A greater focus on respiratory conditions and new research could help us better manage diseases like pulmonary fibrosis.
September is Pulmonary Fibrosis Awareness Month, a time to drive awareness of pulmonary fibrosis (PF) and its most lethal form called idiopathic pulmonary fibrosis (IPF). While PF and its symptoms are often under-recognized by patients, medical professionals like Dr. Sachin Gupta, practicing pulmonologist and Senior Medical Director at Genentech, are hopeful that the COVID-19 pandemic will lead to a greater focus on respiratory health and the impact of other diseases that affect breathing and the lungs.
IPF is a rare, chronic disease that causes scarring in the lungs, which makes it difficult for them to absorb oxygen. In IPF, the cause of the lung scarring is unknown. People with IPF may experience difficulty breathing, which gets worse as scarring spreads through the lungs. They may also have other symptoms, like a persistent dry cough and fatigue.
Because IPF symptoms are similar to other diseases, physicians perform a series of tests and scans to diagnose the condition. For example, they listen for distinctive Velcro®-like crackles in the lungs during examination in someone with IPF.
Dr. Gupta says that when it comes to getting diagnosed with IPF, sooner is better. “Lung scarring from IPF is better managed when it is diagnosed early, and many treatment options are more effective earlier on in the course of the disease,” he says.
While there is no cure for IPF, there are treatment options that may help delay disease progression. One of them is called Esbriet® (pirfenidone), an FDA-approved therapy that may help preserve more of your lung function by slowing disease progression. The most serious side effects that could occur while on treatment with Esbriet include liver problems, sun sensitivity and rash, and stomach problems.
Lessons learned from COVID-19
Dr. Gupta is hopeful that clinicians can improve the diagnosis and management of IPF using some of the lessons they’ve learned during the COVID-19 pandemic.
“One silver lining to the pandemic for people with IPF is how the initial evaluation stages are being streamlined,” says Dr. Gupta. “Many pulmonary clinics are using telehealth visits before ordering diagnostic tests. Then, the patient will come to the clinic for evaluation and to discuss their test results and potential therapies. The continued use of telemedicine could reduce the time to diagnosis and lead to earlier treatment.”
Dr. Gupta also believes COVID-19 research may help scientists advance treatments for IPF.
“COVID-19 studies are giving us a deeper understanding of immune activation and the proliferation of cells that have important implications for lung diseases like IPF. This new research will greatly enrich our understanding of how lung scarring forms and progresses.”
Finally, Dr. Gupta says the increased discussion of respiratory conditions during the COVID-19 pandemic may improve awareness of diseases like IPF and symptoms people should discuss with their doctors.
“Denial can be a strong response to unexpected changes in our health. However, it is very important to describe symptoms like breathlessness and cough to your physician if they persist for more than a few months. I also encourage people to advocate for themselves if the symptoms don’t improve over time.”
Dr. Gupta reiterated that if you suspect you or a loved one might have IPF or another disease that impacts the lungs, it’s important to talk to your doctor, since early diagnosis can make a big difference. “Getting started on treatment is a very important step toward managing your IPF,” he says.
Esbriet is a prescription medicine used to treat people with a lung disease called idiopathic pulmonary fibrosis (IPF). It is not known if Esbriet is safe and effective in children.
Select Important Safety Information
Before you take Esbriet, tell your doctor if you:
- have other medical conditions (particularly liver or kidney problems).
- are a smoker.
- are or plan to become pregnant or breastfeed (Esbriet has not been studied in these patients).
- are taking any prescription or over-the-counter medicines, vitamins, or herbal supplements.
What are the possible side effects of Esbriet?
Esbriet may cause serious side effects, including:
- liver problems. Call your doctor if you have symptoms such as yellowing of your skin or eyes, dark or brown urine, pain on the upper right side of your stomach area, bleeding or bruising more easily than normal, or increased fatigue. Your doctor will also do regular blood tests to check your liver.
- sun sensitivity and rash. When you are outside, use sunscreen (SPF 50) and wear a hat and clothes that cover your skin to avoid getting a sunburn.
- stomach problems. Esbriet may cause stomach problems such as nausea, vomiting, diarrhea, indigestion, heartburn, and stomach pain.
Your doctor may change your dose or discontinue Esbriet if side effects do not go away.
The most common side effects of Esbriet include upper respiratory tract infections, feeling tired, headache, dizziness, loss of appetite, sinusitis, insomnia, or weight loss.
These are not all the possible side effects of Esbriet.
What should you avoid while taking Esbriet?
- Direct exposure to sunlight, or light from sunlamps and tanning beds.
- Other medicines that can make your skin sensitive to sunlight.
- Smoking, which may affect how well Esbriet works.
Call your doctor for medical advice about side effects. You may report side effects to the FDA at 1-800-FDA-1088 or www.fda.gov/medwatch or to Genentech at 1-888-835-2555.
Please see esbriet.com for the full Prescribing Information, including Patient Information, for additional important safety information.