Written by Todd Neff on behalf of National Jewish Health
About 650,000 people in the United States have interstitial lung disease (ILD). That’s an umbrella term for more than 200 conditions that cause inflammation or scarring inside the millions of tiny air sacs that let our lungs collect oxygen and discard carbon dioxide. People with ILD have trouble breathing and, over time find it harder and harder to breathe.
Those diagnosed with ILD used to be given three to five years to live. That’s changed, and setting the record straight on that account is one of seven things everyone should know about interstitial lung disease.
1) It’s Not “Get Your Affairs in Order” Anymore
That three- to five-year prognosis is outdated for all but one form of ILD, according to pulmonologist Joshua Solomon, MD, who leads the Center for Interstitial Lung Disease at National Jewish Health. He adds that early diagnosis and promising experimental therapies also can extend the lives of those with that one form, idiopathic pulmonary fibrosis or IPF.
A trend toward earlier diagnosis – combined with two U.S. Food and Drug Administration (FDA)-approved medications and several other promising drugs now in clinical trials at National Jewish Health and elsewhere – are among the reasons that, as Dr. Solomon says, “ILD is no longer a death sentence by any stretch.”
2) Old Age Does Not Mean You Should Be Short of Breath
Aerobic capacity declines with age. But that doesn’t mean you should find yourself suddenly out of breath from a walk up the stairs.
“We shouldn’t be chalking things up to old age until we make sure there isn’t something else happening,” Dr. Solomon said.
Similarly, while most coughs aren’t caused by ILD, a persistent cough, especially if it comes with more breathlessness, merits a doctor visit. That’s important because, if the cause is ILD, the earlier you are diagnosed, the better your prospects will be.
3) There Are Medications – And More on the Way
The two FDA-approved ILD drugs – nintdanib (Ofev®) and pirfenidone (Esbriet®) – may be joined by several drugs now in trials. The furthest along are pamrevlumab and BI 101550, both of which are in phase 3 clinical trials. Phase 3 trials involve hundreds or more patients. If they show the drugs to be effective, they lead to FDA approval so doctors can prescribe them. Both of these drugs have been shown to slow the progression of the lung scarring – called pulmonary fibrosis – that causes IPF and other forms of ILD-related scarring to progress.
“These two drugs could potentially be home runs in terms of slowing down scarring,” said Dr. Solomon. “I think there’s so much to be hopeful for in the near future.”
4) Lung Transplant Is an Option
There are two big misunderstandings about lung transplants for ILD patients. The first is that the disease will come back. The second is that, if you’re over 65, you’re too old for a lung transplant.
According to Dr. Solomon, neither is true. “Transplant is a cure, and we transplant people into their 70s. They do really well,” he says. “You will live a better life after transplant.”
5) There Are ILD Specialists, and They Make a Big Difference
If you have ILD or suspect you do, get to a center with expertise in treating ILD.
“Treatments for interstitial lung diseases are very complex,” Dr. Solomon says. “It’s not just about prescribing a drug to patients.”
Diagnosis involves more than just understanding what form of ILD is involved. Causes can range from autoimmune disease and environmental influences to reasons unknown. ILD experts also determine whether patients have sleep apnea or acid reflux, both of which may make ILD worse.
“If we think you have untreated reflux, we’re super aggressive in treating it, because we think it’ll slow down the progression of your disease if we get control of it,” explained Dr. Solomon.
IPF specialists consider blood-oxygen levels at night and during exercise to better grasp how serious the disease is. That’s important because patients with declining blood oxygen levels don’t always notice it until it’s a serious problem.
The heart is a special focus. Cardiac specialists look for pulmonary hypertension (which strains the right side of the heart) and blockages to the arteries feeding the heart.
Mood disorders, nutrition and the patient’s tolerance for and willingness to exercise and do pulmonary rehabilitation also come into play. IPF affects the whole person, and so too must IPF care.
6) Exercise Is Medicine for ILD Patients
ILD medications slow the rate of scarring, but they don’t make you feel better, Dr. Solomon says. Exercise and fitness – even walking – can make you feel better.
“We know that, if you participate in pulmonary rehab, or you just stay in shape, your quality of life is better, and your breathlessness is better,” Dr. Solomon says. “It also helps your mental status as you take charge of your own health.”
7) There Are Support Groups Out There for You
If you have any type of ILD or are caring for someone with ILD, know that help is out there. There are support groups such as those at National Jewish Health, other ILD centers, and national organizations such as the Pulmonary Fibrosis Foundation. Caregivers can find resources at the Pulmonary Fibrosis Foundation’s caregiver pages, as well as the Family Caregiver Alliance.
“ILD is a rare disease, but you are not alone.”
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