Wednesday, September 20, 2017

The Drunken Duck

Man! What a day! It started out with my run in with a drunken duck.

Driving down the highway, minding my own business, cruising at about 80 mph and a duck just decides to come in for a landing and sit dead center in my lane of traffic! He seemed drunk on pond scum and sweet bread crumbs.  He was completely disoriented as to where he had just landed. He must have thought he had just landed in a some asphalt duck sanctuary.

Oh man, what a dilemma.  I had a semi on one side of me, the ditch on the other and a confused, sitting duck in front of me.  He had a chance at life if he had of stayed sitting but instead, he decided to stand up at the last minute and stretch his neck, just as my bumper kissed his beak.  It was a disturbing moment.  I'm afraid my car will be choking out feathers for the next few days. Sheesh... I had one nerve left.

Anyways, we arrived safely and just in time for Sam's appointment.  They confirmed that Sam is experiencing an abnormal rhythm since surgery.  They aren't sure at this point what is happening.  They downloaded the last week from his pacemaker and it is functioning normally, the wires are settling in nicely and scarring into his heart.  They removed his stitches and everything looks good that way but his heart rate is a bit of a mess.  They made some adjustments to his pacing and we will wait and see what happens next.  The nurse agreed that maybe it's just his body trying to adjust to this new pace, maybe his nervous system not taking kindly to things...It could be the other issues with heart that are now more prominent.

Downloading the pacemaker

My Happy Camper while he gets his pacemaker 'interrogated'

Who knows?  The bottom line is, we have to continue to have faith that things will work out and either things will resolve or they will figure out what the exact problem is.  It's a little frustrating but getting upset won't do much at this point.  Sam is just happy he gets to shower tomorrow and he only has to keep his arm restrained to his body at night when he sleeps and at work.

We didn't get out of the hospital until 5:30 and since we hadn't eaten all day, we stopped for pizza at our favorite downtown place, Maxwell's.  We got there just as a live jazz band was performing.  It was Heavenly to sink down in the booth and numb out to the smooth sounds of a the sax.  Beside us was an elderly man who had escaped the nursing home...just kidding.  He did have a name tag on that claimed him to a nursing home but he was enjoying some pizza and jazz.

My new friend the Music Man

I have this way with elderly men who have no teeth.  I'm not sure what it is, but he struck up a conversation.  He was an interesting guy; a classical pianist and also played the organ and trombone. People call him the Music Man.  He kept doing donuts with his scooter between our tables.  It was some awesome comic relief.  I was telling him that I played the piano, organ, flute and one summer of the saxophone.  I had him at 'organ'...ha ha.

He said,  "Do you play the organ at church?"
"Yes."  I replied.
"What are you? Protestant, Catholic, LDS?" He asked.
"Ya know what?  I was born Protestant and stayed that way for 33 years until the Mormons got me with their truth and I've been a Mormon ever since!"  And then he picked up his frosty mug of BEER and said, "Cheers!"  Ha ha...I needed a good laugh.   Nice guy.

Well, what a longish day but never a dull moment.  I'm still traumatized about that darn duck!


Tuesday, September 19, 2017


In my scripture study this morning, I was struck by the word Steadiness.  

That word has so many meanings as I thought about it more...


  • Stable
  • Even
  • Constant
  • Resolute
  • Unwavering
  • Undeviating
Wouldn't life be so much better if we could master the art of steadiness?  

It's a word that I can relate to this week. Those steady, 60 bpm I loved last week have not been seen again in Sam.  His heartbeat is all over the place and continues to get higher and higher with the least amount of exertion.  We are seeing up to 160 bpm now in just a slow walk from the living room to the kitchen and in my shoe box of a house, that's about 10 steps.  

I like to imagine myself never wavering in my thoughts and actions, especially when the kids get sick, when we prepare for more hard things, when we face things I can't imagine ever recovering from.   It's almost a distant dream to ever think that I could face a trial and be steady, stable, constant, resolute, unwavering and undeviating from knowing that my Father in Heaven will take care of it all, no matter what, regardless of the outcome.  He proves Himself to be steady and unwavering time and time again; I've learned this over the last half a century.  Yet, I have not quite settled in this steady space.  I continually doubt, question and waver. 

