Yamaguchi Syndrome: No Regrets

Yamaguchi Syndrome Personal Story
Consider the American Dream – we work hard so we can enjoy retirement. What if we spend all the time working to that goal, but never arrive?
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My husband was strong, healthy, and young, but he could drop dead at any moment.

“Yama what?” I asked my husband’s primary care doctor.

“Yamaguchi Syndrome,” she explained. “If not treated, Isaac’s heart could go into ventricular fibrillation, a lethal cardiac rhythm.”

Being a nurse, I knew all too well, what this meant. The lower chambers of the heart quiver and cannot pump any blood. The person collapses, loses responsiveness, stops breathing – and sudden cardiac arrest ensues. Without immediate medical help, death follows. I felt as though my heart had been shocked with two paddles, delivering 360 joules.,

In our fourth year of marriage, we were finally able to look back and recognize that we made it through some major life difficulties. The trouble had started immediately after our wedding. Isaac’s ex-wife took him back to court for more child support and removal of his visitation rights. The result of the two-year battle ended with the estrangement of his two teen-aged children. Legal, financial, and emotional stressors took their toll. At times, our pain was more than we could contain, larger than the house we lived in. Through it all, Isaac maintained that at least we had our health. Finally, we were starting to heal and see some of life’s blessings.

Isaac didn’t have a doctor three years ago. His last physical may have been for high school sports, or college entry, he couldn’t recall. I kept reminding him to get a family doctor. Thankfully, he took my suggestion, perhaps only so he didn’t have to listen to me harp on that subject anymore. His family doctor diagnosed this rare cardiac disorder by appreciating the heart murmur during the annual physical and reviewing an abnormal EKG.

She recalled Isaac’s family history. His maternal grandfather died of a heart attack at age 53. “Are there any other heart conditions in your family?” she probed.

“You know, I think I remember my mom telling me that my grandfather’s brothers all died of heart disease before age 50.”

That was all she needed to hear. Isaac underwent a battery of additional tests, including a 48-hour Holter monitoring, stress echocardiogram, and cardiac MRI.  Second and third opinions confirmed the diagnosis. The walls of his ventricles and septum are double the thickness they should be, causing this hypertrophic cardiomyopathy, a.k.a. Yamaguchi Syndrome. One cardiologist told him, “You can thank your PCP for saving your life.”

The only way to treat this was with protective measures: cardiac drugs for the rest of his life and an internal cardiac defibrillator (ICD) implantation. There was no cure. The defibrillator came with a certain set of rules: no contact sports, avoidance of high voltage areas, quarterly monitoring of his heart rhythm, annual check-ups, and day surgery every five to seven years for battery changes and maintenance.

Then the cardiologist provided two defibrillator options: the Internal Cardiac Defibrillator (ICD), or the Subcutaneous Implantable Defibrillator (S-ICD). The difference being that the ICD leads are placed directly in the heart’s ventricles, while the recently FDA-approved S-ICD leads are implanted along the bottom of the ribcage and breastbone.

Isaac always seems to find humor in most situations. While meeting with the surgeon, he quipped, “Just don’t give the defibrillator remote to my wife!”

Besides the health and logistical concerns, we had to consider whether Isaac would be able to keep his job, as some electromagnetic fields in the paper mill where he worked could trigger the defibrillator to fire inappropriately, resulting in death. By age forty-two, he had worked for the same company for over 18 years. Interestingly enough, he completed college, studying to be an electrician, but never pursued that career after graduation. It is a little unnerving to think that if he were an electrician today, he would probably lose his job.  

It is amazing how physically active Isaac used to be, and how he didn’t know that he was at such a serious health risk. He played three sports in high school, with no adverse effects.  We learned that some athletes with this condition have dropped dead while playing sports such as basketball, track, and cross country running. Now doctors tell him to stay well hydrated and avoid strenuous exercise until after surgery.

