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    • #12251
      Dagmar Munn
      Keymaster

      One ritual all ALS patients share is taking the ALSFRS-R test. The letters stand for “ALS Functional Rating Scale-Revised,” which is a list of 12 questions with multiple choice answers that track the progression of an ALS patient’s symptoms. Originally developed back in 1996 (with one revision in 1999 — thus, the “R”), the ALSFRS-R has been in use worldwide for the past 18 years.

      Prior to being diagnosed with ALS, I worked in a hospital environment for nearly 30 years. So, it was an immense shock to me that there was no medical testing or screening (lab, technology, x-ray, MRI, etc.) in use to track my ALS – – only the subjective self-reporting of 12 questions!

      Currently, researchers are looking into medical bio-markers for ALS; but meanwhile, the 12-questions remain the gold standard in evaluating ALS progression. I voiced my frustration in this column, I’m Still Functioning.

      What are your thoughts about the ALSFRS-R? Do you have confidence in this test as an accurate reflection of your physical abilities and/or ALS progression?

      I’m Still Functioning

       

    • #12297
      Ray Chandler
      Participant

      I don’t think it is a very good measure but like Riluzole it’s all we have.  My current score is 41 but could easily be 38 or 39 depending on how I answer.

      For starters – turning in bed.  At diagnosis I was answering this one with ‘slow and clumsy’.  Turns out it was the bed and not me.  We got a new bed and now the answer is ‘normal function’.  +1 point with no other changes.

      Alternate ways of dressing – some scales include sitting to dress as an alternate method, some do not.  I would consider intermittent assistance or zipper pulls to rate this score but not sitting to get dressed as I often did this prior to diagnosis.  I now cannot get dressed without sitting so maybe this degradation is enough to put me in this category or maybe not.  Another +/- 1 point swing.

      Use of bipap – I was placed on a bipap due to sleep apnea and not degraded breathing.  My respiratory numbers are fairly consistent for at least 2 years.  If I check ‘use at night’ I have a -2 point swing.  It only goes from None – Intermittent – Night with no in between so you instantly get a 2 point deduction for something which may not (or may) have to do with ALS.  I think the first check needs to read ‘None or use other than for breathing difficulties’.

      Lastly – handwriting.  Although I hardly ever hand write anything any more, printing is slow but certainly not sloppy or difficult to read.  Signing my name is becoming more of a difficulty as my hand is becoming slow to respond to my brains commands but it is not illegible.  +/- 1 point.

      The scale very much needs to be more granular.  I saw a presentation given where the scale was broken down to a 71 point scale for use with PLS.  This needs to be considered for all versions of MND if a scale like this is used as our measure of progression.

      My personal scale is broken down into sections:

      Bulbar – 12 total points – speech, salivation, swallowing at 4 points each.  12/12 gives me 100% total bulbar.

      Arms – 8 total points – handwriting, utensil handling (or tube feeding) at 4 points each.  My handwriting total comes out to 75% making my Arm total function 88%.

      Body Core/Coordination – 8 total points – dressing/hygiene, turning in bed at 4 points each.  My dressing score is 75% making my Body Core/Coordination score total 88%.

      Legs – 8 total points – walking, stair climbing at 4 points each.  My walking score is 50% and stair climbing is 25% making my Legs total 38%.

      Respiratory – 12 total points – dyspnea, orthopnea, respiratory at 4 points each.  I currently have respiratory rated at 100% but could be lower due to use of bipap.

      This gives me an overall function score of 85% (41/48).  This gives me personally a better idea of my overall function as well as seeing each function degradation independently.

       

      Ray

       

      • #12300
        Dagmar Munn
        Keymaster

        Good for you Ray, for holding on to those great numbers! But I agree (and am glad you agree with me) that the scale is kind of “squishy.”

        It is helpful as a “guide” to direct conversations with our doctor and medical team – – but still scary to think that drug results and other serious endeavors are totally based on a “squishy” self-reporting mechanism.

