Three months ago we did a podcast with Randy Curits about his recent diagnosis of ALS in March and what it was like for someone who studies and cares for people living with serious illness, to now be someone who is living with serious illness. It was one of our favorite podcasts we’ve done, but also got us to think “wait, how come we’ve never done a podcast on ALS?”
We fix that on today’s podcast. We’ve invited Elizabeth Lindenberger and Kara Bischoff to talk about what every geriatrician and palliative care clinician should know about ALS.
Topics include: prognosis in ALS, disease modifying therapies (and when/if to discontinue, advance care planning in ALS, the role of embedding geriatrics/palliative care in ALS clinics, interventions in ALS (noninvasive ventilation, PEG tubes, and trachs), and symptom management as the disease progress.
So take a listen and if you want to take a deeper dive, take a look at these articles:
Eric: Welcome to the GeriPal Podcast. This Eric Widera.
Alex: This is Alex Smith.
Eric: And Alex, who do we have with us today?
Alex: Today we’re delighted to welcome Elizabeth Lindenberger who is associate professor at Mount Sinai in the department of geriatrics and palliative medicine, and is also program director for the hospice and palliative medicine fellowship and a physician at the Bronx VA. Welcome to the GeriPal podcast Elizabeth.
Elizabeth: Thank you, so happy to be here.
Alex: And we’re delighted to welcome back Kara Bischoff, who is associate professor at UCSF in the division of palliative medicine and medical director of the outpatient palliative care service for non-oncology patients. Welcome back to GeriPal, Kara.
Kara: Thank you so much. Nice to meet with you all.
Eric: On today’s podcast, we’re going to be talking about ALS, all the things that geriatricians and healthcare providers should know about ALS is kind of stemmed I think from when Randy Curtis was on our podcast, talking about his experience with ALS and thinking about man, we need to do a podcast on this disease, but before we dive into the topic, we always ask for a song request, who has a song request for Alex?
Elizabeth: I do.
Eric: Elizabeth, what’s the song?
Elizabeth: My song request is Goodnight Irene by Lead Belly. Lead Belly was really a founder and a legend of blues and folk music. He died of ALS in 1949, actually at Bellevue Hospital in New York. And he as an African American man in the 1930s and forties, he faced extreme racial discrimination and oppression and lived in poverty. And yet his influence on in future musicians has been very great.
Alex: Wonderful choice. This song is near and dear to my heart. I worked at a summer camp in Northern Michigan in high school for a few summers. And I was the campfire guitarist. I’m still kind of the campfire guitar. I haven’t changed that much, but at the end of the night, as the kids were leaving, we would always play and sing this song and the counselors would come up and we’d all be singing it together as the kids filed out. I have a lot of wonderful memories of this song. Thank you for choosing it Elizabeth.
Alex: (singing) “Irene, goodnight, Irene goodnight. Goodnight Irene, goodnight Irene. I’ll see you and my dream. Last Saturday night, I got married and me and my wife settled down. Now me and my wife have parted, going to take another stroll downtown. Goodnight Irene. Goodnight Irene. I’ll see you in my dream.”
Eric: Good job, Alex.
Kara: That was beautiful.
Eric: Okay. ALS, let’s talk about it before we dive into the topic topic. I just want to know from both of you, how you got interested in this from a clinic perspective, from a research perspective, I’m going to start with Kara because I know there’s been a lot of focus on palliative care for cancer patients. A lot of outpatient palliative care grew up around cancer patients, but you kind of bucked that trend here at UCSF. I’d love to hear a little bit about your story.
Kara: Sure, absolutely. Until 2017 at UCSF we really didn’t have any significant outpatient palliative care for people with illnesses other than cancer. We had a very large and robust, long standing palliative care clinic in the cancer center, but I was working primarily on the inpatient side and frequently cared for people with other illnesses and was troubled as were many of my colleagues about the fact that we didn’t have outpatient services to connect them to. That was sort of a long recognized need. And we were very lucky to be able to get medical center funding through an innovative program called the value improvement project, but essentially to launch an outpatient palliative care clinic for people with other illnesses. And in terms of how we made our way to ALS it was actually interesting that we weren’t sure where to start, where to focus our clinic and where to locate it physically.
