Brugada Syndrome: The Rhythm of the Heart

Today I met a patient who had been diagnosed with Brugada Syndrome. It is a genetic disorder which affects 1 in 5000 people and causes sudden, unexpected cardiac arrhythmias which lead to death. This particular gentleman, who had a warm smile and easy manner, had been diagnosed with the disease several years ago but had decided not to opt for the treatment: placement of an implantable cardiac defibrillator.

This peaked my interest. It is not very often that we can offer a treatment as effective as the implantable cardiac defibrillator is for Brugada Syndrome – it can save a patient’s life time and time again, when the heart develops a life-threatening rhythm (ventricular tachyarrhytmias). And it is the gold standard treatment. There are no other options because the arrhythmias present so quickly in this disease that a patient will never have the time to make it to an ambulance or hospital. An instantaneous shock is the only thing that will save his life.

This gentleman had been diagnosed with Brugada syndrome a couple of years ago. Since then, many physicians had tried to convince him to get the defibrillator placed. They could not understand why he refused – this refusal to get care was almost viewed as a voluntary suicide attempt, or as a lack of understanding. They spent significant amounts of time trying to convince him to agree to the cardiac defibrillator.

Although I had a busy shift, I could not let Mr. M leave without understanding his story – and so, as I unhooked him from the cardiac monitor and removed his IV, I confessed, “I have a question for you. I was hoping that you might be willing to tell me why it is that you do not want a cardiac defibrillator.”

He smiled. I don’t think anyone had asked him that question before without trying to push their side, without trying to force reading material – and a decision – on him at the very first moment. He thanked me for asking the question, and went on to explain that his religious and world view was that the heart was central to life and to spirituality. He believed that the rhythm of the heart was the source of his very being. “And if  I was born like this, with this rhythm,” he said with great calm and confidence, “then I don’t want to change it with wires or any other devices near my heart.” He told me, “I want to take care of myself – exercise, eat the right foods, and take my vitamins. But I do not want to change the rhythm of my heart.”

His explanation was so eloquent and well thought out that I could not help but be impressed. I confessed to him that it was difficult for me to let him walk out the door, knowing that he had a risk of dying and that I could do something about it if he allowed me. But we all have a risk of dying at every moment, do we not? And in spite of the frightening statistics and stories associated with Brugada syndrome, this gentleman had lived well into middle age without any problems. He did not seem to be afraid of death. If it were his fate to die as he walked out the door today, he explained to me, he would be content.

I did not try to push a load of pamphlets on this gentleman, nor did I make him feel as though he were making the wrong decision – because although he was not making the best medical decision, he had convinced me that he was making the best personal decision.

I watched him as he left our emergency department. He walked with his head held high, his long stride a comfortable, peaceful, and fluid movement through the swinging double doors. Unlike so many of the patients and physicians whom he was leaving behind in the hospital, he seemed truly happy. Maybe it does all stem from his heart, and maybe there is more to the heart than we discover on an ekg – pumping away with a flawed rhythm, circulating blood in spite of its genetic composition, beating to beat the odds.

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2 Responses to Brugada Syndrome: The Rhythm of the Heart

  1. emmy says:

    Thank you for listening to him without judgement. I have Long QT Syndrome and find that oftentimes those in the medical community don’t understand. I have been living with this faulty wiring my whole life. This is my heart and this is the way it beats, it always has. When it is my time to die, I will die whether it is from LQTS or breast cancer, or from something unforseen. I can accept that. I will take my beta blockers and be reasonable about the lifestyle restrictions, but I don’t want wires going into my heart and regular surgeries to maintain it.

  2. Karen says:

    Great post, but just FYI, it’s ‘piqued’, not ‘peaked’.

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