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Hiroshi Mitsumoto, MD, DSc, discusses palliative care for patients with amyotrophic lateral sclerosis.
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Hiroshi Mitsumoto, MD, DSc, discusses palliative care for patients with amyotrophic lateral sclerosis.
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Segment:0 .
>> Hello, and welcome to "JAMAevidence," our monthly podcast focused on core issues in evidence-based medicine. This is Ed Livingstone, Deputy Editor of "Clinical Reviews & Education" here at JAMA. Understanding when and how to provide palliative care for patients with amyotrophic lateral sclerosis, or ALS, can be difficult. Joining me on the program today to talk about this topic is Dr. Hiroshi Mitsumoto. He's the Head of the Eleanor and Lou Gehrig ALS MDA Center at the College of Physicians and Surgeons Columbia University School of Medicine and the author of a chapter in "Care at the Close of Life" that helps physicians empower ALS patients to prepare for the worst and hope for the best.
Dr. Mitsumoto, thanks for joining us. Could you begin by telling me how patients present with this disease? >> Well, it varies. For example, some patient present weakness of the hand, the fingers, unable to open jar or use fingers, turning of keys, buttoning, using zipper. Those [inaudible] problems. Some other patients develop some walking problems, for example, foot drop. Going up stairs becoming difficult. And some patient also develop some trouble with the speech, slurred speech, and difficulty swallow, coughing with liquid.
So quite variable, but always invariably those problem gradually, gradually spread to other area. That's the most common presentations. >> It sounds like those are fairly non-specific signs and symptoms. When you see a patient with these problems, what differential diagnosis do you think about? >> It could be, of course, ALS type of disease or motor neuron disease. Could be muscle disease. It could be cervical spondylosis myelopathies.
So those disease are more common disease. As you know, there is no test to make diagnosis. For example, send out some lab test coming back that the patient has ALS. So don't have any [inaudible] such test. So how we make diagnosis, essentially, exclude all possible diagnoses step by step. >> Could you tell us a little bit about what those steps are? >> Well, usually, you have to get cervical MRI, brain and neural imaging, but most important thing is full examination, physical, neuro-skeletal examination, and then those blood tests screening some metabolic tests, complete blood counts, and screening of diabetes, and immunofixation, electrophoresis, parathyroid, and all those tests are very important.
So we just screen those disease out step by step, and it is very important to confirm disease is progressive by history, by repeated examination, and then there's a criteria to make a diagnosis. It's called ALS [inaudible] diagnostic criteria for ALS. So we look at the criteria, whether disease meets such criteria or not. So that's the way you make diagnosis. But also important to exclude any possible other causes, as I already mentioned.
>> Could you tell us a little bit about what those criteria are? >> Well, you have to find upper motor neuron signs by examination. And also you have to find lower motor signs by examination and EMG. And then you confirm disease progression and also where the disease involves. For example, cranial nerve, denervated muscles, or arms, or thoracic levels, and lumbosacral levels. There's four neurological regions where the disease involved, and then disease is progressive.
Then you can meet certain criteria. Criteria include definite, probably, and possible diagnosis. Now, new diagnostic criteria is being proposed, so we will have better diagnostic criteria in a couple of years. >> So an upper motor neuron sign is characterized by hyperreflexia and spasticity, whereas a lower motor neuron sign is characterized by fasciculation, hyporeflexia, muscle atrophy, and weakness.
>> Right. >> Once you've given a patient a diagnosis of ALS, how do you treat them? >> Well, there is medication has been approved 1995, Riluzole has been approved. So we have been using almost now 19, 20 years. And this medication slow down the disease a little bit. Extensive studies showed prolongation of survival by two to three months. ALS is not a curable disease, but no cure doesn't mean no treatment.
So much we can do. For example, many symptoms, such as muscle cramps, constipation, and sleep disorders, the problem with the sleep, depression, drooling, those are all manageable. And of course, one medication may not work, but you have to try other medications. So I think symptomatic treatment is extremely important. Also, discussing current research because research is so rapidly ongoing. Yes, it is true we don't have any cure.
We don't have any effective medication except for Riluzole, but research is so rapid. So I emphasize a lot of research going on, and I encourage them to participate because anyone who participates in clinical trials appears to have a more positive attitude in better dealing with difficulties of the disease. So that's what I would encourage. >> Given the fact that the disease is progressive, there must be a need to provide palliation as the patients deteriorate with time.
What sort of palliative measures are needed for this patient population? >> So we had some special advisory board meeting to promote excellence in end-of-life care in ALS that was assembled 2000 to 2004. We gave a recommendation. But everyone felt actually end-of-life or palliative care begins at the time of diagnosis, as you pointed out. But some patients have a very hard time to accept such palliative care.
But all we can do is symptomatic and palliative care. So I think it's any positive actions maintain function as long as they can, and also maintain their strong fighting spirit, and then nutritional care, and if needed, restorative care is critically important. All those are palliative care. >> When you first tell a patient that they have this disease and that palliation should start at the time of being told they have that disease, how do you approach that patient?
What do you tell them? >> Well, ALS is such a highly publicized disease. And almost everyone think if they are told to have ALS, they feel they are told that they are going to die. So that is a major difficulty. So I emphasize disease is a tremendous variation in prognosis. That's very true because more than 10% patient lives beyond the 10 years.
And also, few percent maybe live up to 20 and beyond. So you emphasize the positive aspect of the disease rather than emphasizing negative aspect. And then I'm not going to use the word palliative. So essentially, discussing disease features, pathophysiologies. Let them understand the disease. Emphasize the variation in prognosis. And that's the way to gradually introduce practical sense of palliative care, not using the word palliative or end-of-life care.
That's what I would do. >> So you tell them they have this progressive disease, and they become despondent. What do you do with them then? >> Well, I would suggest a fighting spirit, and maintain function, talking about exercise, clinical trials, and coming into a multidisciplinary clinic, which is extremely important. So anything that they can do, I would suggest. >> Where do you suggest they go? >> Well, there are almost now 90 multidisciplinary ALS clinics throughout the country.
So I would advise they should go to a multidisciplinary ALS clinic. And if they are close to our center, Columbia University, they should come to our center. If they are close to some other centers, certainly I would advise to go to those place. >> So I know that the ALS Association provides special transportation to those clinics and helps provide support for the patients and their families, but what else makes access to a multidisciplinary care team particularly important for patients with ALS?
>> So I think it's very important to come to a multidisciplinary clinic where we have multiple healthcare professionals working with the patient, one after another, and to go over those issues, nutritional issues, pulmonary issues, nursing issues, social worker issues, physical therapy exercise issues, occupational therapies. All those issues are specialized to the condition they're dealing with. They can evaluate. They can answer, and give some positive spirit.
And they are always positive thinkers. So coming to them, even if you are not stopping the disease or reversing the disease, those positive attitude from ALS clinic is very helpful to the patient and the family. >> Well, thank you, Dr. Mitsumoto for this insightful discussion. More information about this topic is available in "Care at the Close of Life," and on our website, JAMAevidence.com, where you can listen to our entire roster of podcasts. I'm Ed Livingston, and I'll be back with you soon for another edition of "JAMAevidence."
