PF Basics: Info for Newly Diagnosed Patients

Your doctor may measure your oxygen level in different circumstances: while sitting at rest, while walking in a hallway or on a treadmill, and during sleep. If the oxygen level in your blood measures below 88%, your doctor may prescribe oxygen therapy. Your oxygen prescription will include:

Pulmonary rehabilitation (PR) is a structured exercise program designed for people living with chronic lung diseases like PF. PR includes exercise training; breathing exercises; anxiety, stress, and depression management; nutritional counseling; education; and more. It’s been found to improve physical function, breathlessness (dyspnea), mood, and quality of life in people with IPF and other types of PF.

In-person, center-based PR is considered the safest and most effective model of PR in the US. Read more on our pulmonary rehab page, watch a short video, or find a directory of in-person PR programs.

Some people aren’t able to attend an in-person PR program because of distance or cost. Although it’s not a substitute for in-person PR, the Pulmonary Fibrosis Foundation’s (PFF) Pulmonary Rehabilitation Toolkit provides some of the information and resources you’d receive through a center-based program, including exercises you can do while watching the videos.

Whether you’re planning to participate in an in-person program or explore the PR Toolkit, talk with your healthcare team before starting any new exercise routine to make sure it’s safe and appropriate for you.

A lung transplant is a life-saving treatment for people with late-stage lung disease after other treatment options have been exhausted. Lung transplantation is an important therapy for some people with PF, but with limited availability of donor lungs, most people living with the disease won’t receive a transplant.

Lung transplants are a specialized type of surgery performed at a limited number of medical centers across the country. If this procedure is appropriate for your care, your doctor may refer you to one or more of these transplant centers. The evaluation process and waiting time can take months, even years, so early referral is very important.

Transplant centers have strict criteria for being a transplant candidate, including your weight, the health of your other organ systems, your history of medical compliance, your support system, and many other factors. If, after extensive evaluation, the transplant center determines that you’re a candidate, you’ll be placed on a waiting list. The order of priority for assigning lungs is based on a lung composite allocation score (lung CAS) computed with each individual organ that becomes available. The lung CAS assigns a score of 0-100 based on several categories: the candidate’s estimated one-year survival while waiting for a transplant, the candidate’s estimated one-year post-transplant survival, candidate biology (including factors like blood type and height), whether the candidate is a pediatric patient or past living organ donor, and the candidate’s proximity and ability to travel quickly to the relevant transplant center. The average wait time for an IPF patient is 3-6 months, but it can be much longer. The transplant surgery is followed by an extensive recovery time.

Most people with PF who have successful lung transplants feel better, reporting that cough and breathlessness are gone. However, lifelong risks after transplantation are serious and include infections, rejection of the new lung, and medication side effects. Read more about preparing for a lung transplant and life after lung transplant, or watch a seven-minute video.