 5th.

Primary Autonomic Failure - This is kind of the biggie that lumps everything all together and caused her spiral downward. PAF is a degenerative disorder of the Autonomic Nervous System where her ANS just slowly started to shut down. In Kayleigh's case specifically, it started with a slow decrease in heart rate and blood pressure over a period of time that was so subtle that it went unnoticed because she was functioning pretty well, just got tired easy. As it progressed her ability to eat a normal meal became a problem. She would get hungry, start to eat, and then become very nauseous because her digestive system wasn't responding properly. Eventually, it got to a point where she wasn't eating hardly anything other than things she knew would not make her sick. The worse it got, the less she ate, and her heart rate and blood pressure drastically started to drop, then her heart wasn't able to sustain a normal sinus rhythm. This eventually left her very lethargic, weak, fatigued, and unable to function at all. This, ultimately, is what took us to the Emergency Room on July 11th.

We are very grateful to the team at Duke Medical Center for putting ALL of the pieces of the puzzle together and being able to treat all of the symptoms involved. She will remain on a slew of medications to keep her heart beat and blood pressure where they need to be, allow the nausea to remain at bay and keep her appetite up, and help her maintain her energy and ability to live a normal life.

So blessed to live in a time where modern medicine can give her her life back!

Looking Back...

I've had some time to sit and reflect over the last few weeks. Not sure that's completely a good thing because it's given me time to think about all of the "what if's...". But, it definitely has me appreciating life and the power of prayer!

I don't know that it completely occurred to me how close we came to losing Kayleigh on more than one occasion. Her illness has slowly been taking her body over, leaving her so weak and fatigued that her heart literally was just starting to give up. Our second night in the hospital, at Baptist, we must have had a roomful of doctors and nurses around when she first went into distress. They moved her bed over to make room for the "crash cart" and prepared us for what "could be". The room was oddly calm, I was in a bit of frozen panic, and all Mike could do was say "I get to yell CLEAR" nervously!

Later that night Mike and I were talking to the doctor in the hallway, leaving Kayleigh in the room with a dear friend. Kayleigh, with tears in her eyes, looked at Jen and said "I felt like I really was dying..." When Jen told me of this conversation, my heart just sunk for Kayleigh.

A few days later, while at Duke Med we found ourselves in a similar situation. The doctor that came in explained to us that they were on the verge of pulling the "code blue" switch in her room because her heart rate had just dipped way too low and she was in a dangerous heart rhythm.

There were several occasions where Kayleigh would go into a sustained dangerous rhythm where all we could do was run our fingers through her hair, speak softly to her reassuring her, and keep her calm. I would send out a prayer text to a group of people that simply would state "Pray NOW..." and within minutes she would return to a normal heart rhythm. The two times where Kayleigh was so dangerously close to cardiac arrest we silently prayed and only His healing hand was needed without any other intervention.

Kayleigh is recovering more each and every day! She is eating well again, has energy, and looks better than she has looked in months! We have learned that life is truly a fragile thing and we are not in control of if, only He is. I thank God at the start and finish of each day for not taking our Kayleigh home quite yet!

Life in the Creamsicle Castle

Once upon a time, a princess named Kaybers, had a short stay in a Creamsicle Castle because she got very very sick. She went there to feel better and get stronger because there were lots of nice people there to help her.... but some were not so nice!

When Princess Kaybers first arrived at the Creamsicle Castle off her chariot, Dr. Confuzzled was nowhere to be found so Princess Kaybers was left to the others! They were all scratching their heads with what to do and enlisted the help of Dr. Stapleyourmouthshut, who was NOT very nice at all! Dr. Confuzzled arrived too late to save Princess Kaybers because Dr. Stapleyourmouthshut had already decided to make Princess Kaybers eat all kinds of strange meals (like salad, white rice, and wheat bread) and disregard the map that Dr. Confuzzled had already drew! Dr. Confuzzled decided to sit back and wait for the go ahead to take charge!

