Monkeypox update; testing an anti-seizure therapy; at-home option in ALS treatment: Upstate Medical University's HealthLink on Air for Sunday, Sept. 18, 2022
Infectious disease chief Elizabeth Asiago-Reddy, MD, discusses monkeypox and how much of a public health threat it might be. Neurosurgeon Harish Babu, MD, PhD, describes how regenerative neural cell therapy might help adults with uncontrolled seizures. Neurophysiologist Eufrosina Young, MD, talks about at-home lung-function testing for patients with the degenerative disease ALS, or amyotrophic lateral sclerosis.
Host Amber Smith: Coming up next on Upstate's "HealthLink on Air," what you need to know about monkeypox.
Elizabeth Asiago-Reddy, MD: ... It definitely seems that the fact that some people don't know they're sick is a reason why this is more likely to be spreading now. So their illness is not severe enough to keep them at home. And because of that, they're able to spread this to other people. ...
Host Amber Smith: A look at how regenerative human cell therapy may help adults who have epilepsy.
Harish Babu, MD, PhD: ... The hypothesis here is that if we could increase the inhibition of the area of the brain that is causing the seizures, we may be able to get seizures under control. ...
Host Amber Smith: And we'll learn how patients with ALS adapted to home breathing tests.
Eufrosina Young, MD: ... 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. ...
Host Amber Smith: All that, and a visit from The Healing Muse, after the news.
This is Upstate Medical University's "HealthLink on Air," your chance to explore health, science and medicine with the experts from Central New York's only academic medical center. I'm your host, Amber Smith. On this week's show, a neurosurgeon tells about a clinical trial with potential as a new epilepsy treatment. Then we'll hear how at-home pulmonary function tests rolled out during the pandemic. But first, what's most important about monkeypox.
From Upstate Medical University in Syracuse, New York, I'm Amber Smith. This is "HealthLink on Air." Monkeypox is a public health emergency, and today we'll go over what you need to know and the ways you can protect yourself and your loved ones with Dr. Elizabeth Asiago-Reddy. She's the chief of infectious disease at Upstate University Hospital.
Welcome back to "HealthLink on Air," Dr. Asiago-Reddy.
Elizabeth Asiago-Reddy, MD: Thank you for having me, Amber.
Host Amber Smith: Let's start with some background. This is not a new disease, right?
Elizabeth Asiago-Reddy, MD: Correct. Yes. This was actually originally identified many years ago as a virus that was circulating in West and Central Africa. And the name monkeypox, in fact, comes from an outbreak that occurred in a lab that was studying monkeys. So the natural hosts of this virus are not monkeys or people, but they're rodents that live in West and Central Africa.
Host Amber Smith: So how did it get to the point where this has become a public health emergency in New York? And do you have any predictions for how things are going?
Elizabeth Asiago-Reddy, MD: Yes. So the first indications that things were different than what we've been used to with monkeypox occurred around April of 2022. And that was when doctors in Portugal started to notice a cluster of cases that occurred there. And very shortly thereafter, multiple cases started to be identified around the world. So the fact that these cases were identified in close proximity to each other timewise suggests that there was some kind of sentinel event that resulted in a lot of spread across the globe.
So this was when we said something is definitely very different in this circumstance compared to what has happened in the past. So in the past, this virus has circulated on and off in West and Central Africa. There are two different versions of the virus. The West African version is the one that has been spreading recently. And because of the fact that these cases typically were easily recognized by the rash, they've been able to get public health involved and stop the spread pretty quickly locally when it occurs. So this was a surprise to see so many cases coming up internationally. There have been international cases before, and they typically have been traced to exotic pets.
Host Amber Smith: So it seems like it kind of popped up suddenly. Could it go away just as quickly?
