Pulmonary Rehabilitation for Idiopathic Pulmonary Fibrosis
What is pulmonary rehabilitation?
Pulmonary rehabilitation (also called pulmonary rehab or PR) is a program that is designed to help people with chronic lung diseases improve their quality of life and reduce their disease symptoms.1 PR is recommended by the American Thoracic Society guidelines for the majority of people with IPF.2
PR programs are often done at a clinic or hospital, with groups meeting once a week for six to twelve weeks. In some areas, PR may be done individually. A few clinics offer home-based PR, where providers come to the patient’s home.
A PR program has many parts1:
- Exercise training: Each participant will have a specially tailored exercise plan that is designed to help improve endurance (how long a person can remain active before becoming tired) and muscle strength. This may include stretching and using a treadmill, exercise bike, or small weights. The program will be very careful to start the participant out very slowly and only work at a level that is safe and comfortable.
- Breathing techniques: Participants will be taught breathing techniques that can reduce feelings of breathlessness and the need to cough. These techniques can improve oxygen levels in the blood for some patients because more air will be getting into the lungs with each breath.
- Energy saving techniques: Participants will be taught how to move about their daily tasks in ways that conserve the most energy and help with breathing.
- Disease education: A healthcare professional will explain more about what happens to the lungs of patients with IPF, what to expect over time, how to avoid situations that make symptoms worse, how to avoid infections, how to take medications, when to contact the doctor, and when to seek urgent medical care.
- Counseling and group support: IPF is a disease that makes most patients feel scared, anxious, depressed, or angry at various times. A mental health provider will provide counseling help to reduce stress, improve emotional coping skills, improve mood, and offer support. Support groups may be available in some PR programs so that patients can share experiences, advice, tips, and understanding in a supportive environment.
- Nutritional counseling: A nutritionist will help patients plan out meals and snacks to provide optimum nutrition. Maintaining a healthy weight can help with breathing, so if a patient desires to gain or lose weight, nutritional counseling can help.
What are the possible benefits of PR for IPF?
People living with IPF tend to feel breathless and tired easily, and this becomes worse over time. Because of these symptoms, most people with IPF tend to do less and less activity to avoid the symptoms. However, this can actually worsen the symptoms because the body is getting less fit.
PR takes a different approach. By teaching people with IPF how to exercise and improve their endurance slowly and carefully, the cardiovascular system (heart and lungs) becomes more fit. Symptoms may then feel less severe.3,4
PR for IPF has been studied using meta-analyses, which are large studies that compile data from many smaller studies. According to three meta-analyses, people with IPF who complete a PR program, on average, have:
- Improved walking distance: The six-minute walk test (6MWT) shows how much distance a person can walk in six minutes. On average, IPF patients who participate in PR have a longer 6MWT distance after finishing the program than when they started. The average difference in 6MWT was about 49 meters in one analysis5, 44 meters in a second6, and 38 meters in a third.7
- Improved overall quality of life: The overall score of the St. George's Respiratory Questionnaire measures quality of life. The average improvement for IPF patients who participate in PR was found to be about eight points in all three analyses.5-7 Patients and doctors consider a five-point improvement or greater to be something meaningful that patients can notice during day-to-day life.
- Improved symptoms: The symptom subscore of the St. George's Respiratory Questionnaire measures symptoms like cough, breathlessness, and wheezing. On average, IPF patients who participate in PR have improved symptom subscores at the end of the program. The average improvement in one analysis was about 18 points.6 This score was not measured in the other analyses.
These benefits are averages. This means that in a large group of people with IPF who complete PR, many will gain benefits. However, not every single patient will have the same level of benefit. A few patients may not have any benefit at all.
Can PR improve survival time?
Only one study has looked at survival time for patients with IPF who did and did not participate in PR. The study found no difference in survival time between the two groups.8
There has been one study on survival after lung transplantation for IPF patients who participated in PR before having the transplant versus those who did not participate in PR before having the transplant. The researchers found that those who participated in PR had 54% improved odds of survival after their transplant compared to those who did not do PR.9
Can PR cure IPF?
PR cannot cure IPF. PR is designed to help with symptoms and quality of life.10
If PR helps me, how long will the benefits last?
Researchers do not fully know the answer to this question.
When researchers study the benefits of PR, they usually study 6MWT distance or the overall quality of life score on a questionnaire. Most often, they are looking at the average score for a group of IPF patients, not the person-by-person individual scores.
Average 6MWT distance and average quality of life scores are improved by PR. If the average improvement lasts a few months before starting to drop again, that would be called a short-term benefit. If average 6MWT distance and quality of life scores stay improved for more than six months, researchers consider that a long-term benefit.
There have been three studies about how long the benefits of PR last for patients with IPF.
The first study found that the 6MWT distance improvement for a group of IPF patients on average did not last more than 6 months after the end of PR. This means PR only had a short-term benefit for 6MWT distance. When the researchers looked at the data person-by-person, however, they found that about one-third of patients had a long-term benefit, while the other two-thirds only had a short-term benefit.11 It is unusual that the researchers looked at the data on a person-by-person level.
