We need to focus on upper limb and bulbar function in progressive MS. #MSBlog #ClinicSpeak
“I saw a patient in clinic last week who has PPMS. He travelled a long way to see me. Tragically I had little to offer him; he had been referred to me for clinical trials. He has been using a wheelchair for several years and is gradually losing upper limb function and has started to have swallowing and speech problems. He is now unable to self-transfer from his wheelchair and needs a hoist. He has an electric wheelchair. He has lost hand function in one hand and uses the the other hand to control the wheelchair, type (single-finger) and eat. He is beginning to have choking episodes when eating and drinking and his speech has become very soft and quite. Despite these problems he has maintained a good quality of life; he has a very caring family, a large circle of friends and gets out frequently. He is desperate to slow down any further progression of his disability and would love to participate in clinical trials. It is my mission to make sure when we take our new neuroprotective treatment strategies forward we don’t exclude MSers with progressive disease who are wheelchair users. Surely, protecting upper limb and bulbar function (swallowing and speech) is something worth doing? I need your help to lobby the charities, Pharma companies and the payers to make sure that if anything gets through the pipeline in this phase of the disease that we make sure it is cost-effective and MSers have access to the drug. When we last did a survey on this topic the results were clear.”
“I saw a patient in clinic last week who has PPMS. He travelled a long way to see me. Tragically I had little to offer him; he had been referred to me for clinical trials. He has been using a wheelchair for several years and is gradually losing upper limb function and has started to have swallowing and speech problems. He is now unable to self-transfer from his wheelchair and needs a hoist. He has an electric wheelchair. He has lost hand function in one hand and uses the the other hand to control the wheelchair, type (single-finger) and eat. He is beginning to have choking episodes when eating and drinking and his speech has become very soft and quite. Despite these problems he has maintained a good quality of life; he has a very caring family, a large circle of friends and gets out frequently. He is desperate to slow down any further progression of his disability and would love to participate in clinical trials. It is my mission to make sure when we take our new neuroprotective treatment strategies forward we don’t exclude MSers with progressive disease who are wheelchair users. Surely, protecting upper limb and bulbar function (swallowing and speech) is something worth doing? I need your help to lobby the charities, Pharma companies and the payers to make sure that if anything gets through the pipeline in this phase of the disease that we make sure it is cost-effective and MSers have access to the drug. When we last did a survey on this topic the results were clear.”
Absolutely agree with you, being able to use your upper limbs is crucially important
Oh God, yes. I would just give up all hope if I lost the ability to play the musical instruments I treasure so much. I'd opt for Switzerland if I got that bad. The malignancy of MS astounds me.
Tragic story. I suspect it's not an isolated case. Puts into perspective the claim that so many advances are coming so quickly in MS research. In a lot of case advanced MS is MND by another name – same dreadful symptoms. I don't share the view that this is a good quality of life. Time to call a spade a spade – this is a dreadful quality of life and the individual has been let down by the so called experts.
The case highlighted by Prof G of the severely disabled PPMSer who sought his help, though went away disappointed, to me typifies the greatly complex nature of this disease.The patient is desperate for alleviation of further damage to his motor functions, yet seems also acceptant that science isn’t advanced enough to help him. He has hope but is used to having neurologists shaking their heads in futility and closing the door on him. He has been let down by health care, Big Pharma, government and life in general.It is at this point one can play the ‘right to die’ card, arguing that the patient should have an option to end their life because there is nothing to offer him currently. But, however, Prof G saying: “[The patient] has maintained a good quality of life; he has a very caring family, a large circle of friends and gets out frequently,” reveals how limited our notions of ‘quality of life’ is.To me this patient is the definition of an MS Warrior; a crusader who has fought and continues to fight despite the elements being against him. I hope that medicine soon is in a position to help him, but nonetheless, I regard him as an example of how this insidious disease separates the heroes from the zeroes.