Elizabeth Asiago-Reddy, MD: The good news is that the data suggests that our trajectory right now is favorable. So it looks like the numbers of cases have started to decline in most places, including the ones that had been experiencing a very large number of cases like New York City, San Francisco, Montreal. And largely this is being attributed to uptake in vaccines in those areas, as well as, probably, too, changes in people's behavior because of concern about the disease spread.
Host Amber Smith: So I know that it doesn't have anything to do with monkeys. But what about pox? Does it have anything to do with smallpox? Do they share any similarities?
Elizabeth Asiago-Reddy, MD: Yes. This is part of the same family of viruses that causes smallpox. And for that reason, we were a little bit better prepared for this outbreak compared to, I would say, COVID for example, where it was more of a de novo (new) virus. We had dealt with a few similar viruses to COVID, but when it comes to monkeypox, we had been preparing for the possibility that smallpox could be used as a biological weapon. And so vaccines and medications had been prepared on that basis. And because these viruses are in the same family, we were able to quickly pivot and try to use those same vaccines and treatments to help with monkeypox.
Host Amber Smith: So, people who've been vaccinated against smallpox, does that offer any sort of protection against monkeypox?
Elizabeth Asiago-Reddy, MD: That's not 100 % clear. The data would suggest that at least three to four years of good, solid protection exists after receiving a vaccine. There's some evidence that it might be quite a bit longer than that, but because individuals are not typically challenged in that setting to find out how long the smallpox vaccine lasts, we're not 100% sure. So people who have been vaccinated in childhood and are wondering whether or not their vaccine is protective, if they're at risk, they would be encouraged to be revaccinated in this setting.
Host Amber Smith: Now, in caring for patients with monkeypox, what have you and your infectious disease colleagues learned about this disease?
Elizabeth Asiago-Reddy, MD: The surprising feature of the current outbreak is the diversity of presentations that we're seeing. So, previously this was considered to have a very standard and classic presentation, where after an incubation period -- which was variable, that could range anywhere from five days to three weeks -- but once people got sick, there was kind of a classic series of events that were thought to occur, which included a few days of fevers and feeling run down, followed by swelling of the lymph nodes, and then followed by this very classic rash that has what we call umbilicated lesions. They're bumps that you can actually feel when you're running your hand across someone's skin. And those bumps often have a depression in the middle of them. That's called the umbilication. And so crops of these would appear and then would disappear, and they were thought to really always be present in every case and visible in various parts of the body.
So what's unique about what we're seeing now is that there are many individuals who appear to have no obvious spots. Or they have very few, maybe one or two. So that's a surprise that we weren't expecting, but that also is very likely the reason why this was allowed to spread more easily than we expected it would be able to. Because whether it's a change in the virus or it was kind of a just "wrong place, wrong time" situation that allowed more spread to occur, it definitely seems that the fact that some people don't know they're sick is a reason why this is more likely to be spreading now. So their illness is not severe enough to keep them at home. And because of that, they're able to spread this to other people.
Host Amber Smith: This is Upstate's "HealthLink on Air" with your host, Amber Smith. I'm talking with Dr. Elizabeth Asiago-Reddy -- she's the chief of infectious disease at Upstate -- about what we need to know about monkeypox.
We've heard that this spreads sexually, but does that make it a sexually transmitted disease?
Elizabeth Asiago-Reddy, MD: You are correct. It does spread sexually, and that appears to have been the primary mode of transmission for this particular outbreak. But we're not classifying it right now as a sexually transmitted disease because of the fact that it could be spread fairly easily outside of a sexual contact, just depending on where the lesions are that the person is experiencing. So if somebody has a rash on their hands, their arms, their face, anything that could come in close contact with someone else, that is the main way that this disease is spread, is the close skin-to-skin contact. It looks like a lot of the early cases involved people who had these rashes in their genital area. And so that's why sexual contact was occurring.
