Home tests for breathing capacity work well for many ALS patients, streamline their care

Host Amber Smith: Upstate Medical University in Syracuse, New York, invites you to be The Informed Patient, with the podcast that features experts from Central New York's only academic medical center. I'm your host, Amber Smith.

Like most medical offices during the pandemic, providers in Upstate's ALS research and treatment center had to adapt the way they cared for their patients with amyotrophic lateral sclerosis. Today, I'm talking with Dr. Eufrosina Young about some things they did that worked so well, they've now become routine practice. Dr. Young is a clinical neurophysiologist who's director of the ALS center at Upstate. Welcome to "The Informed. Patient," Dr. Young.

Eufrosina Young, MD: Thank you for having me, Amber.

Host Amber Smith: Now, many doctors began offering virtual visits during the pandemic, and many continue to offer virtual visits for some things. Did the ALS center switch to seeing patients virtually?

Eufrosina Young, MD: ALS multidisciplinary clinics, like most clinics during the pandemic, utilized video teleconference or telehealth to connect with patients at the start of the pandemic.

It is quite challenging to have multiple therapists engage a patient and caregiver. So we learned early on to be more deliberate in addressing different patient needs. Overall, telemedicine by itself proved inadequate, and the huge gap was monitoring respiratory function.

Host Amber Smith: Let me ask you, before we get too much more into this, to describe the disease ALS.

Eufrosina Young, MD: ALS is a neurodegenerative disease that destroys brain and spinal cord. Motor neurons slowly die, leading to loss of voluntary motor function. Weakness leads to paralysis of different muscles. What starts is an impairment in functions, the ability to speak, pick up a cup, walk unaided, feed by mouth, ultimately ends in complete loss of functions, whether it be gait failure, quadriplegia, loss of speech.

Feeding tube, wheelchair, ventilator -- these are eventually required as the disease takes its course. Ultimately, ALS leads to respiratory failure.

Host Amber Smith: Well, I'm curious, because you mentioned the multidisciplinary team at the ALS center. Pre-COVID, how did things work with patients coming in? Did they see various providers and therapists in one visit?

Yes, you are correct, Amber. The multidisciplinary clinic model saw patients on a quarterly basis and measured pulmonary function, speech, swallowing, nutrition, gait and dexterity with the help of various therapists that come to our clinic.

Our patients had access to an ALS clinic nurse, coordinator, social worker, spiritual palliative care, dietitian and an ALS physician overseeing their treatment plan.

So it sounds like some of those things might be able to be done virtually, but it sounds like you've got things that need to be done hands-on with this patient population, as well.

Eufrosina Young, MD: Exactly. There are just so many things that you can do using the teleconference, or virtual mode, of interacting with patients and caregivers. There is quite a bit of information and examination that needs to be done face to face.

Host Amber Smith: Now, one of the things you measure regularly is pulmonary function. And because that's an aerosol-generating procedure, there was a high risk of COVID exposure. So what did you do about that during the pandemic?

Eufrosina Young, MD: So pulmonary function test laboratories, across the country, shut down to mitigate COVID-19 exposure from, like you describe, an aerosol-generating procedure.

Once Upstate approved the resumption of elective outpatient services based on the trajectory of COVID-19 in the community and the availability of personal protective equipment, the pulmonary function laboratory at Upstate instituted requirements for infection-control measures to mitigate the spread of infection in patients and health care personnel that were returning to an in-person clinic encounter.

So we had dedicated negative pressure rooms with HEPA, or high-efficiency particulate air, filters. These rooms were disinfected and aired for 30 minutes in between patients. And of course staff used N95 (highly efficient) masks with face shields during respiratory testing.

Host Amber Smith: Your center made use of a $5,000 grant from the Upstate Foundation's Tim Green Endowment to Defeat ALS. Can you tell us how the money was spent?

