My PH Diagnosis Story

My pulmonary hypertension (PH) diagnosis was delivered to me as I lay in an ICU bed recovering from a pulmonary embolism, or PE (blood clot in the lung), just days after a category 4 hurricane destroyed my house. I couldn’t tell you what was said or how it was described. I just remember being in a state of total shock!

I didn't know how severe it was

The doctors just kept going on and on about what type of testing would be needed. I had to stop them and say, "I’m sorry, what? My heart is involved?" I really had no concept of how severe my symptoms were or how my future was about to change.

At the time, I was a bit distracted caring for my micro-preemie daughter who was recovering in the NICU from pacemaker surgery just days before.

Doctors suspected CTEPH

About 6 months later, I found myself consulting with specialists at UCSD in San Diego, California. Due to my history of blood clots, it was believed I had a form of pulmonary hypertension known as CTEPH — chronic thromboembolic pulmonary hypertension.

There is a lifesaving treatment for this type of PH, which is an extensive open heart surgery to remove old blood clots from the lungs.¹

I had a series of tests done at UCSD, and I was in recovery waiting for the results of my right heart cath — a right heart catheterization. This is an invasive procedure where they measure blood pressure and oxygen in your lungs and the right side of your heart.²

The doctor finally came in and told me that I was no longer eligible for the much-anticipated surgery because I was in heart failure and, simply put, would not survive if they followed through.

A glimpse of hope extinguished

At that moment, my whole world came crashing down again, now for a second time. The first time was just 2 months prior when my daughter passed away at 8 months old from her own complications with pulmonary hypertension. This surgery was the only glimpse of hope I had to look forward to, and it had just been extinguished.

I stayed overnight, lost over a liter of fluid thanks to IV diuretics, and tried to figure out how I could possibly pick myself back up from this.

Increased difficulty with daily activities

During this visit, I found out that the shortness of breath and chest pain I started to complain about in 2019 were actually the first signs of pulmonary hypertension that went undiagnosed for 3 years.

After this trip, I was officially diagnosed with pulmonary arterial hypertension (PAH) secondary to Sjögren’s syndrome. I had had Sjögren’s for years, and I had no idea pulmonary hypertension even existed. I had complained about these symptoms for years and found myself having increased difficulty performing routine activities on a daily basis. Those activities looked like talking for a prolonged period, walking up stairs to my front door, and demonstrating exercises to my physical therapy patients — really nothing outlandish here.

I didn't think I would have a rare, life-threatening disease

I had every test in the book — echo, stress echo, pulmonary function test, exercise test, CT scans, and endless labs. I had every test except for the gold standard diagnostic test for pulmonary hypertension: the right heart catheterization. All results led to something wrong in my lungs, but no diagnosis was ever found.

At the time, I was too busy to be sick. I acted upon my evolving symptoms, but doctors told me I was fine, so I didn’t question it further. I have an autoimmune disease that brought me unusual symptoms from time to time, and that was the given answer. Not once did I think I would have a rare, life-threatening disease.

My shortness of breath was most prominent, followed closely by my unnerving chest pain. These symptoms continued to wax and wane for years until I found myself in an ICU bed, recovering from a PE and getting the diagnosis of pulmonary hypertension.

Have you been diagnosed with pulmonary hypertension? What symptoms led to your diagnosis? Share an experience with us in the comments below.