In the meantime, Dr. Stapleyourmouthshut put Dr. Perky on the case! Dr. Perky almost immediately decided that Dr. Stapleyourmouthshut was on to something and put Dr. Creeper in charge of peeking in on Princess Kaybers every chance he got! One of Dr. Perky's newbies, Dr. Coolstuff, stopped by late one evening and offered kind words that made Princess Kaybers smile again. Then, the next day, Dr. Perky's knight Dr. Superhero, disagreed with the way that Princess Kaybers had been treated and decided that maybe the help of Dr. Fairygodmother would help get Princess Kaybers out of this mess. So, the King and Queen reluctantly agreed to let the meeting happen.

Dr. Fairygodmother finally arrived a few days later and saved Princess Kaybers from Dr. Stapleyourmouthshut by saying that Princess Kaybers was just too weak before and really needed to be able to make some of her own choices! Princess Kaybers, the King, and the Queen were all very happy!

The next day, Dr. Superhero reenlisted the help of Dr. Confuzzled to make sure that everything was in place and they got back on the right plan! A few little changes did the trick and Dr. Superhero saved the day by rescuing Princess Kaybers on the eve of her birthday! Princess Kaybers was able to finally leave the Creamsicle Castle and return to her own castle on Foxbury Court where the next afternoon a celebration of friends gathered to welcome her home, and she lived happily ever after!

Light at the End of the Tunnel

This is our girl, sitting up on a Sunday morning feeling great! PRAISE GOD!

We also are starting to feel like we see the light at the end of the tunnel! This is what all has happened this weekend...

1) We agreed to let her be seen by a psychologist yesterday morning (the last few weeks have really taken a toll on her). The psychologist said Kayleigh is "delightful", an "old soul", and that she thinks she will be just fine because she clearly has a great support system of friends and family. This little bit of encouragement almost IMMEDIATELY lifted Kayleigh's spirits! To be told you're not crazy is a big thing!

2) The IV Zofran has really helped her nausea and allows her to eat WAY more than she's been eating and her energy level has really improved. She has gone from only able to walk 1 or 2 laps around the unit to 6 laps before she fatigues. Over the weekend, we have successfully transitioned her from IV Zofran to oral Zofran without any trouble! One step closer to going home!

3) Watching the heartrate/blood pressure trends over the last few days finally had them see what we have been seeing and they have decreased her Midodrine dose to 7.5mg and we are starting to see more stabilized blood pressures and heartrates.

4) The multifocal and left ventricular PVCs are still present, but they are less intense and she is getting through them without the miserable scared to death look on her face.

And the best news of the day....

5) She will have a 24 hour holter monitor put on tomorrow morning and taken off Tuesday morning. We hope they read it Tuesday, but it may be Wednesday before we get the results. If the monitor shows that the nature of the PVCs has improved then we get to take her home SURGERY FREE Tuesday or Wednesday!!

So good to finally feel a bit of relief, see a smile on our girl's face, and to see the light at the end of the tunnel!

Friends Make The Days Go Faster!

Nothing like a game of Hungry Hippo with Dean on Wednesday!

Thursday afternoon, Liz and Luke tagged along with Devin... and the Hungry Hippo competition continued!


Andrea tagging along with me on Friday was COMPLETELY unexpected! Loved the smile on Kayleigh's face as she walked in with me!!

A visit from Angie Hutchins was Saturday's surprise!

Community...

Community
com-mu-ni-ti: noun, often attributive
Definition - a unified body of individuals

For us.... it starts at the building above..... River Oaks COMMUNITY Church

One of the sweeping themes through the entire Bible is the idea of God moving people into communities where they will bless others and others will be blessed by them. We have witnessed this direction of the Lord first hand during Kayleigh's illness by the love, support, and prayers of many people! There are many people who have put parts of their lives on hold to help our family out and it brings tears to our eyes each night thinking about the blessings that have came throughout the day.

Jenni and I have been like sisters for many years, and are very close. We met when we were around 5 years old! Our families are very intertwined and we often function as one BIG family! She and Jim have added Chase and Paige to their family for much of the last 8 days while Mike and I are 2 hours away with Kayleigh. Jenni has wiped away my tears after a long drive home, listened to me rant and rave, and offered support and prayer.

