Tuesday, 26 July 2016

Progressive dwindling

PLoS One. 2016 Jul 21;11(7):e0159210. doi: 10.1371/journal.pone.0159210.

Progressive Dwindling in Multiple Sclerosis: An Opportunity to Improve Care.

Martin JE, Raffel J, Nicholas R.

Abstract

INTRODUCTION:

In the general ageing population, 40% of deaths occur following a prolonged trajectory of "progressive dwindling," characterised by chronic accumulation of disability and frailty, and associated with increased dependency and reduced reserves. Those who progressively dwindle are poorly catered for by current healthcare systems and would benefit from a coordinated approach to their medical and social care, known as formative care. People with multiple sclerosis (pwMS) may be more likely to progressively dwindle, and may be appropriate targets for formative care pathways.

OBJECTIVES:

To determine the proportion of pwMS who follow a progressive dwindling trajectory prior to death. To relate trajectory to place of death, and examine what factors predict the progressively dwindling trajectory.

METHODS:

A retrospective observational study of 582 deceased pwMS enrolled in the UK MS Tissue Bank, including death certificates and extensive clinical summaries.

RESULTS:

73.7% of pwMS had a "progressively dwindling" trajectory of dying. This was predicted by those who reach MS disease milestones earlier. 72.5% of pwMS died an MS-related death, which was predicted by an aggressive disease course from onset. Those who progressively dwindled were equally likely to die in hospital as those with other trajectories to death.

CONCLUSIONS:

The progressively dwindling trajectory of dying is very common in pwMS, and can be predicted by earlier disease milestones. Pathways could target pwMS in these years prior to death, to improve care.


 Tipping point

This article brings to the surface the problem with MS research - an enterprise focused on basic science research, or what I call pop science that ignores the immediate needs of our patients. Moreover, frustratingly, what we preach in public is very different from our private thoughts. There is very little impetus to work towards finding solutions for progressive disease. You note, that I don't mention cure. We should be aiming towards the tipping point; the small changes (Sisyphus effect) that have a big impact (snowball effect).

Progressive dwindling is the progressive chronic illness before death. Broadly speaking, in an aging population four broad trajectories of dying have been described (Bowman and Meyer 2004):
  • 20% sudden deaths e.g. heart attack
  • 20% follow a rapid decline e.g. cancer
  • 20% as a result of acute exacerbations of chronic disorders e.g. infective exacerbation of chronic obstructive airways disease.
  • 40% follow a prolonged period of 'progressive dwindling' e.g. Alzheimer's, progressive MS (Figure 1)
Figure 1: cause of death in 582 people with MS (UKMSTB - UK MS Tissue Bank Jan 1998-Feb 2015)

The care pathway for the latter 40% is haphazard at best, focused predominantly at the major centers with access to multidisciplinary teams. There is little in the way of formative care provision in order to improve quality of life. This need is more pressing in MS than any other neurological disorder as it spans most of adulthood.

Martin et al. point out that those who progressively dwindled had an earlier age of onset, progression (earlier achievement of disease milestones suggesting aggressive disease course) and were wheelchair bound.  Moreover, they were more likely to die in hospital (not at a hospice, as in cancers) than in their own residence, which may be interpreted as failure of care.

This research points to a disconnect between active treatment (DMTs) and end of life care in MS with progressive dwindling occurring for several years. This is a serious concern as this research points to at least 74% people with MS having this trajectory before death (NB: this figure may be biased as it is retrospectively collected, and is from a brain bank database). A more formative strategy is needed that can deal early with care planning and readily with complex care needs and increased rates of cognitive disability.

14 comments:

  1. Neuro Doc,
    Thank you for pointing this out.
    You are hitting the nail on the head.
    This has been a taboo subject for to long.

    ReplyDelete
  2. This is an important post.

    Prof G is so focussed on promoting early DMT usage that, as an advanced progressive MSer, I feel this blog fails to address the crisis in adult social services. I feel pretty ignored, yet I have such great needs. Britain has such negative attitudes and concerns when it comes to the disabled. It is like we don't exist.

    ReplyDelete
  3. Are there any statistics for life expecancy from various milestones?

    ReplyDelete
    Replies
    1. There is a relatively old article published in Neurology. 1992 May;42(5):991-4. Life expectancy in patients attending multiple sclerosis clinics. Sadovnick AD, Ebers GC, Wilson RW, Paty DW.

      Abstract

      One multiple sclerosis (MS) is diagnosed, important considerations often include life expectancy and the availability of life insurance. We designed a study specifically to examine life expectancy among MS clinic patients and analyzed the data using standard actuarial methods, both including and excluding suicides. The data show that severe MS disability, as measured by an Expanded Disability Status Score (EDSS) of greater than or equal to 7.5, is a major risk factor for death with case fatality ratios for this group of patients approaching 4 times the rate for controls. Conversely, excluding deaths by suicide, case fatality ratios for those with mild and moderate disability (EDSS less than or equal to 7.0) approach 1.4 times and 1.6 times for age- and sex-matched comparison groups. Life tables indicate that the overall life expectancy for MS is only about 6 to 7 years less than that for the "insured" population without MS.

      Delete
  4. I agree that this is a taboo subject. There are so many interesting (but profoundly depressing) research questions that could be looked at in this area. Does having a DMT make you less likely to die from progressive dwindling? Is there a difference in this area between PIRTs and other DMTs? Since confinement to a wheelchair is a necessary condition for progressive dwindling, is there anything that can be done to keep people with MS from ending up in a wheelchair? I can think of more - I'm sure you doctors can too. Do you have any Ph.D. students who want to look at these questions? Or do these questions suffer from the 'end of life care' curse?

    ReplyDelete
    Replies
    1. A project can be crowd funded, to tackle a specific question. This needs to come from you, the people involved, rather than the generic stuff which the researchers come up with. If we have a set of ideas, and the resource for this project, we can then set up an on line platform with the findings for the first question etc. then where to go from there, and then from there...

      Delete
  5. Very helpful post Neuro Doc. 14.8% of the patients died because of MS. How is it possible given that MS is not a life-threatening disease?

    ReplyDelete
    Replies
    1. Yep. Its not something you read about on the charity webpages.

      Delete
    2. Please see reply to Dale Collins above; the generally quoted figure is 6-7 years lower.

      Delete
  6. Progressive dwindling is just what happens when you have a progressive disease.

    Sure you can treat the side fires and have some success with those.
    but the main fire burns away with no direct treatment at all because
    no treatment exists. Someone has numb sensations..how do you treat and
    fix this..?

    ReplyDelete
  7. Unfortunately, this lack of attention and care of individuals with chronic, progressive, and terminally ill, seems to be so all over the world...
    More lack empathy, not only from the society itself, but the state itself ...

    ReplyDelete
  8. A taboo subject not only ignored by charities charged with acting on behalf of MSers, but actively hidden from view.

    Much better to show inspirational stories of fund raisers climbing killimanjaro, or running a marathon draped in as much of the brand colour as is possible.

    Not good for business to acknowledge the truly devastating effect of progressive MS and to admit the slow progress in this area despite the millions of donations.

    If you're at a loose end play the 'count the wheelchair game' on the photos adorning the charity's glossy media outlets.
    You might be forgiven for thinking you've stumbled upon the website for the up and coming games in Rio.

    Regards as always.

    ReplyDelete
  9. You are so right.
    And when you address the subject with them they get very, very angry

    ReplyDelete
  10. It would be nice to know how many wheelchair bound people visit this blog.
    Or what the ratio waking/waking with a crane/wheelchair/bed is.

    ReplyDelete

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