Something that we actually didn't really realize about monkeypox, we've discovered in this outbreak, which is that it seems like wherever the contact occurred the first time is the likely place where the monkeypox rash lesions are going to show up on that individual So if someone experienced a sexual contact, that's probably where they're going to get sick.So, yes, indeed, it very much has been a sexually transmitted disease, but people can get these rash lesions outside of the area where they were initially infected as well. And so that's where like I said any kind of close skin-to-skin contact could result in an infection through somebody even without sexual contact.
Host Amber Smith: So that would explain why we've heard about children that have been infected, right?
Elizabeth Asiago-Reddy, MD: Correct. If somebody had this, regardless of how they originally got it. Obviously caring for children typically involves skin-to-skin contact. So for sure that would place kids at risk in a situation where this was circulating in their household.
Host Amber Smith: Now, how soon after exposure did you say that someone might start developing symptoms?
Elizabeth Asiago-Reddy, MD: It can range anywhere from five days to three weeks after exposure. we're typically seeing something like closer to five to seven days.
Host Amber Smith: And is there a test that tells a doctor for sure that the person is infected with monkeypox? Or how do you determine that that's what it is?
Elizabeth Asiago-Reddy, MD: The test is done by taking swabs from the areas where the rash is. There have been a couple of studies now which have also looked at genital swabs in high-risk individuals who do not have rash lesions. And that's where we've discovered that people in fact can have basically no symptoms and still have this. Again, that relates specifically to genital swabs. So we're not aware of other forms of transmission, where somebody might have this without symptoms. But, if somebody does have rash, then those would be swabbed and sent to a lab for a PCR test. So that's a molecular test looking at the genetic material involved in the virus. And that is why it takes a couple days for that kind of test to come back. And that's where we would make the diagnosis. There have been a couple of reports of the possibility of false negative tests, so we're just still learning more about this and how common it might be. It appears to be rare, but as a clinician, I'm keeping my radar up for cases that look classic, regardless of the test results.
Host Amber Smith: How are doctors typically treating this disease?
Elizabeth Asiago-Reddy, MD: If it's a mild case, then it's really treated only with rest, symptomatic treatment and isolation. So the individual who's sick should be isolated until any rash lesions that they have are completely dried up and they're feeling better. So no more fevers. Primarily, it would be fevers that we'd be looking at as resolving, indicating that somebody was getting better. But if someone is severely ill then there is a pill or IV medication that can be given either in the hospital or as an outpatient. And Upstate has worked together with a team here of individuals across a number of different areas in the hospital and outpatient to gain access to that medication and make it available to patients if needed. The reason why it was a bit more complex than usual is because it's a medication that was originally approved for treatment of smallpox. So currently the use is, it needs to be monitored by the Food and Drug Administration carefully to assure safety and efficacy for monkeypox, since that's not what it was originally studied for
Host Amber Smith: If a family member has monkeypox and they're isolating, can they safely do that at home with family members around? Or is there any danger that this would spread through the air or from a towel or washcloth?
Elizabeth Asiago-Reddy, MD: Those are great questions. So as I mentioned before, skin-to-skin contact is really the primary mode of transmission but there are other things that family members would want to be alert to if they were staying in the same household. The first one is bedding because of the fact that individuals who have a rash might have some of their skin come off on their bedding anyone who would be cleaning that bedding, so accessing that, taking it to a washing machine, etc. That individual could potentially be at risk from that type of an exposure. And additionally, it's possible that very early on when somebody is first becoming sick that there could be maybe some respiratory spread in a close contact situation. So if somebody's really sitting, talking with somebody for a very long time, that type of thing, even without touching them, it's possible.
That's really more theoretical. And all of the evidence that we have so far is that skin-to-skin contact is required. Again, in the past there have been some circumstances where health care workers have become sick from changing the bedding of patients who have monkeypox without using appropriate protective gear. So that is the other known mode of transmission. We would review this kind of thing with family members to make sure that they understand what would and would not be dangerous.
Host Amber Smith: Would the sheets and towels and clothing for this person need to be sterilized, or can just normal washing take care of getting rid of any of the virus that is shed?
