Your guide to the causes, symptoms, treatments, and more of pulmonary fibrosis.
When Gavin Maitland’s lung problems first surfaced in 2000, he was 35 years old. An enthusiastic runner and swimmer who was born in Scotland and now resides in Boulder, Colorado, he finished the New York City marathon that year one hour slower than he had finished the two previous marathons in 1998 and 1999. It took him hours to get rid of the dry cough that had plagued him at the finish line. That was the first indication that something was seriously wrong, and from then on, his lung function steadily declined.
He sought medical attention, and initially, his doctors “were equally perplexed by my lung disease,” he says. He wasn’t a smoker. He didn’t have a family history of lung disease. He was an accountant and never worked in an industry that featured exposure to hazards that might be associated with lung problems. He was young and otherwise in excellent health. So why was he suddenly struggling for air on his daily training runs and unable to keep the pace that he used to achieve so easily?
Also read-Walking Pneumonia : A Patient’s Guide to Walking Pneumonia And Its Symptoms
Types Pulmonary Fibrosis
The American Lung Association reports that there are over 200 different types of pulmonary fibrosis, or PF, “and in most cases, there’s no known cause.” In these cases, a diagnosis of idiopathic pulmonary fibrosis, or IPF, is made. (Idiopathic simply means the cause is unknown.) The ALA notes that approximately 50,000 new cases of IPF are diagnosed each year, making it the most common type of pulmonary fibrosis, with onset typically starting between the ages of 50 and 70. “It is more common in men, but the number of cases of IPF in women is on the rise,” the ALA notes. In Maitland’s case, his exact diagnosis was idiopathic pleuroparenchymal fibroelastosis, or IPPFE, a subset of IPF.
Risk factors
Simonelli says that because genetic factors likely play a role in the development of many cases of pulmonary fibrosis, people with a family history of pulmonary fibrosis are at higher risk of developing the condition. “We also know that some people who have emphysema can also develop PF,” which is a relatively new finding. “The connection between PF and emphysema wasn’t recognized even a decade ago.”
Signs and symptoms
Pulmonary fibrosis tends to develop gradually over time as scar tissue accumulates in the lungs. “The two most frequent symptoms patients develop are shortness of breath when doing things—walking around, climbing stairs, carrying packages—and coughing. A dry, unexplained, persistent cough is a very common symptom,” Simonelli says.
Symptoms of pulmonary fibrosis include:
- Breathing difficulties, particularly after working out.
- Dry cough that doesn’t go away.
- Shallow breathing.
- Intentional loss of weight (also known as cachexia).
- Weary.
- Joint pain and body aches.
- The fingers and toes become clubbed, or widened and rounded.
Diagnosis
Simonelli says that at any given time there are about 200,000 people in the U.S. with IPF, “and it tends to be progressive. That’s why we want to identify people early in the process.” Making a diagnosis starts with “taking a very detailed history and conducting a physical exam,” he says. Other diagnostic tools for pulmonary fibrosis may include:
Treatments
Because the source of pulmonary fibrosis is often unknown and it’s a progressive disease, it can be difficult to treat. The scarring that occurs in the lungs can’t be reversed, so it’s important to catch pulmonary fibrosis early in order to slow its progression. “A couple of medications came on the market a few years ago” for IPF, Simonelli says, which can slow PF’s progression. These two anti-fibrotic agents—nintedanib (Ofev) and pirfenidone (Esbriet)—slow the rate of scarring in the lungs. “Those medications have proven valuable in slowing progress, but one problem we face is that people with IPF advance at different rates. Some progress relatively quickly; some advance very slowly over many years.” He says finding the right medication for the right person can be challenging and it’s hard to predict how an individual will respond to these medications.
Also read-Walking Pneumonia : A Patient’s Guide to Walking Pneumonia And Its Symptoms
images source: Google
Disclaimer: The opinions and suggestions expressed in this article are solely those of the individual analysts. These are not the opinions of HNN. For more, please consult with your doctor
