Pulmonary Fibrosis

  • Medical Author:
    George Schiffman, MD, FCCP

    Dr. Schiffman received his B.S. degree with High Honors in biology from Hobart College in 1976. He then moved to Chicago where he studied biochemistry at the University of Illinois, Chicago Circle. He attended Rush Medical College where he received his M.D. degree in 1982 and was elected to the Alpha Omega Alpha Medical Honor Society. He completed his Internal Medicine internship and residency at the University of California, Irvine.

  • Medical Author: John P. Cunha, DO, FACOEP
    John P. Cunha, DO, FACOEP

    John P. Cunha, DO, is a U.S. board-certified Emergency Medicine Physician. Dr. Cunha's educational background includes a BS in Biology from Rutgers, the State University of New Jersey, and a DO from the Kansas City University of Medicine and Biosciences in Kansas City, MO. He completed residency training in Emergency Medicine at Newark Beth Israel Medical Center in Newark, New Jersey.

  • Medical Editor: Melissa Conrad Stöppler, MD
    Melissa Conrad Stöppler, MD

    Melissa Conrad Stöppler, MD

    Melissa Conrad Stöppler, MD, is a U.S. board-certified Anatomic Pathologist with subspecialty training in the fields of Experimental and Molecular Pathology. Dr. Stöppler's educational background includes a BA with Highest Distinction from the University of Virginia and an MD from the University of North Carolina. She completed residency training in Anatomic Pathology at Georgetown University followed by subspecialty fellowship training in molecular diagnostics and experimental pathology.

What about pulmonary rehabilitation for pulmonary fibrosis?

Pulmonary rehabilitation along with medical care helps improve the quality of life of people who have chronic breathing problems such as pulmonary fibrosis. It involves:

  • Exercise conditioning
  • Nutrition counseling
  • Patient education on managing the disease
  • Breathing strategies
  • Techniques to help conserve energy
  • Counseling and/or support groups

Patients diagnosed with idiopathic pulmonary fibrosis who are current smokers are encouraged to quit. It is also recommended that patients with IPF receive vaccination against influenza and pneumococcal infection.

What are the complications of pulmonary fibrosis?

Idiopathic pulmonary fibrosis tends to be relentless in its progression. The complications that occur are a reflection of the failure of the pulmonary system. Shortness of breath, decreased activity, and signs of heart failure can occur. As the lungs fail, the blood pressure in the lungs rises. This results in increased work for and ultimately failure of the right side of the heart which pumps the blood through the lungs. This failure can result in fatigue, leg swelling, and overall fluid accumulation in the body. The immobility and sluggish blood flow can increase the risks for blood clots. Depression is frequently seen in this devastating disease.

Can pulmonary fibrosis be prevented?

Idiopathic pulmonary fibrosis has an increased frequency in cigarette smokers. The cause of idiopathic pulmonary fibrosis is unknown, and therefore prevention is difficult. There is a rare form of idiopathic pulmonary fibrosis is hereditary (runs in families). Work is being done at National Jewish Hospital in Denver, CO trying to identify markers for this disease.

Unfortunately, since this is a fatal disease without effective therapy, there are many charlatans trying to take advantage of these stricken individuals and their families. There is no evidence that special diets or supplements or bowel preparations will help this disease in any way.

What is the prognosis and life expectancy for a person with pulmonary fibrosis?

The prognosis of this disease is poor. The survival of patients with pulmonary fibrosis is less than 5 years. It is best to become involved with an academic center in the area where research on interstitial lung diseases is studied in order to receive the latest treatments. These centers often are linked with a lung transplant program. Clinical trials are the best way of treating this disease at this time.

Where can I get information to improve the quality of life for those with pulmonary fibrosis?

For information quality of life and living with pulmonary fibrosis please visit Pulmonaryfibrosis.org.

REFERENCES:

American Lung Association. "Pulmonary Fibrosis Symptoms, Causes & Risk Factors."
<http://www.lung.org/lung-health-and-diseases/lung-disease-lookup/pulmonary-fibrosis/symptoms-causes-and-risk.html

Godfrey, M.K., MD. "Idiopathic Pulmonary Fibrosis Treatment & Management." Updated: Aug 11, 2016.
<http://emedicine.medscape.com/article/301226-treatment>

Kolb, M., et al. "Staging of idiopathic pulmonary fibrosis: past, present and future." European Respiratory Review 2014 23: 220-224; DOI: 10.1183/09059180.00002114
<http://err.ersjournals.com/content/23/132/220>

Nathan, S. MD. et al. "Long-term Course and Prognosis of Idiopathic Pulmonary Fibrosis in the New Millennium." Chest. 2011;140(1):221-229.

Pulmonaryfibrosis.org.

National Heart, Lung, and Blood Institute. What Is Idiopathic Pulmonary Fibrosis? Updated: Sep 20, 2011.
<http://www.nhlbi.nih.gov/health/health-topics/topics/idiopathic-pulmonary-fibrosis>

Pulmonary Fibrosis Foundation. "About PF."
<http://www.pulmonaryfibrosis.org/life-with-pf/about-pf> Raghu. et al. "An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline: Treatment of Idiopathic Pulmonary Fibrosis. An Update of the 2011 Clinical Practice Guideline" Am J resp crit care medicine abstract 2015; 192; e3-19.

Richeldi. et al. "Efficacy and Safety of Nintedanib in Idiopathic Pulmonary Fibrosis." N Engl J Med 2014; 370:2071-2082.

Talmadge. et al. "A Phase 3 Trial of Pirfenidone in Patients with Idiopathic Pulmonary Fibrosis." N Engl J Med 2014; 370:2083-2092.

Medically Reviewed by a Doctor on 9/14/2016

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