Living with ALS – Transcribed
Disclaimer: This transcript was generated by artificial intelligence and may contain errors, omissions, or inaccuracies. While we strive for accuracy, we cannot guarantee that the transcript is completely error-free. We encourage listeners to use the transcript to supplement the audio and exercise their judgment when interpreting its contents. Any inaccuracies or errors should be reported to us for correction. The hosts and guests of this podcast are not responsible for any loss, damage, or injury that may occur as a result of the use of or reliance on any information contained in this transcript.
[00:00:00] Pete Waggoner: This is Neurology Now, the podcast that explores the fascinating and complex world of neurology. Join us as we explore the human brain and beyond with expert guests who share their knowledge and insights.
[00:00:14] Pete Waggoner: Welcome to the Neurology Now Podcast.
[00:00:18] Pete Waggoner: In today’s show, we welcome in, Dr. Praful Kelkar of the Minneapolis Clinic of Neurology.
[00:00:25] Pete Waggoner: As we know, June is ALS Awareness month, and Dr. Kelkar and I will take a deeper dive into ALS and all the things that are engaged and involve with it. And I’m looking very forward to this topic, mostly.
[00:00:38] Pete Waggoner: Doctor, welcome aboard, because it’s one you hear of, but I think a lot of us don’t know of. Would you say that’s a general feel of the public as as to what ALS really is?
[00:00:50] Dr. Kelkar: Yes. I mean I think most people are aware cause it’s also known as blueberry’s disease and a lot of people know about blueberry and the [00:01:00] history and so they’re kind of aware of how serious the disease is.
[00:01:04] Pete Waggoner: What does ALS stand for?
[00:01:07] Dr. Kelkar: It stands for Amyotrophic Lateral Sclerosis, kind of a multiple name there, but that was coined by Dr. Charco back in 1900 something or 1800 something and more than a hundred years ago actually. And it just describes what they found in the spinal cord. In terms of the sclerosis, which means degeneration of certain part of the spinal cord, then led to the name of amyotrophic lateral sclerosis.
[00:01:38] Pete Waggoner: So the way it impacts the spinal cord, it can have multiple, I suppose there’s symptoms that progress, right? I mean, cuz you see how a patient, I mean does it mirror something of multiple sclerosis? Is it kind of the same degenerative type of thing? Or what are the types of symptoms that you can see engaged with this before it really becomes a serious problem?
[00:01:59] Dr. Kelkar: [00:02:00] It’s very different from multiple sclerosis. And in this condition, it’s not limited to the spinal cord, obviously. It goes into the brain as well. And it causes weakness and loss of muscles predominantly or primarily. And that progresses gradually over time in a steady fashion. It does not affect the sensations. It does not affect thinking and feelings in most of the patients. Some of it does.
[00:02:29] Dr. Kelkar: It doesn’t affect the control of the bladder or anything like that, but it leads to progressive loss of muscles and progressive weakness. And basically it can start in one part of the body and then spreads to involve the entire body basically.
[00:02:46] Pete Waggoner: And then when you are going about your active everyday life, suddenly symptoms start to appear, what are some of the warning signs of that? That a person would say, this isn’t normal, this isn’t right. And when they come in, you [00:03:00] start kind of narrowing your focus to that.
[00:03:02] Dr. Kelkar: Very often people start with muscle twitching.
[00:03:06] Dr. Kelkar: We all experience some muscle twitches here and there as you might have experience as well. But in this condition, they just keep happening and that spreads from one area of the body to another. And this is then followed by muscle weakness and atrophy, or loss of muscle bulk. And then that can lead to difficulty walking or difficulty using the hands or difficulty swallowing depending on where the symptoms are beginning.
[00:03:37] Dr. Kelkar: And so in the beginning stages of it, it can be extremely insidious. And it may just be some muscle cramps or some muscle twitching, which you might say, oh, that’s just normal kind of cramps. But then they just keep going relentlessly over time.
[00:03:56] Pete Waggoner: Is there a correlation between [00:04:00] hereditary or any diet related things, or have you learned along the way if there’s some consistencies that you’re seeing within how this appears in people?
[00:04:10] Dr. Kelkar: In less than 10% of the cases, it is familial. And that has been studied quite well over time. And in about 20% of less than 10% of the cases, it is mapped to a particular gene called SOD1 and those patients, so 20% of the less than 10%, they have a mutation in SOD1 gene that causes the ALS. But in 90 plus percent of the patients, we don’t really know what causes it at all.
[00:04:43] Dr. Kelkar: People have looked at genetic factors, environmental factors, food, toxins, all kinds of things people have studied over time, but it just eludes us. And as a result of this complex pathophysiology, [00:05:00] ALS is seen all over the world. So it’s not restricted to any one population or any one area. And it is seen in all different ages, starting from very early, late teens, all the way into 90 and plus age range.
