Pulmonary Fibrosis

  • What is Pulmonary Fibrosis?

    There are approximately 200 types of Pulmonary Fibrosis (PF). The most common is Idiopathic PF (IPF). The actual cause is unknown hence the title idiopathic and fibrosis referring to the scarring that occurs to the lung tissues. Other causes would include exposure to environmental hazards (such as asbestos) and autoimmune diseases (such as rheumatoid arthritis). This scarring makes the lung tissue thickened and stiff which in turn makes it more difficult for the lungs to function properly. As this disease progresses, most people start to feel shortness of breath.This is a chronic or long-term disease process that generally happens in four stages. Mild, moderate, acute, severe, and very severe. As the disease progresses the patient’s lung capacity decreases.

  • What are the Symptoms of IPF?

    If you have IPF, you may have rapid, shallow breathing or a dry, hacking cough that doesn’t go away. When a doctor listens to your breathing through a stethoscope, he/she may hear a crackling noise in your lungs. You may also get “clubbing” — a widening and rounding of your fingertips and toes. 

Diagnosis & Treatment

  • Diagnosis

    Should you show signs or symptoms of pulmonary fibrosis, your medical provider will conduct a thorough examination to determine your current medical history, family history, and possible exposure to certain environmental agents. In addition, tests may be ordered to help confirm a diagnosis of pulmonary fibrosis.  This may include diagnostic testing such as:

    • Blood tests to help rule out other conditions.
    • Imaging tests such as chest X-ray, computerized tomography (CT) scan, and Echocardiogram.
    • Lung function tests such as pulmonary function testing, pulse oximetry, exercise stress test, and arterial blood gas testing.
    • In some cases, a tissue biopsy might also be ordered.

  • Treatment

    There is no cure for IPF but, and no current treatment has been proved effective at stopping disease progression. However, there are specific treatments that may provide temporary improvement of symptoms, slow disease progression, and improve quality of life.  In addition to medications, treatments often include:

    • Oxygen Therapy is commonly prescribed for patients with PF. The therapy is used to treat low levels of oxygen in the blood, which is caused by scarring in the lungs. The scarring inhibits the transfer of oxygen and carbon dioxide to and from the body.
    • Pulmonary Rehabilitation can help manage symptoms and improve quality of life. A pulmonary rehabilitation program typically focuses on physical exercise, breathing techniques, medical nutrition therapy, counseling, and ongoing education about your condition.
    • Lung transplantation may be an option for people with pulmonary fibrosis. However, it may not be appropriate for your condition and there are certain risks involved such as rejection and infection. Your medical provider may discuss lung transplantation if it is determined that you may be a good candidate.

  • Technological Advances for IPF

    • The administration of stem cells to IPF patients may be possible to generate the new lung tissue necessary for effective gas exchange. Mesenchymal stem cells (MSCs) are now being investigated to treat IPF due to their ability to potentially reduce inflammation in the lungs.
    • Researchers continue to study medications to treat pulmonary fibrosis. New medications approved by the FDA include pirfenidone (Esbriet) and nintedanib (Ofev). These medications may help slow the progression of IPF.


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