The importance of monitoring symptoms with pulmonary fibrosis
My husband must be vigilant about any new or worsening issues
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Note: This column describes the author’s husband’s own experiences with anti-fibrotic medications. Not everyone will have the same response to treatment. Consult your doctor before starting or stopping a therapy.
For those of you who haven’t read this column before, my name is Kylene, and my husband, Donnie, was diagnosed with pulmonary fibrosis (PF) in late 2022. While idiopathic PF is the most common type, Donnie’s lung disease was caused by multiple underlying autoimmune disorders, most notably polymyositis, Sjögren’s disease, and scleroderma. While he doesn’t have all the typical symptoms of these diseases, his blood work pointed to them as the likely underlying cause of his PF.
Polymyositis is a disease in which your immune system attacks your muscles, leading to inflammation, weakness, and in some cases, organ damage. Because it causes muscle weakness, it can also affect the muscles responsible for breathing.
Over the past few years, Donnie and I have learned the importance of symptom monitoring and its role in his treatment. When Donnie began to feel unwell in recent months, he first tried to brush it off or attribute it to different causes. When his breathing began to decline and his cough started to return, we knew it was time to address these symptoms with his doctors.
We can’t afford to wait and ‘see what happens’
At his last appointments with his pulmonologist and rheumatologist, Donnie told them he hadn’t been feeling well. It was getting harder to breathe, he was coughing quite a bit again (especially before bed), and all his muscles felt heavy and sore. He was also experiencing an increase in gastrointestinal discomfort and routinely felt bloated and uncomfortable.
We started with the pulmonologist, who reviewed Donnie’s pulmonary function test (which was stable), six-minute walk test (in which he needed oxygen sooner than in the previous test), and ordered an updated CT scan. His doctor also advised him to take a short break from the anti-fibrotic treatment Ofev (nintedanib) to see if it was causing his gastrointestinal discomfort, noting that they could switch him to the newest anti-fibrotic drug, Jascayd (nerandomilast), if needed.
While Ofev has done a great job helping keep Donnie’s lungs stable, we were suspicious that it might be causing some pretty uncomfortable digestive symptoms. Since Jascayd was the first anti-fibrotic drug approved to treat PF in over a decade, it’s a relief to have a new option, especially for those who found Esbriet (pirfenidone) and Ofev to have disruptive side effects.
The next day, we visited his rheumatologist, who reviewed his tests and stated that his blood work showed signs that his autoimmune diseases were active. She ordered a muscle test called an electromyography, or EMG, and told Donnie that, depending on the results, they might need to add another treatment to his regimen.
We left the hospital that day with treatment options up in the air, but things have since started to settle, at least somewhat. The break from Ofev did indeed seem to help with Donnie’s stomach pain and bloating, and he has since started Jascayd instead.
According to Donnie’s pulmonologist, Jascayd can be more tolerable for some people and might be a good option for those struggling with some of the same gastrointestinal issues he was. Donnie has now been on Jascayd for almost a month, and his stomach issues, bloating, and pain have significantly decreased.
Most importantly, after speaking with both of his doctors about increasing symptoms, his pulmonologist started him on a prednisone taper. The medication has slowly helped him to breathe a little better and has definitely improved his cough. We’re still waiting on the EMG test to determine the next course of action for the autoimmune diseases, but we think things are slowly moving in the right direction.
While my husband used to be a “wait and see what happens” kind of guy, it’s become increasingly important that he stays vigilant about his symptoms so that he can monitor for signs of autoimmune activity or progression. Your doctor won’t know if you need to change your treatment unless you are honest about your symptoms and up-front about how you are feeling.
The waiting game has never been my favorite, but we hope the next few weeks and months will bring us more answers (and, hopefully, effective treatments for Donnie). Until then, he’s following his doctors’ orders and trying to stay the course.
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.
Russell Rajani
came down with IPF at mid age of 83. Never smoked or worked in factories/ mines. Flew planes for 38 years. Initially tried JASCAYD for 2 months. ($6500 a bottle. Got 2 sample bottles) Can't afford high prices. No diarrhea, or other problems...except one...heavy, heavy coughing. Went to Ofev. Very mild coughing usually at night and still no diarrhea
Only use OX when needed which is usually when in motion not in resting positions or driving.