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Hi from Claire in Devon, supporting my Dad with ALS bulbar onset

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    Hi from Claire in Devon, supporting my Dad with ALS bulbar onset

    Hi all,
    I've looked at the forum a few times over the last 11 months since my Dad was diagnosed (69 at the time) but this is my first post. His first symptoms were 11 months before (approx May 2020) with slurred speech. As seems to often be the case he was miss diagnosed (mini stroke). 6 months later he had difficulty eating and excess saliva, he eventually had a neurology appointment. He was on his own and told it's not confirmed yet but likely MND and given a leaflet with really worrying statistics. After a difficult month or so, he was finally diagnosed, started riluzole straight away and referred to the Community Team.

    Our Speech Therapist is amazing - helped with swallow, speech to text apps and provided a light writer so he can type and it speaks for him (too late to bank his voice sadly). We also have a great Physio who has provided a neck brace as he has weakness in the muscles in the back of his neck and assists with breathing/cough techniques.

    He had a feeding tube in December, he put it off as late as poss as he is squeamish. By the time we finally got the consultant to approve it and the appointment came through it was nearly too late (thankfully a second doctor managed to place it). Dad has coped brilliantly and I would suggest to anyone to consider it early. Eating was an absolute chore for my Dad and with risks of choking, now he uses the tube for all nutrition and only has the food he likes (pureed chocolate cake and ice cream although this is increasingly difficult.) His meds are now liquid suitable for the tube.

    The current challenge is clearing thick mucus which causes a blockage in his throat as his cough is too weak to clear it. He's just started using a nebuliser and a cough assist machine but any suggestions on this would be gratefully received as it is really horrible for him. He has 2 meds to try to help (Glycopyrronium Bromide and Carbocisteine) but it remains very difficult.

    It's likely his carbon dioxide levels are raised at night. The indication is that Non-Invasive Ventilation will be recommended. Other thoughts are about future mobility challenges with breathing declining, I was thinking of a stair lift however its recently been suggested this may be difficult as core muscles are likely declining and stability on a stair lift may be affected.

    There's so much I don't know and wouldn't have thought about, I like to plan and be ahead of things but feel like I'm always one or two steps behind the illness, despite trying my best to ensure he gets the best care.

    He is lucky to have very good mobility so is living independently, going for walks and gardening. Sadly he has stopped his sports (boxing, table tennis, running and cycling) due to his breathing declining. In amongst all the medical stuff we try to have good times, a simple walk on a sunny day.

    Sorry, this has turned into a long post! I'm not known for being concise!
    Claire

    #2
    Claireflo A warm welcome to the forum Claire, thanks for making your first post.

    So sorry to hear of your dad's diagnosis and of his secretion struggles - secretions can be frustratingly difficult to manage in ALS...

    Is he operating the cough assist on his own? It does feel brutal at the start but hopefully its benefits outweigh the discomfort , which he will get used to over time.

    And the neb - is he using it a few times per day and is it loosening the mucus but it's the coughing up that's the issue?

    Has he a suction machine?

    Is he getting lots of water (~2000mls) daily?

    Re the stair lift: they are fab for maintaining a sense of 'normality' for as long as possible - going up to bed etc - so, what about renting one if one isn't provided free for him?

    Also, may I say, and I hope I don't sound patronising, but you come across as someone who is very competent and full of common sense - great attributes for supporting your dad.

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

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      #3
      Claireflo - welcome. Sorry to hear about your dad's struggles but thus is a fantastic group for support and information. We will do our best to take care of you xx
      Diagnosis confirmed as atypical ALS Jan 2022 (age 46) after several years misdiagnosed.

      Symptoms began in left foot 2017. Now widespread. Powerchair user, useless left arm and clumsy right hand but generally positive!

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        #4
        Thanks Ellie,
        He's just started using the cough assist machine and has a review in a week to tweak the settings.
        He's using the nebuliser several times daily and he's trying a stronger strength solution (3% hypertonic saline). Nothing much seems to be helping so far, though I'm starting to wonder if there isn't something else as nothing is improving.