Today, I had to go through the checklist for Shelbie's testing on Friday.  I completely forgot, from 6 months ago when our Neurology team set up the appointment, that she has go off all of her medications this week.  That is going to be a problem.  A few of them make her so sick if she misses a dose.  They are aware of this but it's the way it has to be.  When anything gets off with her system, she ends up with more seizures and more sickness that will take us another month to recover from.

Again, I never realized until this year, just how important it is to keep the kids' in optimum health, any deviation creates an unstable situation.

I think the weight of this word today was meant to be.  I am dreading Friday even more than I dreaded the pacemaker.   I already have a bad feeling that the complexity and difficulty of this test that Shelbie has to go through could send her into seizures or stop her heart altogether, just like it did to Spencer last October.  I am having some serious PTSD over this but my goal is to be steady, in all things.  (Note to self...when they ask me to wait in the waiting room, I'm going to stay put this time, instead of insisting I go in to watch.  That was a big mistake and had I known Spencer was literally going to die in front of me, I would have chosen not to watch that.  Oh well, live and learn. )

I know, it's a big jump for me to be steady as opposed to wavering from one minute to the next but I'm going to give it the ol' college try!  What do I have to lose?


Monday, September 18, 2017

It's hard to be normal

It's hard to believe that Sam is one week post surgery.  Today, he went back to work but with limited activity and by limited I mean, all he can do is walk around, nothing else.  He gets to shower on Thursday so he is looking forward to that.

I feel like I'm walking around in a fog.  I mostly feel numb after an emotional weekend.  Spencer has been sick since Saturday and that has been frustrating.  Despite the busy, tiring, emotional weekend, I have been able to solidify a few thoughts.

We were very ill-prepared for this life changing event.  We accepted it, I did minimal research and we just powered through, never stopping to consider the difficulties that we face now.  I see that more and more as I witness the response people have to Sam when they see him.

On Saturday, when we were at Walmart, we stood at the front of the store waiting for a friend we had taken with us.  We ran into my cousin who got a pacemaker about 3 years ago.  I asked him how he managed right after and he manages now.  I asked how low is heart rate was before getting a pacemaker and how high it is now.   He said it changed his life.  He felt almost immediately better...he is 85 years old.

After he left, I said, "See Sam, there's hope.  Things will get better."
"Do you know how that makes me feel when you ask 85 year old men how they feel with a pacemaker and think I should feel the same way?"

Nope...that hadn't really occurred to me but I was glad he pointed that out.  It's true, there's a big difference between a 19 year old and an 85 year old.

I wasn't really saying the right thing on Saturday because then I said, "I bet you didn't realize that normal could be so hard."  Meaning...having a normal heart rate has been a challenge but his response was...quite loud!

"What is normal about this?  I'm not normal.  I will never be normal.  Name one other 19 year old you know walking around with a computerized heart!"

I was actually so happy to hear him put actual words to his thoughts.  I almost started cheering but instead I just kept at him til he got a lot of frustration out.  Since Saturday, he's been doing better emotionally but man, his heart is doing weird stuff and I don't get it.

He is still experiencing skipped beats.  He literally takes 15 steps and his heart is racing at 150 bpm...that number just keeps rising each day.  Nothing is really getting better in fact, he would say he feels worse than ever.

All of my kids have a condition called Dysautonomia.  The autonomic system is faulty in them and that plays a part in regulating a heart rhythm.  They also have some clinical symptoms of mitochondrial disease so I'm worried that these issues are relating to their nervous system freaking out with doubled heart rate and the fact they already have energy issues in the cells of their muscles.  I don't really know...just hypothesizing at this point.

We head back to Utah on Wednesday to get stitches out and wound care and I'm hoping they will talk us through what is happening but I have a feeling we will only see a nurse this time.  We will come home late that night and turn around Thursday to get Shelbie down for her little stay the rest of the week...It's a long story why we can't stay down there but on the bright side, I will save money on hotel fees!

We are trying so hard to just be normal about this.  To not let it disrupt our lives too much but it's honestly hard.  There are times I wonder if we did the right thing.  I guess that question only leads to dead ends at this point.  I know we would have watched his heart completely die so we really had no choice but still...I wonder.  I think maybe the hardest thing is just knowing that this is never coming out of his chest for as long as he lives.  From here on out, he relies on a computer to keep him going.