Elizabeth Asiago-Reddy, MD: Yeah, just normal washing would be fine. The virus does not survive well on surfaces for any length of time. So there was a long detailed study looking at a household where two members of the household were sick, and multiple surfaces were swabbed. And despite the fact that genetic material was uncovered on most of those surfaces, none of the samples grew virus. So it was thought that by touching those surfaces, that would not be a viable way of transmitting the virus.
Host Amber Smith: So, what is the typical prognosis for someone who tests positive for monkeypox?
Elizabeth Asiago-Reddy, MD: The prognosis is very good, and the vast majority of people will recover from this just fine. There have been five deaths reported internationally in the context of the current outbreak. Not a lot of details have been made available about the individuals who have died, except to say that they were all severely immune compromised. So, exactly what that means, we just don't have the information to be able to say more about that. But compared to other illnesses that we've been experiencing recently, for example, obviously COVID, this is much, much less likely to be a fatal illness. There have been no deaths in the US, of the cases that have occurred here
Host Amber Smith: If someone is infected with monkeypox, do they have immunity, or would you still recommend that they get vaccinated?
Elizabeth Asiago-Reddy, MD: The current illness is thought to provide immunity at least for several years, if not, again, even longer. So similar to what you would expect from a vaccine.
Host Amber Smith: At this point, who should consider being vaccinated?
Elizabeth Asiago-Reddy, MD: Right now, each state has their own guidelines that they're using to help identify people who are at highest risk. And in most circumstances, that involves men who have sex with men who are outside of a monogamous relationship. So those are the individuals who we've been trying to help access vaccines in Onondaga County and including at our practice, Inclusive Health Services, if such patients are in need of a vaccine.
Host Amber Smith: Well, Dr. Asiago- Reddy, thank you for making time to tell us about monkeypox.
Elizabeth Asiago-Reddy, MD: Thank you so much for having me.
Host Amber Smith: My guest has been the chief of infectious disease at Upstate, Dr. Elizabeth Asiago-Reddy. I'm Amber Smith for Upstate's "HealthLink on Air." The potential of regenerative human cell therapy, next on Upstate's "HealthLink on Air." From Upstate Medical University in Syracuse, New York, I'm Amber Smith. This is "HealthLink on Air." Patients from Upstate University Hospital are part of a national clinical trial of a potential new epilepsy treatment. And today I'm speaking about this with the surgeon who administered the first dose of this regenerative human cell therapy.
His name is Dr. Harish Babu, and he's an assistant professor of neurosurgery. Welcome back to "HealthLink on Air," Dr. Babu.
Harish Babu, MD, PhD: Thanks for having me. Good to be back.
Host Amber Smith: This sounds so exciting. A regenerative neural cell therapy for the treatment of neurological disorders. What can you tell us about it?
Harish Babu, MD, PhD: This, indeed, is very exciting.
Seizure is a result of an increased activation of a group of neurons in the brain, and brain cells that come in two kinds. There are excitatory neurons, which increase in activation, and there are inhibitor neurons, which sort of silence the brains. Now, we think that seizures are caused by an imbalance of this excitation and inhibition in the brain.
The hypothesis here is that if we could increase the inhibition of the area of the brain that is causing the seizures, we may be able to get seizures under control. So in this study, subjects, or patients, will undergo injection or transplantation of cells that will generate these inhibitory cells, which we call interneurons, and they secrete a sort of a chemical that silences the brain. Our hypothesis here is that if there are neurons that will increase silencing of the brain, it'll help control the seizures, if not cure the seizures.
Host Amber Smith: So, to sort of calm things down.
Harish Babu, MD, PhD: Correct. Exactly. We think that the seizures is an area of the brain that just increases the excitation and takes over the rest of the brain.
So if you could calm things down, hopefully, the seizures will calm down, and that'll help the patients.
