Wednesday, November 30, 2011

And the FINAL verdict is...

Tomorrow is a brand new month and a very significant milestone for me. It's the 30th anniversary of my one and only open heart surgery! Below is a copy of the email I sent out to family members and friends this evening. Now that a final decision has been made, it's time to shift the focus of this blog from documenting the play-by-play of this recent journey to a broader focus of generally living a healthy and active life with tetralogy of Fallot.
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I'm not going to have the surgery!

As you know, the last two and a half months have been like a roller coaster ride I've never experienced before. Tomorrow (December 1, 2011) marks the 30th anniversary of my first (and, so far, only) open heart surgery; and I recently set that date as my own deadline to make a final, final, final decision ... and then to go confidently in that direction, to stop questioning, and to cease the second guessing of everything. I'm tired of being tired, exhausted, scared, and unsure.

This morning, I emailed the ACHD cardiologist at the University of Chicago, from where I got the third opinion (and the second opinion that advised against the surgery) to ask if I could change my mind again and to keep him as my regular cardiologist going forward, as well as to proceed with his recommended less-invasive approach. He replied this evening and said that he would be happy to do so.

After I thought I had made a "final" decision multiple times in each direction over the past few weeks, the truly final verdict came down to (at least) a couple key points:
  • The majority of the doctors I met with and/or who reviewed my case actually advised against the surgery. While I didn't meet with him directly, this group of individuals included Dr. Michel Ilbawi, who was the first assistant during my surgery 30 years ago. Dr. Ilbawi is now the director of pediatric cardiac surgery in the Department of Cardiovascular-Thoracic Surgery at Rush University Medical Center; and he was part of Rush's comprehensive medical/surgical conference that reviewed my case (for the second opinion) and collectively advised against the surgery.
  • The ACHD cardiologist at the U of C, who will be my primary cardiologist going forward, had (in my opinion) the best patient-centered approach of all the doctors I met. While I wasn't at all unhappy with any of the care or evaluations I received from Northwestern or Rush, the U of C cardiologist's direct and compassionate approach was unmatched. In addition, he has been very accessible to me and has thoroughly answered and responded in a timely manner to every question and concern that I've raised with him.
Life is uncertain. While there is a very real possibility that I will need another open heart surgery at some point in the future, now is not the time for it. Is it possible that a serious cardiac event could happen to me? Sure! But it's also possible that I'll get hit by a car while walking to the train on my way to work tomorrow morning. While that would certainly be a crappy way to start my 30th anniversary, I won't be losing sleep over it tonight!

Earlier this evening, I sent an email to the doctors at Northwestern informing them that I had changed my mind (again) and that I decided to cancel the second surgery appointment (which I actually just called to schedule earlier this week) and that I would be working with another ACHD cardiologist going forward. Ironically, as I was writing this message, I received a phone call from the cardiologist at Northwestern, who was working late into the evening and just wanted to follow up on my email. We actually had a very pleasant conversation, as I talked briefly about the crazy journey I've been on since mid-September and how I ultimately came to my final decision. He was very cordial and understanding, and he even told me that it sounded to him like I had made the best decision for myself. He admitted that this comment sounded ironic coming from the person who initially recommended the surgery; but he explained again that in unusual cases like this, it's not uncommon for different doctors to have different opinions. He said that he was confident that I would be getting excellent care and wished me well. Truth be told, I'm actually really glad he called; and I feel like there is now positive closure to our doctor/patient relationship.
Next steps: I will be working with the doctors at the U of C to do the following:
  1. Schedule an appointment to have a stent placed in my narrowed left pulmonary artery, which will be done in a cath lab;
  2. Meet with the electrophysiologist, who will assess for cardiac arrhythmias that might have led to my biking accident this past summer; and
  3. Start preparing for my next distance bicycling event in the summer of 2012!
I cannot thank each and every one of you enough for your love and support during this crazy time. Your prayers, good wishes, kind words, and supportive messages—whether in person, on the phone, or online—have meant more to me than you can possibly imagine. I don't think I have been more confident in my recent decision making than I feel right now. Earlier today, when I told myself that this really is my decision, I truly felt like the weight of the world was instantly lifted off of my shoulders. And I took that as a good sign!

