Idiopathic pulmonary fibrosis (IPF) is a rare lung condition in which the tissue in your lungs stiffens. This makes it more difficult for you to take in air and breathe normally. It is unknown why IPF develops. Idiopathic actually means "unknown." Because each case of IPF is unique, it's critical to consult with your doctor about the specifics of disease progression. IPF isn't the only condition that can make it difficult to breathe.

Idiopathic pulmonary fibrosis is a chronic, progressive lung disease, according to MedlinePlus. This condition causes scar tissue (fibrosis) to form in the lungs, preventing the lungs from effectively transporting oxygen into the bloodstream. People between the ages of 50 and 70 are most commonly affected by the disease. Idiopathic pulmonary fibrosis is one of several conditions known as interstitial lung diseases (also known as ILD), which are lung diseases characterized by inflammation or scarring in the lung.

Furthermore, the most common signs and symptoms of idiopathic pulmonary fibrosis, according to MedlinePlus, are shortness of breath and a persistent dry, hacking cough. Many people who are affected also experience a loss of appetite and gradual weight loss. Due to a lack of oxygen, some people with idiopathic pulmonary fibrosis develop widened and rounded tips of their fingers and toes (clubbing). These symptoms are fairly nonspecific; not everyone who has these health issues has idiopathic pulmonary fibrosis. Other, less serious, respiratory diseases can cause similar signs and symptoms.

According to Kimberly Holland and Mary Baucom of Healthline, IPF does not have formal stages, but there are distinct periods in the disease's progression. You may not require oxygen assistance when you are first diagnosed. You may soon discover that you require oxygen during several activities because you have difficulty catching your breath while walking, gardening, or cleaning. As the scarring in your lungs worsens, you'll most likely require oxygen at all times. This includes when you are active, at rest, and even when you are sleeping. High-flow oxygen machines can be used to develop increased levels of oxygen in the later stages of IPF. But with modern medicine, there are portable machines that can provide continuous oxygen when you are away from home.

A variety of tests can be used to confirm IPF and rule out other potential causes. Chest X-ray, high-resolution computer tomography (HRCT) scan, exercise testing, lung function tests, pulse oximetry, which measures your blood oxygen levels, blood gas test, which measures your blood levels of oxygen and carbon dioxide, lung biopsy are all tests used to diagnose IPF. The Pulmonary Fibrosis Foundation reiterated that at least, 50,000 new cases of IPF are diagnosed each year in the United States.

There are treatment options available to assist you in managing and reducing your symptoms. Medical treatment aims to reduce lung inflammation, protect lung tissue, and slow the loss of lung function. This will make it easier for you to breathe. Oxygen therapy and medications are the most commonly used treatment options. Oxygen therapy aids in breathing, and medications aid in the control of inflammation and the reduction of lung tissue scarring. Two IPF medications, nintedanib (Ofev) and pirfenidone have been approved by the Food and Drug Administration (FDA) (Esbriet). A lung transplant may be required as well. Because it is a major operation, a lung transplant is frequently regarded as the final treatment option. It is currently the only treatment for IPF.

Healthline further stated that learning to adopt healthy lifestyle practices that can reduce symptoms and improve your outlook and quality of life is an important part of managing IPF. These lifestyle changes include stopping smoking, if you currently do, maintaining a healthy weight, which may necessitate losing weight, next is staying up to date on all vaccines, medications, vitamins, and supplements, and using an oxygen monitor to keep your oxygen saturation (also known as blood oxygen levels) in the optimal range.

ZDS DMO II Marie-Claire Gaas, RND

References:

1. Everything You Need to Know About Idiopathic Pulmonary Fibrosis (IPF)

Medically reviewed by Adithya Cattamanchi, M.D., Pulmonology — Written by Kimberly Holland and Mary Baucom — Updated on August 2, 2021

https://www.healthline.com/health/managing-idiopathic-pulmonary-fibrosis

2. Idiopathic pulmonary fibrosis

https://medlineplus.gov/genetics/condition/idiopathic-pulmonary-fibrosis/#:~:text=Idiopathic%20pulmonary%20fibrosis%20is%20a,ages%20of%2050%20and%2070.