Are you experiencing shortness of breath? Are you winded simply taking the stairs at home? Although feeling out of breath is normal during a high-intensity workout, there are times when it can be a serious concern. For example, if you are experiencing shortness of breath daily and are over the age of 60, it could be many potential causes, including a rare, and very serious, lung disease called Idiopathic Pulmonary Fibrosis (IPF).
IPF is considered a progressive, fatal lung disease of unknown origin. However, it occurs with a similar frequency to that of stomach, brain and testicular cancers. Incidence of IPF in North America is estimated to range between 2.8 and 18 cases per 100,000 people per year. This disease affects nearly 132,000 Americans, with 30-50,000 new cases diagnosed each year. This interstitial lung disease (ILD) is debilitating and usually takes the life of patients within 3 to 5 years.
Before running out to make an appointment, here’s what you should know about IPF, so you can discuss with your doctor.
Some common early signs of IPF are shortness of breath and dry cough. The telltale sign when listening to a patient’s lung is a dry Velcro-like crackle. Most patient’s see at least three physicians before the diagnosis is confirmed.
Unfortunately, symptoms can vary from person to person, and IPF shares similar symptoms with most common diseases like bronchitis, asthma, COPD or heart disease, and approximately half of these patients are initially misdiagnosed. There is typically a delay of 1-2 years between the onset of symptoms and the actual diagnosis of IPF.
“This is an important disease to educate both the medical and general public about because there are often considerable delays or misdiagnoses,” said Sachin Chaudhary, MD, pulmonologist and ILD program director at Banner - University Medical Center Tucson. “Often, by the time they see me, 1-2 years have gone by. It’s important for doctors to listen closely for those dry crackles, as that may be a sign of fibrosis.”
The exact causes are not fully understood. But according to Dr. Chaudhary, IPF is now generally regarded as a consequence of multiple interacting genetic and environmental risk factors, with repetitive local micro-injuries that progress to fibrosis. Particulate inhalation plays a role both in its development and progression. Most patients with IPF are either current or former smokers. However, multiple other environmental factors have also been implicated, including metal and wood dusts, agriculture and farming and viruses.
If you believe you may have IPF, schedule an appointment with your primary care doctor and discuss your concerns. Your doctor will want to perform a physical exam, listen to your chest to determine if the lungs are producing any abnormal (crackling) sounds and may order a High-Resolution CT (HRCT) scan of the chest.
“To best rule out the disease, you’ll want an HRCT and a pulmonary function test to not only diagnose the disease but also assess its progression,” Dr. Chaudhary said.
Your doctor may also send you to a specialist to have a lung biopsy taken or a series of other tests performed to rule out any other underlying auto-immune diseases.
Once the lungs are damaged, there isn’t anything that can be done to reverse it. However, there are currently a few treatment options available for IPF that can help slow the progression of the disease. There are two FDA-approved therapies that are used to slow the scarring and help preserve lung function. And, some are candidates for lung transplant surgery. Most patients with IPF are also offered an option to enroll in ongoing clinical trials.
When looking for a specialist, Dr. Chaudhary recommends you look for a center that provides a multi-pronged approach to care. At the ILD Program in Tucson, Dr. Chaudhary meets with a team of physicians that include thoracic radiologists, rheumatologists and pathologists every week to help with the diagnosis and formulation of treatment plans.
“It’s important to provide multidisciplinary care to these patients, so everyone is on the same page with our patients’ care,” he said.
He also recommends joining a Pulmonary Fibrosis Foundation (PFF) support group, which meets monthly to support patients and their families.
If you believe you may be suffering from IPF or any type of lung fibrosis, find a Banner Health specialist near you at bannerhealth.com.