From the very bottom of my heart (even though it's still got a leak and an aneurysm in it), thank you!!

-Ken

Tuesday, November 29, 2011

A Cyclist’s Journey

My first posting for the ACHA blog was published today (two days earlier than expected)! Below is a copy of the entry.
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This Thursday marks the 30th anniversary of my first (and, so far, only) open heart surgery. Although I had annual checkups with my pediatric cardiologist as a child, I never really thought of myself as a heart patient. Since I had my surgery when I was only eight months old, the scar on my chest feels more like a birthmark than a reminder of a major life event. My annual visits to the doctor always resulted in a clean bill of health. With the exception of football, I had no physical restrictions growing up.

I have always loved the outdoors, especially bicycling. Over the last few years, I’ve become more of an avid cyclist, having completed multiple distance rides, including centuries and multi-day rides. For me, cycling is the perfect mix of physical endurance and relaxation. While that may sound a bit contradictory, I would bet that most people who ride regularly would agree. It’s hard to describe, but the rush and excitement associated with a distance ride is both physically invigorating and emotionally meditative. For me, the benefits of cycling cannot be replicated elsewhere.

Back in early August, I went for a bike ride after work along Chicago’s lakefront path (a common thing for me to do in the summer), and I later woke up in the ER after falling and having a concussion. Fortunately I was wearing a helmet, and the brain CT scan came back normal. Because the doctors were made aware of my CHD, I was put on a heart monitor while in the hospital and run through the battery of cardiac tests, including an outpatient MRI the following week.

The next month, I had an appointment with the ACHD cardiologist, who reviewed my records and informed me that the MRI found an aneurysm in my main pulmonary artery. During that conversation, he also gave me his preliminary recommendation for open heart surgery.

I, like many adults with a CHD, stopped going for regular checkups when I was in high school. Now it’s hard to know whether or not this aneurysm would have been caught earlier had I been maintaining regular checkups, but that’s a moot point. The last two and a half months have been spent going for a variety of pre-op appointments, seeking multiple opinions, and riding an emotional roller coaster.

The second and third opinions I received actually recommended against the open heart surgery. The doctors told me the likelihood of the aneurysm rupturing is incredibly low. Instead, they recommended a much less invasive procedure to put a stent in the left branch of my pulmonary artery, which has been narrowed as a result of the aneurysm pushing up against it. For many reasons—including my paranoia about the unknown and my desire to not be that first documented case of such a rupture—I anticipate that I will actually be having the open heart surgery, rather than the stent procedure, in the coming weeks.

Truthfully, I’m terrified; but I also know I’m in good hands. The surgeon I met with specializes in adults with CHD, and he has a 0% mortality rate. During our consultation, he even said that he fully expects I will be back on the bike within just a few months after surgery. Hearing that gave me a lot of confidence, and I know that cycling will be a big part of my recovery.

I recently read Back to Life After a Heart Crisis by Dr. Marc Wallack and Jamie Colby. In the book, Dr. Wallack talks about his journey to regain his life after having emergency quadruple bypass surgery, and the final step in his 8-step plan is “Train for a Huge Physical Challenge.” He writes, “To fully recover mentally and physically from heart disease, you need to take on that one thing you fear most. Only then will you prove to yourself and others that you are back and better than ever.” I know that I will be back on the bike by spring, and I am already looking for distance rides taking place next summer that I can register for now to keep myself motivated and my recovery on track.

Friday, November 25, 2011

Reconsidering my options (again)!

If you've been following my blog and feel like you've been riding a roller coaster lately, welcome to my past two months! Even after I thought I made a final decision, I find myself now reconsidering my options. As usual, nothing is definite; but I am, once again, thinking about having the surgery. Even though I have two recommendations against surgery and one for it, my gut is starting to lean back toward having it done as an extreme (but potentially life-saving) preventive measure.

