Melissa Sulpher

Melissa Sulpher, 38 years old, is not your typical person living with pulmonary fibrosis. Most are diagnosed, after several medical consultations, when they are in their 60s. Melissa’s case took quite some time to pin down, especially because of her age. Pregnant with twins when she first experienced symptoms of fatigue, shortness of breath and a cough, her doctor at first thought her breathlessness was a result of her pregnancy. After her delivery, they thought maybe it was post-nasal drip, or a sinus infection, or allergies or asthma.

“It sometimes felt like no one was listening. I would climb one flight of steps and have to sit down for at least five minutes before I could do anything else. That’s not normal,” says the former nurse.

After two years and periods of weekly doctor visits, a chest x-ray, and a referral to a Kitchener respirologist, finally led to a diagnosis of pulmonary fibrosis. At 28 years old, Melissa had just had her third child and believes mold in her older home, along with a predisposition to the disease, was the cause of her condition. After her diagnosis, her family never again lived in the house, although no one else in the family was affected.

“I was so relieved to have a diagnosis,” says Melissa. “I wasn’t crazy, or lazy, or just weirdly obsessed with breathing. There was a reason I was obsessed with breathing. It was because I couldn’t breathe!”

Melissa does not have idiopathic pulmonary fibrosis; her PF is the result of chronic hypersensitivity pneumonitis, one of nearly 200 interstitial lung disease (ILD) conditions that can lead to pulmonary fibrosis. And it affects every part of her life.

She eventually had to cut back her nursing hours to part-time, and then had to stop working altogether two years ago. Although she receives some government assistance, “it’s certainly nothing like working full-time,” she says. She’s grateful that her drug therapies are covered for now and so is most of the cost of her oxygen. Now that the treatment for her condition is approved by Health Canada, she hopes that it will also be quickly approved for coverage by the Ontario government.

Melissa finds the biggest impact has been on her family life. She and her husband Brian Heimpel have four children: 12-year-old twins Emily and Abigail; 10-year-old Thomas and seven-year-old Connor. There are a lot of things Melissa can’t do with them. “I often can’t go on field trips with them because I don’t know if there will be hills to climb or flights of stairs to go up. I can’t always walk my kids to school, and if I do, I might need a two-hour nap to recover,” she says.

If the family wants to do an activity together, for example attend a concert, they need to do a lot of planning. Melissa needs to know where they can park, how many flights of stairs there may be, and how long the concert will last. Will she have the stamina to last and time afterwards to recover?

“It changes relationships, too,” she says. “The people who love you don’t want to see you struggle to breathe. I’ll sometimes try to hide it if I can, but Brian is very observant, he knows when I need to rest or need help. And he’s super fantastic with the kids.”

What do they tell the kids? “At first, they were so little, it was just normal to them that I couldn’t do certain things. Now we refer to my condition as the ‘the black lung.’ We explain things to them as best we can, when they ask. And sometimes we don’t have the answers.”

She does her best to have as normal a life as possible. She does ferry the kids around to their activities and tries to minimize the effects of the cold and flu germs they bring home. “Our family probably has the cleanest hands going,” she laughs. While the kids recover quickly, it can take months for Melissa to recover from a cold.

She feels very fortunate to have stumbled into the care of Dr. Kolb at the Firestone Institute for Respiratory Health in Hamilton about seven years ago. “Having a rare disease comes with some unique challenges and one of them is finding experts who really know the latest research and treatment options. I can very honestly say that I receive world class care from a world class team of health professionals,” says Melissa.

In January 2018, Melissa was selected to participate in a clinical trial of nintedanib (OFEV) for patients with pulmonary fibrosis other than IPF. Recent trial results show nintedanib slowed lung function decline by 57 per cent across the overall study population. These results, and her own experience in the clinical trial, has given Melissa new hope that her slowing decline may give her time to benefit from new treatments and medical advances.

As a participant in the study, Melissa also had access to the clinical trial research coordinator, who was her go-to person with any questions or concerns, about anything. In May 2020, the drug was approved for progressive fibrosing interstitial lung diseases (PF-ILD). It is also indicated for the treatment of idiopathic pulmonary fibrosis (IPF) and to slow the rate of decline in pulmonary function in patients with systemic sclerosis-associated ILD (SSc-ILD). Along with CPFF, Melissa would like to see an ILD nurse available at the clinic, and by phone, as a patient resource and has advocated for this at an Ontario MPP reception.

“Having an ILD nurse in the clinic would be incredibly helpful,” says Melissa. “The specialists are very busy, with long waiting lists, so they are not always available. And while my questions may be simple for my ILD specialists to answer, health professionals in an ER department or walk-in clinic, or a family physician, cannot be expected to have the same level of knowledge of this complex condition. It would be a great relief, knowing there was a specialized nurse I could reach out to with my concerns.”

While she knows she may still need a lung transplant sometime in the future, Melissa feels her future is brighter than ever with progress in research and new treatments.

“I’m the kind of person that looks forward, rather than back.” says Melissa. “And right now I have a wonderful life.”

Some tips for others living with PF from Melissa

1. Take the time you need to process your diagnosis. It’s a lot to take in, for you and your family.
2. Stay as active as you can. Keeping in your best possible shape will make it easier to deal with future medical challenges (maybe even a lung transplant.)
3. Have someone go to all of your medical appointments with you. They can add their observations of your condition to your health care team and take notes about what the professionals are telling you. You cannot do it all alone. Learn to accept help.
4. Connect with others living with PF. The CPFF has support groups. You can also connect with others online. I also met some great people when taking a pulmonary rehab program.
5. Get involved in the PF cause. Fundraise or advocate, be a resource for other patients, or share your story with the media. CPFF has plenty of opportunities for volunteers. It will give you a sense of empowerment and accomplishment.
6. Live your life, the best you can each day.