If you watched any professional football in October, you probably noticed several players donning all manner of pink apparel and equipment. It’s the NFL’s way of using its platform to participate in Breast Cancer Awareness Month and is obviously a worthy endeavor – the American Cancer Society estimates that 40,290 will die from the disease in 2015.
While awareness of breast cancer is unfortunately tied to its large number of annual cases, there are conditions with similar totals in terms of fatalities – and higher mortality rates – which are nonetheless relative unknowns to many, even medical professionals in some cases. Idiopathic Pulmonary Fibrosis (IPF) is one such disease.
Difficult to Detect
Speaking with Temple University Hospital’s first Pulmonary Nurse Practitioner, Michelle Vega-Olivo, MSN, CRNP, FNP-BC, she noted that, as its name suggests, “IPF’s pathogenesis is still unknown, as are several aspects of it, although we know do know more now than we have in the past.”
The disease is characterized by an injury to the alveoli, a specific form of chronic progressive scarring (fibrosis) of the lung tissue which makes the delivery of oxygen to the blood more difficult as it develops. Similar to an autoimmune disease, Vega-Olivo noted that “.as the body is trying to repair itself, the condition actually worsens and eventually the lungs stop working altogether. The scarring causes restriction of a patient’s breathing.”
SEE ALSO: Double Drug Approvals for IPF
While IPF’s aforementioned death toll is about 40,000 lives per year, there are only 128,100 annual diagnoses in the United States, according to the Coalition for Pulmonary Fibrosis. As fatal as the condition can be, Vega-Olivo said that the current lack of concrete knowledge about its origins not only puts IPF patients at significant risk, but also can limit service delivery on the part of doctors, NPs and other caregivers.
“We haven’t had as many misdiagnoses in recent years, thanks to increased research and advancement in CT imaging, but patients mostly come in when they are already symptomatic; they typically present with a persistent cough (lasting longer than 30 days), shortness of breath and a crackling sound in their lungs.”
Some of the issues with misdiagnoses, or with patients arriving when they are already symptomatic and it is effectively too late, are the ways in which IPF presents similarly to other chronic lung conditions, from asthma to COPD. As Vega-Olivo mentioned, a normal chest X-ray or CT scan may show changes, but cannot confirm a diagnosis as a high-resolution computer tomography (HRCT) scan can, which is part of the reason why receiving an IPF diagnosis can take so long.
The initially innocuous symptoms and the time-consuming nature of obtaining an accurate diagnosis aside, Vega-Olivo stressed the critically important role that research plays in fighting this condition.
“Two out of three IPF patients will die from the condition itself, while others will die from comorbidities. Just last year, two drugs designed to treat symptoms of IPF (Ofev and Esbriet) were approved by the FDA. Up until then, the only alternative was a lung transplant, which not everyone is a candidate for.”
Educating, Treating and Advocating
As approved drugs emerge to aid in the treatment of IPF, doctors, nurses, nurse practitioners and respiratory therapists continue to seek out more techniques to care for those affected by it. The American Thoracic Society first published guidelines regarding treatment of IPF in 2002, with updates being made in 2007 and May of this year.
Vega-Olivo cited pulmonary rehab as something she has found very effective in improving the quality of life for her patients who have been diagnosed with the condition, but stipulated that the care model is oftentimes specific to each person, stating that ” . the condition is so unpredictable, treating it varies from patient to patient. They come into our care at different stages, which affects whether we’re making an early or late diagnosis. Some exacerbations are more acute than others.”
While those working in pulmonary care work tirelessly to aid patients battling IPF, epidemiologists and pharmacologists are working around the clock to study the histopathology of the condition in hopes of uncovering where it comes from – and who might be most at risk of contracting it.
“We know that it typically affects patients older than 50 who often have a background smoking, environmental exposures and GERD, although presumed association with microaspiration,” Vega-Olivo said, also touching on the fact that it affects more men than women. “Vanderbilt and the University of Denver are currently studying a gene from patients who have it that may offer more insight. Otherwise, it helps to keep it in mind if you happen to have a brother, sister, cousin or even a parent who’s come down with it.”
With regard to the current treatment landscape, Vega-Olivo touted the progress that’s been made in terms of advocacy and awareness, while being quick to point out that there is still much to be done.
“The PA-IPF Registry has been instrumental in raising awareness about IPF, as well as providing a hotline, support groups, a newsletter and other information about the condition. The founders had a loved one who passed after fighting IPF, so in 2007 they collaborated with five Pennsylvania medical centers (Temple, Geisinger Medical System, University of Pennsylvania, University of Pittsburgh and Penn State Milton S. Hershey Medical Center) to form the registry. Temple even hosted its advocacy event this year.”
For as mysterious as IPF still remains, groups such as the PA-IPF Registry are helping to lead the way to what will hopefully be breakthroughs in the treatment and possible cure of the disease. With enough education and advocacy, there’s a chance that future IPF patients and their loved ones will be able to do something their predecessors could not: breathe easy.
Tamer Abouras is Assistant Editor of ADVANCE for Respiratory Care & Sleep Medicine