This past week, I received a call from the interventional specialist at the University of Chicago who would be doing the stent implementation. He talked me through the details of the procedure and the associated risks, and he explained that it would be much like the cardiac cath that I had done last month at Northwestern. The difference here is that, rather than just observing the heart, this procedure would place the stent in the narrowed portion of my left pulmonary artery just past the aneurysm. When doing these stent procedures, the doctor explained that there is generally a very low risk (approximately 1 in 1,000) of an injury (or tear) to the artery wall. However, because my case is so unusual given the size of the aneurysm, he estimated that the risk of arterial injury here might be closer to 1 in 100, which is actually greater than the mortality risk associated with the surgery (less than 1%). I then asked him this question: “If there is damage done at or near the site of the aneurysm, what, then, would be the likelihood of my needing immediate surgery?” Because my case is so unusual, I understand why he wasn’t able to give me a definite answer. That said, however, this has added yet another piece to the complex puzzle.

While all the doctors I've met with seem to think that the likelihood of my pulmonary artery rupturing is quite low even with my overall level of activity, the lack of documented data cannot really allow them to determine the likelihood with much certainty. (And I surely don't want to be that first documented case!) Truth be told, I'm rather paranoid about it, especially given the active lifestyle I like to lead. I have even been a bit hesitant to exercise with any degree of intensity since first learning of the aneurysm and getting the recommendation for surgery back in mid-September; and I definitely want to feel comfortable and confident again going on future multi-day bike rides.

Just this morning, I emailed the cardiologists at Northwestern and the University of Chicago to ask for more feedback given my latest concerns. I also inquired about endovascular coiling, a minimally-invasive procedure I recently read about online for treating brain aneurysms. While I have no idea if this would be an option for my cardiac situation, I had to ask.

Stay tuned! I'm sure there will be more exciting adventures ahead, even though I don't yet know what they will be!

Tuesday, November 15, 2011

December 7, 2011: Just another day in the office

Two months ago today, I walked into the cardiologist's office at Northwestern for what I expected would be a routine appointment, and I walked out with a recommendation for open heart surgery (OHS). Just about a half hour ago, I called his assistant and left a message telling her that, after seeking a second and third opinion, I have decided to not go forward with the surgery, which had been scheduled for December 7. I think the past two months have been the craziest emotional roller coaster and test of fortitude that I can recall.

After following up with Northwestern to have the last remaining pieces of my medical records (specifically the results of the October 27 heart cath) sent to the University of Chicago, the cardiologist at the U of C emailed me his official recommendation late this afternoon:
The report indicates that the pressures in the pulmonary artery are not significantly elevated, leading [us] to conclude that your risk of rupture of the pulmonary artery is very low. Based on this, we'd recommend (1) proceeding with stent implantation into the left pulmonary artery to attempt to improve flow into the left lung, and (2) an evaluation by our colleagues in electrophysiology to assess for cardiac arrhythmias that might have led to your biking accident last summer.
Ding! Ding! We have a winner!! For those of you that have been following along this journey to any degree, you know that this decision was not made lightly. While I will not be having surgery three weeks from tomorrow, I'm not entirely off the hook, nor will I be for the rest of my life. If nothing else, my biking accident at the beginning of August was a very clear wake up call and a reminder that as a person with a CHD, I need to have lifelong care and monitoring by a cardiologist trained in adult congenital heart defects.

Who knows what procedures I may or may not need down the line? There is a very real possibility that I will need another OHS at some point in the future. But then again, I may not. Life is a journey, and I plan to continue living it one day at a time. From now on, though, I'll just be sure to have the proper medical care and monitoring come along for the ride!

Follow-up from Rush (Opinion #2)

This past weekend, I received an email from the cardiologist at Rush, who informed me that my case was going to be discussed at their comprehensive medical/surgical conference today. He called me this morning to let me know about the conversation that took place. Their recommendation remains the same as when I met him in his office last month: no surgery; instead, put a stent in the left pulmonary artery (LPA). Doing this will help to even out the distribution of blood between my lungs and very likely reduce the pressure on the pulmonary valve (PV), which has been leaking my entire life.

Cool Fact: The cardiologist also informed me that Dr. Michel Ilbawi, who was the first assistant during my surgery 30 years ago, was a part of this morning's conference and weighed in on Rush's collective opinion. Dr. Ilbawi is now the director of pediatric cardiac surgery in the Department of Cardiovascular-Thoracic Surgery at Rush.