I cringe when I think back to the times when he was fully exposed, and we didn’t know it. Two years ago, we started the rigorous P90X program together. Out of shape, he pushed himself beyond what he thought his limits were. In the middle of one session, short of breath, he confessed, “I feel like I might pass out.” Neither of us thought much of it, but now we realize it may have been a warning sign. One Saturday morning last year, he attempted (once again), to start a fitness program. The fresh spring air and gentle sun made it a perfect day for a bike ride. He prepared for his ride with his iPod ear buds already in place, and bent down to tie his shoes while rhythmically bobbing his head, and singing an off-key version of The Monkees’ I’m A Believer. With a brush of a kiss and a wink of his eye, he headed out, saying he’d return in an hour or so. Two hours later, the heavy breathing re-entered the kitchen before my drenched, red-faced husband. It is humbling to realize how close one can get to danger, just grazing it – without even knowing it.

Various scenarios wandered through my mind. What if he hadn’t returned from the bike ride? What if he had collapsed on the side of the road, unable to call for help? The stress of it went to sleep with me. A terrible dream filled one night: he was involved in a fatal car crash because his heart went into ventricular fibrillation while driving. I worried during the times he was alone – driving, hunting, or simply home by himself. Anxiety filled my heart as I tried to whisk away the thought of life without him. 

One evening while we sat reading in the living room, he said, “It’s happening.”

“What?” I asked.

He looked up from his book, and put his hand over his left chest. “My heart is fluttering again.”

Seconds later, he gave an update. “The palpitations are gone.” 

How alarming to know that your husband’s heart may arrest! The 48-hour Holter monitoring confirmed that his heart frequently converted to ventricular tachycardia, the precursor to ventricular fibrillation, even in his sleep.

Isaac agreed to take a daily medication, Toprol. He mentioned the bothersome side effects from the medication: sleep disturbances and fatigue. He combed the internet for drug information, and became more concerned.  “Did you know that I could also experience side effects like dizziness, depression, and impotence? I’ll tell you what, if I can’t get an erection, I will go off the medication!”

Together, we set out to research more about life with an S-ICD. We already knew about the standard risks and complications: bleeding, infection, and faulty equipment. The environmental precautions led to other concerns. His use of cell and cordless phones needed modifying. My left-handed husband would have to hold phones against his right ear, on the opposite side of the implanted device. Medical and dental procedures have the potential to interfere with the device. In addition, he was cautioned that other systems might interact with the device – airport security systems, and the electronic article surveillance systems frequently found at the exits of stores, banks, and libraries. Surprisingly, he could no longer lift our large stereo speakers, as they have magnets in them. Some literature even warned against leaning over a running car engine.

Lastly, we discussed the obvious ‘what if’s’ of not having it implanted – sudden death.

As we dealt with the unexpected concerns that went along with this diagnosis, we began to see that second chances come in a multitude of forms. I don’t believe people spend much time considering how or when they will die. We go about our daily routine, thinking we have all the time in the world. We pretend that we aren’t at risk. What about the movie massacres? The school shootings? Tsunamis, hurricanes, and tornados? Our lives are really like ticking time bombs – we never know when they will go off. There may not be as much time as we think. 

An early morning pillow talk led to a discussion about our mortality and what purpose were we serving during our time here on earth. We are thankful for the time we spend together; however, we realized that much of our time was spent concerned about what is, or what might be. It is easier to focus on every bill that needs to be paid, every flaw in the house, and the annoyance with some workplace issues. How many of us hold onto anger over things that have no lasting significance? I, for one, have lost sleep over things that never materialized. It was stealing our daily joy. I recall the day, some time ago, when I told my breast cancer-ridden mother to fully live each day. “Do not allow your worries to rob you of your joy.” I told her.

Wow, I should heed my own advice!

Consider the American Dream – we work hard so we can enjoy retirement. What if we spend all the time working to that goal, but never arrive? Are we prepared for our last day with as few regrets as possible? A diagnosis can be a wake-up call to stand at attention and salute life – to seize opportunities and deepen connections. Live like we are dying, every day.

Isaac has agreed to have the defibrillator implanted. Best-case scenario, he goes through life never needing the defibrillator to fire, and we have learned to live more fully.If Isaac’s heart is what causes his sudden death, he and I know this is our second chance to say – and do – the things that we may not have otherwise. The diagnosis made us revisit our priorities. We no longer take each day for granted. It motivated us to create our bucket lists, and focus on our passions and our relationships, having no regrets.

Because you never know when your heart will take it last beat.

 

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