My Terrifying Journey To A Diagnosis

The sudden onset of a life-threatening low heart rate turned me into a scared physician-patient and someone living with a serious rare disease

If I had never gone for my routine annual physical, during which my doctor noticed a low heart rate, I would have gone on living my limited remaining days ignorant of the ready-to-blow grenade in my chest. I was completely asymptomatic leading up to my appointment, in the thick of juggling professional and personal commitments. However, on the basis of the ECG, I was referred for acute cardiac evaluation.

Immediately, I texted the recent ECGs to my brother-in-law, a well-seasoned interventional cardiologist. He called me right away and implored me, in no uncertain terms, to go to the hospital that very evening. He spoke to me simply and directly, addressing me as bewildered layperson rather than a trained physician. My wife and I hastily made arrangements for the kids. En route to the hospital, I was in utter disbelief, still not feeling the slightest bit of weakness, fatigue, or chest pain.

By the time I arrived at the hospital, my heart rate was in the low 30s, reflecting development of complete heart block. Such an extremely low heart rate allowed me to get whisked to the front of the emergency room triage line, like having a VIP pass to an event I didn’t want to attend. If left untreated, sudden cardiac death would have certainly been my near-term fate, with a lethal arrhythmia striking me at any random time.

“Just want to confirm that you are a full code, yes? Don’t worry, the defibrillator pads are just to ward off bad juju,” said the on-call resident physician. I was connected (in very obvious ways) to highly amped shock boxes and cardiac monitoring devices. I looked up treatment algorithms on my phone, and indeed being hooked to a defibrillator was the correct medical move even in a hemodynamically stable patient.

After the evening shift change, I was wheeled from the emergency room to my hospital bed in the cardiac unit. By midnight, tethered to the bed with tangled wires, my care orders were in place (NPO with maintenance IV fluids) ahead of a cardiac MRI in the morning. I was interrupted occasionally with vital sign checks and blood draws, but otherwise left to my own thoughts, with restricted visiting hours during the pandemic.

At that moment, without a firm diagnosis, I was left untreated, alone in my hospital room and fearful of dying that night. I was not in pain and otherwise had all my wits about me, which meant there was nothing distracting me from an overwhelming sense of being cornered. All alone in the room, I have experienced no greater terror than watching a screen read out my feeble heartbeat.

I have moderate claustrophobia which, though not debilitating, has caused me to avoid certain enclosed environments. I knew that MRI scanners leave little wiggle room so that the magnetic waves can get close enough to collect detailed images of the body.

During the MRI scan, I felt trapped. Like anyone who has been forced to face deep-seated anxiety, I just wanted to escape and run away to a safe place. I wanted to beg for a timeout to get some fresh air and see the sun, but knew I needed to lay perfectly still in a noisy, narrow tunnel for about the length of a feature film (holding my breath on the radiology technician’s command).

The results of the MRI strongly suggested that I had cardiac sarcoidosis, a rare inflammatory condition. It is one of those diseases in which a diagnosis can be challenging and often made too late. The MRI had hot spots in the areas which control heart rate, explaining my vital signs.

My medical knowledge as a pediatric endocrinologist and researcher helped me understand my diagnosis and discuss technical details with my care team. It also gave me a ready-made facade to hide how much the hospitalization was freaking me out.

The next stop would be surgery to embed a device that would act as both a pacemaker and defibrillator. Without the implant, there was little doubt that I would remain at high risk for sudden cardiac death. I was added on as the last case of the day for device placement, and it was not before long that I was being transported to the operating room.

An unexpected and horrifying twist for me was learning that I would be kept conscious during the device placement. In fact, my desire to avoid procedure rooms was one of the main reasons I become an endocrinologist!

I can recall with detail each step of the operation: the cutting open of my chest skin, inserting of the pacemaker device, attaching wires to my heart muscle, testing of voltages and, finally, suturing of the incision. With all the surrounding monitors and professional chit-chat, it felt like a multiplayer video game was being played inside my body.

I was discharged the next day with follow-up with an assigned cardiac sarcoidosis expert, who placed me on high-dose prednisone after a baseline outpatient whole-bodyPET/CT scan (another ordeal, but not as bad as the cardiac MRI).

Although the pacemaker assures my heart beats at a certain minimum rate, my mind had to learn how to pace itself. In the immediate weeks that followed, I struggled with the sequencing of long-term and short-term goals, not having a secure sense of time as a reliable anchor. My hospitalization not only triggered deep-rooted anxieties and fears, but also generated new worries and uncertainties.

As the weeks have passed, I have tried to adapt and not worry as much about a sudden cardiac calamity. I have stopped looking up survival rates of patients with cardiac sarcoidosis. I had the benefit of receiving prompt medical care and there is every reason to be optimistic. If things take a turn for the worse, I trust my new cardiologist will figure out the next best steps for management.

My experience has given me insights into being a patient during the workup of an unusual diagnosis and how health providers should be sensitive to a patient’s fears. I encourage medical caretakers to actively ask every patient during the often meandering journey towards the diagnosis of a rare, life-threatening disease: “What is scaring you right now and what can be done to help?” Each patient (even one who is a health care provider) undoubtedly faces unique terrors that should be identified and actively managed. And even mild-mannered health care providers can be outright scared out their minds behind a calm demeanor.

Declarations

Conflict of Interest

The author declares that he does not have a conflict of interest.