I know there are so many more feelings yet to surface from both of us.  As soon as I feel things coming up, I'm quick to shut it down.  I'm just not ready for a meltdown yet.


Sunday, September 17, 2017

The Heart of a Mother

I hit the ground running when I got home from Salt Lake.  I had back to back meetings from Thursday night until Friday afternoon.  Sandwiched in between appointments, I said goodbye to Spencer for his official move to Utah.

At noon, I ran through the front door and he was standing there, anxiously patient for me to arrive so he could take off.

Without a breath, I ran through the rapid fire questions.

"Spence, did you pack your medications?"
"Did you get the comforter?"
"Did you grab your 72 hour kit and 1 month food storage bin?"

"Yeah, I got everything.  It's all there.  I gotta go." was his reply.

What kind of a mom would I be if I just waved him off with a simple goodbye? Certainly not a smother mother, the kind he has come to love and adore!

"Okay but first...I need to tell you 4 things...Pay attention...

Spencer, stay away from the world.  You have no business being in the world, that's not where you belong.  Second, don't let your heart fail you.  Stay true to what you know and believe.  Truth is hard to find, don't fall for a shallow view of eternity. Third, never take credit for your success and learn from your failures.  When you are praised for a job well done, consider it a blessing and give thanks.  Always give thanks's the law of abundance. When things fall flat, get up and consider what you can learn from the experience.  Failures are really just an opportunity to try something else. Don't worry about it. Fourth, do something nice for someone no matter what, every day.   There is always something you can do, do it."

With that, we said a prayer and he was gone.  The back seat was stuffed with this and that but buckled in safely beside him, was the dream he has kept alive all year long, through the shadows of a very tough year.

I'm proud of him.  I didn't always see his vision while being at home but I always believed in him.  He has taught himself a valuable skill.  Every day for over a year, my living room has been his classroom.  With determination, he sat there every day with a strict schedule and written goals of what he wanted to learn and he did that, never letting himself get distracted.

We miss him so much.  The heart of a mom is continually bursting open in love that is much bigger than any heart can contain.  With all that love comes a trophy of tears, that we shake high above our heads, a bittersweet celebration, proof that we loved; something to show for the hardest job we have done on earth.

Friday, September 15, 2017

Heart and Home

Sam and I made it home last night.  It was a long, quiet, upsetting ride but when we walked in the door, the kitchen was full of friends cheering for Sam!  It was the greatest thing ever!  It made all the difference to Sam and he felt so loved and watched over. It was Spencer and Shelbie's idea!

They made him this great poster.

They kept him busy all evening so I could go to work.  When I got home, his spirit was definitely lighter and he seemed okay so I went to bed.  At about 2 am, he woke me up and was not okay.  He is having some issues with post trauma I think.  He has not been able to sleep well all week long.

Today has been a little more of the same, just trying to adjust to our new life.  He is still having lots of breathing issues and he is still having palpitations.  He has been asking me to take his vitals every few hours because he is worried something is going wrong.  I gladly check it and each time it's dead on 60 with exception...

He is so winded.  Worse than before getting a pacemaker.  He can't get anywhere without being completely out of breath, even trying to get something from the lower cabinets in the kitchen.  We did a little test earlier today...I put the pulse ox on him and tested his oxygen and resting heart rate then he kept it one while he walked upstairs to his bedroom.  His heart rate shot up to 107 bpm.  He wasn't running or anything, just a slow walk that took less than 10 seconds.  His oxygen dipped from 98 to 92 and his heart rate didn't settle back down for several minutes.

Then, I did the very same thing.  My heart rate went from 68 to 81 and within 2 seconds, it was back down to 68. So, something is not right but maybe it's just his heart and autonomic nervous system trying to figure this new pace out.  There has to be some sort of adjustment period so I'm trying to be patient and let things heal before I get all excited about it.  I do worry that the pacemaker only fixed one issue, as we had discussed, it may not be covering all the other abnormalities.  I kind of cringed when I heard Sam say...I don't want this if I'm going to feel like this!