Host Amber Smith: How unique is this therapy? Have you seen anything like this before?
Harish Babu, MD, PhD: This is indeed very unique in the epilepsy world. Similar things are tried in other diseases, but not for epilepsy.
This is the first of its kind for epilepsy, where cells are being transplanted into somebody's brain, an area where seizures are coming from, in the hope that it'll help with the epilepsy. This is the first of its kind, first in human study, for epilepsy patients.
Host Amber Smith: Well, let's give a little bit of background. Currently, what are the treatment options for adults with epilepsy, if they have epilepsy that's nonresponsive to anti-seizure medication? I'm wondering if this is a therapy that could be attempted in lieu of medications?
Harish Babu, MD, PhD: Currently, if you have epilepsy, or if your seizures are not controlled with medication, most of the treatments would fall within two categories, first being you'll have surgery for removing a part of your brain where we think that the seizures are coming from. And the other is that we think that if your seizures are coming from more than one part of the brain, or your seizures are coming from a part of the brain where we think has more function, important function, then we would do something of what we call a neuromodulation, where we would put an electrode in your brain, so that we can sort of buzz the brain, so that we don't actually cure your seizures, but it can calm your seizures down. You don't completely stop the fire, but sort of don't let it spread, from taking over in the brain.
Host Amber Smith: Now, this being a national study, the Upstate Neurological Institute is one of several sites in the United States participating. Are you still recruiting patients here in Syracuse?
Harish Babu, MD, PhD: That is true. We are still recruiting patients for this study as we speak. We did the first patient, but we are indeed looking, recruiting more patients for this study.
Host Amber Smith: Well, who would qualify for the trial?
Harish Babu, MD, PhD: Generally, the inclusion criteria as we call for a trial is anybody who's in the age of 18 to 65 years of age, male (or) female. You would have epilepsy as your diagnosis and an epilepsy that is not being controlled by medications alone. Another important criteria is, currently, that your seizures are known to come from only one part of the brain. Later on, we may include patients whose seizures are coming from more than one part of the brain, but this being really novel and new, currently we are only recruiting for patients whose seizures are coming only from one part of the brain.
Host Amber Smith: So for people who are accepted into the trial, what can they expect?
Harish Babu, MD, PhD: If you are part of this trial, we want to make sure that your medications are on track, you have the right levels of medications you have taken for months or years, and your seizures are indeed not getting controlled by medications. We make sure that your MRI scans don't show anything that can contribute to your seizures, meaning your MRI scan should not have any tumors or anything that will think may be a reason for your seizures.
And you would also undergo some psychological testing, make sure that you don't have any serious mental health disease. So you would go through a battery of tests to make sure that you are only having epilepsy, and none of the other issues are contributing to this epilepsy.
Host Amber Smith: This is Upstate's "HealthLink on Air," with your host, Amber Smith. I'm talking with Dr. Harish Babu. He's an assistant professor of neurosurgery at Upstate, and he's the surgeon who administered the first dose of a regenerative human cell therapy that's being studied here at Upstate.
Now, will all participants receive the regenerative human cell therapy? I think it's called Neurona, right?
Correct. The name of the company is Neurona (Neurona Therapeutics). All participants in this trial will receive the same cells, from this company. How are the cells introduced into the person's brain?
Harish Babu, MD, PhD: We use a special guidance-based cannula (a thin tube) that will deliver the cells, and we place the cannula in the area of the brain by using something we call a stereotaxis which is like a GPS navigation for surgery that we use to locate precisely where we are in the brain.
So, we'll be combining these sort of very sophisticated techniques and technologies to deliver these cells precisely in the area where we would want those cells to be delivered.
Host Amber Smith: How confident are you that this therapy is safe?
Harish Babu, MD, PhD: This is the first in human studies, so currently we don't have any data for humans, but any trial like this goes through a rigorous process of studies in animals.