"Surely, protecting upper limb and bulbar function (swallowing and speech) is something worth doing?"Absolutely. As I have said before on in a comment on this blog, I have accepted the slow degradation of my walking ability. My hiking poles still get me around, as do wheels, whether it be a car or a quad for the hills. And maybe a mobility scooter or wheelchair some day, depending on circumstance and what happens. But when I contemplate the loss of upper limb function, this is like staring into a dark void. Not only does the loss of upper limb function mean a much higher degree of dependence upon other people, also for intimate care, but the loss of abilities which I feel define who I am, e.g. my handwriting style, my drawing.
It's probably because MS is not a 'fatal' disease as such, less emphasis is put on it. Government, big pharma and researchers are more concerned about the quantity of life rather than the quality.
But complications can be fatal and it seems this is not that well known among neurologists.
"…problems he has maintained a good quality of life; he has a very caring family, a large circle of friends and gets out frequently." As a practicing physician put yourself in his shoes. Would this be a good quality of life for yourself? Would you be able to continue your practice? Would you maintain your ability to be independent and to do the things that make life enjoyable? This case only emphasizes that repair and neuro protective therapies are urgently needed. But we have been saying this for years……sigh.
I think that you're wrong, Anon 1:36pm.When one's MS progresses too much, your expectation from life shrinks too. You become more content and accept things as they are. You stop living in fear and refine what life is and what one wants from life.
Anon 3:56 – yes, I agree. Although I would say that one's expectations must be adjusted, rather than shrunk.And Buddhist philosophy begins to make a lot of sense. The idea that everything changes, that there is nothing that one has that cannot be lost, that one shouldn't cling to things. It's clinging that brings misery. Embracing and adapting to change brings peace.
This is a really limited view, and doesn't ring true with many advanced MSers I know. You don't stop living in fear because now you're facing choking to death on your meals, a life of nurses cleaning and changing you, caregivers embezzling from you while you can't protect yourself. Get real. And even if the MSer is content with a narrower world view, you should be considering the cost of advanced MS on families and society. I can assure you, as a young caregiver spouse to a young advanced MSer, I am angry about the limitations that MS has imposed on our lives and angry that we have been written off.
Anon 6:25 – Your comment makes clear that I, for one, am in no position to comment on behalf of someone else with MS, particularly someone who has experienced more rapid and aggressive progression than I have. I also become riled by broad brush statements about MS, e.g. that a diagnosis of MS per se (or PPMS, in my case) precludes the possibility of a high quality of life. That is simply not true.
I have left side paresis. This is the major factor in my walking but affects manipulating things: books (while reading or actually stopping me reading books), paper work, knife and fork, holding things to cut with a knife, etc. I'm now a one hand typist.(In keeping with many of my work predecessors 30 years ago but now a certain disadvantage. The general population has got better at typing.) Though my deficit in walking reduces my quality of life when I want to go out or move around at home, upper limb deficits affect me all the time. I also have bulbar deficits – mild choking and slurred speech at times. At the lower end of the severity scale but at times very awkward.So though I'm not on the lower limb, then upper limb deficits, trajectory, I would say protecting upper limb and bulbar function is something worth doing for all.
In 1978, I was diagnosed with MS, by Professor Macdonald. A fellow patient in the ward was paralysed from the neck down, unable to lift her head unless a nurse did it for her. Another patient unaware of my condition sat on my bed and said "that's what happens to people with MS". It's really tragic that nearly forty years on, progressive patients attend a hospital and nothing can be done.
"It is my mission to make sure when we take our new neuroprotective treatment strategies forward we don't exclude MSers with progressive disease who are wheelchair users."Whilst I am, on the one hand, delighted to read this, on the other, I am absolutely appalled that anyone could think any differently. Because my legs don't work too well, I would be consigned to the "too late" pile? It's true that my life is not what I wanted it to be 15 years or so ago. If I had known in my early twenties that things would be the way they are now, I would have been absolutely mortified. But I still have much joy in life, a lot of independence, many of my skills, and an awful lot to lose. And I am not the only one, I know.
Re: "I am absolutely appalled that anyone could think any differently. Because my legs don't work too well, I would be consigned to the "too late" pile?"I have participated in about 6 progressive trials and all of them require the participants to have an EDSS lower than 7.0 (wheelchair). In other words they need to be mobile.