Massage & Bodywork

MAY | JUNE 2021

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34 m a s s a g e & b o d y wo r k m ay/ j u n e 2 0 2 1 Pulmonary Fibrosis Scarred Stiff‡ By Ruth Werner, BCTMB education | PATHOLOGY PERSPECTIVES 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,

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