A Guide To Pulmonary Fibrosis

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What is Pulmonary Fibrosis?

Pulmonary fibrosis is a disease in which lung tissue becomes scarred. Long-term damage to this tissue makes oxygen transfer in the body less efficient. Sufferers may find they are short of breath even when performing everyday activities.

Other symptoms include:

  • A dry cough
  • Muscle and joint aches
  • Fatigue
  • Unexplained weight loss
  • Fingers or toes that widen and become rounder at the tips
  • Pulmonary fibrosis patients are at risk of acute exacerbation, a situation where symptoms get worse very quickly

Symptoms stay at the more severe level for days or even weeks. As acute exacerbation wears off, patients return to their usual level of symptoms.

What Causes Pulmonary Fibrosis?

Many cases of pulmonary fibrosis are idiopathic. This means there is no clear cause for the disorder. Idiopathic pulmonary fibrosis generally grows worse over time, but the exact prognosis varies from one person to another.

Pulmonary fibrosis symptoms may come on suddenly and worsen dramatically for one person while taking many years to progress for another. One of the key questions asked by pulmonary fibrosis research is what factors contribute to more rapid disease progression.

Not all cases of pulmonary fibrosis are idiopathic. Environmental factors can cause damage to the tissues between air sacs in the lungs, eventually leading to pulmonary fibrosis. The disease is more common in people in mining, farming, and construction.

Risk factors discovered to date include:

  • Exposure to toxins including silica dust, coal dust, and asbestos fibers
  • Certain forms of radiation therapy for treating lung or breast cancer
  • Some chemotherapy drugs, heart medications, and antibiotic drugs
  • Several other medical conditions affecting the lungs, like pneumonia

Pulmonary fibrosis clinical trials have grown in number in recent years. New clinical resources for pulmonary fibrosis are often offered to patients on a trial basis to determine their efficacy.

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How is Pulmonary Fibrosis Diagnosed?

Pulmonary fibrosis evaluation begins with a standard exam. Here, a doctor will take a medical history and listen to the patient’s breathing using a stethoscope. Imaging tests including x-ray, CT scans, and echocardiogram can be used to look at the lungs and heart.

If imaging tests reveal concerns, lung function tests are performed.

One of the most common is pulmonary function testing. This may include spiormetry, which measures lung capacity when a patient exhales forcefully, or an exercise stress test measuring lung function while jogging on a treadmill.

How is Pulmonary Fibrosis Treated or Cured?

No medication can reverse the lung damage associated with pulmonary fibrosis. While future treatments may prove effective in stopping the course of the disease, none are available yet.

Pirfenidone and nintedanib are two recent medications being prescribed to slow the progress of idiopathic pulmonary fibrosis. Several other formulations are working through FDA approval.

Medication can ease symptoms of disorders that frequently appear alongside pulmonary fibrosis, such as GERD. Oxygen therapy and pulmonary rehabilitation are other options that strengthen and maintain lung capacity. In severe cases, lung transplantation is used.

Pulmonary Fibrosis Lifestyle Changes

Maintaining cardiovascular fitness through a personalized program of regular aerobic exercise is effective in helping many patients sustain healthier lungs.

Patients should be aware of how their body responds to exertion and avoid situations where they must stand or walk long periods of time without rest.

After a pulmonary fibrosis diagnosis, patients should cease smoking immediately. Also avoid situations where secondhand smoke or other airborne pollutants will be inhaled.

Pulmonary Fibrosis Clinical Trials

The following clinical trial are the most recent offered by Clinicaltrials.gov. If you would like us to feature any additional trials, please contact our team.

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