Pulmonary fibrosis means scarring of the tissue of the lung. The fibrosis will continue to progress and eventually destroy the normal lung tissue making it difficult for the body to absorb oxygen into the blood. This will make the levels of oxygen low in body and cause shortness of breath and damage to other organs. More than 200 different types of pulmonary fibrosis affect one out of 200 adults over the age of 60 in the U.S., and approximately 40,000 American’s die from it each year.
Causes of Pulmonary Fibrosis
- Adverse effects of medications, such as chemotherapy, and certain medications that treat bladder infections, heart rate issues, or connective tissue disease.
- Adverse effects of radiation for cancer treatment to the chest such as lung cancer, lymphoma, or breast cancer.
- Effects of other conditions such as autoimmune disease or connective tissue disease like rheumatoid arthritis or scleroderma.
- Over reaction of the body, called hypersensitivities (or hypersensitivity pneumonitis), to pollutions in a work or home environment such as mold exposure, bird exposure, or certain bacteria. No one knows why some people are susceptible to developing a hypersensitivity reaction and others with the same exposure are not.
- Inherited forms related to genetics and can run in families due to other causes like connective tissue disease or hypersensitivities.
When the cause of pulmonary fibrosis is unknown, it is called Idiopathic pulmonary fibrosis. To make this diagnosis, all other potential causes must be ruled out with a thorough history, lab tests, and imaging with CT scans.
Symptoms of Pulmonary Fibrosis
- Fatigue and weakness
- Chest tightness and discomfort
- Weight loss for no apparent reason and decreased appetite
- Shortness of breath
Diagnosing and Treating Pulmonary Fibrosis
Treatment of pulmonary fibrosis is dependent on the cause, which is not always clear. Certain labs, special imaging, called high-resolution CT of the chest, and a physical examination by your provider are needed. If the cause is an autoimmune condition, treatment of that condition is what is necessary. If the cause is medication induced or radiation-induced, these treatments must be stopped. If the cause is environmental or occupational exposures, not only does the exposure need to be removed, but also any potential infection treated.
Causes of pulmonary fibrosis can be treated but the pulmonary fibrosis cannot be cured. The lung tissue scarring does not go away. There are only two approved medications to treat idiopathic pulmonary fibrosis (IPF), type in which cause of fibrosis cannot be determined; medication only slows down the progression or worsening of the disease.
Since pulmonary fibrosis is not curable and is progressive, there have been claims of stem cell therapy as a treatment option. Both the American Pulmonary Fibrosis Foundation and the American Thoracic Society make strong recommendations against patients engaging in stem cell treatments outside of a clinical trial. Clinical trials have some key features: No cost to patient, required written informed consent, designed to help future patients, protected by government agencies and legal standards, and must follow strict research protocol.
If you are concerned you or your loved one may have signs of pulmonary fibrosis, talk to your doctor.
(Deb Fueller and Heidi Shannon are nurse practitioners at CHI St. Alexius Health Heart & Lung Clinic and work closely with pulmonologists to diagnose and treat pulmonary problems.)