Last night, we got his monitor set up in his room.  He has a dedicated cell phone and a home base.  This connects to his pacemaker and information is relayed through the cell phone.  It took some patience getting that set up, it kept showing that something was wrong the pacemaker and I was trying to read instructions, while staying calm and that was tricky!  We finally got the green light and a steady beating heart so that was a relief.  We just have to remain confident that we have done all we can do and the rest is basically up to God.  I know that I will know or Sam will know when waiting and watching is not enough but for now, we are proceeding with caution and trying not to worry.
I got to read the owner's manual in the hospital.  I think this box should have come with a live human being to come home with me for back-up. 

As long as we have a small green light and a steady, white heart, Sam's alive.  Anything else and there's a problem.  He will be monitored everyday, all day for the rest of his life.  I know it shouldn't matter but the bill coming my way has me all freaked out! 

Easier said than done...

How am I...You ask?  This pretty much sums up my state of mind right now.

Like walking into the spider web of life...a little frazzled.   Seriously!

On Wednesday afternoon, as we started pulling out of my stall in the hospital parking garage, a moth flew out from somewhere on the driver's side and...well, let's just say it wasn't a pretty sight.  I freaked out!  You would have thought I was running from a terrorist.  I almost scraped along the concrete wall beside me as I forgot what I was doing- driving! I had a chunk of hair stuck in my mouth, my sweater was caught up on the back of seat, I bruised my arm trying to claw my way out the air conditioning vents... Sam just looked at me in unbelief.  When the moth finally left through Sam's open window, he just looked at me stone faced and said, "You alright?" and then rolled his eyes! Ha ha.  I just cleared my throat and proceeded as if nothing had happened, nonchalantly  putting my hair back in place.

Thursday morning I let him sleep until we had to check out.  I woke him up, helped him get dressed (He can't get his shirts on without help) and then I packed stuff down to the car and returned the room keys.  When I left the room, he was sitting on the bed, ready to go.  When I got back to the room, he didn't answer the door.  I knocked, then pounded, then called through the door.  It was the 'spider web' scenario all over again...I grabbed the nearest housekeeper and begged her to open the door for me.  I was trying to explain to her that my son had just had heart surgery and he wasn't answering the door.  She just kept nodding her head 'no'.  I kept saying 'YES!' , 'Please!  Something is wrong!  He was waiting for me to come right back.'  We clearly didn't speak the same language of panic.  She finally unlocked it but was clearly angry...I did interpret that correctly!

Sam was not in the room but the bathroom door was closed.  I pounded on the bathroom door, no answer so by now, I'm singing in my head..."Another one bites the dust...Another one bites the dust..." just getting into my rhythm for starting CPR and I'm covered in those darn 'spider webs', completely irrational! Now it's funny, then, it was not.  Turns out he was just using the bathroom.  I just couldn't hear him answering me, probably because Crazy Kat had made an appearance.

Anyways, I have a bunch of stories like this...just irrational, crazy stuff.  We are little on edge I guess. It's just a bit harder than I thought it was going to be but we're home for a few days til the fun winds up again next week.  


Wednesday, September 13, 2017

The Bulldozer

Last night, just after midnight, I sunk into the vinyl recliner that sat by the large window in Sam's 4th floor hospital room.  Sam had finally closed his eyes.  I put my head back and thought about maybe falling asleep but then a wave of anxiety hit and there was no way I was going to find my way behind the fallen lids of my eyes.

So...I slipped on my sandals and headed to the stairs.  I ran all the way down the stairs, to the lowest parking level at the hospital and then I turned around and ran all the way up to the highest level of the hospital.  I did that twice...just running as fast as I could, away from all the emotions that threatened to overtake me.

When I got back to Sam's room, I stood by the window that overlooked the valley and watched the airplanes lined up to land at the airport.  They hung in the sky like paper lanterns that had forgotten where they were going.  I thought that it was a perfect analogy for how I was feeling last night.

The natural thing to feel for any normal family is..."Whew, well, that's behind us." And that would be followed by a sigh of relief and something normal would ensue.  I almost dared to think that but just like those airplanes, I can see the next big thing lined up behind us, dangling at the end of my long shadow.  There is no sigh of relief, no moving on to normal, no break, no chance to regroup.  It feels so exhausting and overwhelming.  There is no time for me to sit and just be.  Like a bulldozer that slams into concrete pylons like a hot knife in butter, I keep plowing on; on to the next thing needing to be attended to.

