ARVD Seminar & Another Surgery

To start things off, I have added a very short summary for those who don't feel like reading this lengthy post.  It seems like a lot of writing, well, because it is, but that is because so much was learned this past weekend.  So if you want a short, one paragraph summary, jump to the bottom, and it will be there waiting for you. Otherwise, sit back, take your time, enjoy, and I hope you learn something!

Friday the 13th was the weekend of the 12th annual ARVD seminar.  The actual seminar was on Saturday, but that Friday I had an appointment with Dr.Tandri, who is Dr.Hugh Calkins partner, the head of cardiology.  Dr.Tandri is the one that performs all of the ablations, more importantly the epicardial ablation. He is also the one who has perfected the art of reading MRI's of the right ventricle.

One of the main reasons for my first visit to Johns Hopkins was to seek a diagnosis, which due to false readings of my MRI, my disease remained a mystery.  But thanks to Dr.Tandri, he successfully read the MRI of my Right Ventricle, saw that it was enlarged and that there was indeed scarring of the tissue, sealing the deal for my diagnosis. So this time, when I went back up to Baltimore, I had a chance to meet with him and discuss the ins and outs of an epicardial ablation and how it is different from the previous two ablations I have had, which are referred to as endocardial ablations.

The ARVD team at Johns Hopkins and I decided not to go through with the ablation for at least another six months if not longer because my medicine was keeping my heart stable, and it is a rather major procedure. But the day after we decided that, I was hanging out with some friends in Columbia,SC when my defibrillator went off and proceeded to shock me four times.  That following Monday, I called Johns Hopkins to explain what happened and that is when we decided to go through with the surgery.

The epicardial ablation has been being performed for only four years, therefore, long term success of the procedure is unknown.  However, short-term success is very promising.  So far recurrence of V-Tach is very limited and usually only starts to show after the third year if at all. Completion of the procedure will allow my medicine to be drastically reduced, which will also contribute to re-energizing my body and feeling healthier.

This ablation is different from the other two because of the area where the cells will be burned.  The previous two ablations just targeted faulty cells inside the heart, where the epicardial ablation will focus mainly on the outside of the heart as well as the inside. The first two ablations, endocardial ablations, just used one catheter, inserted through the groin, which then maneuvered up to the inside of my heart that burned the cells produced from the scarring which caused the V-Tach.  With this fairly new procedure, the epicardial ablation, there are two catheters used.  It is basically an endocardial ablation, along with another catheter inserted right under the sternum which will be used to burn cells on the outside of the heart, which is where most of the fat cells and scarring infiltrates.  The doctors use a GPS device to map the heart piece by piece, using colored dye to find the faulty cells.  This is a very lengthy procedure that takes around six hours.  The doctors will keep burning the cells in the scarred tissue until none are left. If he finds there are still cells causing arrhythmias at the end of the procedure, then he will continue until he feels as though he has rid the heart of all the bad cells.

Another thing I learned is that the cells cannot reproduce once they are burned(killed) because the heart is done growing.  Once they are dead, they are gone.  The reason the other two ablations did not work is because most of the arrhythmias are associated with cells from the scarring on the outside of the heart, which is what this ablation will tackle.  I will be in the hospital from one to three nights depending on the amount of burning that my heart incurs. When the cells are being burned, they are resting on top of the heart right under the lining that surrounds the heart.  If they have many areas to burn they could potentially burn the skin that surrounds the heart, which causes severe pain, known as Pericarditis.  So because of this they drug you up real good and then just wait and see what happens. Some feel the effects intensely and others don't at all, it really all depends how much burning goes on.

But the end goal of this surgery is to resume recreational sports and lower my medication.  Athletics is still the kryptonite to this disease, but this ablation will hopefully be able to get me back to swimming once in awhile, or on top of a mountain a few times a summer, or in a kayak in the middle of the lake, or even on a field tossing a frisbee.  I should be able to exercise enough to stay fit and healthy, but no more than that.  Basically this ablation will allow me to do the bare minimum to get in shape, which now is more than I could ask for.  Right now, I can't walk up a flight of steps without tiring.  So Johns Hopkins is in the midst of scheduling the ablation, and I am very excited for that.  I am not setting any expectations as in my journey so far I have seemed to keep falling in that .1% where everything goes wrong.  I remain hopeful, but I also know that things don't always work out as planned, but I am very excited to be having this done.

Now regarding the seminar, it was an all day event Saturday, very informative and interesting, and I'm glad I made it up there.  Dr. Hugh Calkins did a general overview of ARVD, introduced each speaker, and kept the timeline running smoothly.  Dr. Tandri, who will be performing my epicardial ablation, spoke about the procedure and how they have had much success with it.  Then there was Dr. Jeff Saffitz, a leading pathologist from Beth Israel Deaconess Medical Center in Boston, Massachussettes, which is a teaching hospital of Harvard.  He is also the Chairman of the Department of Pathology at the Harvard Medical School.  Most of his presentation was about genes and their mutations and cardiomyopathy.  He went very in depth in to the type of genes involved in heart disease, and the common mutated genes found in ARVD (PKP2), and spoke on current research findings.  Many doctors and labs are now actively researching this disease and making major progress, receiving grants, even winning awards, and now pairing up with researchers from abroad to try and discover what exactly causes this, what makes it worse, and new curative measures for the disease.  The seminar had a lot of information to take in, but it was all very intriguing.