        He was doing OK secretion wise until he had COVID about 5 weeks ago and since then he's had this ongoing issue.

        He has a suction machine but doesn't use it often (uses tissues to remove secretions instead). I think he's struggling with his bedroom looking more like a hospital room with all the machines.

        I've said to him about hydration but he struggles with more than a litre of water. He's currently on 8 fortisip drinks as he's lost weight, plus meds and flushes so it's quite a lot of volume for his stomach.

        Definitely worth looking into the stair lift. I was speaking with a local MNDA supporter and she said there are grants that may be able to assist so I've added that to the 'to do' list!

        Thanks, that's lovely of you to say. It's been a steep learning curve and it's hard feeling useless but I'm trying to accept that I'm doing everything that I can. Thanks for your kind welcome

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          #5
          Thanks TinyLady, really appreciate the welcome. Any information and suggestions will be gratefully recieved!

          It seems the infrequent consultant appointments involve us updating her with no new information gained as to what to look out for or what may come next, hence the feeling of always been several steps behind the illness.

          Comment


            #6
            Claireflo - I totally understand that feeling of being behind and not sure how to get ahead (or even catch up) and prepare.

            If I have issues I usually search on the forum first (loads of useful info from first hand experience) then I might post and ask. My local team are a bit sparse and I lack sufficient OT support, so we improvise a lot. I have rented a stairlift and hope one day to save up enough to put a through floor lift in. It is really important I stay able to access my house and live as normally as possible for my children's sake as well as mine.

            Like your dad, I'm also a big fan of ice-cream 🙂
            Diagnosis confirmed as atypical ALS Jan 2022 (age 46) after several years misdiagnosed.

            Symptoms began in left foot 2017. Now widespread. Powerchair user, useless left arm and clumsy right hand but generally positive!

            Comment


              #7
              TinyLady worth keeping in mind that the MNDA have grants available to help with adaptations. Might be worth exploring in the future.

              A general comment from me is, before you think you need to make changes e.g. stairlift, wetroom etc it’s worth talking to the grants team to check what is available to help. This disease takes enough away without stripping you of every last penny.

              Comment


                #8
                Originally posted by Claireflo View Post
                He was doing OK secretion wise until he had COVID about 5 weeks ago and since then he's had this ongoing issue.
                Ah, that would not help the situation for sure - the virus is known to increase and to thicken respiratory mucus in many people, a double whammy if you have ALS-related respiratory weakness. It might take a medication in the neb to tackle the mucus, stronger than the saline, but it sounds as if your dad's doctor is on top of it (🤞🤞) I wonder if one of the breathing techniques the physio showed him was 'Breath Stacking' - if it was and he can manage it, it can be very helpful.

                Originally posted by Claireflo View Post
                I think he's struggling with his bedroom looking more like a hospital room with all the machines.
                I get that, I really do, we all hate our 'stuff' - it takes a U-turn in mindset to see equipment as helpful, not hateful 😏 Even if he stored it in a cupboard/blanket box/under the bed when it's not in use so as it's not a constant in-your-face reminder that this is his life now... (I have named my most hateful equipment after hot male characters in 'TV' shows, juvenile I know, but hey ho, it gets me through the day 🤣🤣)

                Originally posted by Claireflo View Post
                I've said to him about hydration but he struggles with more than a litre of water. He's currently on 8 fortisip drinks as he's lost weight, plus meds and flushes so it's quite a lot of volume for his stomach.
                That makes sense but (yes, there's a but 🙄) I can't overemphasise the importance of proper hydration and its effect on thinning viscous mucus.