And we study extensively these things in animals, making sure that they are safe, and they don't grow into any cells that we don't want them to grow into, and they're doing the things that we expect them to do. Having said that, it is still animal studies right now.
The human trials are initially meant to show if this is safe, and then, what is the efficacy? And we'll only know the details of how safe this is once we do those trials, as we are doing right now, but from all the preclinical studies, those are the studies that are done in animals, we think that this is a pretty safe trial to go ahead in humans.
Host Amber Smith: Are there any precautions you would offer to someone who's considering whether they'd like to participate?
Harish Babu, MD, PhD: Those will be the checks that we will be looking for. If we think that you are a candidate for this, you would be going through a battery of tests and checks to make sure that you are safe for getting this treatment.
Those are some of the inclusion and exclusion criteria that we use for trials.
Host Amber Smith: So how long does a patient who's in this trial stay in the trial? And how often do they come for appointments, to be assessed?
Harish Babu, MD, PhD: We will be checking them routinely for two years. Having said that, if your seizures are not getting controlled after you have been given this therapy, if you think you do not want to continue, you can always drop out of it. And you'll always be given the standard of care, which is what you would have received if you did not participate in this surgery. But in this trial currently, we are evaluating patients for two years from the time they had surgery.
Host Amber Smith: What's the best way for someone to learn more. Do you have a phone number or a website to share?
Harish Babu, MD, PhD: If anybody who is interested in participating or knowing more, our Upstate Neurological Institute website -- https://www.upstate.edu/neuroinstitute/about/locations.php -- is a point of information. You could also contact my office, 315-492-3172, or Dr. (Robert) Beach, who is also the principal investigator in this study, a neurologist here, his office (315-464-4243), to know more about this.
Host Amber Smith: Now, I know you had your first patient in the study at the end of June. Is it too soon to know how effective the therapy is for the subjects in the trial?
Harish Babu, MD, PhD: We had our first patient in June, and it's been about two months since that surgery. We are expecting that, once we transplant these cells, it'll take about four to five months before the cells integrate into the existing cells within the brain. And that's when the cells will be expected to act in a way that we would think it would act. So we expect that'll take about. That time, four to five months' time, before we know, to have any results of efficacy or of therapeutic benefit for this.
Host Amber Smith: These cells that come from the company called Neurona, are they created from stem cells, or where do they come from?
Harish Babu, MD, PhD: These cells are derived from stem cells. There are lines of stem cells that are used for these trials all around the world. These particular cells are derived from the stem cells and have been made or programmed to generate interneurons or these inhibitory neurons. And the cells that we transplant are already so-called differentiated or restricted to generating only these interneurons in the brain. But the starting point of this cell is a stem cell .
Host Amber Smith: Is it hard to be patient, you, as the physician, and the patient who's trying this, if you have to wait four or five months to see whether it's working? Isn't that difficult to do?
Harish Babu, MD, PhD: It is indeed. We live in a world of instant gratification, and medicine is no different. We would like to see the results of this yesterday, but we just have to be patient to know what works and what doesn't work.
So we are waiting, with our patients, to see how this pans out.
Host Amber Smith: Upstate's Neurological institute offers the region's only level 4 epilepsy program What does level 4 mean?
Harish Babu, MD, PhD: Levels of epilepsy care were developed by the National Association of Epilepsy Centers, and Level 4 epilepsy care such as an epilepsy center at Upstate Medical University provides the highest level of care to people with epilepsy and other seizure disorders Now in level 4 we would perform the most complex forms of intensive neurodiagnostic monitoring. Level 4 centers also offer a complete evaluation for epilepsy surgery, and that'll include intracranial electrodes placement for knowing your seizures better, locating which part of the brain they're coming from. At Level 4 centers, there's a broad range of surgical procedures for epilepsy that are provided for patients. It also provides for extensive medical, psychological and psychosocial treatments and support for our patients with epilepsy. The guidelines are that if your seizures have not been under control with medications after 12 months, you should request a referral to a specialized epilepsy center, such as a Level 4 epilepsy center at Upstate medical University.