Kara: Space is a big issue at our medical center and of course there were many places we could have started. So we were talking with colleagues in primary care and cardiology. And then one of my colleagues, Dr. Brooke Calton actually ended up riding an elevator with a colleague in neurology who runs our ALS clinic, Dr. Kathy Lomen-Hoerth and Kathy said, “How about you co-locate with us? Our patients have a ton of needs and our team would love to work with you.” And so that’s what we did in short.
Alex: Fortuitous elevator ride.
Kara: Absolutely. So I can’t say my interest in this topic was particularly premeditated, but perhaps it’s a lesson in being open to going where there’s need and enthusiastic partners, which basically summarizes my career to date and to a large extent.
Alex: Sometimes I teach about elevator pitches and how to craft an elevator pitch. This is actually a real world example where the elevator pitch worked and led to a whole new clinic. It’s incredible. Thank you.
Kara: I’m grateful to both my colleagues for that absolutely.
Eric: Problem with Zoom now, you don’t really have those elevator experiences anymore. It’s now random Zoom interactions. It’s all pre-planned. Elizabeth, how did you get interested in this?
Elizabeth: I actually love to tell this story, particularly to my mentees and fellows, because it’s really a story of how careers are generally very nonlinear often can be nonlinear and unpredictable. My story started about two decades ago. I am not a neurologist. I did internal medicine residency, and then I did a geriatrics fellowship at a place called UCSF and that’s your stomping ground. And when I was finished with all my training, I had seen a sum total of two patients with ALS in my entire medical training. I can actually remember the two. And my first job at a fellowship was in Washington, DC. I was the medical director of a home based primary care program at the Washington DC VA. And when I arrived on the job, I found myself with a panel of 120 patients and seven of them had ALS and I thought I had absolutely no idea how to take care of these patients.
Elizabeth: And it was really a fork in the road for me because I thought, gosh, these patients are all so complex and have so much need and I don’t know what I’m doing so I can either fly by this heat of my pants or I can really figure this thing out. And so I chose to do that. We were fortunate. I was fortunate that there was a very wonderful multidisciplinary ALS clinic nearby. I spent some time there. I asked lots of people, lots of questions. I got myself trained up in ALS care and I got my team trained up in ALS care.
Elizabeth: And I was also really fortunate that we had a few really amazing champions from other disciplines at our medical center. In particular, there was an amazing speech therapist and also respiratory therapists who were really interested in ALS care and we didn’t have a program. We decided to create a task force and create a clinic. We worked with a neurologist, we got a clinic going and we had, just because we started out of home-based primary care, we had embedded home based care and we had embedded palliative care. And that’s where it all started.
Eric: Wow. And tell us a little bit where you are now.
Elizabeth: I joined the faculty at Mount Sinai in 2009. And my first position with Mount Sinai was 100% clinically based at the VA. I’ve actually been a VA doctor for 20 years now. And so I was at the James J Peter Bronx VA, and I still am very part-time there. And when I joined the [inaudible 00:08:53] at the VA, I decided to start an ALS clinic there as well. And that was a pretty exciting time nationally in the VA for ALS care, because it had become a service connected disease. There were tremendous supports for veterans with ALS because it was increasing understood that the incidence was significantly higher among veterans, but many VAs didn’t have clinical care programs for these patients to receive their care within. I started an ALS clinic there. It literally started with me in a speech therapist and then we recruited a neurologist and then fortunately spinal cord injury took it over and it’s the vision center for both spinal cord injury and ALS care.
Alex: And on that note about higher incidents among veterans, isn’t it specifically veterans of the Gulf Wars?
Elizabeth: Such a good question. Interestingly, when I first started this work, that’s what the early studies showed was that there was a higher incidence among Gulf War veterans. But then and actually a couple years after those studies came out, there was a really large study that didn’t include Gulf War veterans that showed that there was an increased incidents among all veterans. And there were no Gulf War veterans in that cohort, in fact and then the knowledge of risk is about 1.5.
Alex: Oh wow. Any ideas why? Probably speculation…
Elizabeth: Yeah. It’s all speculation. I mean, we really don’t understand what causes ALS.
Eric: Maybe we can take a step back for our listeners. What do we know about ALS? What is it?
