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L i s te n to T h e A B M P Po d c a s t a t a b m p.co m /p o d c a s t s o r w h e reve r yo u a cce s s yo u r favo r i te p o d c a s t s 35 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 O 2 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 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.