        Teach patients to roll over, I say, rather than medicate and accommodate. Here’s my favorite go-to for fundamental movement, Tim from Original Strength, teaching us how to roll: https://youtu.be/C7Vxcb1yOro

         

         

    • #12302

      I think that the problem stems from a weak definition of what ALS is. There is no strict medical definition of ALS, there are many mimics and probably 10% of ALS cases are misdiagnosed. There are no known pathogen agent, no biomarkers (lab works), so the diagnostic is differential, which means that when doctors have proven it is not another well known disease, and if it is obviously a motor neuron disease, then it is labeled “ALS”.

      ALSFRS and the other scales are just the reflection of the fact that it is a motor neuron disease and they are very practical, easy to understand and compare from patient to patient.
      However the scoring method which aggregates its components has been criticized, for example if the patient’s symptoms did not changed but if she/he received a treatment for excessive salivation, then the overall score increases.

       

    • #12345
      Bill Ott
      Participant

      I’m a hypochondriac. If you tell me the symptoms and give me a week I’ll have them. As such, for me, subjective measurements are useless. Subjectively, I am a mess. 🙂

      But I do have a few objective measurements that help mitigate my hypochondria. I take 22 pills a day, 11 in the morning and 11 after dinner. Even though I am convinced I have a swallowing problem, I swallow all 11 pills in one gulp. So, maybe my swallowing isn’t so bad. 🙂  I exercise every day, convinced it’s the last day I’ll be able to do the exercises. At the end of the exercise I get off the floor w/o any assistance, mechanical or otherwise, so maybe I’ll be able to exercise tomorrow. 🙂  The objective measurements do more for me in bolstering my confidence than any AFRS score.

      I am looking for more objective measurements. Anyone have suggestions?

    • #12346

      >> I am looking for more objective measurements.

      So are scientists as well 🙂

      There are many proposals for biomarkers for ALS, for example neurofilaments. The easiest to test by yourself is your body weight: Eat proteins and lipids!

      A successful biomarker is something that is sensitive but highly specific: It must be able to detect small changes in the progression course, while not be sensible to markers that are common to several diseases.

      If you have access to cheap labs tests, then you can ask for periodic tests indicating muscle waste and liver dysfonction: Creatine kinase, Gamma Glutamyl Transferase, Bilirubin…
      The idea is that if ALS progresses there will be more muscle waste, which had to be recycled by liver and kidneys and they suffer from overload. So it shows in blood works.

      I am not a doctor, nor a scientist so ask to your doctor what makes sense for her/him.

      I wrote a small Web page on ALS biomarkers with the common value (beware when it comes to medicine, nobody is average)
      https://padiracinnovation.org/en/ALS/

      • #12357

        Sorry for the people who tried to access my Web site: The server was down for the last 24 hours, everything is OK now.

    • #12364
      Dagmar Munn
      Keymaster

      Jean-Pierre, your website is very interesting and I appreciate the explanation of laboratory tests and ranges.

      The ALSFRS-R remains for me, a guide as to how to design my short-term and long-term lifestyle routines and regimens. My every 3-month lab screenings are concrete monitors of the effectiveness of those regimens.

      What I wished ALS Clinics also measured or included?

      • Lean muscle/body fat
      • Depression index scale
      • Posture

      What additional tests/screens do our forum members think should be included in regular doctor/clinic visits?

    • #12366

      Many thanks Dagmar,

      Out of curiosity, you are interested in a “Lean muscle/body fat” measurement, some retail scales give that information, but how would you use this information? What do you expect to learn from it?

      I ask because I have access to the PRO-ACT database and there is no such high level information in it. They seems to be interested in muscle degradation and recorded it in more than 10,000 pALS: Creatine Kinase, Triglycerides, Total Cholesterol, Lactate dehydrogenase and 44 other measurements.

      If you (or another person) are interested in the mean value of those parameters, or something else not too hard to compute, it would be a pleasure for me to provide it.

