JoAnn Volpe and family
Jeannette Morrill initially thought she would never do anything useful or meaningful after her pulmonary hypertension (PH) diagnosis in 1976. Then 23, she was told she had six months to live. Doctors told her parents to make her as comfortable as possible.
But Jeannette wanted to make the most of the time she had left. “I decided that I would make sure I did something every day,” she recalls. “It didn’t matter how I was feeling. Even on my worst days, I would at least make sure I got up, got dressed and got out of my bed.”
In 1987, she and her husband David adopted two sons, Brian and Ian.
She found a doctor who told her, “If you are willing to try, I will do everything I can to help you survive until, perhaps, some new medication, comes along.”
In 1995, a medication did come along. The Food and Drug Administration approved Flolan, or epoprostenol. It has transformed Jeannette’s life and the lives of thousands of other people with PH since then.
“It was a complete game changer,” says Murali Chakinala, M.D, chair of the Pulmonary Hypertension Association’s Scientific Leadership Council. “It provided the first real hope that PH patients had.”
Kevin and Jeannette Morrill
Targeted therapy
Before Flolan, there was no targeted therapy for PH. Physicians prescribed vasodilators, blood pressure medication and other drugs that worked on arteries throughout the whole body, rather than arteries in just the lungs. For many patients, a lung transplant was their only hope.
Flolan was different. Administered through a catheter placed surgically into a large vein in the chest, it targets arteries in the lungs. It relaxes and dilates narrowed blood vessels, increasing blood supply to the lungs and reducing the workload of the heart. A small battery-powered pump keeps the medication flowing continuously into the body.
“People literally were able to get out of bed and start living again,” Dr. Chakinala says.
No longer couch-bound
In 1992-93, JoAnn Volpe was the head secretary of a Connecticut high school. At 44, she had no energy. She huffed and puffed going to and from the parking lot. She passed out trying to shower. Her daughter worried so much about her that she didn’t want to go away to college.
Eventually, JoAnn was diagnosed with PH and had to leave the job she loved. “I think they thought I was going to drop dead on their premises,” she says.
After her diagnosis, a doctor at Hartford Hospital referred her to the University of Pittsburgh Medical Center, where she was evaluated for a heart and lung transplant. During her two-week stay, a heart doctor told her about a clinical trial for a new drug, Flolan. JoAnn decided to join the trial if it meant she might not need a transplant.
She returned to Connecticut with a central line and the ability to properly mix and store her medication. “I thought I was too old to learn how to do it,” she recalls.
Twenty-six years later, she still receives IV therapy, but she’s since switched to Remodulin, which is pre- mixed and doesn’t have to be refrigerated. The drug eliminated the need for a transplant.
“What a difference,” she says of the drug that saved her life. “I can breathe without problems. I only use oxygen at night. I used to be couch-bound.”
New opportunities
Jeannette was practically couch- bound as well. Like JoAnn, she had to quit her job teaching physical education. But after she started taking the calcium channel blocker Nifedipine, which controls blood pressure, she felt well enough to return to teaching. She became a classroom teacher, instructing fifth graders on a variety of subjects.
Eventually, the Nifedipine lost its effectiveness.
“I was like I went back in time,” Jeannette says. “I couldn’t do stairs. I got very short of breath doing anything that required physical exertion.”
In 1997, Jeannette was referred to Robyn Barst, M.D., who told her about Flolan. After many tests, including a heart catheterization, Dr. Barst told her she was a good candidate for Flolan.
In April 1997, a Groshong catheter was inserted in her right chest, and Jeannette began using the CADD pump for constant infusion. She stayed on Flolan until September 2006.
“I was so happy to find that there was something else that would help me,” she says.
Game changer
The therapy was equally remarkable for health care professionals. “You now had therapy that made people feel better, that turned PH into a treatable condition,” Dr. Chakinala says.
When Jeannette starting taking, Flolan, she found that she could do more than just get up and get dressed.
“I started challenging myself. I started walking from one telephone pole to another until I was walking a mile on a regular basis.”
Her shortness of breath gradually decreased. She got a cart and started golfing almost every day. She taught her nieces and nephews how to play four-square, badminton, basketball and soccer. “I was able to play with them without having to worry about fainting in front of them,” she says.
Although she had to stop teaching to protect her immune system, Jeannette tried to get back to the many things she had lost before Flolan.
“Did I ever think I would get back to normal, like before PAH? No, but I was going to try very hard to balance activity, rest, family, household chores.”
Critical care element
Twenty-five years later, epoprostenol and treprostinil remain the most potent, effective PH treatments, Dr. Chakinala says.
“We still rely on them for the sickest and most compromised patients,” he says, noting that scientists and researchers continue to develop more convenient delivery methods with less risks. “That class of drugs is not going away. It’s such a critical part of our patients’ care management.”
Meanwhile, Jeannette continues trying to live well with PAH, accepting what she can’t change and working to change what she can.
Today, Jeannette and David live in Old Orchard Beach, Maine, where she leads a PHA support group, golfs and spends time with her 7-year-old grandson son, Kai, her “joy.”
“Flolan really saved me a second time from death,” she says. “I think of all the other patients it has helped and feel very blessed.”
This story appeared in the December 2020 issue of Pathlight, the Pulmonary Hypertension Association (PHA)’s quarterly member magazine. Pathlight is a benefit of PHA membership. To read more inspirational PH stories and receive a copy of the magazine, join PHA.