The second study looked at the average 6MWT distance and average quality of life score for a group of IPF patients before they did PR, right after PR, and then at six and 12 months after PR. The study found that the average 6MWT distance and average quality of life score for the group was improved after PR and stayed at that improved level 12 months after PR was over. This is a long-term benefit. The researchers did not look at the data on a person-by-person level.12
The third study looked at the average 6MWT distance and average quality of life score for a group of IPF patients before they did PR, right after PR, and then at 12 months after PR. The study found that the average 6MWT distance and average quality of life score for the group was improved after PR and stayed at that improved level 12 months after PR was over. Again, this is a long-term benefit. The researchers did not look at the data on a person-by-person level.13
Long-term benefits from PR are more likely when patients are early in the course of IPF when they start the program. The first study found that higher lung function at the start of PR predicted a longer-term improvement in symptoms.11
If PR is helpful but the benefits do not last as long as you would like, repeating PR may help. Repeating PR often helps people with chronic obstructive pulmonary disease.14 However, no one has yet studied repeating PR for people with IPF to see if it will help again.
Do I have to choose between PR and other therapies for IPF?
No. PR does not replace other treatments for IPF. It can be used together with medication and oxygen supplementation. PR can also be used alone for patients who do not take medication or use supplemental oxygen for IPF.2
Patient Perceptions of Pulmonary Rehabilitation
In a survey study of patients undergoing PR, a small number noted there can be downsides to PR. These included too much time and energy required to get to sessions or not feeling a sense of belonging during sessions.15
However, most patients reported enjoying their PR program.
“I looked forward to attending. Under other circumstances a hospital is not a welcoming place. But those sessions were special. Obviously, we are ill, but I kind of forgot it, because it was so nice to be there. The two other participants from my village agreed; it was just pleasant and cozy! I will hold onto this memory for a long time.”
“We gained enormous trust and a sense of security from the start. Of course, we had conversations about our illness, but we enjoyed small-talk as well. We were able to share; this was no problem, because we all understood one another. Sometimes these matters are just too difficult to talk about with people who don't have the disease.”
- U.S. National Library of Medicine. Medline Plus. Pulmonary rehabilitation. Last updated August 30, 2019.
- Raghu G, Collard HR, Egan JJ, et al. An official ATS/ERS/JRS/ALAT statement: idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management. Am J Respir Crit Care Med. 2011;183:788–824.
- Vainshelboim B. Exercise training in idiopathic pulmonary fibrosis: is it of benefit? Breathe (Sheff). 2016;12(2):130–138.
- Holland AE, Dowman LM, Hill CJ. Principles of rehabilitation and reactivation: interstitial lung disease, sarcoidosis and rheumatoid disease with respiratory involvement. Respiration. 2015;89(2):89-99.
- Yu X, Li X, Wang L, Liu R, Xie Y, Li S, Li J. Pulmonary rehabilitation for exercise tolerance and quality of life in IPF patients: a systematic review and meta-analysis. Biomed Res Int. 2019:8498603.
- Gomes-Neto M, Silva CM, Ezequiel D, Conceição CS, Saquetto M, Machado AS. Impact of pulmonary rehabilitation on exercise tolerance and quality of life in patients with idiopathic pulmonary fibrosis: a systematic review and meta-analysis. J Cardiopulm Rehabil Prev. 2018;38(5):273-278.
- Cheng L, Tan B, Yin Y, et al. Short- and long-term effects of pulmonary rehabilitation for idiopathic pulmonary fibrosis: a systematic review and meta-analysis. Clin Rehabil. 2018;32(10):1299-1307.
- Vainshelboim B, Oliveira J, Fox BD, et al. Long-term effects of a 12-week exercise training program on clinical outcomes in idiopathic pulmonary fibrosis. Lung. 2015;193(3):345-54.
- Florian J, Watte G, Teixeira PJZ, et al. Pulmonary rehabilitation improves survival in patients with idiopathic pulmonary fibrosis undergoing lung transplantation. Sci Rep. 2019;9(1):9347.
- Rochester CL, Vogiatzis I, Holland AE, et al. An official American Thoracic Society/European Respiratory Society Policy Statement: enhancing implementation, use, and delivery of pulmonary rehabilitation. Am J Respir Crit Care Med. 2015;192(11):1373-86.
- Holland AE, Hill CJ, Glaspole I, et al. Predictors of benefit following pulmonary rehabilitation for interstitial lung disease. Respir Med. 2012;106(3):429-35.
- Wallaert B, Duthoit L, Drumez E, et al. Long-term evaluation of home-based pulmonary rehabilitation in patients with fibrotic idiopathic interstitial pneumonias. ERJ Open Res. 2019;5(2):pii: 00045-2019.
- Perez-Bogerd S, Wuyts W, Barbier V, et al. Short and long-term effects of pulmonary rehabilitation in interstitial lung diseases: a randomised controlled trial. Respir Res. 2018;19(1):182.
- Atabaki A, Fine J, Haggerty M, et al. Effectiveness of repeated courses of pulmonary rehabilitation on functional exercise capacity in patients with COPD. J Cardiopulm Rehabil Prev. 2015;35(4):272-7.
- Halding AG, Wahl A, Heggdal K. ‘Belonging’. patients' experiences of social relationships during pulmonary rehabilitation. Disabil Rehabil. 2010;32(15):1272-1280.