Remaining hopeful through a difficult diagnosis
After a PF diagnosis, we must educate ourselves, prepare, and stay resilient
As my husband Donnie’s diagnosis of pulmonary fibrosis (PF) slowly unfolded, I found myself oscillating between anger, sorrow, and disbelief. At the time his symptoms appeared, he was only in his 30s. He had an extremely labor-intensive job, walked over 10,000 steps a day, and was incredibly active. He spent weeknights and weekends coaching our daughter’s elementary basketball team, and we were just a typical, busy family of four.
Somewhere between the onset of his symptoms and his diagnosis, I stumbled upon an article about PF. Since Donnie was exhibiting many of the symptoms of PF, I kept reading. When I read the prognosis of a three-to-five-year survival rate, I felt physically ill. How could this be happening to my husband? How could this be happening to our family?
Even though doctors initially suspected asthma, I knew something wasn’t adding up. I often found myself reading articles and real-life stories about PF, which gave me the courage and knowledge to help my husband push for an accurate diagnosis.
Relief is possible
For starters, the prognosis was merely a prediction of what might happen without treatment. It’s also a median figure, meaning some people may live for a shorter time, while others may live much longer. Because PF is considered a rare disease, many people wait years before receiving an accurate diagnosis, which I believe contributes to the lower survival rate, as many individuals do not get the care they need in a timely manner.
Some people with PF can achieve long periods of stability in their lung function, especially with the advent of medications such as Ofev (nintedanib), Esbriet (pirfenidone), CellCept (mycophenolate mofetil), and the newly approved Jascayd (nerandomilast).
Secondly, a prognosis is just an educated guess. It’s somewhat like watching the weather. We can see a storm brewing on the radar, but we don’t know exactly what will happen until it arrives. Sometimes, the weather forecaster is spot on; other times, they are not.
My husband’s PF has remained mostly stable since he started Ofev about two and a half years ago, although doctors debate whether a slight progression may have occurred. We know this is a progressive disease, but I’ve read stories of many people whose PF has remained stable for years, even decades. The only thing we can do is educate ourselves, prepare, and stay resilient.
Thirdly, one of the most concerning things I read when my husband first got sick was that he would never feel better than he did at that moment. I can tell you that has not been true in his case. While PF isn’t currently curable, it is treatable, and relief from symptoms is possible.
Donnie feels so much better today than he did three years ago when his symptoms were untreated and uncontrolled. In 2021 and 2022, his cough was relentless and violent, and severely affected his sleep and daily activities. Today, his cough is under control and typically only bothers him during times of illness or allergy season. He can sleep, works full time in a career he loves, and remains an engaged and involved parent. He hasn’t stopped living the life he wants, and he has no intention of doing so.
Lastly, it’s 2025, and medical treatments are evolving more rapidly than ever before. Earlier this year, our young daughter was diagnosed with type 1 diabetes. Just over 100 years ago, that would have been a death sentence. Today, thanks to modern medicine, years of research, and the experiences of all the type 1 patients who came before her, she lives a happy, healthy, and active life.
Who’s to say that PF won’t experience the same kind of rapid evolution in the near future? Until then, my family chooses to remain thankful for the available PF treatments and hopeful about the advancements that are sure to come. A diagnosis of PF will change your life, but life is for the living, and my husband intends to live it to the fullest.
Note: Pulmonary Fibrosis News is strictly a news and information website about the disease. It does not provide medical advice, diagnosis, or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website. The opinions expressed in this column are not those of Pulmonary Fibrosis News or its parent company, Bionews, and are intended to spark discussion about issues pertaining to pulmonary fibrosis.
About the Author
Karl
Articles that describe a 3 to 5 year survival rate do a terrible disservice to people entering the discovery phase of PF, or in my case IPF. I am fortunate to be a 7 year (so far) survivor with no intention of allowing this disease to take me down. At 88 my attitude remains positive which I believe is the key to minimizing the effects of Pf. In my opinion COVID is a worse threat to life.
I what makes me feel terrible is the fact there are so many with PF that are so many people much worse off than me.
Kylene Henderson
Karl, I love to hear that you are doing well and are maintaining a positive attitude! I love to hear stories like yours, as they are so encouraging to me, my husband, and our family. Take care!
Jane
Hi Kylee, my name is Jane and I read your post! I am glad to know your husband is doing well! I am in NC and have PF, too! You are going to be surprised that I have had it about 11 yrs! Don’t understand how I have had it this long, and no treatment for it until this past Feb! Of course, mine was slow to progress, that what I was told! Have PFTs every 3-6 months and seem to be stable!? I am a retired OR nurse, just turned 79, and I never stopped! Retiring at the age of 70, I was fine! As time moves on, still hoping to stay stable, not your regular 70ish lady, but with hope, prayers, and extended research this terrible diagnosis will become a thing of the past! Please keep living your lives, and enjoy each day! Look at me! 11 yrs—- it can happen! Just been on OFEV since Feb and was on Cellcept, as well, but the both together was tooo much! OFEV for me, has really bothered my GI tract, so I am on a lower dose of OFEV to see if that helps! Trial and Error, the name of the game! Keep in touch— Good Luck!
Kylene Henderson
Hi Jane! I love to hear that you are doing so well! Donnie is on a similar schedule - he gets his PFTs every 6 months (sometimes more often), and so far they've remained stable. He's also taking CellCept and OFEV, and we're very thankful that the medicine seems to be working. Thank you so much for the encouragement, and take care!
Catherine Berntsen
My prayers for you and your family dealing with a dreadful disease at such an early age, in your prime, raising a family. My husband developed PF at age 75; probably had it for years without being diagnosed. Where it came from no one knows; he never had any pulmonary issues but he did work as an auto mechanic for many years and was the supervisor of the NYC DOT shop where the trucks laden with dust and debris from the World Trade Center clean up were taken to be repaired. We don't have an answer. He was on Esbriet but i don't think it did anything. Not much available at the time. He died in Jan 2021. Seems like medicine has more to offer you now.
Kylene Henderson
Thank you, Catherine, and I'm so sorry to hear about the loss of your husband. Donnie also worked in the collision industry for many years, and we initially thought his disease might be linked to some sort of chemical or dust exposure. In the end, testing determined it wasn't related. We're thankful for the medicine available, but of course hope the future will bring even better treatments.
Jim Connors
I see negative AI in more segments of daily events.It is scary to depend on it.
Dulecia Lunde
Hi there
I was diagnosed in 2014 with ILD and lupes after the anaethetist failed me for surgery. I stubbornly stayed on at work went from full to half days. One day I collapsed at work and spent more than a month in hospital. Lupes has affected my brain, lungs, heart and skin. I signed up online to support groups and Googled for more information for knowledge is power. After the long stay in hospital and seeing the fear in my husband and 2 kids eyes, I decided to leave the job I loved. I take it one day at a time and my faith kept me positive and strong to encourage others. I am also in a wheelchair due to medical negligence that they are still not admitting to but rather gaslight me instead. But God! Presently Im looking for a remote position and I believe it will find me. Strongs! 🤗 ❤️ 🙏