Elizabeth: ALS is a neurodegenerative disease that is characterized by degeneration of both the upper and lower motor neurons. And you have to have evidence of signs and symptoms of both upper and lower motor neuron dysfunction to my criteria for ALS, if it’s only upper motor neuron we call it PLS or primary lateral sclerosis. And it causes weakness in really all of the muscles of the body due to that degeneration and ultimately it is generally a fatal disease.
Alex: And is it only a motor disease, motor symptoms?
Elizabeth: There are a lot of non-motor symptoms as well. About two thirds of cases are spinal and about one third are bulbar and when it’s a bulbar onset ALS it means that speech and swallow are very highly affected.
Alex: Yeah. And bulbar is a type that Randy Curtis has and talked about on our podcast and has talked about publicly. And my grandmother had bulbar ALS, I’ll just mention briefly. And I remember she was in her seventies at the time. This is my mom’s mom, my Hawaiian grandmother. And I remember her losing her speech first and then her swallowing and then eventually getting a feeding tube.
Kara: It’s a really challenging illness, particularly bulbar onset. And I experience, and I also might mention that another challenging aspect of this illness is that up to 50% of people have mild cognitive impairment with up to 15% actually meeting criteria for frontotemporal dementia in this illness. This is a very, very challenging situation when the two syndromes occur together. And so I think the cognitive impacts and symptoms in ALS are under recognized, but important to screen for.
Eric: Yeah, we did a podcast with Benzi Kluger talking about Parkinson’s disease. I think it was always a good reminder that it’s not just the motor disease, a lot of non-motor symptoms, same thing here with ALS, including the cognitive issues. What’s the role of a geriatrician or a palliative care clinician in the care of ALS patients. Elizabeth, I’m going to turn to you first.
Elizabeth: I would say the palliative care needs of patients with ALS are really vast. On the one hand you have the tremendous symptom management needs, some of the many symptoms that we see with ALS are shortness of breath, fatigue, weight loss difficulties with speech and swallow, drooling, complex neuropsychiatric symptoms, pseudobulbar affect. And many of these symptoms are actually highly treatable and manageable with good care.
Elizabeth: And then secondly, you have progressive disability. So people often lose their ability to walk, to feed themselves, to do the other ADLs that are so important in life, to speak. Dysarthria is a big part of this disease for many. And certainly palliative care and particularly geriatricians are very adept at dealing with disability and helping people to have the best function that they can given the disability that they have. And then lastly, really is the need for skilled communication, because as I’m sure we’ll talk about, there are many, many important treatment decisions that need to be made in this complicated disease and having a space to safely talk about those issues is really important so communication is really key.
Eric: Kara, you actually published a paper recently on your team’s experience of an embedded palliative care ALS clinic. You want to talk a little bit about that paper?
Kara: Yeah, absolutely. I’d love to. Our study aim essentially was to describe our collaborative care model that we developed to provide longitudinal, interdisciplinary, palliative care to patients and families facing ALS, examine its impact and share lessons learned that might be helpful to other groups developing such services. We report on 55 patients with ALS who received longitudinal interdisciplinary palliative care in close collaboration with our ALS care. We had in October 2017, basically embedded an inner a disciplinary palliative care team of doctors, a nurse, a palliative care social worker, and a palliative care spiritual care provider within the ALS clinic, as I mentioned, and we were seeing a lot of patients that were referred by the ALS team, and we were working closely with the ALS team in managing these patients. We were really glad to examine what happened with this care.
Kara: In short, the average age of patients we saw was 66 half were women, three quarters were white, three quarters were English speaking. Patients were referred to the palliative care team from the ALS team by the ALS team based on the ALS provider’s discretion, a median of six months after they established care with the ALS team. So these patients had sort of moderate ALS, and they were referred for symptom management, advanced care planning and/or support for the patient or family in roughly equal frequency. All of those broad categories were very frequent reasons for referral. And many patients were referred to palliative care for more than one of those reasons.
Eric: Can I just ask. The structure of this, so you had a palliative care team, chaplain, physician, nurse, social worker, how integrated were they in the ALS team or they were just getting consults from the ALS team?
Kara: Yeah. Great question. In building our collaboration, we were co-located with them in the same clinic space and we are seeing patients on the same days, two days a week. To build the collaboration, we started by shadowing each other. So we shadowed the ALS team to really understand their practice, as well as to learn about the illness, to be honest. And then they shadowed our team to understand more about palliative care and what we do. And because we were sharing space, we frequently ran into each other in the hallways honestly, and had lots of opportunities to ask questions of each other, to really make sure that we understood each other’s perspectives and practice. And we were delivering care in a unified collaborative way. We initially were seeing patients on the same day, so a patient would come and they would see members of the interdisciplinary ALS team and also see members of our team. But we actually found that this was quite exhausting for patients. It was a lot of visits for one day.