[00:05:16] Dr. Kelkar: So it can happen at any time to anybody and anywhere, and we just don’t know what the triggers are.
[00:05:23] Pete Waggoner: What is the most effective way for diagnosis of ALS?
[00:05:28] Dr. Kelkar: So what it needs is a good clinical exam and a history.
[00:05:33] Dr. Kelkar: So when you see the patient in the clinic, you go over their symptoms and how it’s progressing, and the examination will reveal certain signs that are telltale signs of ALS.
[00:05:45] Dr. Kelkar: But you might see muscle twitches all over the body or muscle atrophy and weakness. And in addition to that, there reflexes the deep tendon differences that we check in the office. They become very brisk or [00:06:00] hyperreflexia it’s called.
[00:06:01] Dr. Kelkar: And when you see a combination of those two things, and in addition to that, their mentation is normal and the sensations are normal. So it’s a purely motor syndrome. So when you see that combination, then you start suspecting that this is ALS.
[00:06:18] Dr. Kelkar: And then a is often always done, it’s called EMG or electromyography. And that allows us to study the nerves in different parts of the body. And see what’s happening to them. So by using the clinical exam and the EMG, then that takes you to the diagnosis.
[00:06:37] Pete Waggoner: One of the things that you mentioned on your profile on the website was that you enjoy the opportunity to work with a number of neurological disorders and things that are happening with patients, and among that was alleviating some things along the way too. Are there things that you can do after you’ve diagnosed it and you’re okay? This is the hand we’re dealt here.
[00:06:59] Pete Waggoner: [00:07:00] What are some of the things that you can do or help at least get through the process? Is there anything?
[00:07:06] Dr. Kelkar: Yes. I mean, you can break it down into two or three different ways of approaching the patient with ALS. The one, of course, the holy grail would be to cure it or stop the disease completely in its track. And we might come back to that research and such, but that hasn’t happened yet, unfortunately.
[00:07:26] Dr. Kelkar: The second then goal is to keep the patients comfortable and functional as long as you can. So that’s the second very important goal. In fact, one of the quotes from neurology well-known professor was that he told his residents that, I don’t expect you to cure ALS necessarily, but learn to take care of the patients. That’s what I want you to do.
[00:07:49] Dr. Kelkar: And in doing so, patients need help with physical therapy. They need help with assistive devices such as treatment of the foot drop or hand [00:08:00] weakness or difficulty with ambulation. And then eventually patients do experience trouble swallowing and eating. And then we discuss feeding due, for example.
[00:08:12] Dr. Kelkar: And then they often have trouble with breathing, and it starts at night and then progresses in today. So very often we will use something like a CPAP, it’s called BiPAP, to support their breathing. People will often experience anxiety and depression that you can treat. So while using all these different modalities and multidisciplinary approach, you can keep the patients comfortable and functional as long as you can.
[00:08:39] Dr. Kelkar: And that’s a very important part of taking care of the patients with ALS.
[00:08:43] Pete Waggoner: And then in terms of patients and where they’re best served, I’m sure there’s a progression over time, whether that’s an in-home scenario or you get into some form of assisted type of care. Can you talk a little bit about the progression of that and then what the [00:09:00] psychological components and the support groups that could go into the patient and family provider caregivers?
[00:09:07] Dr. Kelkar: Absolutely. So the progression of the ALS. The speed of progression is variable between one patient and the next. By and large, 50% of the patients will succumb to the disease within two years. So it’s a very aggressive disease and there is a long range of course. Some people progress very fast within six months, some can have a slower progression over 10 years. But by and large, two to three years, or two to five years is the range of the disease progression.
[00:09:38] Dr. Kelkar: And that partly depends on where it starts. For example, if it starts in your foot, then slowly spreading up the leg, then spreading into the arms and eventually into your swallowing and breathing mechanism takes a bit longer.
[00:09:54] Dr. Kelkar: Whereas if it starts in your throat, It’s called Bulbar ALS. It starts in the swallowing [00:10:00] area. Then the progression is faster cause the breathing and the swallowing centers are close to each other.
[00:10:06] Dr. Kelkar: So then the patients experience a lot more life threatening even sooner than. If it were to start in the leg, for example. So it can start in one part of the body and then slowly progress and eventually does affect the entire body.
[00:10:21] Dr. Kelkar: In terms of center for taking care of these patients on long-term ongoing basis, there are ALS clinics in our state and there is one at University of Minnesota, and that’s where I used to work. And there is one at Mayo Clinic and there used to be one at Hennepin Medical Center as well.
