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What Exactly Is Idiopathic Pulmonary Fibrosis?

Pulmonary fibrosis (minus the “idiopathic” part) is a medical condition characterized by scarring (fibrosis) of the lung tissue. It is a type of interstitial lung disease, a large group of lung diseases affecting the interstitium of the lungs.

The lung scarring from pulmonary fibrosis makes the lungs stiff and thick, which can lead to difficulty in breathing. Pulmonary fibrosis can be caused by environmental exposures (chemicals, toxins, dust etc.), radiation therapy, and some connective tissue diseases. The scarring is irreversible and in many cases continues to worsen even if the cause is removed.

Idiopathic Pulmonary Fibrosis

When the exact cause of pulmonary fibrosis is unknown (a common occurrence) it is referred to as idiopathic pulmonary fibrosis (IPF). IPF is a fatal, progressive disease with an unpredictable rate of decline. In some cases the disease can slowly progress over many years, but in others, start slowly at first and then worsen quickly over months. The life expectancy from diagnosis is usually 3 to 5 years.

IPf is not well understood, but there does seem to be a genetic link for many sufferers. In my case, my mother died from the disease and I learned at the time that I would be at high risk for the disease. 

Symptoms of Idiopathic Pulmonary Fibrosis

Symptoms of both idiopathic pulmonary fibrosis and pulmonary fibrosis are the same and may include shortness of breath, a persistent cough, fatigue, unexplained weight loss, and clubbing of the fingers and toes (enlargement and rounding of the fingertips).

Diagnosis of Idiopathic Pulmonary Fibrosis

Diagnosis typically involves a combination of medical history, physical examination, lung function tests, imaging studies (such as chest X-rays or CT scans), and sometimes lung biopsy. 

In the early stages, diagnosis can be very difficult and my personal perspective of the disease (from both myself and my mother’s experience) is that the sufferer can be very aware of the symptoms long before they can be tied to IPF. In these beginning stages there can be many misdiagnoses until eventually the disease worsens enough for a proper diagnosis to be made.   

Treatment Options for Idiopathic Pulmonary Fibrosis

While there is currently no cure for idiopathic pulmonary fibrosis, various treatment options may help manage symptoms and slow down the progression of the disease. Treatment may include medications to slow the scarring (nintedanib and pirfenidone), oxygen therapy, pulmonary rehabilitation, and in some cases, lung transplantation.