Pulmonary fibrosis (PF), the accumulation of stiff, intractable scar tissue deposits in the lungs (typically seen in mature people), has been a topic of interest for me for many years. But because it has until recently been fairly rare, I have never taken the opportunity to look at it in detail. Now that we know COVID-19 can have long-term repercussions for the lungs, it is clear that diagnoses of PF may be on the upswing—in people of all ages, not just those over 50 years old (see COVID-19 and Pulmonary Fibrosis sidebar on page 35).

My exploration of PF has been an interesting exercise in critical thinking, since there is virtually nothing published about the role of massage therapy in this context. One of my most exciting discoveries has been seeing the rich potential for collaboration with respiratory therapists to bring comfort and improved function to clients who live with this condition.

I want to offer special thanks to respiratory therapist (and massage therapist) Ravi Fernando, Clinical/ECMO Specialist, RCP, RRT, ACCS, at Northwestern Memorial Hospital, for his help in unraveling some of the confusing information I encountered along the way.

Definition

Pulmonary fibrosis is more than a single condition. It is a group name for many diseases that involve scarring and loss of function in the lungs. Another umbrella term for this collection of conditions is interstitial lung disease. This refers to problems with the extracellular matrix of the lungs, as opposed to the epithelial cells that comprise the alveoli, or the endothelial cells of circulatory and lymphatic capillaries. PF is a disease of scar tissue; it doesn’t involve an attack directly on airways or blood vessels, although these structures can sustain damage with long-term interference.

Many types of PF have been identified, but the most common form is called idiopathic pulmonary fibrosis (IPF). This is a chronic, progressive, and irreversible form of scar tissue pathology in the lungs. It has a very poor prognosis, its incidence is on the rise, and we understand very little about it.

Demographics

The prevalence of PF is unclear, but some estimates suggest the numbers of people with IPF in particular are increasing. IPF is diagnosed about 50,000 times a year in this country, and it has a very high mortality rate, causing about 40,000 deaths each year. IPF is more common in men than in women, and it is strongly associated with age. Although it can occur in younger people, most cases are identified in people over 50. Other forms of PF are not specifically connected to age or sex.

Pathophysiology

While we don’t yet understand IPF, the pathophysiology of some other forms is not always mysterious. PF may be linked to identifiable factors, and sometimes it can be successfully managed. But for many patients, the causative agents are not clear.

The most commonly recognized causes of PF include these factors:

• Idiopathic PF is a disease of unknown origin, although major risk factors include a history of cigarette smoking, male gender, and age. Many people with IPF also have gastroesophageal reflux disease (GERD), so that is also being explored as a possible contributor and treatment strategy.

• Drug-induced PF is linked to the use of certain chemotherapeutic drugs.

• Radiation-induced PF can be a complication of radiation therapy for cancers that affect the thorax.

• Environmental PF is the result of exposure to irritating triggers like molds or animal dander. It is sometimes called hypersensitivity pneumonitis.

• Occupational PF is related to contact with potentially hazardous airborne substances like fibers, vapors, and asbestos.

• Autoimmune PF is connected to several autoimmune diseases, including scleroderma, rheumatoid arthritis, polymyositis, and several others.

• Infection-related PF is the consequence of lung infections with viruses, bacteria, or fungi. This is not the outcome of most lung infections, but when a person develops acute respiratory distress syndrome (ARDS) related to an infection, then PF is a possible complication.

The development of excessive scarring in the lungs causes stiffening of tissues that should be elastic and stretchy. The distribution of scar tissue can be patchy or diffuse; different patterns suggest different subtypes of the disease. As the lungs become invested with scar tissue, the exchange of oxygen and carbon dioxide becomes much more effortful, so hypoxia (low blood oxygen) is common. This causes shortness of breath, especially during any kind of exertion. And the scarring also causes specific lung sounds that indicate pulmonary dysfunction.

Most cases of PF are progressive and terminal. Unless they get a lung transplant, patients usually succumb to respiratory failure within 3–5 years after diagnosis.

The precise cellular changes that happen in the lungs of people with PF is the subject of a lot of research. Discoveries about the genetic expression of certain proteins have led to the prospect of biologic therapies that might one day brighten the outlook for people who live with this disease.

