It’s a disease for which there is no cure, but advocacy and awareness can help change that. Many patients assume pulmonary fibrosis symptoms are related to old age, being overweight or a heart condition. While the signs – shortness of breath, dry cough and fatigue – are nonspecific, the condition itself is more serious than just breathlessness after a walk, says Amy Hajari Case, M.D., a pulmonologist at Piedmont Physicians Georgia Lung.

Pulmonary fibrosis is an interstitial lung disease, meaning it causes progressive scarring of lung tissue. The scar tissue makes it difficult for the lungs to work properly. For idiopathic pulmonary fibrosis (IPF), the cause of the disease is largely unknown.

Diagnosis

IPF patients often first seek care from their primary care physician or cardiologist. They usually don’t see a pulmonologist or have a pulmonary evaluation until they are much further along in the disease process, says Dr. Hajari Case. During a pulmonary evaluation, the pulmonologist will discuss the patient’s history of potential exposure to toxins and pollutants, as well as other conditions that can interact with lung function. Pulmonologists can sometimes diagnosis IPF based on those factors, along with images from a high resolution CT scan. “We rely heavily on imaging,” says Dr. Hajari Case. “High resolution CT scanning gives a lot of details about the lung tissue and pattern of lung injury.”

Treatment options

There is currently no cure for IPF and no FDA-approved therapies in the United States. “I encourage them to look into clinical trial options,” she says. “We have some of those available here at our practice.” Dr. Hajari Case says there are options that can improve a patient’s quality of life, including:

• Therapies that help manage symptoms like coughing and shortness of breath
• Prescribed supplemental oxygen, when appropriate, to help patients maximize the activities they are able to do
• Physical therapy through a pulmonary rehabilitation program
• Support groups

“When I’m talking to patients, we have to understand the limitations of our current medical therapies,” says Dr. Case. “That’s important, but the other thing I always focus on is what we can do to help a person. Education is very empowering.”

She says her patients benefit from meeting others, learning about the disease and how to manage it, and staying up to date about new treatments on the horizon – including some to which they could possibly contribute. “They could potentially be contributing to that knowledge by participating in research therapies,” she says.

While there are limited options for those with IPF, communities can raise awareness of the disease and advocate for patients – all of which will hopefully lead to a cure in the future.  

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