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    Good afternoon Doblett

    I see you are now back on the forum after being thrown off after you declared information about me that I shared here outside this forum against the rules thereof.

    If I find you been sharing any more information about me outside this forum, I will find some way of throwing the book at you.
    Warmly


    Andy

    ​Diagnosed 03/2015. One sided limb onset (arm) sporadic ALS/MND. MND hitting - now 50% left arm and 90% right arm, plus other bits including left shoulder

    "Things turn out the best for people who make the best of the way things turn out"

    Comment


      Hi, Our mum was diagnosed in Nov 2017 and begun using RCH4 in Jan 2018. She hasn't used any other ALS drugs. Her condition has stabilised since commencing RCH4 and there have been no side effects.
      We as a family are extremely grateful to the charity that supplies RCH4 free of charge along with ongoing support.
      Our hope is that the charity gains the recognition they deserve, to bring this treatment option to the market and help other PALs dealing with this devastating disease.

      Below are her latest ALS scores.ALSFRS-R 09-19.png

      Comment


        Hi Noow17,

        Pleased to hear that your mum is still getting the drug OK because I was led to believe that Dobett couldn't get it.

        Love Terry
        TB once said that "The forum is still the best source for friendship and information."

        It will only remain so if new people post and keep us updated on things that work or don't work and tips.

        Please post on old threads that are of use so that others see them and feel free to start new subjects and threads.

        Comment


          Hi all,

          Neater Robo-arms are out of stock - sadly for me it will be a 4~5 week wait.

          In other news: I have always doubted the veracity of ALSFR-S scales since a final number is used to assess the progress of ALS. I was doodling along with my usual research on ALS/MND when I came across a more detailed variant that breaks the final number down to specific area - respiratory et etc.

          https://www.mdcalc.com/revised-amyot...rs-r#use-cases

          Wondering if any other on the forum think it more useful to see the breakdown and improve a prognosis - note the original research document is from 1999.

          I think it a much better scale than an extract from a spreadsheet..

          What does everyone think?

          Kind regards

          Chas

          Comment


            Hi Chas,

            Sorry to hear about the arms being out of stock. There was one advertised but it was adapted for a small person. I doubt that it is still available but I could check if you have short arms.

            Love Terry
            TB once said that "The forum is still the best source for friendship and information."

            It will only remain so if new people post and keep us updated on things that work or don't work and tips.

            Please post on old threads that are of use so that others see them and feel free to start new subjects and threads.

            Comment


              Chas, I may have misread your post, but that ALSFRS-R scale is the scale being used, not only by the posters on this thread, but in MND Clinics and by other healthcare professionals too. (ALSFRS-R = ALS Functional Rating Scale Revised)

              As someone who has completed many, many ALSFRS-R sheets over the years, my issue with the ALSFRS-R is that it is a subjective, self-reported system, with no scope for nuance in the blunt scoring system.

              That said, there has to be some standardised way of, for example, having inclusion/exclusion criteria for clinical trial participation, so the ALSFRS-R is often one of the criteria used for enrolment.

              Love Ellie.
              ​Diagnosed 03/2007. Sporadic Definite ALS/MND Spinal (hand) Onset.
              Eye gaze user - No functional limbs - No speech - Feeding tube - Overnight NIV.

              Comment


                Thanks for that Terry. However I am - was - 6ft - and longish arms.

                I will make enquiries though about modifications.

                Kind regards

                Chas

                Comment


                  Originally posted by Ellie View Post
                  Chas, I may have misread your post, but that ALSFRS-R scale is the scale being used, not only by the posters on this thread, but in MND Clinics and by other healthcare professionals too. (ALSFRS-R = ALS Functional Rating Scale Revised)

                  As someone who has completed many, many ALSFRS-R sheets over the years, my issue with the ALSFRS-R is that it is a subjective, self-reported system, with no scope for nuance in the blunt scoring system.

                  That said, there has to be some standardised way of, for example, having inclusion/exclusion criteria for clinical trial participation, so the ALSFRS-R is often one of the criteria used for enrolment.

                  Love Ellie.
                  Exactly my views too Ellie. I also checked with my neurologist in Sheffield - they still use the version I tagged.

                  Kind regards

                  Chas

                  Comment


                    Sportingmac said

                    I have always doubted the veracity of ALSFR-S scales since a final number is used to assess the progress of ALS. I was doodling along with my usual research on ALS/MND when I came across a more detailed variant that breaks the final number down to specific area - respiratory et etc.

                    He then correctly directs us to the more modern ALSFRS-R scale

                    https://www.mdcalc.com/revised-amyot...rs-r#use-cases

                    which is highly accurate in consideration of the fact that it is the only validated system. There is no validated biomarker ("test") to measure MND.
                    Basically this is exactly the scale that the RCH4 charity uses for everyone's monthly monitoring report. Actually they have added further datum points to their form which is also dynamic in that it also encompasses predictive analysis etc. which builds over time.

