My life has definitely had some unexpected twist and turns and from August of 2010 to present day has probably been the most surprising of all.
It all started last year.
I was working in a veterinary emergency room on the midnight shift. I started to notice in the hectic chaos of the ER I was having trouble physically keeping up. My heart started to do weird things and was beating more like a bad techno remix than a regular heartbeat. It would speed up, slow down, stop, skip and feel like a butterfly flapping its wings and doing somersaults like it was on the olympic gymnastics team. Yeah, I have always had palpitations (skipped beats or funny rhythm from time to time) but nothing that seemed to happen everyday, several times a day and for long periods of time. So… I did what I didn’t want to do and made an appointment to see my cardiologist.
It took several appointments, echocradiograms (ultrasounds), and 24 hour ECG monitors for them to tell me I was definitely having rhythm issues. They clocked my heart going 180 beats a minute at times. They wanted to place me on Cumadin and advised me that I should not be working. I escaped the Cumadin only slightly, I had to alternatively agree that I would start taking 325mg of Aspirin each day instead of the 81mg Aspirin that I had been taking to avoid possible blood clots. Awesome, preventative headache therapy each morning!
After going home and breaking the news to my husband, who rightfully so is now my ex husband, he informed me that if I couldn’t work I needed to go live with my mother or someone who could take care of me because he couldn’t afford to “keep” me. I called my mom and told her I had to come stay with her and moved to Arizona after surviving the beating of my life from a six-foot, 210 pound man (versus me that’s a giant lol). Don’t worry, I called the cops and he was arrested the next morning. I mean, it was either that or my brothers mob of friends and since I love my brother and wanted to keep him out of trouble I handled it the legal way. I have not seen him since that night and don’t care if he falls off the face of the earth. I arrived in Arizona the morning after the domestic brawl and have been living with my mother ever since.
Moving to a new state meant finding a new doctor. I was set up with the Adult Congenital Heart Center at St. Joseph’s Hospital in Phoenix. I began seeing Dr. Ed Rhee, or as I like to call him Dr. Smiley. It doesn’t matter what the man is going to tell you, good or bad, he is always smiling. I often wonder how he does that.
After being evaluated with several home monitoring devices Dr. Rhee explained that what was happening to me was not uncommon in patients that have undergone the old style Fontan. There is no way that back in the 80’s they could have known the Fontan they preformed on me would become outdated and that there would be complications as patients got older. It’s all part of being what I like to call the “Crash Test Dummy Generation” of congenital heart patients. Think of your heart as a giant circuit board. Now what would happen if you place a paperclip in the middle of it? It would conduct the electricity right? RIGHT! So what was happening to me is when I had my second surgery they used metal stitches in my actual heart muscle. Over time my right atrium (upper chamber) became the size of a grapefruit and cause the electricity to get “stuck”. The current was flowing around the stitches which was causing all the crazy beats.
Old Style/Classic Fontan anatomy after surgery: 
Tachycardia around suture lines:
My frame of mind? How do we fix this? Is it fixable? Medicine? Surgery? Well, kind of. Dr. Rhee told me that if it didn’t interrupt my life I could live with it but obviously it was interrupting my life. Next step? I was offered to undergo a procedure called a cardiac catheter ablation.
Catheter ablation is an invasive (means they mess with your insides) procedure used to remove a faulty electrical pathways that cause arrhythmias (funny heart beats) .
It involves advancing several flexible catheters into the patient’s blood vessels, usually either in the femoral vein (this is where they go in me and did it in BOTH legs), internal jugular vein, or subclavian vein. The catheters are then advanced towards the heart and high-frequency electrical impulses (microwaves!) are used to induce the arrhythmia, and then ablate (destroy/burn out) the abnormal tissue that is causing it.
Ablation Procedure:
So in December of 2010 I had an ablation. I was sure this was it and I would be good to go. I have never had any severe issues in the time between my childhood surgeries until now so I was confident this was another quick fix. When you have an ablation you get to reside in a comfy hospital suite in the children’s wing of the hospital for the night (insert sarcasm here). After the anesthesia had mostly worn off early the next morning Dr. Smiley made his appearance to tell me how it went. It is never good when your doctor’s first sentence after a procedure is “Well, there’s good news and there’s bad news.” In my head I was thinking “HUH?” “What do you mean there is bad news??” “You cannot be serious.” However it came out in the form of
ME: “O..K… what would the bad news be?”
