Julie Mizrahi at Bryce Canyon National Park
In September, Julie Mizrahi visited six national parks with her companion, Milo, by her side. Milo was there as she walked for hours in Zion National Park; rode a horse down 2,000 feet into the bowls of Bryce Canyon; hiked at the north and south rims of the Grand Canyon; and took a helicopter ride over Canyonlands National Park.
For Mizrahi, a 42-year-old auditor and artist in Delaware, it was a thrilling feeling to conquer the mountains, canyons, and sudden elevation changes. The last time she visited those parks, before she had Milo, she felt so sick she couldn’t get out of the car.
Milo isn’t a partner or a pet. It’s the name Mizrahi calls the three-pound pump that sits in a pack around her waist and delivers a continuous intravenous (IV) infusion of medication, through a tube in her chest, to treat her rare lung disorder. It will stay with her unless she receives a lung transplant.
“It’s like a whole new life, having this pump,” Mizrahi said. “It’s keeping me alive, so I feel like I have a very close relationship with that machine.”
All her life, Mizrahi had been more active than most people. When she wasn’t traveling internationally for her job, or taking trips for pleasure with friends and family, it was nothing for her to run 25 miles a week and spend hours gardening, painting, cooking, or organizing her home.
Then in 2012, she began to experience breathlessness, headaches, swelling in her legs, and frequent fainting spells. She continued to travel, occasionally fainting at famous sites including the Christ the Redeemer statue in Rio de Janeiro, the Great Wall of China, and the Old City neighborhood of Jerusalem.
By 2018, she was passing out with the slightest physical exertion. Her local doctors couldn’t figure out what was wrong, and the mysterious illness was taking a toll.
“I’d go outside to mow the lawn, and after 10 seconds, I'd go into the garage to pass out. Then I'd come back and drink some water and I'd go back out, continue, and pass out again,” Mizrahi said. “Finally, I had to stop mowing the lawn. I couldn’t even make my bed in the morning because if I leaned over I would get this intense headache and I would have passed out.”
Finally, after passing out and hitting her head in the Frankfurt Airport in Germany, she got some answers from Steven Kawut, MD, MS, director of the Penn Pulmonary Hypertension/Pulmonary Vascular Disease Program. Kawut diagnosed Mizrahi with idiopathic pulmonary arterial hypertension, a rare disorder characterized by high blood pressure in the arteries of the lungs. Over time, this can cause the right side of the heart to become larger and can lead to heart failure.
“You don’t look sick from the outside, so physicians can be fooled by the fact that this person is saying they have shortness of breath and they look totally fine,” Kawut said.
Managing Pulmonary Arterial Hypertension
In Mizrahi’s case, the disorder is idiopathic, meaning the cause is unknown. While there is no cure — except lung transplant, which has its own risks and benefits — several treatments for severe pulmonary arterial hypertension, including oral, inhaled, subcutaneous, and IV, or pump-infused, medications, have become available in the last two decades.
“Researchers found that people who have this condition have low levels of a molecule called prostacyclin, which opens up the vessels and has some other features,” Kawut said. “The pump infuses a medicine which imitates and replaces that molecule.”
Kawut has seen patients who have been on pump therapy for 10 to 15 years or more.
Mizrahi named her pump Milo after the Nestlé chocolate drink mix she loved as a child in South America. Whenever she drank it, she convinced herself she was powerful and full of energy.
As soon as Mizrahi learned how to prepare her medication and keep her IV line clean, she was ready to get back to traveling. But traveling on pump therapy is no minor feat. For one thing, the medication, while life-saving, also has side effects, including leg pain, nausea, and jaw pain with the first bite of food. Then there are the supplies. Along with a wheelchair, which Mizrahi uses if she’s having pain from the medication, for a two week trip she must pack two big duffel bags with all of the supplies to mix her medication, plus two back-up pumps. If her pump malfunctions, the medicine has a short half-life and symptoms can return quickly.
“She wanted to go to Switzerland six weeks after she got the pump, and we said, ‘You have to give it some time,’” Kawut said. “But that’s Julie, and we don’t want to hold her back. Our whole approach to the care of our patients is not to make them into somebody else, but to return them to their usual lives as much as possible.”
She did take that trip after reassuring Kawut that she would line up a specialist in Zurich in case anything went wrong.
Mizrahi credits Kawut and Nurse Practitioner Christine Archer-Chicko, MSN, CRNP, with saving her life, but there’s a whole team of physicians, nurse practitioners, nurses, pharmacists, technicians, medical assistants, coordinators, and a social worker who all come together to provide her care.
Returning to a Life of Adventure with Pulmonary Hypertension
Mizrahi often thinks about the days, prior to her diagnosis, when she could barely walk five to 10 steps without feeling like she was going to pass out — or actually fainting.
“Within one and a half years, Milo helped me start walking again, and since June 2021, I've been walking five miles every day in the woods. Even when traveling I make sure that I get the five miles in, whether outside or on the hotel gym's treadmill,” she said. “Being able to move this much is freedom at its fullest. So much so, I forget at times that I have lung disease."
To Kawut, Mizrahi’s story shows the Penn Medicine team’s ability to help patients with pulmonary hypertension live life on their terms, whether they want to hike the Grand Canyon, like Mizrahi, or simply want to keep working or play with their grandchildren, like some of his other patients.
“Here’s somebody who, despite being dealt an illness, is living a full life,” Kawut said. “Julie can’t do everything she wants to do, but she can do a lot. She’s incredibly positive, motivated, smart, talented, and funny. Her story is a nice example of how at Penn, we’re equipped to help people live their best lives, even with health challenges.”