      This list of measurements is easily available in the PRO-ACT database:

       [0] = “Absolute Eosinophil Count”;
       [1] = “Albumin”;
       [2] = “Albumin/globulin ratio”;
       [3] = “Alkaline Phosphatase”;
       [4] = “ALPHA1-GLOBULIN”;
       [5] = “ALT(SGPT)”;
       [6] = “AST(SGOT)”;
       [7] = “Basophils”;
       [8] = “BETA-GLOBULIN”;
       [9] = “Bicarbonate”;
       [10] = “Bilirubin (Direct)”;
       [11] = “Bilirubin (Total)”;
       [12] = “Blood Urea Nitrogen (BUN)”;
       [13] = “C-Reactive Protein”;
       [14] = “Calcium”;
       [15] = “Chloride”;
       [16] = “CK”;
       [17] = “Creatinine”;
       [18] = “Fibrinogen”;
       [19] = “Free T4”;
       [20] = “GAMMA-GLOBULIN”;
       [21] = “Glucose”;
       [22] = “Hematocrit”;
       [23] = “Hemoglobin”;
       [24] = “IMMUNOGLOBULIN A”;
       [25] = “Lactate Dehydrogenase”;
       [26] = “Lymphocytes”;
       [27] = “Mean Corpuscular Hemoglobin”;
       [28] = “Mean Corpuscular Hemoglobin Concentration”;
       [29] = “Mean Corpuscular Volume”;
       [30] = “Monocytes”;
       [31] = “Partial Thromboplastin Time”;
       [32] = “Phosphorus”;
       [33] = “Platelets”;
       [34] = “Potassium”;
       [35] = “Protein”;
       [36] = “Prothrombin Time (clotting)”;
       [37] = “Red Blood Cells (RBC)”;
       [38] = “Segmented Neutrophils”;
       [39] = “Sodium”;
       [40] = “Uric Acid”;
       [41] = “Urine Appearance”;
       [42] = “Urine Bacteria”;
       [43] = “Urine Color”;
       [44] = “Urine Mucus”;
       [45] = “Urine Specific Gravity”;
       [46] = “Urine Squamous Epithelial Cells”;
       [47] = “Urine Uric Acid Crystals”;
       [48] = “White Blood Cell (WBC)”;

    • #12369
      Dagmar Munn
      Keymaster

      My “wish list” for all three of my suggested additions center on providing feedback and motivation for the patient. If everyone in the patient’s circle of care operate from the opinion simply assuming ALS patients will lose muscle strength & mass, become depressed and lose posture – – then the patient is on a sure course in that direction.

      Yes, a bathroom scale can easily provide Bioelectrical Impedance Analysis (BIA); percent of lean muscle, body fat and water weight. I use mine for feedback that my exercises and nutrition & liquid intake are maintaining good health levels. If this same measurement were a part of routine medical visits: patients could be encouraged to exercise or continue passive ROM, increase healthy eating and maintain hydration. Patients are often encouraged to generally “gain weight” – – but excess body fat can stress the body’s organs – – giving specifics goals would be helpful. Just tracking degradation is not “medical care” in my book.

      Same goes for depression and posture. Yes, excess sitting leads to weak muscles and poor posture – – but we can do things to slow down degradation in these areas. We should expect more education and motivation from our medical team (and change the perception of ALS).

    • #12375

      I understand now: You are on one of the most difficult task in today’s human communities: Changing the medical perception of ALS.

      I had colleagues (I am retired) and I know a few univ professors that wanted to offer help to MDs. They had to let go, MDs are a very conservative group, look for instance at the stethoscope. Not only it is completely obsolete but most doctors claim they master it, yet most studies shown they do not.

      Another example is the use of checklist in hospitals, it is simple, life saving, yet nobody wants them.

      Providing advice is complicated, it depends on the context, there are not textbook or quick answer. And to complicate the matter (and to explain why MDs behave this way) patients ask for quick answers. A lot of patients do not care about life style, they just want to swallow another pill and forget the disease. Current medical practice fills this need.

    • #12459

      About body fat and ALS, I did a little research through the literature on this topic:

      Many times, those diagnosed with ALS have led healthy and active lifestyles. In fact, it has been repeatedly shown that ALS patients have lower rates of antecedent disease than that of the general public [4] .

