What if the Diagnosis Is Pulmonary Hypertension?
BYApril 27, 2021
Newly diagnosed with a rare and severe form of high blood pressure in the lungs, Chere McGrath initially planned to ignore her cardiologist’s advice to make an appointment with specialists at Yale Medicine.
“I was at my local hospital, where they told me they didn’t treat my condition. I live in Enfield, and I’m very territorial. I smiled politely and said OK, but I was thinking there was no way I was going all the way to Yale when I have a doctor right near my town,” McGrath recalls of that day several years ago.
McGrath’s cardiologist told her he was going to make the referral, and called Inderjit Singh, MBChB, director of the Yale Medicine Pulmonary Vascular Disease (PVD) Program. He told Dr. Singh that he had a patient he planned to discharge and refer to him as an urgent outpatient case.
“Tell me a little bit about her first,” Dr. Singh told the cardiologist. After hearing the details of McGrath’s illness, Dr. Singh instructed the physician to put her in an ambulance and send her to Yale New Haven Hospital’s Medical Intensive Care Unit (MICU).
“It’s just a wonderful story of how I met Dr. Singh. I hadn’t realized how serious my disease was,” McGrath says. “But once I met Dr. Singh, he explained everything to me, and I’m so glad I ended up at Yale.”
Pulmonary hypertension explained
McGrath, who is 61 and works in customer service for a nonprofit organization, spent two weeks in the Yale MICU as Dr. Singh began to treat her rare form of pulmonary hypertension (PH).
Pulmonary hypertension is a general term for high blood pressure in the lungs. Whereas regular hypertension affects the blood vessels throughout the body, PH primarily affects the vessels in the lungs. What’s more, PH is far less recognizable to patients and physicians compared to regular hypertension, adds Dr. Singh.
There are several different types of PH, including pulmonary arterial hypertension (PAH), which McGrath has. PAH is a chronic disease where the arteries of the lungs thicken and narrow, so the heart must work harder to push blood through confined spaces. Over time, the heart can lose its ability to pump blood throughout the body. Currently, there is no cure for PAH, but treatment can slow its progression.
McGrath had been experiencing shortness of breath to the point where she was unable to exercise—a common symptom of PAH—for about a year before she was diagnosed. “I had been about 15 pounds overweight and my primary care doctor had been telling me to exercise more, but I would get winded doing simple things,” she says.
On a trip to San Francisco with her daughter in 2018, the problem became impossible to ignore when McGrath got extremely fatigued walking up the city’s many hills. “My daughter said, ‘Something is going on here,’” McGrath says.
A month later, she couldn’t even lift a bag of heavy garbage at her house and nearly fainted. She went to see her doctor the next day and said, "I am more than out of shape." Her doctor ordered an electrocardiogram (EKG) study. When he saw the results, he called an ambulance to take her to the hospital for further heart evaluation.
There, doctors admitted her to the ICU, where she stayed for two weeks undergoing various tests before her PAH was finally diagnosed. PAH is sometimes caused by diseases including HIV and connective tissue disease, chronic liver disease, and congenital heart disease. It has also been linked to certain genes or it can be idiopathic (meaning there is no clear cause), which was the case with McGrath. Though rare, PAH affects women twice as often as men and the average age of diagnosis is 35.
PAH is often missed by community physicians, who don’t have the training and expertise to pick up its symptoms. “Often, patients go on for months or maybe up to a year or two without being diagnosed,” says Dr. Singh, adding that it is a disease that develops slowly over time. “Patients may be told that it’s asthma or COPD [chronic obstructive pulmonary disease] because of the symptoms.”
Yale’s Pulmonary Vascular Disease Program: One of just a few nationally
McGrath was referred to the Yale Medicine PVD Program because it is one of the few centers in the nation accredited by the Pulmonary Hypertension Association, and the only such program in the state. The PVD Program allows pulmonary specialists to collaborate with physicians in cardiology, rheumatology, hepatology, cardiothoracic surgery, diagnostic radiology, and pathology, making it a single destination to manage complex cases. Depending on how serious the condition is, treatment options include medications taken orally and those delivered intravenously through a continuous pump.
At the time, however, McGrath, a married mother of three and grandmother of five, had no idea how complicated her disease was or how much her life would change because of it.
There are four functional classes of PH disease with class 4 being the most severe, Dr. Singh explains. When he first met McGrath, she was already at class 4. Because of that severity, Dr. Singh put her on two oral medications and a continuous IV medication delivered through an external pump. Today, McGrath wears the pump, which is about the length of her smartphone, at all times on the side of her body. The pump has a 4-foot IV line that attaches to a catheter in her chest and delivers medication to a vein near her heart. McGrath receives the medications by mail and puts them in her pump.
“This was a life-altering change for me,” McGrath says of the PAH diagnosis and treatment. “Knowing that I would have to wear a pump for the rest of my life was hard. I’m a young 60 and when Dr. Singh told me, I just burst into tears. I thought because I had a family history of heart problems that maybe the worst would be a stent. I had no clue, and this was a long haul.”
Part of what helped, McGrath says, is how personable and helpful Dr. Singh was. “He drew pictures of the left and right sides of my heart and explained how the pump would work. He stood right there while I composed myself, and he answered all of my questions and spoke to my daughter in California over the phone,” she says. “And now, at every appointment, he asks how she is doing.”
Adjusting to an IV pump
Life soon became more manageable, McGrath says. “I went from being very sick and very anxious about everything I was feeling to suddenly feeling better,” she says. “I didn’t have to worry about being out of breath. At work, I spend eight hours a day talking on the phone, and I was able to do it. I went from hating my pump to considering it my friend. I even dress it up in designer bags.”
In order to shower, she has to cover her catheter site with tape, which also means she can no longer go swimming. But she says it’s worth it for the increased energy she now has. “I feel 80% better than I did before,” she says.
Initially, McGrath had check-ups with Dr. Singh monthly, but as her condition improved, they were able to move to every three months. Her condition has improved to the point where it is now considered functional class 1, Dr. Singh says.
“For Chere, getting from severe disease to now having no limitations is quite a turnaround,” he says. “And from first being told what this disease even is to being a patient advocate herself is very rewarding to see.”
These days, whenever a Yale PVD Program patient is apprehensive about starting IV therapy, Dr. Singh and his team reach out to McGrath and ask if she’d be willing to talk to the patient.
Without hesitation, McGrath always agrees.
“I remember when I was first diagnosed, Dr. Singh asked me to talk to another woman with the disease. She changed my life—and my perspective. She is several years younger than I am, and so positive,” McGrath says. “She explained everything and gave me hope. We have remained friends—she is the one who sent me my first designer bag to make carrying this pump around a little more pleasurable. Since then, I am now doing the same for other people. Dr. Singh is a natural matchmaker for those of us who are in need.”