Wednesday, November 9, 2011

Third Opinion = A New Direction?

Yesterday morning, my parents and I went to the University of Chicago Medical Center to get a third opinion. After the confusion I faced following the second opinion, I went into yesterday's appointment with absolutely no expectations one way or another. I figured I would just go with the flow and see what they said.

We met with both the medical director and the medical co-director of the University of Chicago's Center for Adults with Congenital Heart Disease. During our conversation, it became clear that these two gentlemen agreed with the cardiologist from Rush University Medical Center, who, during my appointment for the second opinion, recommended against the surgery.

Prior to yesterday's appointment, I had another phone conversation with the cardiologist from Northwestern, who called to discuss the results of my recent lung scan and heart cath procedures. He confirmed what the other doctors told me about the heart cath, which was definitely a good thing. Regarding the lung scan, he told me that the most significant finding (and one of the main reasons for having the test done in the first place) was the percentage of blood going to each lung. While the ideal distribution is 50/50, he told me that it's not uncommon for the distribution to be more along the lines of 45% to the left lung and 55% to the right lung. My lung scan showed that the blood distribution is approximately 20% to the left lung and 80% to the right lung!

Fortunately, the cardiologists at the University of Chicago received this test result prior to yesterday's appointment. (Some of my other medical records, for some reason, never made it to the University of Chicago. Before I left, the nurse had me sign the release paperwork so that she could follow up and re-request the rest of my records from both Children's Memorial Hospital and Northwestern Memorial Hospital.) Just like the Rush cardiologist had said previously, the University of Chicago cardiologists recommended having a stent inserted into the narrowed left branch of my pulmonary artery, which should correct the balance of blood distribution to the lungs.

One of the things that I've found the most interesting (confusing?) is the fact that the primary concern of the doctors at Northwestern (the aneurysm in my pulmonary artery) seemed to be the least of the concerns of the doctors at both Rush and the University of Chicago. When I followed up with the U of C cardiologist via email regarding this discrepancy, he—like the cardiologist at Northwestern—said that there isn't much data regarding the risk of rupture of the pulmonary artery. However, he added that
"it is almost unheard of for the pulmonary artery to rupture except in a few rare disorders (not tetralogy of Fallot). Also, we never seen pulmonary artery patches 'wearing out' over time - after surgery they form really tough scar tissue that just doesn't fail. Given this, it's our opinion that the risk of surgery, despite being very low (probably less than 1%), is still higher than the risk of rupture, so we would not recommend it. The only caveat here is I need to see the cath report details to know exactly what your pulmonary artery pressure is. I'm assuming its relatively normal, but just want to make sure."
The cath report is clearly one of the missing pieces of data that is being requested again by the nurse. The cardiologist expects to be able to follow up with me early next week after he's had a chance to review both the cath report from Northwestern and the original op report (from 1981) from Children's Memorial.

While I have yet to make a final decision about my immediate next steps, there is a very good possibility that—based on this information I received from the doctors at the University of Chicago—I will actually not be going to Northwestern for the surgery that is currently scheduled for four weeks from today. However, because nothing is definite at this point, I have not yet canceled anything at Northwestern.

On one hand, knowing that there is a very real likelihood that I will not need to be having a major surgery at this point is a huge relief. On the other hand, it's all still very confusing and overwhelming. As I was telling my family members and a couple friends, the whole idea of surgery has been on my mind every single day—in one form or another—since the initial recommendation came in mid-September. Physically, I have been feeling fine. Mentally and emotionally, however, I am beyond exhausted. Within just the last month alone, I went from meeting with the surgeon at Northwestern and beginning to prepare myself mentally for this major procedure, to being thoroughly confused and unsure of what would be next after getting the second opinion, to going back to thinking that the surgery was still the better option, to (just as of yesterday) going back to grasping the very real and legitimate possibility of choosing to not have the surgery at this time.

At this very moment, I think I know what I'm going to do (or not do) in the immediate future. But then again, I've said that before. The jury is still out.