      Scientists known that a conventionally ‘risky’ cardiovascular profile, such as a high body mass index (BMI), or diabetes mellitus type 2, might protect individuals from ALS by delaying the onset of symptoms and/or slowing clinical progression. Hypertension, hyperlipidemia, arthritis, COPD, thyroid disease, and non-ALS neurological disease are found to be significant factors associated with a delay in ALS age of onset. These conditions have also been found to be less prevalent in the ALS population.

      On contrary, and equally surprising, a ‘beneficial’ cardiovascular profile, with a low body mass index, an athletic lifestyle, and low blood cholesterol levels, may increase the risk or worsen the prognosis [5][6]  .

      In 1978 scientists[2]  were surprised to see that insulin was strongly increased in ALS patients which suggested insulin insensibility. This leads to a flurry of drug trials with variations of IGF-1, Insulin growth factor until 2000. In 1997 a study shown that IGF-1 slowed ALS progression by 26% [3] , which is very similar to current ALS drugs such as Masitinib and much better than Riluzol.
      One study in 2010 seems to recapitulate the 1978 study as it finds that patients with ALS show abnormal glucose tolerance that could be associated with increased free fatty acids levels, a key determinant of insulin resistance [1]. However no suggestion was made to explain the association between ALS and insulin resistance.

      So my own conclusion is: there is no clear indication that fat is good or bad for ALS, but insulin resistance is probably a problem in pALS. However take it with a grain of salt 😉

      [1] https://www.ncbi.nlm.nih.gov/pubmed/20184518

      [2] https://www.ncbi.nlm.nih.gov/pubmed/661736

      [3] https://n.neurology.org/content/49/6/1621

      [4] https://www.ncbi.nlm.nih.gov/pubmed/25720304/

      [5] https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6278047

      [6] https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4810157/
      (An uncommon large study)

      So my own conclusion is: there is no clear indication that fat is good or bad for ALS, but insulin resistance is probably a problem in pALS. However take it with a grain of salt 😉

       

    • #12477
      Diana Belland
      Participant

      Thank you Dagmar and Jean-Pierre for this very interesting discussion.

      These lines in Jean-Pierre’s comments stood out for me:  “On contrary, and equally surprising, a ‘beneficial’ cardiovascular profile, with a low body mass index, an athletic lifestyle, and low blood cholesterol levels, may increase the risk or worsen the prognosis [5][6”.    I have always worked hard to do the “right” things to stay healthy, and I definitely fit the “beneficial” cardiac profile.  It’s been bewildering for me to learn that my “healthy” lifestyle may have contributed to the onset of my ALS. But I accept that many forms of cancer develop in individuals with this profile and we don’t know why either.

      This line in Dagmar’s response stood out for me:  “We should expect more education and motivation from our medical team (and change the perception of ALS).”  I heartily agree.  My first ALS clinic was April 15, 2019.  Since then I’ve seen my PT twice and I have had three sessions with the Pilates instructor associated with the rehab clinic but not part of my ALS clinic.  My neurologist and my PT are caring and personable individuals but I am disappointed in the lack of clear guidance I have received from in terms of how to manage my disease or as Dagmar says, how to “live well with ALS.”

      Since my diagnosis on March 18, 2019, I’ve learned far more about exercise and nutrition from reading Dagmar’s blog, posts and e-book, than I have from the members of my medical team.   This leads me at times to wonder if the grass may be greener, so to speak, at other ALS clinics.   My diagnosis was made at the Cleveland Clinic and I am still considered a patient there although my ALS clinic is in my home city four hours away. I am actually participating in both ALS clinics, and this arrangement is fine with both of my neurologists.     I will have my first ALS Clinic at the CC in August and I am wondering if they will take a more “proactive” approach in terms of specific recommendations for exercise and nutrition.

       

       

    • #12499
      Dagmar Munn
      Keymaster

      Jean-Pierre, I cannot find any reference to your statement “On contrary, and equally surprising, a ‘beneficial’ cardiovascular profile, with a low body mass index, an athletic lifestyle, and low blood cholesterol levels, may increase the risk or worsen the prognosis [5][6] ” in the studies you provided. ??

      I personally (and from what I have read and observed) that fit individuals are at a higher risk than the unfit. Many people are diagnosed with ALS who are unfit, overweight and/or generally inactive. The athletic folks simply stand out in media reports.

