Dr. Leslie Tolle, MD, interim section head for diffuse parenchymal lung disease in the Respiratory Institute at the Cleveland Clinic, talks about a new treatment, called “lung washing,” that is helping people with rare autoimmune disease.
Interview conducted by Ivanhoe Broadcast News in May 2022.
What is PAP and what does it stand for?
DR TOLLE: PAP stands for pulmonary alveolar proteinosis. It’s a disease where the lungs ineffectively metabolize proteins and protein-rich fluid fills the lungs instead of air.
Is it a rare disease?
DR TOLLE: Yeah, it’s super rare. It’s what we consider an orphan lung disease, meaning incredibly rare.
What causes the condition and what are the symptoms?
DR TOLLE: There’s a number of different causes for the disease. But most commonly, we don’t know why it happens. The most common symptoms are shortness of breath, cough, and the need for supplemental oxygen.
Does PAP masquerade as any other conditions? Is it sometimes difficult to diagnose?
DR TOLLE: Yeah. Unfortunately, many lung diseases present shortness of breath and cough, and patients most commonly are diagnosed with bronchitis or even COPD or emphysema. So, if you’re not thinking about it, you’ll never diagnose it. It can be kind of tricky to diagnose, especially if it’s not on the tip of your tongue, so to speak.
What is considered the first line of treatment?
DR TOLLE: Oftentimes, we will do a whole lung lavage as first line treatment and sometimes we will use some off-label inhaled medications which are currently under investigation.
Is PAP potentially fatal?
DR TOLLE: Absolutely. It can be fatal because, ultimately, if it gets bad enough, it can lead to respiratory failure. If you don’t treat it in time, people can absolutely die from it or necessitate a lung transplant.
Can you describe lung washing and walk us through the process?
DR TOLLE: A lung wash is a procedure where they independently ventilate each lung. What they do is put a patient under general anesthesia and shut off the breathing to one side through the breathing machine. What they’ll do is put fluid in that one side and suck that fluid back out. They keep doing that until the fluid coming out is the same color as the fluid going in, which is clear. So, it starts out as this milky white fluid and then, as you wash it out, it gets clearer and clearer. After 20, 30, 35, 40 liters, it will become crystal clear. Then, you switch and do the other side. Sometimes they do them together and sometimes they do them one day apart. But that’s the general procedure.
How effective is the process?
DR TOLLE: The procedure itself can be quite effective. Patients can go from needing a lot of oxygen and being very symptomatic, walking room to room across their house to not needing any oxygen and having no shortness of breath at all. So, when done well and done successfully, it can be remarkable and life changing.
Do patients need additional procedures?
DR TOLLE: Some patients do need more than one whole lung lavage and some patients can get away with one in their entire life and not have it again.
Can you tell us a little bit about your patient, Paula?
DR TOLLE: She’s had kind of a complicated course. She’s had a number of whole lung lavages. I believe her most recent one was in 2019. Since that time, she’s done incredibly well and has only had minimal shortness of breath and hasn’t really needed oxygen in a few years, now and has been pretty stable off of any treatment for at least six months now.
How is she doing now, and will she need any additional follow-ups?
DR TOLLE: She’s doing quite well, now. We’re going to follow her probably every six months for the foreseeable future with breathing tests, X-rays and sometimes CAT scans, as well just to get a sense of how she’s doing and if her disease is coming back.
Is there a difference during the pandemic? Is PAP more harmful to patients like this that have had lung lavages or that have this condition? Is COVID a complicated factor for them?
DR TOLLE: Yes, certainly anyone with underlying lung disease is going to be at higher risk of complications from COVID. And I don’t think, in that sense, PAP is unique to any other lung disease. It certainly can complicate things, especially if you have indolent disease or somewhat low active disease. COVID could make that worse or certainly hit you harder than the average person who doesn’t have any underlying lung disease.
What exactly are you looking for with that lung function test that Paula did earlier?
DR TOLLE: What we’re doing is looking at two different things. We are looking at how much air she’s able to take in and blow out when she takes a maximum breath. Because what happens is, when your lungs fill with fluid, you’re not able to take in and blow out as much air. It’s a reflection loosely of how much air she’s able to take in and blow out. And then, the other thing we’re looking at is how well her lungs absorb oxygen, because you can imagine, if your lungs are filled with fluid, you don’t absorb oxygen as much as you should. And so that’s the other number we’re looking at is how well she’s absorbing oxygen.
Is there anything else you want people to know about either about PAP or the lung wash?
DR TOLLE: I think it’s important for patients to get together and congregate about their disease. Because it’s so rare, it’s hard to study, so, there’s fantastic networks of patients out there to help advance research as well as for patient support so we can learn more about this disease, learn more about how to treat it, how it behaves and how to treat it. And the more patients we have, the better. So, if there’s anyone out there that’s diagnosed and isn’t involved in these patient support groups or in these large centers that treat the disease frequently, I would advise them to go to those centers so that they can help themselves and help advance the disease and help others.
END OF INTERVIEW
This information is intended for additional research purposes only. It is not to be used as a prescription or advice from Ivanhoe Broadcast News, Inc. or any medical professional interviewed. Ivanhoe Broadcast News, Inc. assumes no responsibility for the depth or accuracy of physician statements. Procedures or medicines apply to different people and medical factors; always consult your physician on medical matters.
If you would like more information, please contact:
Alana Wyche
Sign up for a free weekly e-mail on Medical Breakthroughs called First to Know by clicking here
