'I Was Diagnosed With Pulmonary Arterial Hypertension At 25—This Was the First Unexpected Symptom I Experienced'
Katrina Berry was 24 years old when she first started experiencing symptoms of pulmonary arterial hypertension (PAH), a progressive health condition that leads to reduced cardiac output and right heart failure.
PAH affects between 500 and 1,000 Americans each year. It’s rare, but it’s fatal. Here, Berry shares what her initial symptoms were and the long road to finding the current treatment that has changed her life.
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The Initial Symptoms of Pulmonary Arterial Hypertension
As a healthy 24-year-old, Berry, who is now 29, was surprised when she started finding herself winded after activities she was used to doing effortlessly. “I was always the fastest walker in any group and would run up a flight of stairs,” she says. Suddenly, she couldn’t do that anymore. She couldn’t understand it. She had a healthy diet, having been vegan for the last 10 years, and didn’t have a history of health problems. What was going on?
“I had significant shortness of breath, my resting heart rate was 125, stabbing chest pain and was coughing up blood,” she says of her initial symptoms. Doctors—and she consulted several—told her she had asthma and gave her an inhaler.
“No one believed me when I told them I was sick. I knew there was something wrong with me,” Berry says. Despite her worrisome symptoms, Berry, by now 25, decided to head to Greece for six weeks to volunteer at a public pediatric hospital. One morning three weeks into her trip, she started experiencing nausea and became unable to breathe, gasping for breath. She truly felt like she was going to die. “At that moment, I knew I was having a heart attack and was completely alone,” Berry says.
Fortunately, her symptoms subsided and when she was able, she called her mom who arranged for Berry to fly home the next day.
Finally Getting a Diagnosis
When Berry arrived Stateside, her mom took her directly to the hospital. Her oxygen had dropped to 82% and the doctors were extremely concerned. A CT scan showed dozens of blood clots in her lungs causing very severe heart damage. “The cardiologist said I should not have survived that flight with dozens of blood clots in my lungs and that my level of heart damage wasn’t typically found until autopsy,” Berry says.
Berry was put on blood thinners and underwent a highly specialized open heart surgery called pulmonary thromboendarterectomy (PTE) to remove the blood clots from her lungs. “When they opened me up, they didn’t find the clot burden they were expecting. Instead, my arteries were collapsed. They also found a hole in my heart,” Berry says. This is when doctors finally realized she had pulmonary arterial hypertension, a far cry from the prior misdiagnosis of asthma.
While in surgery, she went into cardiac arrest and was placed on ECMO, the most advanced form of life support. During this time, the doctors still could not close up her chest, which was packed with medical-grade stuffing and wrapped in a material similar to Saran Wrap. After five days, they were able to take Berry off ECMO and close her chest. She woke up a week later not knowing what had happened.
Katrina Berry
It was a month before Berry was discharged from the hospital. There is no cure for PAH, but there are treatments that reduce symptoms. Though stable enough to go home, she was given an oxygen tank, which she had to be connected to and bring with her everywhere, told she would have to be on it the rest of her life. She also had to stay connected to an IV medication that threaded directly into her heart, pumping the medication there directly. “If anything happened to this med pump, I had about three hours to get to the hospital before the situation became fatal,” Berry says. She also continued the blood thinners, which she said had horrible side effects.
Her calendar was full of doctor's appointments, including seeing a pulmonologist weekly (and eventually every other week) as well as physical therapy since she had lost much of her muscle mass in the hospital. “I had to teach my muscles how to walk again,” Berry says.
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The Clinical Trial That Changed Her Life
Though she was home and had machines and medication to help manage her symptoms, Berry was not getting better. “I could feel my body become weaker. I was truly dying,” she says. With nothing to lose, she enrolled in a clinical trial for a new experimental drug for people with PAH called sotatercept. The drug works by inhibiting proteins called activins that cause blood vessel walls to thicken, which then causes the heart to work harder. The medication helps blood vessels relax so this doesn’t happen.
After a couple of months of receiving the injection medication every three weeks, Berry’s body was remarkably stronger. “I was still hooked up to oxygen, but I could go on walks and some very minor hikes,” she says, adding that before starting the medication, it was difficult for her to even put her laundry away.
Nine months after starting the medication, Berry was able to come off the IV medication and switch to an oral form of it. “That completely changed my life and was the first step toward me getting my life back,” she says. She was also able to come off the oxygen machine, no longer having to bring it with her everywhere she went. (Though she says she still needs it from time to time, such as if she’s doing a strenuous hike.)
It’s been more than two years now and Berry says she’s back to solo traveling, having gone back to Greece and even zip-lining in Pakistan. Berry still has PAH and says it impacts her every day. Before starting sotatercept, Berry was only given a few years to live. While the medication is not a cure, it has extended her life and greatly improved her quality of life.
Sotatercept became FDA-approved earlier this year under the brand name Winrevair, produced by Merck. Berry is a patient consultant for Merck but has not received any compensation for sharing her story here, or other places. Besides Berry, 322 other people participated in the clinical trial, either receiving sotatercept or a placebo. After six months, 5% of people taking the medication had died or their PAH had worsened. In the placebo group, 26% of people had died or their condition had worsened.
Berry knew something was seriously wrong with her long before doctors did, serving as an important reminder of how important it is to be your own health advocate. It bears repeating that PAH is rare, but because it’s so serious, it’s important to know what its initial symptoms are (fatigue, shortness of breath, chest pressure or pain, dizziness or rapid heartbeat) so if you are experiencing them, you can seek medical care ASAP. Remember, no one will ever know your body better than you.
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Sources
Pulmonary Arterial Hypertension: The Clinical Syndrome. Circulation Research. 2015
Learn About Pulmonary Arterial Hypertension. American Lung Foundation
Pulmonary Thromboendarterectomy (PTE). Cleveland Clinic
Pulmonary hypertension. National Health Service
Phase 3 Trial of Sotatercept Treatment of Pulmonary Arterial Hypertension. The New England Journal of Medicine. 2023
Winrevair. Merck.
What are the symptoms of PAH? PAH Initiative