SMILEY: “Well, the good news is that we did destroy the two spots that you had causing the tachycardia. The bad news is while we were in there we discovered a new problem that we couldn’t see on the monitors. It appears you now have chronic a-fib.”
Normal Heart Rythm vs. Atrial Fibrillation:
ME: “What does that mean??”
SMILEY: “Well, when you have a few spots that are short circuiting it’s usually an easy fix with a procedure like an ablation. A-fib means that your entire heart’s circuit board is shorting itself out all at once and since it’s so many circuits causing the problem the only way to permanently fix it is to perform open heart surgery, the Maze procedure. The Maze surgery is where they will cut your enlarged chamber to a third of the size it is now and make a lot of small cuts into the whole muscle so the electricity can’t run over each other and cause it to short out. In doing that though, we would cut out your sinus node (your heart’s brain which is how your heart knows when to beat) and you would need a permanent pacemaker.”
Extracardiac Fontan/Fontan Conversion:
ME: “So I need my chest cracked open again, cut my heart in a billion pieces and make it so a machine is making my heart beat???”
SMILEY: “No, not at the moment. Sometimes this type of thing can be controlled by medication for a few years. We will try the medicine first but the medication only usually works for a period of a year and half to two years before it is no longer effective. It’s called Multaq and we will start you on it now.”
ME: “Ok, start the meds and let’s see where we go from there.”
I was up for taking the medicine if it meant I wouldn’t have to go under the knife again… until I started reading about the medicine. Multaq is not great for the liver and has even been indicated in a few liver failure cases. I’m sorry, I have enough problems with my heart, I don’t need a liver specialist too. It is black labeled by the FDA meaning if you are not prescribe to take it, it can potentially kill you. Why? Well it changes the electrical impulses in your heart. That is dangerous. I mean, when you are getting warnings in the mail from your pharmacy on how bad this medication is something is not right. On top of that, the medication only lessened the occurrence of the episodes and did not do away with them.
After six months of Multaq and still being symptomatic I went in to the doctor again. This time I needed a stress test. If you have never had one, thank your lucky stars. My idea of a good time is not running uphill until I wanna puke and can’t breathe. I did it anyways and Smiley said that for people with my condition I did average. However, if I had a normal heart and ran the way I did I would be considered in heart failure and in need of a transplant. Which is technically what I am classified as at the moment. Heart failure. Smiley said it was time to talk about surgery.
As time goes on my condition will not improve, it will get worse. He said the surgery would be good as soon as possible because I am young and otherwise healthy and if I wait there is no telling what other complications I could run into with a surgery of this magnitude. They not only will perform the Maze procedure but will also do what is called a Fontan Revision. This means they will go and undo everything they did to me when I was two and create the same type of blood flow system in me that they give to children present day with my condition. I am currently waiting for my appointment with my surgeon so he can tell me exactly what the risks will be and what the mortality rate for me will be. On average the mortality rate is low, around 2%. I am definitely going to make sure this surgeon understands that if he is my surgeon I am a fan of breathing and would like to keep doing it for another three to six decades. I fully intend on waking up if I have to do this and he better make sure that happens. Smiley says I may never not be tired all the time or feel that, besides my rhythm issues, anything is much better but that the blood flow will be a hundred times better and that this surgery should last me another 30 years or so at least.
Thanks to the wonderful Obama administration and the crap that is our Social Security/Disability Administration I have to wait to be approved by them to get my surgery. I was denied disability on my first try and am now on the first appeal. It baffles my mind how someone with ADD or alcoholism can be approved for disability but those of us who have half a heart, diabetes, cancer or any other REAL condition have to fight until the death to get the help we desperately need. If it wasn’t for having a supportive and loving family I would be on the street living under an over pass. As soon as the government gets it through their thick heads that my prognosis will NEVER get better and they approve my disability I will be undergoing surgery number three.
Luckily: Disabled 