It hit me again, when pharmacy came in to explain meds and then 10 minutes later when the tech from Boston Scientific came in to 'Interrogate' his device.  With Sam laying on his back, they turned his heart rate clear over 100 bpm and then back down to 50 and dialed it back to 60.  Sam felt his heart racing, being remotely controlled by a man sitting beside his bed at a computer and then it seemed so surreal.  This surely can't be real...and then a woman came to run through the list of all the things Sam can and can't do for the next week, the next 4 weeks, the next rest of his life.  In between all the people coming and going with more instructions, more appointments, more requirements for our new life with a pacemaker, we both fell asleep and it felt like I had slept a month's worth in just 10 minutes.

Sam was discharged early afternoon but we are staying at a hotel so he can get on top of the pain for one more day before we travel home.  We had to make a stop for some things we needed and the guys at the register saw that Sam's left arm is tied to his body.
They said, "Dude!  Did you break your wrist or something?"
"Ahh, no. I...ahh...well...I just had surgery and can't move my arm."
"No way! Surgery on your wrist?"
"Ahh, no.  I had a pacemaker implanted in my heart."

Both young men stood still with a slacken jaw.  "What? Dude! No way! *cuss word* Man!  How old are you?"   At this point, somewhere between the 'Dude' and the cuss word, I wanted to scream and cry.
Sam replied, "19.  I'm 19"
"Oh shoot man.  So sorry.  That sucks but I guess you get to live.  That's something."

This is why I hate leaving my house.  I never realize, from day to day how rough things are until we become this staggering story of unbelief.  It's really hard to find my way back to denial after days like today.  These are the things that stop my bulldozing methods in their tracks.

Anyways, it was just a hard day.  They were already talking about the next surgery when his battery needs to be replaced.  We have to come back next week for a device check, get the stitches out and wound care.   Sam has been having some bad issues with controlling his body temperature.  He spent all of the night, last night, in a major sweat.  The nurse was doing everything she could to cool him down.  We had the air conditioning in his room cranked, ice packs, cool facecloths and he was so uncomfortable.  Today, it's been extreme hot then really cold.  I'm wondering if his autonomic nervous system is a little shocked at the new, increased heart rate?  The doctor wasn't exactly sure what to think, it's not a typical side effect.

Sam has also been having a hard time getting deep breaths and is having heart palpitations.  I'm trying to take this in stride.  I'm trying to be patient and let him get a couple more days of recovery behind him before I get too anxious.  Tomorrow, we will get the receiver set up at home and then they will be monitoring his heart 24/7 for forever!  That's a long time. We've been told that his heart is going to be going through lots of changes and scarring the wires into the tissue of his heart so I'm sure strange things are to be expected.

He's been a little depressed and I hate to say it but...cranky today.  He has been snapping at me and really short on patience.  I know he has been through so much.  When we were eating dinner, I said, "Sam, you can't just not talk.  I want to know how you are feeling about things.  You need to process this."
"I have processed this." He said
"I don't believe you." I told him
"I just can't.  I don't want to do this."
"I know but this is not going away.  It's not like your heart is going to mend and they will take it out in 6 weeks and you'll be good as new.  Your heart is being kept alive by a computer, for the rest of your life...and before the end of the year is up or early next year, you will be receiving someone else's donated plasma to keep your immune system going too.  We have to talk about this."
"I know but I can't.  Not yet." He said

We have some rough days ahead.  He can't even shower for one more week.  He can't bend his legs, lift any weight or exert himself in any way for another week.  After that, he can shower but no swimming, soaking etc.  He can't use his left arm for 4 weeks.  At night, he has to have his arm tied to the side of his body or the side of the bed.


Tuesday, September 12, 2017

Keeping The Pace

What a longish day!
Prior to surgery

Off he goes

One of the bravest boys I know!  I couldn't do this knowing I would be awake! 

This is a picture of Sam's defective heart and where they were going to place both wires and where they would access the the two lower chamber and the junction in the middle where more issues reside

Post Op

Lucky number 60! More than doubling Sam's heart rate! 

One overwhelmed young man but better than ever.

A view from Sam's room.  

Everything with Sam's pace maker surgery has gone so well; such a tender mercy.  Our doctor has been so amazing and his Fellow was outstanding.  They spent so much time prior to surgery educating Sam on what is wrong with his heart.  They even drew pictures to help him understand the procedure.

