Idiopathic pulmonary fibrosis treatment available today

Idiopathic pulmonary fibrosis (IPF) is a type of chronic lung disease. It is characterized by the buildup of scar tissue in the lungs, making it difficult for oxygen to pass into the bloodstream.

The exact cause of IPF is unknown, but it is believed to be caused by a combination of genetic and environmental factors. The American Lung Association, The American Thoracic Society, and Pulmonary Fibrosis Foundation are excellent sources of information for overall lung health-related quality content. It is also a great source of patient education material.

IPF causes slowly worsening respiratory problems, such as shortness of breath, a dry cough, shallow breathing, gradual weight loss, tiredness, and aching joints. Many of these symptoms can be seen in other conditions; however, the continuous worsening of these despite decongestants and inhaled medications symptoms distinguishes IPF from the common cold, asthma or even COPD/ emphysema.

Once scar tissue has formed, it is irreversible. There is no cure for IPF, but treatments can help slow the disease's progress, such as Kinase inhibitors Nintedanib (Ofev) and Pirfenidone (Esbriet, Pirfenex, Pirespa).  

These medications aid in slowing the scarring (fibrosis) caused by IPF. They may also assist with preventing an unexpected and striking worsening of symptoms over a few days or weeks, allowing patients treated to live longer. These are the only medications officially approved by the FDA specifically for the treatment of IPF. 

The average survival time following a diagnosis of IPF patients is 3 to 5 years. Keep in mind that this is an average and that some individuals survive much longer than five years with IPF. With early diagnosis and appropriate management, many people live normal lives for years after being diagnosed.

IPF is typically diagnosed through a combination of tests, including a physical examination, a chest X-ray, pulmonary function tests, heart tests such as an echocardiogram, blood work, and a CT scan. A bronchoscopy or a lung biopsy may also be performed to confirm the diagnosis.

There are four stages of IPF disease progression, each with its own set of symptoms:

• Stage 1: Mild symptoms, such as shortness of breath and a dry cough

• Stage 2: Moderate symptoms, such as fatigue, weight loss, and difficulty breathing

• Stage 3: Severe symptoms, such as respiratory failure and heart failure

• Stage 4: End-stage disease, which is fatal

IPF is a progressive disease, meaning it gets worse over time. The rate of progression can vary from person to person. IPF can lead to lung failure and death.

The exact cause of IPF is unknown; however, certain risk factors may increase your risk of developing the disease, such as:

• Age: IPF is most commonly diagnosed in people between 50 and 70.

• Gender: IPF is more common in men than women.

• Smoking: Smoking is the most significant risk factor for IPF.

• Family history: People with a family history of IPF are more likely to develop the disease.

• Environmental factors: Exposure to certain airborne toxins, such as those found in certain occupations (e.g., farming, construction) or hobbies (e.g., woodworking), may increase your risk of IPF.

Complications of IPF can include:

• Respiratory failure: This is the most common complication of IPF. Respiratory failure occurs when the lungs cannot adequately exchange oxygen and carbon dioxide.

• Heart problems: IPF can strain the heart and may cause heart failure.

• Kidney problems: IPF can cause kidney failure in some people.

• Depression: IPF can cause depression and anxiety.

• Infections: Patients with IPF are at an increased risk of developing infections, such as pneumonia.