Kara: With the advent of telemedicine and moving increasingly in that direction, we’ve actually evolved to a practice pattern of seeing patients on a different main palliative care, see patients on a different day than when they see the ALS team, but still because of the relationships that we built through shadowing each other, through working shoulder to shoulder over time and our systems of frequent communication, we’ve been able to still maintain very collaborative care.
Eric: What does your clinic look like Elizabeth? Is it the same?
Elizabeth: Our clinic is a little bit different. We are definitely fully embedded, because it’s actually me, who is the palliative care clinician for that clinic. The disciplines are slightly different from the usual interdisciplinary non-VA ALS clinic. In that we have the luxury of having a psychologist on the team. We also have primary care embedded within the team and we have a nurse care coordinator and he just kind of makes everything work … So there’s a lot of really intensive case management for patients and there’s also some embedded home care.
Eric: Is pulmonary involved?
Elizabeth: In my clinic, it is not pulmonary. In the clinic that we had at the VN Washington, we had a respiratory therapist clinic. It varies place to place. I don’t know about you Kara.
Kara: Yeah. The ALS team does have a respiratory therapist as well as a physical therapist, occupational therapist, registered dietician, a speech language pathologist and a part-time social worker. So they’ve got a very robust team to start with, but still we found there were additional needs that our team is able to help with.
Eric: Did anything surprise you about doing this Kara.
Kara: Yeah, I think from the first day that I shadowed, it was remarkable to me that, oh, we do do things differently. Oh, we do have things to add. I assumed that there might not be that much left for us to manage, but it turns out that with this incredibly intense illness experience, these really substantial losses that just continue to come one after another and affect both patients and family caregivers so profoundly, there was a lot for everyone to do and contribute.
Alex: And when you’re saying the losses that come one after another, I wonder if you could just sort of paint a picture for our listeners of what the course of ALS is typically.
Kara: Sure. There’s not a single phenotype, but just to provide an example, someone might even before they received a diagnosis of ALS, they may have lost significant physical function in their limbs, for example, lost their ability to hike, perhaps that was a really important activity for them. And now they’re struggling to walk without tripping and falling. And that may occur even without knowing their diagnosis, which often takes many months to make. And then after a diagnosis, they may go on to lose more physical function so that they’re no longer able to walk at all. They’re needing a wheelchair they’re needing help with ADLs. They also may lose the ability to feed themselves and need help. They may then develop some bulbar symptoms and have difficulty with chewing and swallowing and weight loss or choking episodes, which can be quite scary in association with that. As they’re adjusting to all those changes, they may then lose some ability to speak, develop dysarthria, be very difficult to understand, and even become anarthric.
Kara: During this time there’s also really significant emotional symptoms as people have a change in their function, their roles, their relationships, how they find meaning in their life. All of that is sort of going on simultaneously.
Eric: I guess there’s a lot of support for family and caregivers too, going on.
Alex: And prognosis for ALS, thoughts about how you estimate that, what ranges are differences for sort of standard type and bulbar?
Kara: Yeah, absolutely. The overall rule of thumb is about three to five years from the time of diagnosis, but it certainly does depend on their type and their history with the illness. I think looking at their illness trajectory and how things have progressed thus far can give us a lot of information for an individual patient about what’s likely to come and what they can expect.
Eric: Yeah. Elizabeth, we got two FDA approved disease modifying treatments, really all being one, does that change significantly prognosis?
Elizabeth: So unfortunately not so much. The average increase in life expectancy with the use of [inaudible 00:23:25] is really on the order of an additional two to three months of life. And we don’t even really quite know at what point in the trajectory are we prolonging that survival time? Yeah. And it’s generally well tolerated though some patients have side effects. The other FDA approved drug is Radicava or Edaravone and that’s a pretty complicated drug to take with …
Eric: It’s an infusion, right?
Elizabeth: It’s an IV infusion. That involves you need a pick line, you’re pretty medicalized and it’s essentially a strong antioxidant. And there’s a little bit evidence of a little bit of benefit for some patients if given early.