      I believe being fit prior to ALS is an advantage; the patient has better muscle mass and cardiovascular health. But they are at risk emotionally – – feeling that their past health efforts were for naught, and, unwilling to accept a new normal.

      The unfit ALS patient begins at a lower muscle mass and dedication to exercise; thus they atrophy quicker and feel helpless.

      Maintaining fitness as a patient, I believe helps the body and reduces mental stress. Measuring body fat and muscle mass may be a motivational benchmark to maintain their fitness – – and could be an addition to the strength testing done at ALS Clinics.

    • #12504

      Dagmar, first I completely agree with you that reducing mental stress is extremely important.

      The sentence that you quote about “beneficial’ cardiovascular profile” can be found in the reference [5] in the fourth paragraph of the background section. I would say that it is a rather common view.

      In second I am not a native speaker, and finding the right words is not so easy for me. In addition there are some limitation in vocabulary, “fat” means many different things depending on the context. It can means being overweight or refer to one chemical which is a building block of life. When I see fat, I understand lipid, but not being overweight.

      I do not want to push the idea that becoming unfit is a good thing to pALS, that would be stupid and having a BMI > 30 is proved to worsen the prognostic. Rather I think that when doctors tell patients that they need to not loose weight, it is very important. And lipids are very important for pALS, the brain and nerves are bathed in lipids. There would be no life without lipids.

      You say also “The unfit ALS patient …; thus they atrophy quicker“. It seems to me that scientists found the contrary to be true. But you are right, scientists agree with the need of exercise in ALS to maintain the neuro-muscular junction. For example in this article. Targeted and slow exercises are needed and you are probably one of the best experts on this subject.

      Finally I would argue that having a criteria to measure something is intellectually a satisfying perspective, but the reality is probably very complex and it may be very unproductive for a pALS to focus on their body fat. And I have no alternative to offer.

    • #12507

      I read this article which has this nice picture:

      At left is the relation between ALS risk and BMI for men, and at right for women.

      While the relation for men seems to be what I wrote (medicine books are written by and for men), for women the story is much more complicated: A BMI of 27 seems to give the best outcome.

      The study was to investigate the association between body fat and risk of amyotrophic lateral sclerosis (ALS), it included 518,108 individuals recruited from the general population across 10 Western European countries.

       

      • #12524
        Amanda
        Keymaster

        When was this article published?

    • #12525

      The article where one can find those pictures was published in:
      Neurology. 2013 Feb 26; 80(9): 829–838.
      https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3598455/

      It was cited 32 times!

      What I am telling is not some outlandish view of someone in Internet. On contrary it is the standard view on dieting and ALS since four decades:
      1. Lipids are as much important as proteins.
      2. Survival in ALS is best for people who have a BMI a bit higher (see figures) than what is usually recommended.

      But I am not a doctor, so I may be wrong, but I would like to read research that tells a different story.

       

    • #12571

      Amanda Sifford:
      You asked me: Did you read the article posted on ALS News today?
      As I can’t answer directly to your notification (you have a strange forum software), and as it is not a private issue, I answer here.
      I assume that you mean the article about BMI that was posted the 28 of June?

      Yes I did, it is another article on this subject that confirms that having a high BMI means a lower risk of having ALS.
      Nakken and al did several nice studies, including on multiple sclerosis risk for women in Norway.

      Several words of caution about ALS and BMI:
      1. My understanding is that for people who have ALS, it is dangerous to get a low body mass index (<22), particularly for women. For ALS women it seems that a BMI of 27 is perfect.
      2. For me (ask a scientist) it does not mean that for a healthy person, being lean creates a risk of having ALS. What I read in the scientific articles is that people with ALS have a high metabolism, so they are naturally lean and active. This is why I pointed to articles about insulin resistance in ALS.
      3. I do not think either that for a person diagnosed with ALS, getting overweight protects against ALS. pALS have to have a diet fitted for people with a high metabolism. ALS centers have a role here.
      4. All those articles that make statistics on large populations, may only apply to sporadic ALS cases, not to familial cases.

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