[00:10:40] Dr. Kelkar: And right now I’m not working at the ALS clinic, but when I see patients with ALS in our clinic, I do send them to the ALS clinic for care because they have well established multidisciplinary care in one center.
[00:10:58] Dr. Kelkar: So they see a neurologist, they [00:11:00] see a psychologist, they see a social worker in. They see physical therapist, occupational therapist, breathing, therapist, all in one place, in one visit. And then they track the patients every three months or four months. And in addition to all of this, they have research trials sometimes going on with new medications that one can participate in.
[00:11:23] Dr. Kelkar: They also have funds through ALS Association and Muscular Dystrophy Association, which can help with youth chairs and walkers and those kind of things to the patients. So it’s probably best for the patient to get enrolled in a ALS clinic that is directed that way.
[00:11:44] Pete Waggoner: Well, it sounds like it to me because if I learned one thing here today, it’s just the level of different things that need to occur for one individual. When you mentioned all of the different forms of therapy, from psychology to swallowing to [00:12:00] physical therapy. I mean, there’s a lot of different things.
[00:12:03] Pete Waggoner: It’s a lot coming at one person at one time, and I know you deal with this quite a bit with your patients, all the people you see. Is there one thing that you’ve drawn from people’s journeys, from your perspective that maybe you wouldn’t have thought when you went into this practice?
[00:12:21] Dr. Kelkar: Yes, there is something unique about patients with ALS and people have written research papers on this as well. They are absolutely phenomenal human beings.
[00:12:31] Dr. Kelkar: Somehow there’s a quote that ALS only happens to nice people. These people are just extremely loving and caring. And so when you take care of somebody like that with an attitude that’s extremely positive, even though they’re going through a terminal illness, they make you feel good inside that you’re taking care of them.
[00:12:52] Dr. Kelkar: It’s a very unusual interaction. These patients are going through all these life-changing events and at the same time, [00:13:00] they’re grateful, they’re thankful. They just give you a positive energy back as you take care of them. So it’s very, very, very interesting in that way.
[00:13:09] Dr. Kelkar: And in some ways the burden of the disease also takes a toll on the caretakers. Sometimes even bigger burden psychologically, because they see their spousal loved one go down slowly, step by step, and they’re trying to stay with them and take care of them. So when you treat these patients, you’re not just treating one person, they’re treating whole family really.
[00:13:33] Dr. Kelkar: And counseling them.
[00:13:34] Pete Waggoner: Yeah, it’s a very large component that goes beyond just one individual. It requires a lot from the medical perspective and also from the family perspective. Don’t know if you have the number, but roughly how many people per year get diagnosed with it?
[00:13:52] Dr. Kelkar: It is estimated that there are about 30,000 patients in the US with the disease at any given time. [00:14:00] More or less, what? 30,000? About four or 5,000 patients are diagnosed every year, new patients with ALS. So it’s not a super common disease, although we see a fair number of them, obviously.
[00:14:15] Pete Waggoner: So, question to that. How much do you see what the research has been doing and how it’s evolving. I’m sure you see new things and ideas. Do you stay pretty in tune to that? Do you get involved with that? What does that look like?
[00:14:30] Dr. Kelkar: Yes, I am in tune with it. Although personally I’m not involved in the research right now. When I worked at the ALS clinic at the university, over time there have been a little bit of current breakthrough or good news here and there with treatment.
[00:14:45] Dr. Kelkar: Several years ago, our medication was approved. It’s called Reutech . And it showed that you can actually slow down the disease. And when it came out, there was a lot of excitement that, yes, we can build on this and make it better and better and better. [00:15:00] But that has not unfortunately occurred. So right now, this medication is available for giving it to patients.
[00:15:07] Dr. Kelkar: It’s called Reutech. It does have a little bit of efficacy in slowing down the disease, but unfortunately, the effect is very, very small. To summarize it, it’s about two or three month prolongation of slowering down of the entire illness, which it’s not zero, but it’s not a great number.
[00:15:28] Dr. Kelkar: And more recently, another drug was approved. It’s called radicava. This is an infusion that is given once a month and that has been shown in some research studies to maintain the muscle strength better. With treatment as compared to without treatment, and so it’s available. But what we don’t know about this drug as yet is whether it actually has an impact on patients survival and quality of life. We don’t know that yet.[00:16:00]
[00:16:00] Dr. Kelkar: So only two medications have been approved so far in the last 20 or 30 years, and lots of research has been done in trying to understand the disease. What causes it, what’s the mechanism and how can we stop it? And various different drugs have been tested and we were part of many of those trials.
[00:16:20] Dr. Kelkar: And unfortunately none of them have worked as yet.
[00:16:23] Pete Waggoner: Did that surprise you a little bit? I mean now you kind of the two, are you surprised or is that just how tricky this is?