Eric: $150,000 a year for that drug. Again, it sounds like potentially beneficial, maybe marginally have modest benefits if that. Does everybody get put on it basically and if so, do you ever consider stopping them?
Elizabeth: I think this is considered the evidence based standard of care to offer that medication to all patients. I think many of us have a low threshold for stopping it, if there’s any kind of side effect and for stopping it late in the disease. I think the one other point I would make Eric about these medications is that it’s also important to know that we have other interventions that are actually more efficacious and the medications that are not drugs. Non-invasive ventilation, for example, BiPap actually increases life expectancy by up to a year to 18 months. And that goes along with improved quality of life for patients who have any respiratory compromise. That’s an intervention, it’s not a drug, but it increases survival time and makes people feel better. They feel more alert. They don’t have their daytime morning headaches anymore. They have less dyspnea. All in context.
Alex: Back to the BiPap for a second, you’re talking about BiPap, is it 100% time BiPap or are we talking about intermittent BiPap.
Kara: Intermittent as well.
Kara: Usually where people start and they may use it at night initially, and then start using it for naps during the day or rest periods during the day and find that it can boost energy considerably and the quality of sleep and so it’s not only used for shortness of breath. Great. Thanks.
Eric: What about PEG tubes? Do they fall into that BiPap?
Kara: Yeah. There’s not randomized controlled evidence about PEG tubes, but there’s observational data that shows that PEG tubes can slow the progression of weakness in ALS when there is weight loss associated with it. PEG tubes in ALS are an extremely different thing than PEG tubes and advanced dementia. And I think that’s a real important clinical pearl for clinicians to remember. We frequently see people with ALS for whom feeding or eating becomes increasingly difficult burdensome and even scary if they’re choking frequently. And it can be burdensome in taxing on family caregivers as well when feeding is really an uphill or eating, I should say is an uphill battle. We often talk to people about the option of having a PEG placed and feeding tube feeds delivered via PEG, which can improve the burdens of feeding as well as slow the progression of the weakness. Even in people who don’t necessarily want to extend their life with this illness, I often recommend tube feeds in order to improve their quality of life.
Eric: And my understanding is it probably is best used from a nutritional supplement standpoint. The evidence for PEG tubes and aspiration, I would say are pretty darn weak, but from a nutritional supplement standpoint. Potentially people can still enjoy food by mouth, but they’re just getting supplements. Is that right Elizabeth? Is that how you think about it?
Elizabeth: Yeah. That’s exactly right and I think that’s so important to remember. The other thing I will say is, speech and language therapists too can really help patients with swallowing techniques is also a hugely important part of the care of these patients. That’s why interdisciplinary care is the standard of care of best practice care for ALS. We need all of these disciplines and the SLP on the team can help with textures of food and thickness of liquids and techniques for swallowing that can help patients to have the safest kind of eating experience and to be able to eat it by mouth as long as possible.
Elizabeth: I would say the majority of our patients who get feeding tubes are still eating by mouth. I mean, certainly patients with severe bulbar disease and advanced stages often can no longer eat anything by mouth, but most of our patients do still eat, but it takes the pressure off of those meals as Kara pointed out that can be really onerous to you. And it’s not even just the dysphasia sometimes people have weak arms. And so just bringing the fork to their mouth is exhausting. And so it’s a way to really help that quality of life.
Eric: Noninvasive ventilation, PEG tubes we talked about, when does a trach fall into the picture here?
Kara: Sure. We typically consider a tracheostomy, an endotracheal ventilation when people’s forced vital capacity that’s below 30 per cent. The ALS team regularly monitors patient’s respiratory strength with pulmonary function tests. And I think it’s really important to talk about this option in advance of a crisis. This is something that is you’d likely to come. We sort of have that knowledge and that expectation. And if we think about it in advance and talk to people about it in advance, we can talk with patients and family caregivers in a calm, controlled environment when we’re able to talk to them about the realities of living with a tracheostomy and endotracheal ventilation at home, what that is like from the patient’s perspective and perhaps connect them with other patients who are in that situation. And then also talk about what that’s like for family caregivers and sort of the what’s required of the family system to make that work well.
