Pulmonary Hypertension: A Patient’s Advice
Jan 12, 2017 Cedars-Sinai Staff
When Josefina Rodriguez felt unshakable fatigue and shortness of breath, she chalked it up to having a new baby and trying to get back into shape.
When the symptoms persisted, she was eventually diagnosed with pulmonary hypertension, a disease that disproportionately affects women in their 20s, 30s, and 40s—young women who seem unlikely candidates for lung or heart disease.
Pulmonary hypertension is high blood pressure in the lungs. Over time, this chronic high blood pressure taxes the lungs and one side of the heart, which has to work harder to pump blood through the constricted blood vessels. Over time, the disease can lead to heart failure.
The early symptoms are tiredness and shortness of breath—common complaints for many diseases, so the condition often goes undiagnosed until more serious symptoms arise. Those include chest pain, dizziness, swelling in the arms, legs and abdomen, and fainting. Because the disease is fairly rare, many doctors do not recognize it.
"If you're not responding, you should immediately seek another opinion and think about the possibility of pulmonary hypertension," said Dr. Antoine Hage, a cardiologist in the Cedars-Sinai Heart Institute and Pulmonary Hypertension Program. "Most patients have had symptoms for one or two years. They can visit two, three, four doctors. It causes delays in the diagnosis."
Rodriguez, 35, is treated with a combination of medications, a strict low-sodium diet, and mild exercise.
"I feel 100% normal," Rodriguez said. "I don't even feel like I have PH. I am able to go on walks, and I'm doing monitored cardio. I can take care of my children, which is the most important thing. I can tend to them without feeling out of breath or tired."
Josefina's advice for pulmonary hypertension patients
There is hope
Don't let your early internet explorations frighten you. Every patient is different. Therapies can be very effective for managing pulmonary hypertension, and only you and your care team can determine what's right for you. If left untreated, the disease can lead to heart failure and even death. Getting diagnosed and having an effective treatment plan leads to much better outcomes.
Trust in your doctor, not Google
"I'd never heard of pulmonary hypertension before," Rodriguez said. "I Googled it, and I freaked out." A simple web search turns up numbers that can be overwhelming and discouraging to people newly diagnosed with the disease, which has about 200,000 new cases each year. However, advances in treatment have made the disease manageable, and more new treatments are being studied. With the right combination of therapies, many patients are able to resume their usual activities and have a promising prognosis. "When I first Googled it, I thought I had an expiration date," she said. "There isn't an expiration date. Don't have one. I would tell anybody who has PH that there is hope. There are doctors who go beyond to treat their patients."
The solution is not that bad
Rodriguez was initially overwhelmed by changing the way she eats and cooks, but she said she got used to it quickly. Rodriguez separates her portion of the meal before seasoning a dish to be served to her family. She also monitors salt intake more closely for her whole family.
Rodriguez says her husband, Tony, has been crucial to managing her pulmonary hypertension. He comes to every doctor's appointment, has cut salt from his diet in solidarity, and keeps tempting foods out of the house.
Take it one day at a time, or just one hour at a time
Pulmonary hypertension patients don't know how they're going to feel tomorrow, or even later today. Listening to your body is important. "Really take seriously that you have a second chance at life and just start slow."
Advocate for yourself
It's your health. Rodriguez initially saw a cardiologist who gave her a diagnosis and promised a referral—then never followed up. She was aggressive in seeking out care. Organizations like the Pulmonary Hypertension Association pointed her in the right direction.