Signs and Symptoms

The symptoms of PF typically begin with a gradual onset of a dry, unproductive cough. (Interestingly, the mechanics of what causes this cough are not understood.) Exertional dyspnea, or shortness of breath with minimal exercise, is also common and predictable. And because oxygen/carbon dioxide turnover is slowed, muscles are under-supplied, and stamina is very low. This can affect the ability to exercise, of course, but it can also cause general weakness, fatigue, loss of appetite, and unintended weight loss. PF is chronic and progressive, so symptoms continue to worsen over time.

A standard test for PF and other lung problems is the six-minute walk test. If the patient’s blood oxygen levels (as measured on a finger pulse oximeter) drop below 88 percent during this procedure, it is a strong predictor for severe disease. Doctors also look for a distinctive sound on auscultation of the lungs called “Velcro crackles,” because it sounds like Velcro being separated. Pulmonary hypertension develops in late-stage fibrosis, and heart issues—including right-sided heart failure—can follow. And for reasons unclear, people with PF often develop digital clubbing: the distal phalanges of the fingers and toes become enlarged, and the curve of the nail becomes distorted.

All these phenomena—cough, low stamina, hypoxia, lung noises, and digital clubbing—sound like they might fit together in a predictable picture that is unique to pulmonary fibrosis. Unfortunately, this is not true. Many conditions that affect oxygen levels and lung function can cause these signs and symptoms, including chronic obstructive pulmonary disease (COPD), which is extremely common. For this reason, PF diagnoses are usually confirmed with some combination of pulmonary function tests, biopsies, and a procedure called alveolar lavage, in which a sample of fluid from the alveoli is examined for signs of infection. Different types of PF show different patterns of the distribution of scarring, so high-resolution computed tomography (CT) scans may be used to identify which type might be present.

Treatment Options

One of the reasons it’s important to discern the cause of PF is that some types have a more positive prognosis if the right treatment is administered. While idiopathic PF remains a mystery, a version of PF that accompanies autoimmune diseases can be controlled and even reversed with appropriate medications. But for most cases of PF, treatment focuses on slowing the progression of the disease and maintaining quality of life.

Patients with PF are counseled to quit smoking, if that is an issue, and to vaccinate for flu and pneumococcus. Oxygen may be supplemented, although some doctors note that this may counterintuitively increase anxiety, as patients fear being without their O₂ equipment. Medications to treat PF include steroidal anti-inflammatories, immune system suppressants, antifibrotic agents, proton pump inhibitors for GERD, and others. Biologic therapies designed to address cellular expressions of specific proteins are in development.

In addition to drug therapies, PF is treated with a complex regimen of pulmonary rehabilitation, usually led by respiratory therapists. Pulmonary rehabilitation can incorporate several strategies and modalities, including nutritional advice, postural adjustments, airway clearance techniques, breathing exercises, stretching, manual therapies, and physical activity. The “manual therapies” referred to in this context include therapists who apply vibrating machines to improve alveolar function, devices that help knock mucus into airways so it can be more easily expelled, and several others. But look over that list of pulmonary rehabilitation interventions again: Can you see opportunities for the integration of massage therapy in this setting?

If medications and rehabilitation are not sufficient, then PF patients may consider a lung transplant. Between 2,000 and 3,000 patients receive donated lungs each year in this country. Improved techniques and strategies for postsurgical care mean the prognosis for survival is good: About 50 percent of transplant recipients live for more than five years after their surgery—and some live for much longer.

COVID-19 and Pulmonary Fibrosis

Pulmonary fibrosis is one of the many possible long-term complications of infection with SARS-CoV-2, the virus that causes COVID-19. Because massage therapists are likely to have many clients who live with the aftermath of this infection, I felt it was important to anticipate what we might be dealing with.

I am happy to report that my main concern—that people with mild or asymptomatic disease might be at risk for life-shortening lung problems later in life—does not appear to have come to pass. However, for people who survive severe infections, PF does seem to be a possibility.

One of the consequences of severe COVID is acute respiratory distress syndrome (ARDS), a known factor in the development of pulmonary fibrosis. Signs of fibrosis affect about one-third of hospitalized COVID patients, but PF does not appear (yet) to be a problem for survivors who were not hospitalized. Risk factors for post-COVID pulmonary fibrosis include age, severity of infection, and how long a person needed mechanical ventilation.

For patients whose lungs are so badly scarred they cannot survive without breathing assistance, a lung transplant becomes their last recourse and best hope for survival. This surgery is obviously risky, but the prognosis for living with long-term lung scarring is very poor, so it is a viable option. COVID-related PF is now a leading reason some patients get on the lung transplant waiting list.

