My Journey to BiPap

Two years ago, I was hit with some bewildering symptoms. A loud roaring noise invaded my left ear during a visit to my sister in Maryland over the summer. I didn’t want to think anything of it at first, but when I returned home, it didn’t subsided.

Was it just a sinus infection?

Knowing something was off, I went to see my family doctor. Initially attributing it to sinus issues, we treated it as a sinus infection with antibiotics. This provided brief relief before the roaring resurfaced.

I was hearing the roaring in my ear more then someone talking to me or the television. I could even hear it over music flowing through my AirPods.

A mystery to all my doctors

Frustrated, I consulted my family doctor, who, in turn, referred me to an ear nose and throat doctor (ENT). Despite undergoing hearing tests that yielded normal results, my bewilderment grew. I had perfect hearing in both ears and no damage to the eardrum. Everything with my ears was considered well within the realm of normal.

There’s nothing more frustrating then feeling bad and going to the doctors for tests and yielding no results. I was incredibly grateful nothing was wrong. It was killing me not having any answers or plan of care put in place to fix the issue.

A spiking heart rate

Unexpectedly, tachycardia emerged, causing my heart rate to spike to 150bpm even when at rest. My body was instantly thrown into a deep flight or fight response. I had no control over it.

These episodes, accompanied by persistent dizziness and fluctuating blood pressure, led to multiple ER visits. I was given beta blockers to trial. They only offered a temporary solution.

A dangerous drop in heart rate

A month later, I found myself in the ER with a dangerously low heart rate and sky-high blood pressure. Thus, prompting withdrawal from the beta blocker and a referral to a cardiologist for a comprehensive cardiac workup.

The uncertainty surrounding my condition was agonizing, fueling my daily nerve-wracking routine of checking heart rate and blood pressure. This obsession led me to purchase my own pulse oximeter for at-home oxygen tracking. It revealed lows of 80% that promptly bounced back. My anxiety also spiked from checking it so much.

My Apple Watch became a constant companion, tracking a heart rate that persisted at 150-160bpm even during moments of inactivity. I tried everything I could to get my heart rate down. Feeling like I had no other options, I even went so far as to google home remedies for lowering your heart rate during the high times. I sat outside in the winter because I read the cold exposure could help lower the heart rate, and it worked! But as soon I went inside it bounced right back up.

Was this connected to my SMA?

Enduring numerous cardiac tests, including EKG, ECG, ultrasound, and bloodwork, all returned frustratingly normal results. Collaborating with a cardiologist, a cardiac electrophysiologist, and a sleep specialist uncovered erratic nerve activity around my heart, leaving the connection to spinal muscular atrophy or mere happenstance uncertain.

Despite seeking guidance from my SMA-specialized neurologist in Cleveland, little assistance was provided, compelling me to navigate this journey with various doctors over several months.

Going offf Evrysdi

I’ve since got myself a new SMA specialist due to her lack of help at the time. My general family doctor decided to pull me off Evrysdi since I had just started it a few months prior to these wacky starting. She wanted to be sure there wasn’t any correlation. I was heartbroken to stop the medicine for my SMA.

Even after stopping it my issues persisted.

A diagnosis of sleep apnea

A sleep specialist's at-home pulse oximetry test and sleep study unveiled the revelation of sleep apnea, explaining my heart's nocturnal stress during apneas. Finally! I had a somewhat concrete answer. Now I was faced with having to grapple with using a BiPap at night as an adult.

My turning point was when a noninvasive ventilator (BiPap) was introduced. Setting up the machine at home with a compassionate respiratory therapist proved challenging initially. But with  adjustments, guided by my pulmonologist, rendered it both tolerable and beneficial.

Relief with using a BiPap

With the correct settings in place, my sleep quality improved significantly, and my heart rate normalized, bringing relief. While occasional heart racing persists, understanding it's related to nerve activity provides reassurance.

Reflecting on this prolonged journey, I am grateful for heeding my body's symptoms, as I now feel much better.

Living with spinal suscular atrophy naturally tunes you into your body's quirks and signals with the unique challenges the condition throws our way. It's like developing this sixth sense for your own well-being

Personally, this heightened awareness kicked in when my body started dropping hints that something was off. Learning to read and respond to these subtle signals became a game-changer in my journey to figure out what was going on health-wise.

There's this profound connection between us SMA folks and our bodies, underscoring the importance of picking up on even the tiniest shifts and taking a personalized, proactive approach to our health.