[00:16:30] Dr. Kelkar: It is just how tricky the disease is, you know, so when we try to understand the treatment for a disease, we try to establish what are the mechanisms leading of the disease?
[00:16:40] Dr. Kelkar: And with ALS, unfortunately all we know is that the motor neurons are lost and people have looked over and over and over. So what is causing that motor neuron loss? What is the mechanism or, what are the mechanisms so we can block them? And this just completely eluded us. All great scientists are [00:17:00] working on it, and nothing has been found yet.
[00:17:02] Pete Waggoner: When it does, that’s gonna be huge. We all know that. And you never give up, right? So, you know, we’ve kind of gone through the various components of it, and one of the things I think you mentioned the caregivers and the groups of people and the patient. What are some of the tips that you can give both groups in terms of improving quality of life, making it the best you possibly can?
[00:17:23] Pete Waggoner: Is there anything out there that you could do a broad brush on first for the caregivers?
[00:17:28] Dr. Kelkar: Well, particularly for the caregivers, they also need a support system. That is very, very important because the burden of caring for ALS is very huge and it is very common for the caregivers to have depression and anxiety.
[00:17:43] Dr. Kelkar: So in the ALS clinic, in fact, the social worker and the psychologist will also meet with the caregivers and address that need as well as it arises. And also setting up home care visits because patients will need help [00:18:00] with activities of daily living, like taking showers and getting dressed. And so one caregiver doing all of this 24/7 has a huge price tag attached to it in terms of their own health and safety.
[00:18:15] Dr. Kelkar: So we try to organize assistance at home. So the caregiver gets a break and they have help with other people physically and emotionally, both.
[00:18:26] Pete Waggoner: And then how about for the patient just to be as comfortable as they can. Are there a couple tips that you can add to them? Maybe someone picks us up and says, I never was told that.
[00:18:36] Dr. Kelkar: I think we sort of touched on that, that they need multiple different people looking at different things like the physical therapist, you look at the ambulation use of legs and the occupational therapist will then help with the function of the hands and then the bss, the swallowing and the breathing.
[00:18:53] Dr. Kelkar: Well, another big part of that is communication. And people lose their ability to talk or use [00:19:00] their hands and communicate. And that gets very depressing and frustrating to the patients. And yes, there are, in kind of a tip, would be that there are some high-tech, so to speak, devices and also low tech devices that we, we use with patients where they can point to different things with their eyes or with their fingers.
[00:19:20] Dr. Kelkar: And the machine then speaks for you and it seems like a maybe relatively simple thing, but it’s not. When you have the ability to communicate your feelings and thoughts to other people, that somehow is very liberating to the mind and very important for us to communicate.
[00:19:37] Dr. Kelkar: There’s an aspects sometimes not addressed as much as it should be.
[00:19:41] Pete Waggoner: Can you share what type of organizations are out there to assist individuals and families that are impacted by ALS and how they can benefit from them?
[00:19:50] Dr. Kelkar: Yes. There are two main resources. One is called ALS Association, and that’s a huge organization that offers a lot of help to the [00:20:00] patients and the families.
[00:20:01] Dr. Kelkar: Also they do research funding and other funding for the ALS clinics directly. So that’s a very important resource for the patients to connect with. Other one is Muscular Dystrophy Association. ALS is not really a muscular dystrophy disease, but the M D A does cover a lot of different parts of aLS and taking care of ALS. So the patients should definitely start with those.
[00:20:27] Dr. Kelkar: In addition to that, many big cities, at least like in the Minneapolis area, there are patient support groups that can be quite helpful to connect with for the patients and for the caregivers. So and so one can look into the local area for support groups.
[00:20:43] Dr. Kelkar: So that can be helpful.
[00:20:44] Pete Waggoner: That’s great stuff. Dr. Praful Kelkar of the Minneapolis Clinic of Neurology, we know you have a busy schedule that you have to keep, and we appreciate you taking time to talk about an area you’re very close to and are incredibly knowledgeable about, and we [00:21:00] do appreciate that you can share this with our listeners.
[00:21:03] Pete Waggoner: So thanks for joining us here today.
[00:21:05] Dr. Kelkar: Oh, thank you, and nice to meet you as well. Thank you.
[00:21:07] Pete Waggoner: Likewise. Thank you.
[00:21:08] Pete Waggoner: And thank you all to listening and joining in with us here on this episode of Neurology Now. We hope you found it informative and engaging. If you’ve enjoyed this episode, please subscribe to our podcast to stay up to date and help us educate our community and beyond.
[00:21:23] Pete Waggoner: We welcome your feedback, comments, and suggestions for future topics. So please feel free to reach out to us through our website or social media channels. That’s gonna do it for Dr. Kelkar. I’m Pete Waggoner. So long everybody.