Jay and Cindy Dietrich have become very dear friends of ours from church. Cindy is referred to as Kayleigh's "Mama D" and Paige just adores her "Mr. Jay". Cindy has been with Kayleigh from day 1... even beat me to the school the night it all started! These two have given Jim and Jenni a reprieve from Chase and Paige and Cindy is always on call for support for Kayleigh (and me!).

Donna and Steve Heinz have helped us to pick up the pieces with Paige on several occasions over the last month. During our church's VBS, Paige stayed with them a few nights just so that she could attend and we could focus on Kayleigh. This week, while we have been 2 hours away, then again took Paige for a night that was a bit tough on Paige and helped to wipe away the tears and pray with her.


Angie Hutchins has been a HUGE support for Kayleigh, and for Mike and I. She has really helped us understand a lot of the medical terminology, taught us how to do Kayleigh's finger sticks and injections, and has traveled to Duke Children's for appointments with us to be that second set of ears. She and her husband Todd are AMAZING people with so much love for the Lord and for others!

Chip Herrera is Kayleigh's Youth Pastor at church. He and his wife Krista are so good to her and are just wonderful people. The night that Kayleigh was EMS'd to the hospital after collapsing, Chip met us there (without telling his sleeping wife I might add!) and stayed with us until almost 2am!! He always puts his youth kids first and Kayleigh adores him!

There are many, many other people who have also been with us every step of the way. Doug Todd, Eddie and Michelle Schapira, Wendy Warden, Kim and John Riggins... just to name a few!

Mike posted a few nights ago that he was "amazed by the love and charity being poured out upon my family through these trying times.... Honestly I have no clue how to respond to it all". Other than saying Thank You.... I have to agree with him! The community that has surrounded us, this huge body of individuals that have supported us these last few weeks is unlike anything we've ever experienced.

We have been blessed!

Santa Claus Came Today!!!

The highlight of our day was Santa Claus! Yep... he was HERE! The look on Mike's face when Santa walked in the room was priceless and Kayleigh smiled great big! She even got a nailpolish gift set!

Other highlights of the day...

- She was deprived from breakfast and lunch for a radiology test that involved eating egg whites with a radioactive nuclear material in them... they ended up being FLORESCENT YELLOW! But she choked them down like a champ!

- Dr. Kanter came and talked to us about the findings of her halter that they did over the weekend. She had over 900 PVCs on it that were multifocal, some came two at at time, and some even came 3 at a time. He said he couldn't explain it, but wasn't comfortable with it and is looking into a few things.

- We started working very closely with nutrition to work on strategies to help her with meals. Through all of this she just hasn't been eating well. It was very comforting tonight to hear the doctor look at her and say "you do not meet the criteria for anorexia". I wanted to look at her and say duhhhhhhhhhhhh!!!!!!!!! Think of it like a cancer patients... they just don't feel like eating so they have to come up with strategies to help out a bit.

- She had a special EKG done called a Signal Averaged Electrocardiogram that apparently gets down to the nitty gritty of each heartbeat.

- She also had a special echocardiogram done with some extra measurements. It was so cool to watch!!! Don't have a clue what all I was looking at.... but it was really neat to see the heart pumping and blood swooshing around!

- And did I mention Santa came?

We hope to get some results back tomorrow. Still talking about her being here several days. She's a bit sad about it, but is doing ok!

Now... time to finish our mother/daughter mani/pedi!

Today Was a Good Day

Dr. Kanter is back!!!! Yeahhhh!!!!

He came in early this morning to see Kayleigh and started by saying that he has put the pacemaker on the back burner. He wants to get a good idea of the whole picture before he does it because he isn't convinced that it would fix all of what's going on now in light of recent overall decline. So - today we started with another stress test, tomorrow she'll have an echocardiogram done as well as another test down in radiology that will take a few hours. After he gets those results he will decide what's next.

Overall, today was a good day. She was feeling a little better, less nauseous, ate a little more, AND she had all of her family in the same place! My good friends, Donna and Cindy, brought Chase and Paige up for a visit, and Devin came a little later. It was so good to see them all together again! Can't wait for it to be back to "normal" around here!

