What is pulmonary fibrosis?

Pulmonary Fibrosis (PF) is a debilitating disease where there is progressive scarring of the lung tissue and interferes with the person’s ability to breathe. It was only recognised as a disorder in its own terms in 2001 and before that was grouped under other similar lung disorders and was referred to as other disease titles.

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It can in some cases be linked to a specific cause such as environmental exposures, chemotherapy, radiation therapy, residual infection or due to an autoimmune disease such as rheumatoid arthritis. However in cases where the cause is unknown it is referred to as idiopathic pulmonary fibrosis (IPF).

While the cause of PF remains a mystery it is suspected that PF involves changes in the lung’s normal healing process. 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 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.

Symptoms of Pulmonary Fibrosis usually have a gradual onset and may include:

  • Shortness of breath, especially after exertion
  • Dry cough
  • Gradual, unintended weight loss or weight gain
  • Fatigue and weakness
  • Chest discomfort
  • Clubbing (enlargement of the ends of the fingers or toes) due to a build-up of tissue

 

PF affects each person differently and progresses at varying rates. Generally, the patient’s respiratory symptoms become worse over time and activities (such as walking or climbing stairs) become more difficult.

In addition:

  • The patient may require supplemental oxygen
  • Advanced PF makes it difficult for a person to fight infection
  • Pulmonary Fibrosis puts a strain on the heart and on the blood vessels in the lungs, and may lead to high blood pressure in the lungs
  • PF has also been associated with heart attacks, respiratory failure, strokes, blood clots in the lungs (pulmonary embolism), lung infections, and lung cancer.

 

An important part of the treatment is the use of supplemental oxygen to provide your body with the required level of oxygen it needs but cannot get due to the scarring in the lungs.

Supplemental oxygen can:

  • Decrease your shortness of breath
  • Improve your ability to carry out daily tasks
  • Improve your overall level of fitness
  • Improve your quality of life
  • Increase life span by decreasing the extra work your heart is doing because of low oxygen saturation levels

 

PF patients should also discuss the possibility of a lung transplant with their GP as soon as possible as unfortunately 30% PF patients succumb to their disease while waiting on the transplant list. This is due to the unpredictable progressive nature of the disease in combination with long transplant waiting lists.

There are new treatments being currently tested, which look promising at reducing symptom severity and aiding in decreasing the progression of the disease and ultimately aiding in improving life expectancy.

 

References: http://www.coalitionforpf.org and http://www.breathingmatters.co.uk

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