Kara: I’ve had a couple patients, unfortunately, who have found themselves in a crisis where they developed respiratory failure before we were able to have these conversations and got and intubated in an emergency setting. And I found, unfortunately, it’s a lot more difficult for patients and families to make the decision about either extubating in that situation if they’re not able to recover the respiratory strengths efficient to have that happen without the patient dying, than it is to sort of make that decision in advance. I’ve also found that when people are suddenly thrust into that role of having a tracheostomy and a ventilator at home without having thought about it in advance, it can be really hard on patients and families.
Eric: Because you’re looking at that point 24 hour caregiver support like somebody with the knowledge about what it is, how to brainstorm when things go awry.
Kara: That’s exactly right. Someone has to be there ready to respond to an alarm 24/7.
Alex: Is there evidence for outcomes with tracheostomy and ventilator support compared to alternatives?
Kara: Right. People can live a lot longer with tracheostomy and ventilator or at home. Elizabeth, last time I looked into this, I didn’t find any statistics about how long people with ALS on average live with endotracheal ventilation. Are you aware of that data?
Elizabeth: I believe, it’s very variable and it’s an average of something on the order of three years, but thought to be less when patients are over 60.
Kara: Certainly I’ve seen people live for years and in some case many years, for 10 years, which is quite incredible. It can prolong life certainly but I think people just have to really understand what they’re signing up for and make sure that this is a condition that they want to prolong.
Eric: Yeah. I think the majority die within one or two years after a trach placement. I guess another question, I want to go back to disease modifying treatments, let’s say, is it real use all. My understanding it’s, the best evidence is it kind of prolongs the time that you need a trach or potentially a PEG. If they’re already have a trach and a PEG, do they still need to be on these treatments? Do they do anything, once somebody is in that stage of ALS?
Kara: It does slow the progression of the weakness felt in other areas, their body too. It would depend, I suppose, on their level of function that they had at that time, but it could make sense to continue.
Elizabeth: I think it’s also important to keep in mind that the conversation isn’t over just with whether they want a trach or not. First of all, it’s important to note that in the US it’s about five to 10% of patients with ALS who make that choice. The majority of patients do not choose tracheostomy and invasive mechanical ventilation. It’s a small percentage of patients. And some of those are emergent kind of intubation situations in which was obviously essentially often a medical error really of advanced care planning, I would say, not always, but often. I would also say even as Kara pointed out, it doesn’t stop the progression of disease. And so one of the things that’s really important is even if a patient makes the choice to put tracheostomy and invasive ventilation, you want to talk to them about is there any quality of life, any condition of progression in which quality of life would no longer feel that the right level for them.
Elizabeth: And so some patients will tell us, and I’ve encountered this several times. I want the ventilator, but if I am no longer able to use my communication device and my eye gaze because I locked in, then turn it off. I think it’s important to remember, it’s nuance, there’s a lot of pieces to this.
Alex: I wonder if you could walk through our listeners of what the alternative is to ventilation, how you present that and paint a picture of what that’s like, and who’s involved, is this when hospice gets involved for example, and using medications to treat dyspnea.
Kara: Sure, absolutely. Many people are on non-invasive ventilation, BiPap either at night, and then during an increasing number of hours of the day. And some people actually can live on 24/7 BiPap for something very close to that for even years. Others do not tolerate it or live that long. Using BIpap is certainly one tool that a vast majority of our patients use. We also use opioids, low doses of an opioid such as sublingual morphine, or roxanol starting with, for example, five to 10 milligrams, sublingual every two hours as needed for dyspnea, depending on the patient’s age and organ function, of course. And that can be very helpful for people. We find that even low doses of opioids can take the edge of dyspnea, help people feel much more comfortable and we rarely have to escalate the doses very high. At the same time, people are usually coping with the change in their function and in order to accommodate in depth to their Dyspnea.
Eric: I want to talk about, is advanced care planning, because we’re talking about how to present this with folks. When we think about ALS is advanced care planning, how different does it look? And I think about this too, because Randy Curtis just wrote a great Channel piece called Three Stories about the values of advanced care or value of advanced care planning. And one of those stories is his own story, having ALS and the value that he sees in it. Elizabeth, when you think about advanced care planning, how different is it in this population?