                8 Fortisips daily is hard - 2 bottles, 4x daily, plus flushes and meds in between, sounds like a full-time regimen in itself. I hesitate to suggest this... my favourite piece of equipment is my little feeding pump and, if your dad is unaware of their existence, he could use the pump to get his calories overnight and be free to nibble and flush during the day. Feed is delivered at a set, comfortable rate, over 8-10hrs (or whatever suits) and doesn't leave you feeling full/icky. Of course the pump can be used during the day too and/or for shorter periods - food for thought... xx
                ​Diagnosed 03/2007. Sporadic Definite ALS/MND Spinal (hand) Onset.
                Significant bulbar impairment - No functional limbs - No speech - Feeding tube - Overnight NIV - Eye gaze user
                .

                Comment


                  #9
                  TinyLady I'm sure the first hand experience will be really helpful as the medical team seem hesitant to share what might come next. I appreciate everyone is different and the disease affects everyone differently but having an idea of what happen hopefully enables you to pick up on it early and try to prepare for it.

                  The MNDA have mentioned the grants available and there's one for home adaptions so definitely worth looking at.
                  ​​​​

                  Comment


                    #10
                    Ellie he coped much better with COVID than I feared he would except for the mucus issue. The physio is the one advising what we should try but the GP has generally been good and prescribed what's recommended. Dad's tried the breath stacking and manual assisted cough but just isn't enough to clear the mucus. He's been trying to make himself sick as its the only way of clearing it 😔.

                    My dad is very stubborn and if he doesn't think something is working immediately its hard to get him to stick with it. I like the idea of naming the machines!

                    The feeding is very time consuming, dad has so far been sleeping great (until the mucus issue) so we hadn't wanted to risk affecting that. However it's definitely something to think about as it may be more tolerable for his stomach over a longer period and free up his day. Thanks for the suggestion!
                    ​​​​​​

                    Comment


                      #11
                      Claireflo I started with bulbar onset about 6 months before your dad, and really appreciate your dad’s problem with mucus. I haven’t had Covid but impressed that your dad has got through it except for this horrid problem. I did have a chest infection briefly (caught in time with antibiotics I think), but it started off mucus problem for me for the first time. At the time I was on glycopyrronium bromide for having too much saliva, but I stopped it then in case it was thickening my mucus. I was started on carbocystine which thinned my mucus down and I have had a period without mucus being a problem (though just starting again to a small extent). But my saliva problem isn’t that bad at the moment, so not needing anything like glycopyrronium.
                      I have been struggling to use cough assist. I live on my own and have carers twice a day, but hands too weak to hold the mask on with much force, and not finding it useful so far. I wish I did, as it must be great if it helps you cough up when you need it.
                      So , many sympathies with your dad, and to you too (I often think it’s a full time job getting informed about everything).
                      love to you both, from Heather
                      Diagnosed July 2020, ALS bulbar onset. PEG and ventilator (mainly at night), and pretty poor speech, but legs still about functioning!

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                        #12
                        Welcome along ""
                        Bulbar started Jan 2020. Mute and 100% tube fed but mobile and undefeated. Stay Strong 🤗😘🤗😁xx

                        Comment


                          #13
                          Thanks Heather R​​​​​​, it does feel like a full time job trying to keep up with all the appointments and ringing around sorting out meds etc. I live 2 hrs away from dad which doesn't help.
                          Dad coped amazingly with COVID, it was a worrying time as most of my family had it including my elderly nan, who had been living with dad until a hospital stay in late Feb. She's now moved into a home near me so I can make sure she's looked after and visit. It also means she doesn't have to watch dad deteriorate.
                          Dad's issues with mucus also originally started after a chest infection (in December after having the feeding tube fitted) but were manageable until he got COVID.
                          Dad hasn't found the cough machine that helpful but I hope they can increase the settings and it will be more help. Can your carers help you to hold the mask on while you use it, or is there a strap that can be used with the mask to hold it on better?
                          Sending my sympathies back and I hope your saliva issues continue to remain manageable.
                          ​​​

                          Comment


                            #14
                            Thanks matthew55

                            Comment

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