Where Does Massage Therapy Fit?

The intersection between massage therapy and PF is unexplored terrain. There is no published research on how massage might help or hurt people with PF, so we must use our critical-thinking skills to explore the possibilities. In this situation, we can consider what living with breathing problems means for people with PF—specifically about the vicious circle between chronic shortness of breath and anxiety that exacerbates dyspnea, which worsens anxiety, ad infinitum.

Breathlessness can be an emotionally frightening and disabling symptom affecting mobility and quality of life. —Somogyi

Dyspnea, even in its mildest forms, can induce a fear of suffocation and the anticipation of a worsening of symptoms, including acute respiratory distress syndrome (ARDS) and its most feared outcomes (i.e., hospitalization in intensive care units, intubation, and death). This, in turn, might heighten fear response, false alarms, and exacerbate vigilance towards respiratory symptoms both in affected and unaffected individuals. —Javelot et al.

We have some information on massage therapy for other lung problems, especially COPD and asthma. Not surprisingly, people with those conditions have positive responses to massage, with reports of reduced anxiety and less resistance in their breathing.

Massage therapy, with its muscle alignment techniques, and trigger point treatment for relaxation applied to the accessory muscles of respiration contributed to muscles length improvement, and by consequence, its force, considering that until then this group of muscles were overloaded by increased respiratory work, and COPD’s exacerbation. —de Alvarenga et al.

The experience of anxiety about breathing is inextricable with any reduction in respiratory function. Because unrestricted breathing is so central to our experience of feeling safe and healthy, it is not surprising people with breathing disorders don’t have an accurate sense of interoception—the ability to gauge our own internal condition—so the cycles continue.

Specifically, when anxious individuals receive body signals, they cannot easily differentiate between those which are associated with potential aversive (or pleasant) consequences versus those which are part of constantly ongoing and fluctuating interoceptive afferents. As a consequence, these individuals imbue afferent interoceptive stimuli with motivational significance, specifically, an internal body signal, e.g., an inspiratory breathing sensation, is associated with negative valence and linked to belief-based processes, e.g., “I am not getting enough air,” which results in an increased “fight/ flight” response and potential withdrawal or avoidance behaviors. —Paulus et al.

Wouldn’t it be amazing if it were possible to interrupt the self-fulfilling cycle of anxiety and breathing problems? Maybe with loving, educated, soothing touch? And indeed, the research supports this idea.

Interoceptive awareness facilitates regulation and an integrated sense of self, and thus contributes to health and well-being. —Price et al.

We have research about massage therapy and anxiety. We have research about massage therapy and improved breathing. And we have research about massage therapy and an enriched sense of interoception and self-efficacy. To my knowledge, no one has put these elements together to create treatment plans that include massage therapy for patients with PF, but it doesn’t seem like a stretch. This idea particularly resonates when we see factors in pulmonary rehabilitation protocols include attention to posture, stretching, breathing exercises, and manual therapy.

PF is often a progressive, irreversible, and ultimately fatal disease. It can be controlled and slowed, but not reversed. Massage therapy won’t change that. But I am eager to explore ways that skilled massage therapy, with an emphasis on breathwork, calmness, and imbuing a sense of peace, might integrate with respiratory therapy to add to the precious quality of life for those who face this challenge.

Resources

American Lung Association. “Interstitial Lung Disease.” Accessed February 15, 2021. www.lung.org/lung-health-diseases/lung-disease-lookup/interstitial-lung-disease.

Bharat, Ankit et al. “Lung Transplantation for Patients with Severe COVID-19.” Science Translational Medicine 12, no. 574 (December 2020): eabe4282. https://doi.org/10.1126/scitranslmed.abe4282.

Boggs, Will. “Fatal Pulmonary Fibrosis Can Occur With COVID-19.” Medscape (August 7, 2020).                      www.medscape.com/viewarticle/935302.

Chioma, Ozioma S. and Wonder P. Drake. “Role of Microbial Agents in Pulmonary Fibrosis.” Yale Journal of Biology and Medicine 90, no. 2 (June 2017): 219–27.