Elizabeth: I think the things that make it different are one that the decision making that’s involved is a little bit unique in that you have a better idea of what’s coming than in many diseases. You actually know for most patients, what particular decisions are going to need to be addressed. For example, if use of PEG feeding, not every patient that need it to be addressed because some patients will die before they ever lose their ability to swallow. But most patients develop the dysphasia and that’s a topic that needs to be discussed and then respiratory support because you know the majority of patients will die respiratory failure. So that’s unique. And I will also say a lot of the decisions that need to be made, that shared decision making that is court advanced care planning are really scary things to talk about for patients because it’s a really scary disease.
Elizabeth: I mean, you just get the diagnosis and you Google ALS, it’s terrifying because it’s a really scary disease. And so I think the other piece that is incredibly important is for us to be really aware of the importance of those psychotherapeutic elements of advanced care planning that are the core of what we do as well, which is really holding a safe space for patients to even be able to tolerate having conversations about the future when they’re all already dealing with difficult coping of loss of disability, of symptoms, of scary future, that piece is so, so very important. And I will say, Randy Curtis is such a role model to so many of us in this field and I just found his piece to so very beautiful and just another gift that he gives us. And I don’t remember exactly how it was worded, but he said being able to talk to whether it’s doctors or friends or colleagues about the future and about these issues and the decisions actually promoted a lot of positive stuff for him, joy gratitude.
Elizabeth: And I think that that reminds us really all of the value of the unmeasurables in advanced care planning about the importance of holding a safe space for holding hope and navigating uncertainty and also supporting the decision making.
Eric: Right. We’re going to do a quick lightning round of pearls. Feel free anybody jump in with a pearl. I’m going to start us off, because have questions about pearls. Cramps are really common in ALS any good treatments for cramps in ALS?
Elizabeth: not great.
Kara: To be honest, not great. I think that’s why we’re both quiet, but honestly it’s rare I think in my experience to have cramps be as significant issue.
Eric: Spasticity though, right?
Kara: Spasticity certainly. And for cramps, sometimes we use baclofen, it’s not my favorite medicine, but it is what we use in addition to heat and gentle massage [crosstalk 00:39:51] repositioning.
Elizabeth: Yeah. And certainly baclofen and tizanidine for spasticity and for people who have very, very extreme spasticity, and we have some patients like this, there are some patients who need a backlift and pump.
Eric: Yeah. Botox too if it’s located in a specific area?
Kara: Can be helpful where I suggest Botox a lot in these patients is for sialorrhea actually excessive saliva that’s difficult to manage. That’s a very frequent symptom that comes up. We typically use glycopyrrolate as our first line, something on the order of one milligram up to three times a day as needed can increase from there. There are other agents that can be added to that, including a scopolamine patch, for example, but one medications as well as suction and cough assist are not enough to manage the sialorrhea. We often refer patients for salivary gland Botox that can be quite helpful.
Eric: Great. What about for fatigue, any quick tips on fatigue?
Elizabeth: Yeah. I think that fatigue is one of the more, newly evidenced based unmet palliative care needs that’s been found. It’s a really, really common symptom. Certainly, I think Ritalin and Modafinil are the go-tos.
Kara: There’s a syndrome in ALS was mentioned briefly by Elizabeth pseudo bulbar effect, which is not actually a mood disorder, but a change in effect that a characteristic in ALS and you’ll recognize it after you’ve seen it once or twice. It’s essentially leads to uncontrolled and perhaps inappropriate laughing or crying. I see the crying more often the laughing to be honest and sometimes with sort of a whaling face and this symptom and issue can be very effectively treated with a medication called new Dex. That is a sort of unique-
Eric: Dextromethorphan and quinidine, tussin and tonic. They have been trying to push that one on dementia patients a lot. And I think the best evidence we have is for pseudobulbar affect, not really in dementia. Any other common symptom that you see?
Kara: Well, I think a really common symptom is an adjustment reaction with depressed mood or anxious mood. And we see this both patients and family caregivers. And of course it makes a ton of sense. This is a devastating and unrelenting illness as I’ve mentioned, but I think that’s a huge part of what our team and our interdisciplinary team, particularly with the help of our spiritual care provider and social worker address, helping people think about how their life can still have meaning, richness, adequate quality, how they can sort of adjust as their relationships and roles change. It’s really big. One of the things
Eric: One of the things that we see sometimes in ALS because people are diagnosed sometimes in their forties is they have young kids. Do you work with child life specialists or how to think about how they’re doing with this diagnosis?
