Written by Todd Neff on behalf of National Jewish Health
There are more than 200 forms of interstitial lung disease (ILD). There are treatments available, but getting an accurate diagnosis is an important first step.
Jeff Swigris, DO, MS, a pulmonologist who specializes in ILD at National Jewish Health, describes diagnosing ILD as “detective work.” ILD experts look for clues and evidence to help diagnose and develop effective treatment plans for patients.
A patient’s background can be telling. About one-third of ILD patients at National Jewish Health have disease that is caused by long-term, repeated inhalation of things like mold or bird droppings. Side effects of medications also can end up hurting the lungs. “When we first meet a patient, we’re asking about what they’ve done, what jobs they’ve had, what they like to do in their spare time – anything that might lead to a worrisome exposure,” said Dr. Swigris.
Another one-third of patients have autoimmune disease (such as rheumatoid arthritis), which is why ILD specialists carefully examine the hands and joints. They also listen to the lungs, focusing closely on the backs and bottoms of lungs, where many forms of ILD begin. High-resolution chest CT scans help narrow things down. As an ILD Center, National Jewish Health brings together different experts in weekly conferences to help sort out complex cases.
With the diagnosis in hand, therapy can begin.
Commonly prescribed medications for ILD target inflammation by calming either the immune system or fibrosis (scarring). For pulmonary fibrosis, several promising medications are now in clinical trials. Currently, only two medications are available for doctors to prescribe for patients with fibrosis: nintedanib (Ofev®) and pirfenidone ( Esbriet®). These drugs slow progression of fibrosis, but they do not reverse fibrosis that is already in the lungs. Whatever drugs are prescribed, Dr. Swigris monitors for side effects and communicates with patients closely. “It’s about really partnering with patients and setting expectations, both in terms of potential benefit and possible side effects.”
Oxygen therapy is a treatment for some patients with ILD. The patient’s voice is especially important here, says Dr. Swigris. Being on supplemental oxygen involves a significant lifestyle change. Patients need to talk through their concerns and get help to overcome challenges.
Oxygen therapy allows patients to be more active in their daily lives. It also can give them relief from symptoms like shortness of breath. Over time, having too little oxygen in the body can be damaging to the brain, heart and overall health.
Pulmonary Rehabilitation and Exercise
Dr. Swigris stresses the importance of physical exercise for all patients with ILD. But he notes that exercise does not heal the lungs of ILD patients. It keeps the heart and other muscles strong and in shape. He recommends focusing on exercise for the legs. Legs are the body’s biggest muscles and they use the most oxygen.
Sleep is a “hugely important” aspect of ILD care that gets far too little attention, according to Dr. Swigris. Many patients with pulmonary fibrosis have sleep apnea. It’s important that patients with any form of ILD have their sleep apnea treated. Sleep apnea, like ILD, is associated with pulmonary hypertension, a condition that can harm the heart.
Centers specializing in ILD may participate in clinical trials to find new medications for ILD. Drugs in trials may or may not work – figuring that out is the goal of the trials. Patients who enroll in the trial have a chance to receive the medication or an identical looking placebo that does not have the drug. The ILD team carefully reviews clinical trials before asking patients if they want to participate. Patients who do sign up are giving their time and effort to help future patients with ILD.
“Through trials, we gain a better understanding of the disease and whether a particular drug works in general to improve the condition,” said Dr. Swigris.
In many trials, all patients who complete the trial are offered a chance to take the drug free of charge in what is called an open-label extension study.
While drug therapy may eventually change things, lung transplant is still the only cure for pulmonary fibrosis. Dr. Swigris says he and his colleagues discuss it with nearly all patients. That discussion is important because there are pros and cons to this major surgery. The biggest positive is that “most patients are going to have an improved quality of life without needing supplemental oxygen – at least for a while – and they will be able to do whatever they want to do without severe shortness of breath or air hunger,” according to Dr. Swigris.
But there are other things to consider. Full recovery from the surgery takes at least three months on average. Also, patients must take quite a few drugs, including strong anti-rejection medications. There’s a lot of ongoing testing and monitoring involved.
“If you believe a patient is going to live longer than five years with a reasonable quality of life without a transplant, you don’t want them to go through transplant surgery,” explained Dr. Swigris. “But for many patients it’s a good option.”
When it comes to ILD, the best decisions about any treatment are made by well-informed patients and their physician partners.
“It really is about figuring out a patient’s values and preferences and trying to meet them,” said Dr. Swigris.