Prior to 2010, I had never heard the term pulmonary fibrosis. But now, more than 5 years later, pulmonary fibrosis (PF) has profoundly affected my life both personally and professionally.
In March of 2010, my father Jeffery was diagnosed with idiopathic pulmonary fibrosis (IPF) after several years of an unexplained cough and increasing shortness of breath with exertion. He remained relatively stable for several years but suffered an acute exacerbation of symptoms in November of last year and eventually succumbed to the illness on May 19, 2015.
September is Global Pulmonary Fibrosis Awareness Month and awareness is crucial to a disease that kills more people than breast cancer each year yet very few people have ever heard of until they are personally affected.
Pulmonary fibrosis (PF) in the simplest terms means scarring in the lungs. It is an interstitial lung disease (ILD), a group of more than 200 different diseases that affect the interstitium of the lung, the place where gas exchange between oxygen and carbon dioxide occurs. Over time, the lungs becomes increasingly thickened and scarred, causing shortness of breath and low oxygen levels in the blood.
PF can occur from several known causes like autoimmune disorders such as rheumatoid arthritis and scleroderma; certain occupational and environmental exposures like coal and metal dusts, animal proteins, and mold; infections or drug-induced exposures from radiation or chemotherapy. The disease is termed idiopathic when the cause cannot be found. There is also a genetic or familial form that makes up about 10-15% of cases. Researchers believe that an abnormal healing response occurs after repeated injury to the lung, which leads to inflammation, and scarring; however, the exact process is still unknown.
The prevalence of idiopathic pulmonary fibrosis is between 150,000 and 200,000 in the United States; however, the full impact of ILD is thought to be much higher. While PF affects people of all ages, including children, IPF is more common in older adults and nearly 1 in every 200 people age 65 and older carried a diagnosis of IPF in 2011 in the United States.
People with PF can present with shortness of breath during exertion, a dry cough and fatigue, and it is not uncommon to take over 2 years for a diagnosis of PF to be made. Diagnosis can be made through a focused history and physical examination followed by a high-resolution CAT scan, which helps diagnose the type of PF, including IPF. Some patients may undergo a lung biopsy.
The clinical course of the disease is unpredictable and can vary greatly from patient to patient and treatment options are limited. In October 2014, the FDA approved two therapies for IPF, pirfenidone and nintedanib that help to slow the progression. Many patients also utilize supplemental oxygen and pulmonary rehabilitation programs to help reduce symptoms, and comorbidities like GERD and sleep apnea can be treated to help improve quality of life. Lung transplantation is an option for some patients. Currently, PF is the number one indication for lung transplantation in the United States.
After my father’s diagnosis we turned to the Pulmonary Fibrosis Foundation (PFF) for information and support. In 2011 I began working for the PFF, where I am now the director of programs, which includes managing our Patient Communication Center, our Support Group Leader Network and the PFF Care Center Network (CCN), comprised of 21 medical centers specializing in treating patients with fibrotic lung diseases. Because the disease is relatively unknown and the diagnostic process can be long, it is important for patients and families to find accurate information about the disease, find a pulmonologist who specializes in ILD to discuss treatment options, and find a local support group to help deal with the psychosocial issues that arise from living with a chronic and life limiting disease. The PFF offers many programs to help patients, caregivers, and families navigate the unknown world of PF and helps create a community so that no one feels alone.
From a public health perspective, there are several challenges that pulmonary fibrosis presents. Since the natural history of the disease is poorly understood, observational and epidemiologic studies are important to understand the current incidence and prevalence as well as identify known associations. The PFF has set up the PFF Patient Registry that will collect data from all types of pulmonary fibrosis the CCN sites across the United States to help researchers better understand the disease. Eventually, the PFF Patient Registry will become a tool to quickly enroll clinical trials that will lead to more treatments. The data collected from the CCN will lead to the identification of care patterns at each site and compare patient outcomes to potentially determine a standard of care.
On the policy side, while the FDA did approve the first two drugs last year, it was only for one type of PF, idiopathic pulmonary fibrosis, and while the drugs help to slow the progression of the disease, they do not improve patient’s most debilitating symptoms like the cough and shortness of breath. IPF was included in the FDA’s Patient-Focused Drug Development program and a meeting was held in September 2014 when the FDA sought input from patients and caregivers affected by IPF to better understand the needs and expectations for therapeutic treatments from the community itself.
Another pressing issue for the PF community has been regarding Medicare reimbursements for supplemental oxygen use and pulmonary rehab (PR) programs. Medicare data for both of these treatments are based on data from COPD patients; however, PF patients utilize both oxygen and PR very differently. For example, if COPD patients use more than 10 liters per minute (lpm) of oxygen, they can suffer from carbon dioxide poisoning. But at the end of my father’s life he was using 25 lpm that was administered through a stationary oxygen concentrator set at 10 lpm delivered through a nasal cannula with an additional 15 liters administered through an e-tank with a face mask. He could have greatly benefited from the use of liquid oxygen but we could not get an oxygen company to supply the equipment because of Medicare’s Competitive Bidding Program that changed how durable medical equipment (DME) companies were being reimbursed. After Competitive Bidding went into effect, suppliers stopped carrying liquid oxygen because the Medicare reimbursements were not covering the costs of supplying the equipment.
This September, the PF community including patients, caregivers, family members, healthcare professionals and industry come together to let the world know about pulmonary fibrosis by attending education events and fundraisers, wearing a Breathe Bracelet or participating in the #BlueUp4PF campaign. There are many ways to get involved this month so I encourage everyone to visit the PFF website at www.pulmonaryfibrosis.org to learn more and be sure to like us on Facebook and follow us on Twitter and Instagram.