Kara: Absolutely. ALS is such a visible illness. It’s something that no member of the family can forget ever because it’s there in your face every day. And so it certainly does have an effect on children and also because we know where it’s going, as Elizabeth mentioned. Our social worker and spiritual care provider frequently work with children and patients around their concerns about their children.
Eric: Elizabeth, any last pearls from your end.
Elizabeth: One more I realized we didn’t touch on that I think is really important, really is with the feeding tube issue, timing is very important in terms of monitoring respiratory function, because we know that putting a feeding tube in is safest when people still have good respiratory function. It’s not that you can’t put it in later, but it gets a little bit riskier with the conscious sedation of a PEG. And so that’s again where monitoring respiratory function and all also really having those early conversations and ongoing in a continuous manner are really important.
Eric: And Kara, can I go to dyspnea like in hospice, in the end of life. One of the issues is that the respiratory failure is from hyperventilation. You get hypercapnia, it’s not necessarily oxygen levels are dropping. Should we avoid oxygen in those patients? How do we think about opioids for dyspnea in this population? Is dyspnea common or what does the end of life look like?
Kara: Right. You’re right, the issue is with ventilation, not oxygenation. So we’re generally not recommending oxygen and frequently correcting inpatient teams if they end up on a lot of oxygen in an effort to help their dyspnea. But at the end of life, this can go multiple ways of course. Some people do stop breathing, even with the support of BiPap they may stop breathing fairly suddenly and can die in that way. I mean, not suddenly because they’ve had a progressive illness, but that is one common way that people with ALS can die of hypercarbia, leading to a cycle where they breathe less. Another way that some people die is by making a decision that they no longer want to continue their life prolonging treatments, including noninvasive ventilation. And they may opt to use medicines for comfort, like opioids, perhaps benzodiazepines with removal of the BiPap when they’re getting to a point where they’re needing it 24/7, and they’re not finding their life to have acceptable quality.
Kara: And that ties in with the fact that in our cohort of patients, we also had very frequent requests for medical aid and dying a full 30% of patients that we cared for did ask us about medical aid and dying, which is legal in California since 2016 and 28% of patients who died in our cohort died after ingesting medicines, medical aid and dying meds. Which really surprised us was a higher number than we had anticipated when we kind of really examine the numbers. But it is actually consistent with previous reports out of Oregon and Washington that have showed that in this patient population, they consider medical aid and dying very, very frequently and are really concerned about the loss of control and loss of sort of autonomy that this illness.
Eric: I know we’re out of time. I got one quick question. In advanced ALS for you to qualify for medically and dying, you have to be able to give yourself the drugs.
Kara: That’s right.
Eric: You could do it through a PEG tube, but how do you with severe motor dysfunction?
Kara: Yep. In this illness, there’s a window period where people who are very interested in medical aid and dying have reached a point where they want to pursue this and have a prognosis of less than six months, but are still able to self ingest via control route, meaning the other PEG tube or by mouth, if they’re able to swallow which is less common. There’s actually a lawsuit that’s been filed in the State of California around a particular patient with ALS case trying to challenge this requirement that patients self ingest internal route saying that it’s discriminating against people with certain types of-
Eric: Fascinating. Because that’s usually where we draw the line between physician aid and dying and euthanasia. I could go on and ask both of you questions for another hour, but running out time. I want to thank you. But before we end this, Alex, I think you got a little bit more of that song.
Alex: Irene goodnight, Irene goodnight, goodnight Irene, goodnight Irene, I’ll see you in my dreams. Sometimes I live in the country. Sometimes I live in the town. Sometimes I have a great notion to jump in that river and drown. Irene goodnight, Irene goodnight, goodnight Irene, goodnight Irene. I’ll see in my dream.
Eric: Elizabeth, Kara big thank you for joining us on this podcast. Really, that was tremendous. I learned a ton and amazing work with what you’re doing really moving geriatrics palliative care forward for these patients. To all of our listeners, thank you for your support and Archstone Foundation thank you for continued support. For anybody else who wants to continued support the GeriPal podcast we appreciate. We just launched a new donation website to help support some of the work that we do. Mainly some of the transcription costs the audio video production. You can learn more. We got a video on that site too if you’re interested in helping support the GeriPal podcast. Good night, everybody.