Eufrosina Young, MD: We, first of all, inquired with Upstate pulmonary services, pediatric cystic fibrosis clinic and ultimately consulted with the U.S. Cystic Fibrosis Foundation to learn how they were maintaining the care for their patient population. We learned that internet-based respiratory remote spirometry, using handheld, portable spirometers (devices to test breath capacity) and software application, downloaded in a patient's personal smartphone, can be used to obtain pulmonary function tests from home.

And this is where the Upstate Foundation came into the picture. The Tim Green Fund of $5,000 was used to purchase 50 portable spirometers, and these spirometers cost about a hundred bucks each, and they were single-patient use and issued to patients willing to monitor their vital capacity from home.

Host Amber Smith: How do those handheld spirometers that are done in the home compare to the pulmonary function tests you do in the office?

Eufrosina Young, MD: That's a good question. And it was actually a question that we ourselves wanted to answer. So the accuracy and the feasibility of measuring respiratory function has been shown in pulmonary research, but not in ALS. We started by testing baseline respiratory function two ways, using our conventional spirometer and portable spirometer, then deployed portable spirometers to patients' homes. Now, except for the rare occasion where patient anxiety was aggravated with testing, majority of patients and caregivers used this with ease. If they had a strong internet connection, patients are coached by Upstate respiratory therapists to perform vital capacity measurement with results that are generated in real time. So facilitating remote monitoring for patients allowed for our patients to avoid travel to clinic at the height of the pandemic. And this was a game changer, particularly for patients that were quarantined or patients that were homebound.

Host Amber Smith: So, these home pulmonary function tests have already been used in diseases like cystic fibrosis. Are there other applications for it? Do you know, are there other patients that are making use of this technology in the home?

Eufrosina Young, MD: Yes, Amber, this technology has been used in specialized pulmonary clinics for cystic fibrosis, asthma, COPD (chronic obstructive pulmonary disease). So these are pulmonary diagnoses, but not in ALS clinics. There is a practice gap. I can describe this as a practice gap that exists between ALS neuromuscular and pulmonary practices. Now, this technology has been tested in pulmonary and ALS research, but not used in ALS clinics.

So we tried to bridge this research-to-practice gap by implementing what we now call at-home telespirometry, or AHT, in collaborative effort with our colleagues in the pulmonary department. So that's Dr. Dragos Manta, Dr. Birendra Sah, and respiratory department physiotherapists Elizabeth Rescorl, who's now retired, Scott Hildebrant, Christine Cottet and others.

The technology has existed. The research to support its use has existed. And with this collaboration, we were ready to transform our ALS clinic.

Host Amber Smith: This is Upstate's "The Informed Patient" podcast. I'm your host, Amber Smith, talking with Dr. Eufrosina Young. She's the director of the ALS center at Upstate.

How are you able to get more grant funding to expand the home spirometry program?

Eufrosina Young, MD: I believe that you really must get out of your clinic, get out of your offices, get out there and find people that are just as dedicated to improving the care of our ALS population. So early on, we partnered with Atrium Health in Charlotte, North Carolina, and (neurologist) Dr. Benjamin Rix Brooks, whose guidance and personal mentorship helped launch at-home spirometry even further. For an investigator-initiated study involving these two institutions, we were able to expand home monitoring to more ALS patients and extend availability of services through the now third year of the pandemic.

Host Amber Smith: Do all patients with ALS have the ability to use the pulmonary function at home? You did mention some had some anxiety about it, but in general, physically and medically, are they able to use it at home?

Eufrosina Young, MD: Yes, Amber, we actually encourage patients and their caregivers to participate in testing.

It is true that over time, even as a patient is able to use the equipment independently, the need for assistance from caregivers, more often than not, would be necessary. And I think that our patients have been quite happy with the ease of use. And so have the caregivers.

Host Amber Smith: How does it work? Can you describe what it looks like and what the patient has to do? Do they breathe into a tube?

Eufrosina Young, MD: Yeah. So, it's a small, handheld, portable device, and essentially the device, the portable spirometer, talks to the software application on a patient's smartphone. So the application is downloaded prior to the visit, and the access to the clinical dashboard and the respiratory therapist happens through the patient's smartphone.