Cleveland Clinic. “Record Number of Lung Transplants Performed in the U.S. in 2017.” Consult QD (April 17, 2019). https://consultqd.clevelandclinic.org/record-number-of-lung-transplants-performed-in-the-u-s-in-2017.

de Alvarenga, Guilherme Medeiros et al. “Physiotherapy Intervention During Level I of Pulmonary Rehabilitation on Chronic Obstructive Pulmonary Disease: A Systematic Review.” The Open Respiratory Medicine Journal 10 (February 29, 2016): 12–19. https://doi.org/10.2174/1874306401610010012.

Drillinger, Meagan. “Lifelong Lung Damage: A Serious COVID-19 Complication that Can Hit People in Their 20s.” Healthline (June 22, 2020). www.healthline.com/health-news/lifelong-lung-damage-the-serious-covid-19-complication-that-can-hit-people-in-their-20s.

Godfrey, Amanda M. K. “Idiopathic Pulmonary Fibrosis (IPF): Practice Essentials, Background, Pathophysiology.” Medscape. Updated July 15, 2019. https://emedicine.medscape.com/article/301226-overview.

Harding, Anne. “SARS-CoV-2-Exposed Donor Lungs Transplanted Successfully.” Medscape (December 15, 2020). www.medscape.com/viewarticle/942514.

IPF Foundation. “Home page.” Accessed March 2021. www.ipffoundation.org.

Javelot, Herve et al. “COVID-19 and its Psychological Consequences: Beware of the Respiratory Subtype of Panic Disorder.” Respiratory Physiology and Neurobiology 282, no. 103530 (November 2020). https://doi.org/10.1016/j.resp.2020.103530.

Noble, Paul W., Christina E. Barkauskas, and Dianhua Jiang. “Pulmonary Fibrosis: Patterns and Perpetrators.” Journal of Clinical Investigation 122, no. 8 (August 2012): 2756–62. https://doi.org/10.1172/JCI60323.

Paulus, M. P., and M. B. Stein. “Interoception in Anxiety and Depression.” Brain Structure and Function 214, no. 5/6 (May 2010): 451–63. https://doi.org/10.1007/s00429-010-0258-9.

Price, Cynthia J. and Carole Hooven. “Interoceptive Awareness Skills for Emotion Regulation: Theory and Approach of Mindful Awareness in Body-Oriented Therapy (MABT).” Frontiers in Psychology 9 (May 2018): 798. https://doi.org/10.3389/fpsyg.2018.00798.

Rai, Deependra Kumar, Priya Sharma, and Rahul Kumar. “Post COVID-19 Pulmonary Fibrosis—Is It Reversible?” Indian Journal of Tuberculosis (November 2020). https://doi.org/10.1016/j.ijtb.2020.11.003.

Sauleda, Jaume, Belén Núñez, Ernest Sala, and Joan B. Soriano. “Idiopathic Pulmonary Fibrosis: Epidemiology, Natural History, Phenotypes.” Medical Sciences 6, no. 4 (September 2018): 110. https://doi.org/10.3390/medsci6040110.

Schwartz, Robert A. “Clubbing of the Nails.” Medscape (March 2020). https://emedicine.medscape.com/article/1105946-overview.

Smith, Maxwell L. “Update on Pulmonary Fibrosis: Not All Fibrosis Is Created Equally.” Archives of Pathology and Laboratory Medicine 140, no. 3 (March 2016): 221–29. https://doi.org/10.5858/arpa.2015-0288-SA.

Somogyi, Vivien et al. “The Therapy of Idiopathic Pulmonary Fibrosis: What is Next?” European Respiratory Review 28, no. 153 (September 2019): 190021. https://doi.org/10.1183/16000617.0021-2019.

Spagnolo, Paolo et al. “Pulmonary Fibrosis Secondary to COVID-19: A Call to Arms?” The Lancet Respiratory Medicine 8, no. 8 (August 2020): 750–52. https://doi.org/10.1016/S2213-2600(20)30222-8.

Wang, Tina J. et al. “PM&R and Pulmonary Rehabilitation for COVID-19.” American Journal of Physical Medicine and Rehabilitation 99, no. 9 (September 2020): 769–74. https://doi.org/10.1097/PHM.0000000000001505.

Yu, Xueqing et al. “Pulmonary Rehabilitation for Exercise Tolerance and Quality of Life in IPF Patients: A Systematic Review and Meta-Analysis.” BioMed Research International (March 2019): 8498603. https://doi.org/10.1155/2019/8498603.