About 10 years ago, at the age of 22, Evans was putting in 70-hour weeks as a pastry chef when she started feeling breathless and unusually tired. Over the next 11 months, she visited an allergist and five other doctors, all of whom gave her different diagnoses, including the old standby “It’s all in your head.” By then, she says, “I was so sick I could hardly speak without stopping to catch my breath.” Finally one of her doctors ordered a stress echocardiogram, which involves gathering ultrasound images of the heart before and after a six-minute walk on a treadmill. But Evans never made it to the six-minute mark. “I passed out and turned blue three minutes in,” she says. She was placed on epoprostenol sodium (Flolan), which she says immediately improved her breathing. “I didn’t realize how sick I was until I could breathe again,” she says. However, Evans remained on the drug for only six months because of a host of complications. She had nausea, aches and pains, and infections in the catheter feeding the drug to her heart. Since then, she has tried many of the medications approved to treat pulmonary arterial hypertension (PAH). “I’ve been on a number of other therapies — inhaled, subcutaneous, various pills,” she says. Of the 12 current approved drugs for PAH, “I’ve taken seven of them — soon to be eight!” She’s also beaten the odds, living longer than her doctors first predicted. In the year after Evans’ pulmonary hypertension diagnosis, she says, “I was excited just to wake up every morning. I was never given any expectation that I’d live past two years, so I just focused on a day at a time.” With the approval of multiple new treatments and more in the pipeline, Evans says she feels more able to look toward the future. “Instead of just making it through the day, I worry about my long-term health, my emotional health, and, most importantly, being happy,” she says. “Those weren’t things that were even on my radar my first year in.” With so many more options available now, she says she advises others to be proactive about their treatment and ask not only about other medications that might be more effective but also about pulmonary rehabilitation and exercise. RELATED: Your Pulmonary Hypertension Care Team
Rising to the Challenge of Pulmonary Hypertension
Pulmonary hypertension is a progressive, incurable disease, but Evans warns that it’s crucial to avoid succumbing to self-pity or despair. “The most important thing is to be optimistic and to have a strong-willed personality,” she says. Evans still has enough energy to attend the live music concerts she loves. She requires a handicapped sticker on her car and laments the outraged stares she sometimes gets from passers-by. “It’s hard because you look perfectly healthy,” she explains. “People just have to take your word for how sick you are — that’s probably the hardest part of it.” Evans’ passion now revolves around building awareness and improving the patient experience. “I started with fundraising and awareness, holding two local benefit concerts and getting an article published in the local paper,” she recalls. Because she felt strongly that young adults like herself needed dedicated awareness programs, she helped create and served on the advisory board for Generation Hope, an online support group for patients in their twenties and thirties. Evans also speaks publicly at conferences about her experience and mentors other patients online. She has received one of 13 Tom Lantos Innovation in Community Service Award grants, which she’s using to develop an information and resource handout to give to people newly diagnosed with pulmonary hypertension. “One of my biggest concerns has always been patients’ diagnosis experiences and how we can make them less traumatic,” she says. “Thanks to the Lantos grant, I was able to create small notepads where each sheet is printed with the Pulmonary Hypertension Association’s most important online resources. My hope is that doctors and nurses will give these to new patients or patients who need a little extra support.” Evans says she hopes those pads will direct people to sources of support more quickly after diagnosis. The notepads are available through the Pulmonary Hypertension Association’s website.
Pulmonary Hypertension Prognosis
“Pulmonary hypertension is progressive and it’s non-curable,” says pulmonary hypertension expert Samuel A. Allen, DO, director of the Pulmonary Hypertension Center at Beaumont Hospital in Troy, Mich. However, he adds, with the correct treatment, people living with pulmonary hypertension can improve their quality of life and slow the progression of the disease. If you have symptoms, such as shortness of breath that occurs without strenuous activity, be proactive about seeking a diagnosis and getting treated by health professionals with expertise in managing the condition.