Later was Dr.Samuel Sears, Professor and Director of Health Psychology at East Carolina University.  I enjoyed listening to him the most.  Although he was a physchologist, he also has studied up in the medical field and was well informed regarding any health and heart questions.  He was a well spoken, charismatic guy, who threw in some humor, and just had a lot of good stuff to say in general.   I would have loved to have him as a professor.  He was speaking about how family members, not just the ones with the disease, are affected by the disease and how to cope with it, and then he went into family being the support team for one another largely based on communication. He also showed statistics of partners, the ones without ICD's, being equally anxious and scarred of living with a defibrillator.  His main focus in his practice, is in regards to post traumatic stress syndrome of people who have been shocked by their ICD's and also dealing with their general anxieties of living with such a device, which I found very interesting. I never knew people counseled others just regarding their ICD's, but after being shocked, I can see how someone would have such emotional trauma. I was a little apprehensive, the following day, after leaving the hospital when I was shocked, fearing I would be shocked again.  Then I realized you cannot live in fear of the device, you cannot let it have such control over your mind, but the memory replays in my mind over and over and the thought of recurrance is almost daily. It's a mind game, and I would like to believe I have the mental capabilities to free my mind and find peace.

After the professionals spoke, three people got up to share there stories.  One was the father to a son with the disease who was now in college.  One was a man of the age of 76 who was diagnosed in 1999, when the disease was first discovered. Subsequently, he had been shocked around 84 times since then by his ICD. He was a college professor himself that once fought in the Korean War, and said speaking during this seminar was the hardest speech he has ever given out of the thousands in his life. And the other one was a marathon runner who was just recently diagnosed, holds a record for the 400m hurdle at Purdue, and is trying to figure out what to do with his life now that running is not an option. He is in his 40's and has spent his whole life running, everyday, all day. Last year it was reduced to simple jogs but this past January he was shocked by his ICD while out for a jog, so now his challenge is to find another passion, another love for life.  And that is the hard part for most of us with this disease.  Most of us are avid athletes, who have spent the majority of our lives doing something involving sports and activity, and now it has been taken from us.  Being active and physical is such a natural thing, that it is hard to cope with the idea of not getting to enjoy the lifestyle we were so accustomed to.

But many questions remain, and that is why Hopkins is actively delving deeper into the genetic characteristics and implications of the disease.  Over the last 5 years great strides have been made in understanding the genetic cause of ARVD.  We now understand that in many cases, ARVD is a desease of the cardiac desmosome.  The desmosome is the structure that holds heart muscle cells together.  Much still needs to be done to better understand how, when, and why the desmosomes fail in ARVD and to develop treatment for patients and family members.  Just a week ago they discovered a way to take a cell from your arm, grow it and transform it within a petri dish and eventually change it into a cardiac cell which will then produce a heartbeat.  They showed us a video of one of these transformed cells in a petri dish beating, and it was pretty incredible.

Just an interesting side note of something else I learned in the seminar.  I learned they refer to the person in the family who first seeks medical treatment for a condition the proband. That would be me, I'm the proband.  The proband, in relation to ARVD, has a high percentage rate of sudden cardiac death.  I was very lucky to be spared such a thing, although I came very close when I went into cardiac arrest last October.  But not only was I lucky that one time, as Dr.Calkins told me, I could have easily died any one of the many times I had a syncope episode (passed out).  So in reality, I was spared death many a time.  Also in hearing others' stories during the seminar, there were a few widow(ers) who had stories of their spouses dying from the first or second time they passed out, so I am just thankful to be breathing and walking.  I also met a younger fellow who gave bad advice to another person who was even younger than he was. His words, "THIS SUCKS."  I put that in all Caps because he said it with such emphasis.  But many things in life suck, and this is just one of them, but there are much worse diseases to be had and many worse situations to be in, and I was very taken back and slightly annoyed/angered by his advice to such a young person dealing with this disease.  It is a rare heart disease and I think we should support one another, not the opposite.  Anyways, I learned many things from this seminar and I am looking forward to this ablation and hoping to be physically active, at least just a little, by the end of the summer.  There is always Hope.  I never really thought that, but I certainly do now!  Enjoy your days!

Quick Summary:
I will be having my 3rd ablation, which is different from the previous two and considered major surgery. It is in the works but hopefully will happen sometime within the next two months.  We decided to go through with this since I was shocked four times by my ICD a couple of weeks ago.  Also, I attended the 12th annual ARVD seminar and learned a great deal, and heard from some leading doctors from across the nation, and shared stories with other families afflicted with this disease. Then I flew home and wrote this post for you to read.