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Interstitial Lung Disease: Complex Condition Warrants Special Care

Interstitial lung disease presents serious challenges not only to patients, but often to their doctors. It's a complex set of lung conditions involving the interstitium. Usually superfine, not even visible on chest X-rays or CT scans, the interstitium can thicken. When it does, several different dangerous conditions can emerge.

The interstitial lung disease (ILD) program at The University of Kansas Health System is a national center of excellence and one of the few programs in the Midwest to offer comprehensive care for these rare diseases that can lead to pulmonary fibrosis. Several causes may drive the condition, including autoimmune disease – such as rheumatoid arthritis, polymyositis and scleroderma – or environmental exposure – as seen in patients with bird fancier's lung, farmer's lung or exposure to mold in one's own home. It can be idiopathic, which may indicate an underlying genetic cause that can result in more rapid progression to respiratory failure.

The outlook

In the case of idiopathic pulmonary fibrosis (IPF), it was only a few years ago that the average survival was just 27 months, with more than 40,000 people dying from the disease annually, a figure on par with breast cancer mortality. In many cases, the condition developed slowly, remaining a subclinical disease for a year or more. This could often be attributed to the fact that the symptoms of pulmonary fibrosis seem minor and are easily dismissed. In the early stages of the disease, many patients may only notice shortness of breath on stairs and inclines and attribute it to older age or deconditioning. Others may experience chronic cough symptoms easily attributed to more benign causes.

A study of data from a patient-reported outcomes registry recently revealed that patients, on average, were misdiagnosed 2-3 times – often believed to have chronic obstructive pulmonary disease or asthma, or treated for reflux or allergies – and spent a year or more working toward an accurate diagnosis. The study indicated that, by the time patients were correctly diagnosed with interstitial lung disease, almost half were irreversibly oxygen-dependent.

A specialty team

Our multidisciplinary team provides experience to help make an impact. Comprised of pulmonologists with advanced training in interstitial lung disease, as well as dedicated thoracic radiologists, lung pathologists and rheumatologists, our team offers the highest levels of expertise in both diagnosis and treatment. In 2014, the Pulmonary Fibrosis Foundation (PFF) began to identify and develop a network of Specialty Care Centers. Our program was among the first 20 of a current total of just 43 centers across the U.S. Programs awarded this distinction are those that "embrace a multidisciplinary approach that delivers comprehensive patient care." The collaborative team strategy ensures patients benefit from accurate diagnoses, access to comprehensive care and resources to fully manage their diseases, and the opportunity to participate in innovative research with our partners at the University of Kansas Medical Center.

Leading the way

One example of our position at the forefront of diagnosis and treatment of ILD is our approach to lung biopsy, which is critical in determining the precise form of ILD. This procedure traditionally entailed surgery, and we partner with our cardiothoracic surgeons when a traditional approach is necessary. But, with increasing frequency, we perform cryobiopsy, an outpatient procedure that presents lower risk and fewer complications while obtaining the tissue sample essential to effectively diagnose and treat ILD.

We participate in clinical trials leading to the approval of the two current FDA-approved therapies. Studies have shown up to a 50% discontinuation rate of these therapies in some community clinics; our long-term experience with these drugs has enabled us to fine-tune these therapies to manage the serious side effects, allowing 80-85% of patients to more easily tolerate treatments that are helping most patients live at least 2 years longer.

The power of collaboration

In addition to our advanced diagnostic options and access to clinical trials, we actively explore ways to improve diagnostic efforts early.

One program partners with the University of Kansas Medical Center's telehealth program to provide continuing education for providers utilizing Project ECHO. Project ECHO is an interactive, case-based learning platform that connects providers with our specialists for real-time discussion on diagnostic and treatment nuances of these diseases. We see this as an opportunity for community providers to incorporate their practice into the larger Pulmonary Fibrosis Care Center Network.

Another program that garnered national attention is the Cough 55 Initiative. The goal was to improve early diagnosis of the disease before extensive fibrosis develops. Part of the difficulty of diagnosing ILD early lies with failure to recognize subtle symptoms in the initial stages of the disease. Chronic cough may allow us to improve early detection efforts. Patients with asthma or COPD may cough 70-200 times per day. The pulmonary fibrosis patient typically coughs 450-1,400 times per day. Consequently, we are focusing on those cough symptoms to enhance early recognition of the disease.

In 2016, we started to partner with primary care providers across the state of Kansas as part of the Cough 55 Initiative. Primary care providers obtain high-resolution CT scans in patients over age 55 reporting chronic cough symptoms unresponsive to common management strategies in the chronic cough guidelines. Thus far, patients have been identified early with preserved lung function.

This engagement resulted in our seeing new patients during that timeframe with an average forced vital capacity (FVC) of 90% and diffusing capacity of the lung (DLCO) of 70% with none of the patients requiring oxygen. This is in significant contrast to the Patient Reported Outcomes Registry, which showed an average FVC of 72% and DLCO of 46%.

We continue to research barriers to early detection and recognition of the disease among primary care providers and patients.

To consult with a physician or refer a patient, call 913-588-5862 or 877-588-5862.

Mark Hamblin, MD, pulmonary and critical care specialist

Dr. Hamblin's clinical practice is focused solely on interstitial lung diseases and most of the clinical trials in IPF. Our ILD program is recognized as a center of excellence by The World Association for Sarcoidosis and Other Granulomatous Diseases, The Rare Lung Disease Consortium and the LAM Foundation. As such, Dr. Hamblin also sees patients with pulmonary and extrapulmonary sarcoidosis, lymphangioleiomyomatosis (LAM), many diseases designated by the Rare Lung Disease Consortium as well as provides follow-up care for lung transplant recipients with ILD

We offer a variety of appointment types. Learn more or call 913-588-1227 to schedule now.

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