When we first checked in, the nurse told me that Sam would in fact be under general anesthesia and I felt like a ton of worry had been lifted.  All along,we were told he would be awake.  That amazing feeling lasted all of 20 minutes until Anesthesia came in and said, "No, I don't feel comfortable putting him under with the condition of his lungs and liver." awake he was.  We came here just planning on a one lead, pace maker.  They determined that because of his Right bundle branch block they needed to place two wires in his heart.  One in the upper right atrium and one in the lower right atrium.  They also needed to access the lower parts of his heart so they decided to do a second procedure and enter the main artery in his groin.  That procedure was to get a better look at why his heart isn't initiating a beat and to get more precise measurements of each ventricle, left chamber, junction nodes etc.

I am so thankful that they didn't run into any complications with his Arterior Venous Malformations or lung problems. No excessive bleeding. His biggest risk at this point is infection.  He has no immunity left so they are running IV antibiotics tonight and tomorrow.  As soon as he gets healed from this, we will head back to Immunology and start on immune therapy to keep him in better health this winter...finger's crossed.

When they brought Sam back to the recovery room and hooked him back up to all his heart leads the monitor started beeping out a rhythm that sounded so beautiful and glorious!  A beat of 60.  Steady, solid 60 beats per minute!  I couldn't even speak!

 I just started crying. The doctor looked at me funny and I said, "It's such a steady, amazing number!  My new favorite number!"  He smiled and said, "Yes!  It is.  I guess that you have missed that."  I had never thought how much I have missed seeing that number on any of my kids.  It's the number of life!  It's the number that infused me with hope!

Sam has been on strict bed rest all afternoon.  He has had to have his arm restrained because he can't move it above his elbow for 4-8 weeks.  They also needed him flat until the aorta at the groin site had clotted off and was not bleeding.  That took all afternoon.  He's been up walking and guess what...not dizzy!  Not out of breath!  Not like an 80 year old!  He's my boy, my 19 year old boy with a normal, albeit computerized heartbeat.

I am amazed!  I am amazed at God and I am amazed at science and I am amazed at our skilled team of cardiologists.  We are truly blessed.

Sam had a panic attack during the procedure and hyperventilated so that was traumatic for him.  When I kissed him in the recovery room, I said, "Listen to your heartbeat Sam!  It's normal!" He got pretty emotional and his lips quivered but he was afraid to cry, just looked shocked and panicked.  He is overwhelmed.  It's been a rough day with everything he has witnessed and been through but we will work through that.

Thank you for so many prayers and warm thoughts. Sam was able to have a blessing from his brother and cousin before we left and all of his friends serving missions have been praying for him and that has given him so much strength to feel all that love.  He has the greatest friends!

Tomorrow, I will be trained on how to care for things from here on out.  I'm still so overwhelmed with what we have been through this year and what lies ahead even still.  I mostly did okay today but it took every ounce of energy I had to stay in a place of hope and faith.  At 11:33 this morning, I was hit with the most profound feeling that I needed to pray at that very minute.  At the same time, I felt extremely panicked but I sat on the edge of my chair and prayed, then waited for a nurse to come through the door to tell me Sam had coded...they didn't and I'm sure now that he didn't and since I was crying when the doctor came out to let me know things went well, I forgot to ask what happened at 11:33.

Whatever that was's okay now.  I know we will have some trauma to work through once the adrenaline tide retreats and all of this will hit me hard, like it always does when the dust settles.

PS...It's been really hard to find any literature on heart conditions and Dyskeratosis Congenita.  All the doctors say...Nope...not related.  In my mom of mom hearts, I am having a hard time jumping on their bandwagon.  I was shocked to pieces when the Fellow came in and told me that he did find one Journal article describing a similar situation in a DC patient.  I asked him to print me a copy and I hope he remembers.  I also asked if he and Dr. F would be willing to write up a case article on Sam so that it is at least documented that a boy with DC has conduction heart issues as well as cardiac AVM's.

It was priceless to see the look on the Fellow's face when I asked him for that favor!  It was like a kid at Christmastime.  I love having doctors who love the opportunity to learn.  He was almost giddy which made me smile.  I actually wanted to hug him but refrained...Ha ha.