We continue to have our socks blessed off by all the people around us!!

Thank you all!!!

Update on Kayleigh

(A little note left on her wipe off board from big brother Devin!)

Today was a hard day for Kayleigh. She really wasn't feeling well and I think she is starting to feel a little down. I would too... tomorrow marks 1 week that she's been in a hospital!

Kayleigh did have a few surprise visitors that got a few little smiles out of her (thank you Michelle & Eddie Schapira, and big brother Devin!), but she didn't really interact a whole lot with anyone. Then around 5pm the "witching hour" started and she went into another long run of irregular heart rhythms that set all the monitors off like crazy. When they finally ended she was left with a bad headache and dizzy spells.

Tomorrow HER primary EP Cardiologist will be back and will see her first thing. She is excited as she knows that he knows her best and will be able to give us a good game plan on how to help her feel better... whatever it takes!!

Know that she is getting GREAT care here at Duke Children's!! All of the doctors, nurses, and support staff have been fantastic and very very attentive to her needs.

We continue to be grateful for the outpouring of love from our friends at River Oaks who have stepped in to take care of Chase and Paige, to love on and pray over Devin, planning meals for when we get home, and all those who have continuously prayed for our sweet girl; and our family! We simply can not come up with the words to express how we feel to each and every one of you!!!

Much love to all!

Peace...

Psalm 29:11 "The Lord Blesses His people with peace...."

After a crazy night of PVCs, trigeminy, bigeminy, pauses, and whatever else the crazed out monitor was mad about... she Kayleigh is finally resting... peacefully!

Watching Prayer at Work...

(Sent to me by Chip Herrera - River Oaks Community Church's youth group on a mission trip gathered to pray for Kayleigh Monday night as she was admitted to the hospital)

This week has certainly been full of ups and downs. It isn't anything I ever thought we would be going through with our own child, and I feel so helpless as a Mom when Kayleigh is having one of her spells. All I can do is sit and watch and keep her calm.... and ask for prayer.

When we left Baptist on Wednesday I was very uneasy about taking her home. Something deep down just didn't feel right, but we decided to give it a try. I prayed that night that when I woke up Thursday morning I would feel peace and and direction. Later that night, Mike posted on his facebook wall:

"So, I stepped away on my own and gave Him a piece of my mind about Kayleigh's situation. I'm pretty sure that He chewed it up and returned it based on the headache that I currently have. Just as I was starting to think about how all of my focus has been on Kayleigh and work lately and relatively none on Him, this song popped up on Pandora...Yep, I need to step back and find my center again."

Yep... Even Mike has turned to having conversations with Him about all Kayleigh has been through!

I woke up Thursday and the thought immediately crossed my mind to call and get her seen by her doctors here at Duke - so I called the clinic to get her an appointment. The receptionist said she'd call me back (this was at 8:30am). We waited and waited and waited and waited some more! While I was waiting they pulled her Baptist Hospital records... then we got the call.... "bring her to admissions in the main hospital, we are getting a bed ready for her now".

I prayed along the trip that Kayleigh would be fine until we got here so that she wasn't miserable in the car. Well, we were here just a short time when Kayleigh went into a long run of PVCs. So glad we were here where she felt safe and could stay calm through them!

Yesterday afternoon was a great day for her... she smiled a lot and even had a solid run of heart rates in the 60's! We were thrilled.... then it happened! At first it started easy and she was handling the PVCs well, but the longer they went the worse they got and by the end it was the worst run she's had. On the monitor you can tell how strong they are by a number that goes from 0 (no PVCs) on up... she was in the high 20's and low 30's. When they start to go away the number slowly drops.

About 45 minutes in I sent a prayer request out to please pray they would stop and she would feel relief. Within 5 minutes of that prayer request she went from PVCs with a high intensity to ZERO! No tapering... straight to ZERO! Talk about the power of prayer!!!