Host Amber Smith: Do you foresee offering other trials for epilepsy treatment in the future?
Harish Babu, MD, PhD: We're always looking for ways to improve care for epilepsy patients.
If there are trials that are novel that we think will benefit, or they are improvements in the current treatment modalities, we would definitely be participating in those trials, so we can provide better care for epilepsy patients in Upstate New York.
Host Amber Smith: Well, Dr. Babu, thank you so much for making time to tell us about this potential new epilepsy treatment.
Harish Babu, MD, PhD: Thank you. It's my pleasure.
Host Amber Smith: My guest has been Dr. Harish Babu, an assistant professor of neurosurgery at Upstate. I'm Amber Smith for Upstate's "HealthLink on Air."
Next on Upstate's "HealthLink on Air": pulmonary function tests -- at home.
From Upstate Medical University in Syracuse, New York, I'm Amber Smith. This is "HealthLink on Air." 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 is director of the ALS center at Upstate.
Welcome to "HealthLink on Air," 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?
Eufrosina Young, MD: 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.
Host Amber Smith: 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 "HealthLink on Air" with your host, amber Smith .I'm 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. I'm Amber Smith for Upstate's "HealthLink on Air."
Here's some expert advice from Dr. Timothy Shope from Upstate Medical University. How much weight does someone lose after bariatric surgery?
Timothy Shope, MD: It's not immediate. But the majority of people will notice a substantial amount of weight coming off within that first month or so. When we talk about the weight loss for these operations it's generally a year to a year and a half is the timeframe that we talk about for maximum weight loss. But that is pretty well front-loaded, meaning that the first six or eight months is where are they going to experience the majority of that weight loss, and then the next eight months or so, a little bit here and there will come off. It really depends on the starting weight as far as how much weight they will lose in that first month. But I think on average, somewhere around 20 to 30 pounds in the first month is pretty typical. They don't wake up lighter from surgery. In fact, because of the fluid that we give during surgery and during the hospital stay, some patients will go home up a pound or two. That's not unusual, but realistically within those first couple of weeks they are really starting to drop that weight.
Host Amber Smith: You've been listening to chief of bariatric surgery Dr. Timothy Shope from Upstate Medical University.
And now, Deirdre Neilen, editor of Upstate's literary and visual arts journal, The Healing Muse, with this week's selection.
Deirdre Neilen, PhD: Two of our poets this issue offered us contrasting ways to depict anxiety. Mark Danowsky is a writer from Philadelphia and the author of a chapbook entitled "As Fall Trees." His Muse poem is called " Racing Thoughts":
Like lotto balls
They race around
(Unless given attention)
Save the few voiced
Except when they ricochet
Off the mind's walls
Leaving little bruises
Daisy Bassen is a physician and poet who completed her medical training at Brown and her undergraduate degree at Princeton. Here is her poem, "Analysis":
She wonders how hard it will be
To get the couch to her third floor office
In the old psychiatric hospital.
It was built when the only treatment
Was beauty, long windows disciplined with mullions,
The river curving around the acres.
Oaks carefully placed to dapple light,
Spangle the sky that reaches all the way to earth.
She will sit behind the couch's mohair shrug
And make sure she can see the clouds,
Strata or veil, the atmosphere's underbelly,
And the branches, buttresses.
Up the narrow staircase, there are no questions.
Host Amber Smith: This has been Upstate's "HealthLink on Air," brought to you each week by Upstate Medical University in Syracuse, New York. Next week on "HealthLink on Air:" an update on polio. If you missed any of today's show or for more information on a variety of health, science and medical topics, visit our website at healthlinkonair.org. Upstate's "HealthLink on Air" is produced by Jim Howe, with sound engineering by Stephen Shaw. This is your host, Amber Smith, thanking you for listening.