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WHAT IS PULMONARY FIBROSIS?

Pulmonary Fibrosis (PF) is a debilitating disease-marked by progressive scarring of the lungs-that gradually interferes with a person's ability to breathe. Pulmonary Fibrosis belongs to a family of approximately 100 related diseases, called interstitial lung diseases, which have similar characteristics and can result in lung scarring. This scarring is most often referred to as pulmonary fibrosis.

Your doctor may have referred to your condition by one of a number of names that are sometimes used to describe Pulmonary Fibrosis. This confusion may be due, in part, to similarities between Pulmonary Fibrosis and the other forms of interstitial lung disease. Only since 2001 has Pulmonary Fibrosis been recognized as a distinct clinical disorder, meaning that specific clinical criteria were developed to determine a diagnosis. Your physician combines clinical information derived from a medical evaluation and certain diagnostic tests to diagnose Pulmonary Fibrosis according to these criteria.

Sometimes pulmonary fibrosis can be linked to a particular cause, such as certain environmental exposures, chemotherapy or radiation therapy, residual infection, or autoimmune diseases such as scleroderma or rheumatoid arthritis. However, in many instances, no known cause can be established. When this is the case, it is called idiopathic pulmonary fibrosis or IPF.

Whether you are an Pulmonary Fibrosis patient, a family member of a patient, or are close to someone with Pulmonary Fibrosis, the CPF is always just a phone call away. Our staff have counseled thousands of patients, and we are always here to provide you with the resources and support you need. Please call (888) 222-8541 to learn more.

WHAT CAUSES PULMONARY FIBROSIS?

While the cause of Pulmonary Fibrosis remains a mystery, what researchers suspect is that Pulmonary Fibrosis involves changes in the lung's normal healing process. Pulmonary Fibrosis patients may have an exaggerated or uncontrolled healing response that, over time, produces excessive fibrous scar tissue - or fibrosis - in the lungs. This scarring, in turn, causes the lung's tiny air sacs, called alveoli, to thicken and stiffen-rendering them less able to function and provide the body with the oxygen it needs.

Exactly what sets this abnormal tissue-repair process in motion is unclear. The body's own immune response may play a major role. Researchers are investigating a number of potential risk factors that may make a person more likely to develop Pulmonary Fibrosis. These risks may include:

  • Cigarette smoking
  • Occupational exposure to dusty environments (e.g. wood or metal dust)
  • Genetic predisposition (10-15 percent of cases)
  • Viral or bacterial lung infections
  • Acid reflux disease
Pulmonary Fibrosis hinders a person's ability to take in oxygen. It causes shortness of breath and is usually associated with a persistent dry cough. The disease progresses over time, leading to an increase in lung scarring and a worsening of symptoms. Unfortunately, Pulmonary Fibrosis is ultimately disabling and fatal.

WHAT SYMPTOMS MIGHT YOU EXPERIENCE?

Symptoms of Pulmonary Fibrosis usually have a gradual onset and may include:
  • Shortness of breath, particularly during or after physical activity
  • Spasmodic, dry cough
  • Gradual, unintended weight loss or weight gain
  • Fatigue and weakness
  • Chest discomfort
  • Clubbing, or enlargement of the ends of the fingers (or sometimes the toes) due to a buildup of tissue

As you can imagine, Pulmonary Fibrosis symptoms can greatly impact quality of life. Fortunately, there are things you and your doctor can do to help minimize lifestyle-altering affects of the disease

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