What is Idiopathic Pulmonary Fibrosis?

Idiopathic pulmonary fibrosis is a lung disease. Severe breathing difficulties for affected individuals are caused by fibroid (scar tissue) buildup in the lungs, and the buildup makes the lungs unable to transport enough oxygen into the bloodstream. The scar tissue forms around alveoli, which are the air sacs in the lung that fill with oxygen each time a person inhales. The walls of the alveoli are very thin, and because of this the thickness and fibrous nature of the scar tissue is especially debilitating for them.

Idiopathic pulmonary fibrosis tends to be a progressive condition, meaning it becomes worse over time. The breathing problems experienced by sufferers may get to the point that they need an assisted breathing device in order to not die from this disease. There is ongoing research into idiopathic pulmonary fibrosis treatment, but as of now there is no cure and a maintenance approach is the only option for those with this disease.

What Are The Causes of Idiopathic Pulmonary Fibrosis?

When a condition is deemed idiopathic it means that the exact cause of it is not definitively known. With IPF the consensus in the medical community is that it is environmental in nature and connected to what a person inhales into their lungs. There are known risk factors for idiopathic pulmonary fibrosis though, and most notable are cigarette smoking, and Epstein-Barr virus, hepatis, or herpes infections. 

GERD (gastrointestinal esophageal reflux disease) may also factor into a person getting idiopathic pulmonary fibrosis. The belief is that people with GERD may be breathing small amounts of refluxed stomach acid into their lungs. IPF is also familial, meaning you are more likely to develop idiopathic pulmonary fibrosis if a predecessor in your family has had it. 

Idiopathic Pulmonary Fibrosis Symptoms

In addition to difficulty breathing, people with IPF may also be experiencing chest pain or tightness, appetite loss, leg swelling, joint pain and muscle aches, excessive tiredness, unattributable weight loss, or clubbing. Clubbing is when the tips of your toes or fingers get wider. A crackling noise coming from their airway when they exhale is also possible with idiopathic pulmonary fibrosis. 

Note as well that idiopathic pulmonary fibrosis symptoms can be the same ones seen with tuberculosis, so it is advisable to obtain a proper medical diagnosis if you are experiencing them. This will usually be with a chest X-ray scan but may also involve pulse oximetry testing. 

Idiopathic Pulmonary Fibrosis Treatment

There are prescription medications that can slow the growth of fibroid scar tissues in the lungs. Doctors will often put IPF sufferers on a course of treatment with medications like Esbriet or Ofev  as the standard approach for idiopathic pulmonary fibrosis treatment. Oxygen therapy treatment can also be a possibility to make breathing easier and prevent complications from low oxygen levels. 

Doctors may advise patients to start pulmonary rehabilitation if they see it as potentially helping with the management of idiopathic pulmonary fibrosis. This can involve physical exercise, breathing techniques, nutritional counseling, and emotional counseling and support.