Putting Away Pulmonary Fibrosis

You can hardly breathe. Every breath feels like your last. You have a dry cough that won’t let up. You might have idiopathic pulmonary fibrosis.

Pulmonary fibrosis describes the scarring of your lungs. Dense fibers make a scar. Strands of scar tissue crowd the spaces between the lungs’ millions of air sacs. The causes are many for this condition including lupus, rheumatoid arthritis and sarcoidosis. Most patients fall into the idiopathic category. Idiopathic signifies no cause can be found.

The dense and widespread scars prevent oxygen from diffusing through the lungs and into the blood. Patients, therefore, live with a chronic oxygen shortage. Taking only a few steps leaves them panting for air and exhausted.

A dry, hacking cough is a notable symptom. It makes falling asleep difficult. Pulmonary fibrosis causes progressive lung scarring and eventually suffocation.

Pulmonary fibrosis patients may have an exaggerated or uncontrolled healing response that, over time, produces excessive fibrous scar tissue, or fibrosis, in the lungs. It’s not known what sets this abnormal tissue-repair process in motion.

The condition typically affects those over 50 but doesn’t discriminate, debilitating runners and smokers alike. Although this illness has been recognized for the past century, relatively little is known about pulmonary fibrosis.

Some people with pulmonary fibrosis may develop other serious lung conditions such as lung cancer, blood clots in the lungs (pulmonary emboli), pneumonia, or high blood pressure in the blood vessels that supply the lungs (pulmonary hypertension).

Idiopathic pulmonary fibrosis has an estimated prevalence of 13 to 20 per 100,000 people worldwide. About 100,000 people are affected in the United States and 30,000 to 40,000 new cases are diagnosed each year.

There are certain risk factors for developing pulmonary fibrosis such as smoking. Other risk factors include:

·       Male biological sex: The condition affects more males than females. But note that cases with women have been rising over the years.

·       Older age: Most people who develop pulmonary fibrosis are age 50 to 70.

·       Smoking: Cigarette smoking increases your risk of getting pulmonary fibrosis.

·       Working around dust or fumes: Regularly breathing in chemicals or hazardous substances can damage the lungs. Hairdressers, stone cutters/polishers, farmers, ranchers, and metal workers may be at increased risk.

·       Other medical conditions: In some cases, another medical condition like rheumatoid arthritis may lead to pulmonary fibrosis.

·       Other factors: Radiation exposure, such as radiation therapy that is used to treat cancer, can damage lung tissue.

Pulmonary fibrosis is diagnosed through a variety of ways. Your doctor may order tests such as blood tests, imaging tests (e.g. chest X-ray, CT scan), breathing tests (pulmonary function tests), oxygen desaturation tests (measures oxygen levels in the blood) and a biopsy (tissue sample from the lungs for examination).

Treatment is difficult. Cortisone drugs are sometimes used to slow the scar-formation progression. If they fail to change the course of events, then drugs that moderate the immune system can be tried. Cyclophosphamide is one such drug, which can be used to stop fibrous strands within the lung tissue. Supplemental oxygen always helps. The ultimate treatment, however, is a lung transplant.

With a diagnosis of pulmonary fibrosis, you want to stay in the best shape possible. That means eating the right foods and avoiding other sick people because it is harder for you to fight off infections. You should also keep up with vaccinations as well.

It is really hard to predict the progression of this disease. Every individual is different. Some people last months after diagnosis, others may last years. Hopefully science will catch up to the condition sooner rather than later.