All about interstitial lung disease, what is is and how to treat it.
For most of us, most of the time, a cough means a bad cold or flu. In 2021, we might think COVID. But for Cynthia Kear, a nagging cough and a “weird” sensation in her chest turned out to be something else entirely.
Healthy until then, she found herself facing a tough diagnosis. She had interstitial lung disease, also known as ILD, and would need a lung transplant in only a few years’ time.
ILD is rare, but early diagnosis may give doctors a chance to slow or delay its progression. Here are some things to know about ILD and its treatment, including lung transplant.
ILD is not a single disease.
ILD actually refers to a group of 200 different lung conditions that affect a part of the lung called the interstitium. This network of tissue surrounds and supports the lung’s tiny air sacs.
Interstitial means “lying between things.” This tissue lies between the air sac and the blood vessels. When you breathe in, oxygen crosses this tissue into the bloodstream to be delivered throughout the body. This process happens in reverse to expel carbon dioxide.
What these lung conditions have in common is that they cause the interstitium to thicken, with scarring, inflammation or fluid.
Causes of ILD vary – and may be unknown.
Many things can contribute to development of ILD including:
- Bacterial, viral or fungal infection can cause interstitial pneumonia.
- Breathing in dust or other particles as part of your job can cause disease, including “black lung disease” in coal miners.
- Inflammation can cause ILD, including autoimmune diseases or sarcoidosis, which can affect many organs but especially lungs and lymph nodes.
- There are some genetic causes of ILD as well.
And some ILD is considered “idiopathic,” meaning the cause is not known. One of these is idiopathic pulmonary fibrosis, which scars the interstitium.
Symptoms of ILD and its treatments
Most everyone with ILD will be short of breath. This symptom may get worse over time. ILD may cause a dry cough, and in some cases, people with ILD will experience weight loss.
For many forms of ILD, symptoms develop over time. For some, especially caused by infection, symptoms may come on quickly. A lingering cough, and especially being short of breath, should never be ignored and warrant seeing your doctor.
How ILD is treated depends on the severity. Treatments can include medicines, like steroids or anti-fibrotic medications, supportive therapy, like oxygen or pulmonary rehabilitation, and in severe situations, a lung transplant.
What makes a good lung transplant candidate
Cynthia Kear was sicker than she realized at first. Her illness was managed with medicines and pulmonary rehabilitation for nearly 2 years before her lung specialist referred her to his colleagues in the Vanderbilt Lung Institute.
“I was the last to admit I was getting worse, because that would make it real,” she said.
Dr. Ciara Shaver, one of Kear’s Vanderbilt pulmonologists, said Kear made an ideal candidate for lung transplant for three reasons:
- She was highly enthusiastic and motivated for her transplant. “I look for someone who is motivated to fight for their health,” Shaver said.
- She had a strong support system. “Whether it’s family or friends, it doesn’t matter, as long as you have an army of people that will work with you to get you through the tough parts of transplant and be able to celebrate the good parts.”
- She was realistic and flexible. “There’s lots of wonderful things about transplant, but there are some bumps in the road,” Shaver said. “When our patients get to a bump, they need to understand that we have a plan and they need to be flexible to work with us, to accomplish the goals necessary to get them through those bumps.”
It is also important, Shaver said, for individuals with ILD and their doctors to consider referral to a highly specialized team of lung disease experts, like VLI, early in the illness.
“When we meet with patients referred for lung transplant, we first make sure we’ve exhausted all other options,” she says, adding that patients can be scheduled at VLI within a few weeks at most. “There may be modifiable things we can work with a patient to make transplant more successful.”
The Vanderbilt Lung Institute brings together experts in diagnosing and treating lung disease from pulmonary medicine, thoracic surgery and allergy. This unique approach gives hope through coordinated, personalized and compassionate care for even the most complex conditions that affect the lungs and breathing.
