Can I be cured?

I had an appointment with my cardiologist/electro-physiologist today. I don't have these appointments often, thank goodness. My EP monitors my Long QT Syndrome and the defibrillator I have implanted in my chest.

I got a call, reminding me of the appointment, Sunday night. It told me it was today at 3:30. In reality it was 2 PM and by the time I got there the doctor that I was scheduled to see (whom I'd never met) wasn't there. My regular EP was though.

These appointments are all the same. They hook me up to the machines from Medtronic (the company that makes my device) and check its memory to see if I've had any shocks or weird rhythms recorded (my device fires if my heart rate exceeds 207 bpm and records anything over 187 bpm - which I frequently reach). Then they test the leads which involves using the device to pace my heart fast - which is quite uncomfortable and wierd feeling. Then I get a lollipop for being good and am sent on my way, but not until after I pay my $10 copay and $5 to get out of the parking garage.

I've been through 5 EPs in 8 years. The first two were at Beth Israel Deaconess Hospital in Boston - I really didn't like either of them. They didn't take my passing out spells seriously. The first one I saw actually told me he wouldn't be able to help unless I got copies of my parents EKGs (Long QT is genetic). At the time I wasn't talking to my parents and that was out of the question. The second one was almost as bad. One weekend I had a really bad spell that I didn't come out of too easily so Shawn took me to the BID emergency room and I was promptly admitted. The next morning that EP came into my room with some intern and simply said "Well, maybe we'll look at installing a defibrillator" and left. I loved the word 'installing', like I was the borg® or something.

The next EP I saw was at Massachusetts General Hospital in Boston. Her name was Theofanie Mela and she saved my life. She gave me my first defibrillator on August 22, 2000 - my second birthday.

That device saved my life six times in December of 2000 while I was in an aerobics class. I'll never forget that day, the ambulance ride or the confused doctors at the small town hospital I was at. The worst part of that day was about 4 hours after it happened. Shawn and I were in the emergency room and he had gone to Wendy's to get us dinner. He got me a hamburger because I was starving. I unwrapped it and while salivating brought it up to my mouth prepared to take a bite when a nurse came into our room, snatch the brown goodness from my hands, threw it in the trash and yelled at me about not eating that junk with a heart condition. This is just one of the ways people don't understand Long QT. You can't cause it by eating bad, having elevated cholesterol/blood pressure of anything like that - you inherit it.

I was sad when we moved to Rhode Island because then the trek up to Boston was too much and seeing Dr. Mela was no longer an option. I had to find someone local.

The first EP I saw was Dr. Michaud. His wife, also a doctor, got a job at the Lahey clinic in Boston so he left which I was very sad about. He was also a big savior. My first device was made by St. Jude Medical. Somehow there was a glitch in the programming of the device and it randomly reprogrammed the day before I saw him for a regular checkup. If I had gone to the gym that day I would have set off my device multiple times, called and electrical storm, which can be deadly (Electrical storm is an uncommon and dramatic but usually treatable syndrome of recurrent ventricular arrhythmias.) He recognized it, which is hard to see, and reset my device for me. My device was labeled defective and had to be replaced, which is was just two weeks later - 2 days before he left RI Hospital for Lahey. He also was able to help me get a Medtronic device rather than the St. Jude I was supposed to be replaced with - this is also my current device.

After Michaud left I got Dr. Kirk. He wasn't my first choice. But he turned out to be a good choice for me - he gave me the freedom my condition took away from me. He helped me become a skydiver (writing letters to many governing bodies) and gave me 'permission' to do whatever I wanted. Without that 'permission' I never would have realized my leg pain was actually due to compartment syndrome and not just being 'fat and lazy'. This was great for me when I was younger and wanting less structure in my treatment. Dr. Kirk didn't care what I did as long as I didn't get any shocks from my device. He even dropped my medication in half and then to nothing! I have my device - the ultimate protection!

Unfortunately 6 months after my Medtronic device was implanted that particular model was recalled. This would mean 2 devices in less than 5 years. They told me that my first device would last at least 5 years. I was not happy! Thankfully Medtronic said that if some people didn't want to have it replaced they could monitor it with a magnet, daily. I don't monitor daily anymore (its been more than 3 years) but I do monitor occasionally.

What's even better is that Medtronic has a home phone-in system so now I don't have to go into the office every 3 months. If my device wasn't on recall I wouldn't go in unless I got shocked, I go every 6 months instead.

So that brings you up to date with today's visit.

I told Dr. Kirk that we may plan to add to our family at the end of the year and that would mean I'd need a device that would last at least 2 to 2.5 years.(most devices average 5) I got good news. My device was implanted on June 13, 2003 - so about 4 years ago. As of today I have 56 months left on my battery, thats more than 4 years!! I really hope it lasts that long, the surgery is very painful as its implanted under my collarbone, in my shoulder. I have a really cool scar *wink*. I voiced my concern over having it replaced if I was pregnant and he said "oh we could do it with just local anesthetic too, like at the dentist". I wanted to laugh.

Part of installing a new generator (a defibrillator is made up two parts - leads (wires into the heart) and the generator (battery). The leads last > 20 years. The generator ~ 5 years), involves doing NIPS testing which is where they put the heart into a violent arrhythmia and make sure that the device shocks you back into rhythm. Um, no, I don't want to be simply 'dazed' for that, thankyouverymuch! So I really want to make sure that I won't need it replaced due to its natural battery depletion for the next 2 years. 4 years sounds good to me!

I also got other good news. My last two EKGs were completely normal! I had an EKG in November and one today, both normal. I haven't had a normal EKG ever. My QT interval typically measures at .64. Long QT intervals in women are anything over .40. A typical interval lasts for ~ .8 - so mine is EXTREMELY long. So long, in fact, Dr. Mela almost admitted me for emergency ICD placement when she first met me. I remember her awe at how I'm still alive, with that interval I should have been dead years ago. So to have a completely normal EKG is just amazing.

Then Dr. Kirk invited this German doctor into the room. She was very nice. She is doing a study on the effects of hormones on women with Long QT Syndrome. They believe that my Long QT interval is shortened due to hormones experienced during pregnancy and lactation - and since I'm only about 2 months post-breastfeeding I may still be in that period. This is very exciting news for me as it may means in the future I can be completely medicated with hormones/device combo rather than medication/device combo. The beta blockers they put you on are horrid, and although I'm not on them now I probably will be as I age.

So all of this is very promising.

Due to my Long QT, when I was in preterm labor with Arianna they wouldn't give me any drugs other than mag sulfate to try and slow the contractions. All the studies say that mag sulfate is the only drug proven to actually stop contractions but I would have liked the option of some others too. Now that we hypothesize that my body is actually better protected when pregnant Dr. Kirk sees no reason why we can't push for better tocolysis drugs if I need them. God willing, I don't.

This all makes me wonder - can I be cured? Can my device be removed so I can move my shoulder freely, walk through security without a pat down, wear a strapless dress without a huge bump in my shoulder? Wow, just the thought is exciting.

But my device is my very own personal emergency room - its not a safety net I will let go of without a fight... to the death.