So all the patient has to do is to set up an appointment with the respiratory therapist. They know to get online at that point in time. The dashboard is activated, and the patient breathes into the spirometer with the respiratory therapist on the screen, coaching and providing guidance as to how to do the procedure. And the data is generated.

The vital capacity can be seen on the screen, both by the patient and the respiratory therapist, in real time. And so, you know, it sounds a lot, like it might be a lot for someone who's not technology savvy. And in fact, most of our patients are within the elderly age group, and either they are able to engage by themselves, or they have family helping them.

Most of our patients tell us it takes about five to 10 minutes to get all this set up and done, from the comfort of home.

Host Amber Smith: Is this something where if the patient was having symptoms or feeling as if they were having more trouble breathing, they could do a test that day rather than trying to make an appointment to come in to the center? And does this kind of allow them a little more flexibility?

Eufrosina Young, MD: That's a very good question, Amber. In fact, when I have had meetings with our team, the question that has come up is if this can be available on demand, and really the technology is there to allow for that availability.

However, there's more to it than just the patient calling to say that, "Hey, I'm having symptoms, and I need to get tested now." So, a couple of things have to be lined up. One is the availability of respiratory therapy to get online. The physician overseeing the treatment plan has to be informed and able to direct the care because, essentially, what you're running is testing from home, wherein a patient is quite symptomatic and can very well show that the data is abnormal and requires immediate attention, not "I'll see you at clinic in a month."

There is nursing care that needs to be available, physician care, respiratory therapists ... so these have to be integrated with the technology availability in order for this technology to be useful and helpful for our patient population.

Host Amber Smith: Can you tell us what it means that Upstate's center is part of the ALS Association's network of certified treatment centers of excellence?

Eufrosina Young, MD: Yes. So we've expanded services, and we have a hybrid model of telehealth and in-person, and this allows for flexibility in meeting individual needs, as well as accessibility to clinic services. But pre-pandemic, we've had therapists and physicians, and we've had research involvement in these, built up over time.

The ALS clinic at Upstate has had this designation of certified treatment center for excellence for clinical care and research since 2016 and access to multidisciplinary clinic services and research with clinical drug trials have been in place since. So the clinic has grown to a dozen team members providing care to patients in Central New York and beyond.

And, we've developed this mantra, and we tell our patients, when they come into our clinic, "We've lined up an army for you."

Host Amber Smith: And in addition to the medical care you and that army you spoke of provide, you must have to deal with the exhaustion and frustration that the patients and their families and caregivers face as this disease keeps bearing down.

Eufrosina Young, MD: Yes. So, we deal with a lot of frustration, right? And we also deal with a lot of thankfulness.

And I think it can take its toll on caregivers the same way it can take its toll on the clinic, the nurses, the doctors, but I think at the end of the day, we just think about, "Hey, what else can we do more? And what else can we do better?" So that anything and everything bad that's happened throughout that day or throughout that week kind of just fades away in the background.

So what else can we do a little more, a little better? And that's what the remote monitoring is about. It's amazing, but it's really a story, you know, one that I like to tell my kids. My kids are little. I like to tell them that. And I think I'll tell my grandkids, "You know what we did during the pandemic? We were all losing our minds, and we were all scared out of our wits, and we were all like, oh, my God, what's going to happen to our patients?

"But then we did this."

Host Amber Smith: And what a difference it made. Well, I really appreciate you making time for this interview, Dr. Young.

Eufrosina Young, MD: Well, thank you so much for having me, Amber.

Host Amber Smith: My guest has been Dr. Eufrosina Young. She's the director of the ALS center at Upstate "The Informed Patient" is a podcast covering health, science and medicine, brought to you by Upstate Medical University in Syracuse, New York, and produced by Jim Howe. Find our archive of previous episodes at upstate.edu/informed.

This is your host, Amber Smith, thanking you for listening.