Understanding Pulmonary Fibrosis

Ahead of the American Lung Association’s event at the Detroit Zoo, the organization’s national volunteer medical spokesperson speaks with Hour Detroit about the disease
Pulmonary Fibrosis
Photo: IStock

More than 1.4 million Michigan residents live with lung disease, including asthma, chronic obstructive pulmonary disease, lung cancer, and pulmonary fibrosis. On Sept. 29, the American Lung Association is bringing its Lung Force Walk to the Detroit Zoo, a family-friendly fundraising event that features speakers, entertainment, and activities all while increasing awareness of these lung diseases, providing educational sessions, and sharing information on local resources and support groups, like the Better Breathers Club. Those interested in participating can register at no cost on action.lung.org.

Prior to the event and in honor of Pulmonary Fibrosis Awareness Month, Hour Detroit spoke with Dr. David Hill. As the national volunteer medical spokesperson for the American Lung Association, Hill is knowledgeable on all things pulmonary fibrosis, which affects up to 132,000 people in the US. The disease occurs when lung tissue becomes damaged or scars, and symptoms include shortness of breath, unexplained weight loss, and aching muscles or joints. Here, Hill answers some of the most pressing questions about PF.

Hour Detroit: Autoimmune and environmental factors are the most common causes of PF in the United States. What contributes to this?

Dr. David Hill: Some cases of PF are caused by autoimmune diseases like rheumatoid arthritis, scleroderma, or Sjogren’s syndrome. Certain viral infections and gastroesophageal reflux disease (GERD) are also risk factors for PF. GERD is a condition in which acid from your stomach backs up into your throat. Some people who have GERD may breathe in tiny drops of acid from their stomachs, which may injure the lungs. Other problems with swallowing or esophageal function can lead to recurrent aspiration and lung fibrosis. PF can be caused by exposure to hazardous materials. Examples include occupational exposures such as asbestos or silica. Some cases of PF are caused by chronic allergic reactions (hypersensitivity) to breathing in things such as bird droppings and other animal related exposures, molds, or other biologic materials. Radiation treatments and certain types of medications can cause PF. Cigarette smoking also increases a person’s risk of developing PF.

Do genetics play a role in the probability of developing PF?

Familial PF is very rare. PF is considered familial when two or more members within the same family have idiopathic pulmonary fibrosis (IPF) or any other form of idiopathic interstitial pneumonia (IIP). There are genes that have been linked to PF but much is still unknown about this field. If someone in your family has had any type of interstitial lung disease, be sure to speak with your doctor and a genetic counselor about your risk.

Are there any steps you would recommend to someone who has been diagnosed with PF in order to keep their daily life as “normal” as possible?

Managing the symptoms and side effects that come from pulmonary fibrosis and its related treatments is something every PF patient has to face. Pulmonary rehabilitation can be an effective way of maintaining strength and conditioning to maintain an active lifestyle. With more advanced disease a palliative care specialist can be helpful. Once referred by your doctor, this specialist will provide you with personalized side effect management strategies. While you can’t cure pulmonary fibrosis, there are a variety of things you can do to maintain a good quality of life and stay as healthy as possible: eat right, stay active, reduce your stress, protect your lungs, and manage your side effects (including coughing, fatigue, acid reflux).

With the wave of E-Cigs and Juuling, are you seeing direct links between these habits and the development of PF?

E-cigarettes have only existed for roughly a decade. Little research has been done on e-cigarettes, so we don’t know the long-term implications yet. This is one of many reasons to either quit vaping or not to start in the first place. Some cases of pulmonary fibrosis do not have a known cause, and although we do not know yet if there is a direct link between e-cigarettes and PF, we do generally advise avoiding smoking to lower the risk of getting the disease. The current epidemic of acute lung disease related to e-cigarette use has included a variety of forms of lung disease some of which are associated with the development of pulmonary fibrosis.

How can people lower their risk of a PF diagnosis?

There are over 200 different types of PF and in most cases, there’s no one known cause. Avoiding exposure to lung toxins particularly tobacco products including e-cigarette use is a wise decision. Making sure that proper ventilation and respiratory protection is utilized in occupational settings is also important. If you have a family history of pulmonary fibrosis or other interstitial lung disease, you should discuss your own risks with your health care provider. Finally, seek medical attention when you have persistent respiratory symptoms such as cough or shortness of breath so that any chronic lung condition can be identified early when treatment may be most effective.

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