We hate watching Kayleigh go from sitting up and smiling feeling great, to crashing hard. It's the hardest thing we've done as parents and we feel pretty helpless. But it's clear that if we pray and ask for prayer that He shows us He is right here guiding the way for His will to be done because He's just cool like that!

Thank you ALL for your love, support, and ongoing prayer!

Happy 62nd Birthday, Mom!

My Mother was born, a beautiful baby girl who grew up to be a beautiful woman both inside and out. I miss her so very much. I still remember every detail of her face, how she smiled as she baked in the kitchen, and watching her give her grandkids the biggest hugs ever!

While she's not here with us, we know she is celebrating her special day with the angels in Heaven.

Much love to you, Mom!

Happy Birthday!

Finally! Some Answers!!!

What a day it has been! Mike, Kayleigh, and I got up at 5:30am and were out the door by 6:30am to head to Duke Medical Center for Kayleigh's Autonomic Studies to determine what's going on. We were checked in by 9am and at 10am they got her prepped. Not one... but TWO tables of needles, IV tubing, meds, and more for 1 young girl! Yikes!


She had to have an arterial line placed as well as a traditional IV. The arterial line goes directly into a major artery into the wrist and is quite painful to place and tolerate! She handled it like a champ though! Squished up her nose just a little with the numbing medicine, but that was it! I think the IV was a bit harder on her (it's up a little higher in the same arm!) because her vein started spasming on the nurse as she was placing it! In addition, she had a constant EKG going and a Transcranial Doppler done.

Then we waited... a LONG time! They needed her to become calm and peaceful so that they would know and capture her "normal". She didn't want a picture of her face... but I did get her beautiful feet!


Not having Justin with her was hard this go around! But... she listened to a video of him singing and playing the guitar and that made her smile!


Then it was time to start..... I still don't know exactly what all was done in the testing. But - I do know the results.

The first thing they told us was "after about 23 minutes she went asystolic....". My mouth hit the floor! Mike looked a bit clueless so Dr. Kanter then said "she had no pulse or blood pressure.... she was clinically dead." Thankfully, it was a very controlled environment and Dr. Kanter and his nurse Angela are FANTASTIC and were right on the ball!

When her heart started beating again though, it was way out of rhythm and took a long time and some meds to get it recovered. (If you look at the picture of the EKG strip below you can see how right before she flat lined her beat was very slow, then afterwards it was VERY erratic!)


So - what does all this mean?

Well... she has 2 different things going on, and possibly even 3.

First - She without a doubt has Neurocardiogenic Syndrome. In simple terms, this means that her autonomic nervous system is hyperactive and causing an extreme response to certain stimuli.

Second - She "more than likely" has a form of Dysautonomia called Postural Orthostatic Tachycardia Syndrome. The labs he drew during the test today will give us the 100% definitive on this, but he said he expects them to come back positive based on what he saw. The only thing that he saw that indicates it may NOT be POTS is that her heart rate only went up to about 90-100 beats per minute. With POTS it typically goes up to 120+ when the body is standing.

Third - She may very well have a problem with her SA node in her heart. This is evidenced by the arrythmia she was in from the second they hooked her up to the monitors as well as the difficulty her heart had recovering from her asystolic episode. The SA node should have put her heart right back into rhythm and it needed some help.

Where do we go from here?

She has been taking Midodrine and Florinef. She is to continue those medications just as she has been taking them for the NCS and POTS. She was started on these meds a while ago with suspicion that she had some form of Dysautonomia.

They've also added something called Scopalamine. It will help calm her nausea and hopefully allow her to eat, and it can increase her heart beat and help settle her system down.

He wants to put her on a beta blocker, but can't do it at this time. If she has no improvement in 2 weeks he has another drug he can try before we start giving serious consideration to a pacemaker. It is looking like this is going to be part of her future, but he wants to be certain that he's tried every other avenue first and we are ok with that.

She is home tonight and feeling very blah, weak, and tired. But she is stable and doing ok. Mike and I are doing ok too... just very tired!

Thank you for your prayers, love, support, meals, phone calls, emails, texts, and more! We are so very blessed with a community of friends and family who love our girl so very much!!!