What are the stages of idiopathic pulmonary fibrosis?

Idiopathic pulmonary fibrosis, or IPF, can be a frightening diagnosis. IPF is a rare, progressive lung disease that develops differently for everyone, and managing its development can be difficult. However, some individuals are able to live with the disease and stay active for many years. 

While there are no formal categories or stages in regard to IPF, medical professionals and patients often like to organize the condition into a few different sections defined by a person’s symptoms and treatment requirements, which is what we’ll take a look at below.

A guide to IPF stages

Stage 1: Recently diagnosed

When a person is first diagnosed with IPF, doctors typically conduct diagnostic tests to further understand the progression of the disease and what treatment options might be most effective. They will ask about symptoms and severity, and perform Pulmonary Function or Lung Function Tests to gauge how much air the lungs can hold. They may also conduct a walk test to measure an individual’s shortness of breath during activity.

The early symptoms of IPF can sometimes include fatigue, shortness of breath, and a distinctive, dry cough, but some people do not experience symptoms at all in this first phase. For patients at this stage, there are two FDA-approved IPF treatments, Esbriet and OFEV, which doctors may recommend in addition to quitting smoking and continuing exercise.

Stage 2: Oxygen is needed only with activity

As IPF advances, it becomes more common for patients to experience coughing more frequently, in addition to shortness of breath with activity. Doctors will typically advise using oxygen at this stage, and patients can use a pulse oximeter to make sure they are utilizing it appropriately. Though this can make exercising more cumbersome, it is important to stay active at this stage.

Stage 3: Needing oxygen throughout the day 

In the third stage, patients will feel shortness of breath with activity and will experience low oxygen levels at rest. Cough and fatigue will continue to be bothersome, but patients will not typically feel shortness of breath without exertion. 

Stage 4: Advanced oxygen needs

Eventually, a portable, lightweight oxygen delivery system will no longer be sufficient for a patient’s needs, and doctors will then recommend high-flow oxygen delivery. Often, high-dose oxygen will require a patient to stay at home or in the hospital, meaning the fourth stage can be an isolating one.

While receiving an IPF diagnosis and managing the condition’s progression can be difficult, there are options for those affected. Clinical trials are routinely taking place to examine new treatment options — click the button below to learn more.