                    From the `Net
                    Measuring the progression: The ALSFRS-R score

                    As the ALSFRS-R score is a subjective observation, it will likely be scored differently by any two different people. It is said that the PALS must be scored by a specialist medical professional - but this is not the case.
                    See the evaluation study in the accuracy of on-line self reporting https://www.tandfonline.com/doi/full...68.2011.633268 (Journal Amyotrophic Lateral Sclerosis Volume 13, 2012 - Issue 2)


                    In fact the precise ALSFRS-R score is not particularly relevant. What matters is the CHANGE in the score over exactly the same time periods.
                    Provided that the SAME person (e.g., the patient or a care giver) does the scoring every month over a long time, the submitted change in data is very accurate. Further, scoring by the same person over extended time ensures elimination of patient or carer bias as the submitted data would rapidly exceed the envelope of credibility.


                    Again from the `Net, the patients own reporting is very consistent. In this case for the RCH4 drug which was given to everyone for free but the charity has no money now.
                    als-2-year-117 chart.jpg

                    The ALSFRS-R score does not reflect loss of neurons or other subtle changes, but someone rightly said that if the ALSFRS-R score never changed then we would all die from extreme old age or something else. Speaking as a medical doctor, I must accept that is true.

                    Comment


                      Hi Originalthought,

                      Thank you for point. However - it remains a subjective method.

                      Also- the results depend largely on the type of motor neurone disease - ALS versus PLS versus Bulbar onset versus limb onset. Each would have a different trajectory.

                      There has been research into objective testing. The research used two different methodologies: MRI scans and EMG results. The EMG determines the number of neurones used to move a selected muscle and the size of the neurones . Using these two objective tests it was shown how much degradation was occurring in the patient.

                      I was part of that research.

                      Harvard University picked up the ongoing research - sadly I have yet to see its outcome - I declined my participation through my lack of mobility to get to Sheffield. Ongoing research for me.

                      Hope that helps explain my position.

                      Meantime I wish you all the best in your research.

                      Kind regards

                      Chas

                      Comment


                        Hi Chas,
                        Thanks for your best wishes for my research. Sorry there is a misunderstanding. I am not smart enough to be a scientist or researcher! Just a retired GP dealing with this awful condition.

                        My “research” only went so far as to learn that Riluzole did nothing to slow the progression and RCH4 definitely does in my case. My concern is how long the RCH4 charity, who supply it for free, will last as they have no funding.
                        We all know that MND is a most heterogeneous condition so outcomes will vary a lot. The charity http://www.rch4.com say it is ineffective in 16% of the treated population. However I came on this interesting table from PLM and also see that it has the highest votes on ALS Untangled (our American cousins call it ALS).
                        PLM Efficacy Table.png

                        To address your point about the ALSFRS-R score. You are absolutely right that it is subjective, but it has been validated as the gold standard for measuring progression in the absence of any biomarker being available. As I posted before, what the actual score is for each patient is not in fact relevant. What matters is the CHANGE IN THE SCORE OVER TIME.

                        Even if an accurate objective protocol or biomarker were developed tomorrow, it would still be academic as there is no “cure”. All an accurate biomarker would do is enable earlier definite diagnosis. Sadly, that’s little help to us.
                        Last edited by originalthought; 12 October 2019, 03:20.

                        Comment


                          Oct 2019.png

                          Mums latest ALSFRS-R chart. She's still doing really well.

                          Comment


                            Originally posted by Sportingmac View Post
                            Hi all,

                            Neater Robo-arms are out of stock - sadly for me it will be a 4~5 week wait.

                            Chas
                            Hi Chas;

                            Any new on the arms.

                            Love Terry
                            TB once said that "The forum is still the best source for friendship and information."

                            It will only remain so if new people post and keep us updated on things that work or don't work and tips.

                            Please post on old threads that are of use so that others see them and feel free to start new subjects and threads.

                            Comment


                              Hi Noow17,

                              Pleased to hear that your mum is still getting the drug OK.

                              I was led to believe that Dobett couldn't get it and that was the reason for his anger.

                              Love Terry
                              TB once said that "The forum is still the best source for friendship and information."

                              It will only remain so if new people post and keep us updated on things that work or don't work and tips.

                              Please post on old threads that are of use so that others see them and feel free to start new subjects and threads.

                              Comment


                                Originally posted by Terry View Post
                                Hi Chas;

                                Any new on the arms.

                                Love Terry
                                Ongoing saga with NHS. NHS say NO. MNDA need reason for no before offering assistance. Have written to CCG asking for their help. Also have investigation by Neurologist in Rehab Team into reasons too.

                                Meantime I now have a GridPad - Yeahhhhh Robo-Arms would be good to reach the screen. Eyegaze if no robo-arms.
                                Last edited by Sportingmac; 21 October 2019, 20:19.

                                Comment

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