Monitoring for Disease Progression of Pulmonary Fibrosis

Pulmonary fibrosis is a group of lung diseases in which the area around the air sacs of the lungs have scarring. The scars or the fibrotic tissue block the movement of oxygen from the air sacs of the lungs into the capillaries or small blood vessels that surround the air sacs. This results in low blood oxygen levels and can cause people to feel short of breath. 

Progressive lung scarring means scarring of the lungs becomes worse over time. It causes the lungs to become thick and stiff, making it harder and harder for oxygen to pass from the air sacs into the bloodstream. This damage is thought to be irreversible and the lungs’ ability to function normally is permanently affected. 

However, the clinical course of pulmonary fibrosis is highly variable among individuals. In some, lung fibrosis can progress very rapidly. In others, progression can happen very slowly. In still others, it may stay stable over a long period of time. Pulmonary fibrosis can also be complicated by episodes of a sudden decline in lung function, known as acute exacerbation. Because disease progression is often difficult to predict, monitoring progression is an important part of managing the disease. 

Your pulmonary fibrosis is said to be progressing if two of the following three criteria are met:

1. Worsening symptoms – that is if cough, shortness of breath, fatigue etc. are increasing in frequency and/or duration. It is important to monitor if your breathing is becoming more difficult, if you have an increased demand for higher flow of oxygen, you are coughing more, feeling more anxious or depressed etc. 
2. Worsening pulmonary function test results – This is physiologic evidence that your condition is progressing. A reduction in Forced Vital Capacity (FVC) of 5% or a decrease in Diffusing Capacity of the Lung for Carbon Monoxide (DLCO) of 10% over a period of one year indicates progression
3. Worsening findings on chest CT – This shows radiographic evidence of progression

Some patients do not fully recover after an event of acute exacerbation. In such cases, it becomes more important to monitor for heart failure, pulmonary hypertension, or high blood pressure in the lungs and other diseases. 

Ready to take action?

1. Learn more about measuring progression here: 

• How to monitor & measure:
  • https://pulmonaryfibrosisnow.org/2019/05/22/how-quickly-does-pulmonary-fibrosis-progress/
  • https://pulmonaryfibrosisnow.org/2021/07/11/monitoring-inflammation-in-your-body/ 
  • https://pulmonaryfibrosisnow.org/basics-old/how-to-measure-your-progress/#measure
• Recordkeeping:
  • https://pulmonaryfibrosisnow.org/2021/10/13/recordkeeping-for-patients-with-pf-part-1/
  • https://pulmonaryfibrosisnow.org/2021/10/27/recordkeeping-for-patients-with-pf-part-2/
  • https://pulmonaryfibrosisnow.org/2022/03/31/recordkeeping-for-patients-with-pf-part-3/
• FAQs:
  • https://pulmonaryfibrosisnow.org/2021/07/31/faqs-about-pf/ 
  • https://pulmonaryfibrosisnow.org/2021/08/25/faqs-about-pf-part-2/ 

2. Learn more about acute exacerbation here:

• https://pulmonaryfibrosisnow.org/2019/10/04/what-is-acute-exacerbation-of-idiopathic-pulmonary-fibrosis/
• https://pulmonaryfibrosisnow.org/2019/09/18/causes-symptoms-prognosis-treatment-acute-exacerbation-ipf/

3. Learn more about heart health in the winter here:

• https://pulmonaryfibrosisnow.org/2021/12/24/heart-health-in-the-winter/

4. Be sure to also sign up for our upcoming support group meetups and to join our online community forum for 24/7 support. 

Stay In Touch

PF NOW! hosts virtual support groups on Teams multiple times each month. Not only will you strengthen your network of connections but you’ll learn firsthand how those with PF best look after themselves. PF NOW! also has a Facebook group whose active community shares their PF journey and their tips with others. New to online support groups? Download our free step-by-step guide for online advocacy here.

Never disregard professional medical advice or delay in seeking it because of any information received from us.