Idiopathic pulmonary fibrosis is one of the most common causes of pulmonary fibrosis (scarring). It is a chronic condition that can lead to scarring in the lungs, which can cause chronic cough and breathing difficulties. While it’s not completely clear what causes idiopathic pulmonary fibrosis (IPF), there are treatment options.
IPF is a very serious lung disease that can end with a lung transplant in some patients. Your lungs function to move oxygen into your body and remove excess carbon dioxide from your cells. Your lungs are elastic and stretchy, and this allows them to have a large surface area for the gas exchange of oxygen and carbon dioxide. When IPF occurs, your lung tissue becomes thickened and scarred. Lung scarring makes it difficult for oxygen to diffuse across the cells in your lungs, making you feel short of breath. With less oxygen in your lungs and your cells, you may grow tired more easily and develop some concerning symptoms that become hard to ignore.
The symptoms of idiopathic pulmonary fibrosis (IPF) can range from mild to severe and often come on gradually. Patients with IPF often first develop shortness of breath or cough, and then as the disease progresses, they may develop worsening symptoms including:
The above-mentioned symptoms are often vague and may be brushed off as just growing older. However, the mainstay symptoms of IPF include worsening shortness of breath and a dry cough. Often the cough becomes persistent and patients develop coughing fits that are difficult to recover from, while also experiencing worsening shortness of breath. Patients may feel like they are just getting out of shape, but even with regular exercise, shortness of breath and coughing will not go away.
Doctors are not sure what the cause of idiopathic pulmonary fibrosis (IPF) is, but they have pinpointed a few risk factors. The name idiopathic translates into “unknown cause,” while there is not an exact known cause of IPF, there are some well-researched risk factors.
Risk factors for IPF include:
Due to idiopathic pulmonary fibrosis (IPF) having very vague symptoms in its beginning stages, it sometimes is difficult to diagnose. Unfortunately, IPF also shares a lot of symptoms with other very common lung diseases. The first step to getting a diagnosis is making an appointment with a Pulmonologist or a lung specialist.
To diagnose IPF, a Pulmonologist may run a variety of tests including a chest x-ray, chest CT scan, a spirometry test to look at lung function, and possibly a lung biopsy. With this variety of tests, you could be diagnosed with IPF.
Treatments for idiopathic pulmonary fibrosis won’t cure the disease, but treatments can help your symptoms. Treatments include medications that can slow scarring in the lungs such as corticosteroids, using oxygen for shortness of breath, and trialing pulmonary rehabilitation.
Some patients benefit greatly from pulmonary rehabilitation. This often includes you working directly with physical therapists, respiratory therapists, and physicians to find the best way to decrease your shortness of breath and increase your exercise capacity.
If all treatments fail, or if your shortness of breath worsens drastically, then a lung transplant may be the best option. You must fit rigorous criteria to match with a donor, but if you do, then a lung transplant can give you and other patients living with IPF a new outlook on life.