Late-stage Multiple Sclerosis

Late-stage Multiple Sclerosis

You may find this conversation unsettling, so please decide for yourself if you want to read on. I am discussing the worst case scenarios for people with aggressive, progressive MS, and what may be referred to as late-stage MS. This is in response to the recent MultipleSclerosis.net MS in America survey and what the respondents asked for in additional information.

When I began writing about multiple sclerosis, a common question from the newly diagnosed had the theme of ‘am I going to die from my MS?” I would answer quite earnestly – MS won’t kills us. The life expectancy for someone with multiple sclerosis is very similar to the general population and the leading cause of death for people with MS is heart disease, cancer and stroke, according to the MS Foundation.  They point out that MS affects the quality of life but not the quantity.1

Rethinking my answers

I was challenged by more reading, research, and some other people with multiple sclerosis to rethink my answer. While MS technically is not a killer by itself (except in rare circumstances), it can cause enough problems and symptoms with complications that can be fatal, and multiple sclerosis can at the very least be named an accomplice in death for some people.

Expanded Disability Severity Scale (EDSS)

The majority of people with MS will live a life about the same length as their peers, but there is the small percentage of people, estimated at 10-15%, who will have a very aggressive form with a high degree of disability.2 This is the face of multiple sclerosis that is rarely talked about – the people with MS who are on the far end of the Expanded Disability Severity Scale (EDSS), who are totally dependent on others and are facing certain death from complications of this disease.

EDSS ratings

If you aren’t familiar with the EDSS, those are the numbers used by our neurologists to rate our level of disability. Simplified, the numbers on the EDSS look like this –

0 = Normal

1-1.5 = No visible disability, but some abnormal neurological signs

2–2.5 = Minimal disability

3–4.5 = Moderate disability, affecting daily activities, but still not severe

5–8 = Increased disability where the person adjusts their routines because of the MS, uses a cane, walker or wheelchair at least some of the time.

8.5–9.5 = Very disabled, bedridden

10 = Death

It is primarily the people rated 0 through 6 or so that we see and talk about. We slip in some occasional mentions of the problems of people with an EDSS of 6-7, but we are pretty much silent about the 8.0-9.5 folks. The 10’s are only mentioned in obituaries unless they are famous and get a headline about their death. Wheelchair Kamikazee, Marc Stecker, shares his intimate thoughts and fears through his writing in Fear Factor as he ponders “What demonoid could come up with such a disease, a fiendish thing that forces you to watch yourself disappear but then doesn’t have the good manners to finish you off?”

The explanation of 8-10

A more complete description of the Kurtzke EDSS can be found at many places on the web, including a thorough explanation at the Veteran Administration’s  Multiple Sclerosis Centers of Excellence, but the following is their explanation of 8.0-10 on the EDSS.3

8.0 Essentially restricted to bed or chair or perambulated in wheelchair; but may be out of bed much of the day; retains many self-care functions; generally has effective use of arms.
8.5 Restricted to bed for much of the day; has some effective use of arm(s); retains some self-care functions.
9.0 Helpless bed patient; can communicate and eat.
9.5 Totally helpless bed patient; unable to communicate or effectively eat/swallow.
10 Death due to MS.

 

Common complications

MS is a disease of the central nervous system, and although it doesn’t directly cause death it can create problems that cause other forms of physical disability to create complications. Being in a wheelchair or bedridden can make the person more susceptible to infection, blood clots, and pneumonia. According to the NMSS common complications for people with advanced MS include:4

  • Pneumonia, especially aspiration pneumonia which happens when someone is choking and inhales food or liquid in the lungs
  • Pressure sores occur when people are limited in their positions and spend long periods of time in one position seated in a wheelchair or lying in bed. Pressure sores can become infected and very difficult to heal.
  • UTI’s – the dreaded urinary tract infections for most of us can become life-threatening for people who are not moving about as much. Their kidney and bladder infections can go unnoticed for long periods of time allowing infections to possibly spread through the blood stream.

Any of these problems can be fatal for someone who has health problems, but especially for a person who is already bedridden and medically vulnerable.

For an up close and often intimate and brutally honest look at what life is like for someone on the upper end of the scale and their caregivers, I highly recommend you read some of the articles by Donna Steigleder about her life with her husband Lynn, and his progression over the past few years. Donna writes on the challenges of fighting infections and keep Lynn healthy.

Preparing for the future

Another of the reasons we should talk about this part of MS is the need for people to have the opportunity to prepare for what may or may not come, through discussions with their family and others. This is a difficult subject but needs to be tackled head on. We often avoid discussing death, but it is inevitable for all of us, and whether we have a chronic disease or are in perfect health it is a conversation we should hold.

I would wager the majority of you reading this have not updated your wills, your medical power of attorney forms or your advance directives. I confess my forms are now 15 years old and written before I was diagnosed with MS. All of us need to prepare these important legal documents now and not wait until we are possibly incapacitated and unable to make our wishes known. This is important for everyone to do and not just people with progressive forms of MS.

Tough discussions need to be made to review the types of care that might be available and preferred, including hospitalization, hospice or at home care. “Everyone with MS has the right to make choices about the care they receive now, as well as in the future. People severely affected by MS might have trouble with this. Speech difficulties, fatigue or memory problems can make it hard for them to express their wishes,” according to the UK MS Society.5

Health care provider perspective

For a provider’s thoughts on late stage MS, I turned to my neurologist, Aaron L. Boster, MD, Systems Medical Chief of Neuroimmunology and Clinical Research of Ohio Health. Dr. Boster has a unique perspective for this discussion because not only does he treat people with MS, he had an uncle with progressive MS who fit the profile of this discussion.

He quickly points out that the use of disease modify therapies in the past 20 years of so has changed the statistics quoted above, and although he can’t give me a specific number, he believes there are now fewer people with MS who reach the EDSS mark of 8.5 or higher. Dr. Boster also points to studies that show physical and mental conditioning can be useful in slowing or stopping MS progression, except in the most dramatic instances. He acknowledges there is variability among individuals and not everyone can stave off progression through conditioning.

Slow shift in thinking

He is encouraged there is a slow shift in thinking toward the medical care of people with late stage MS and more people continue to be treated by a neurologist who understands multiple sclerosis. “People in nursing care still need to be followed by a neurologist because there are medical care needs which are specific to MS and those needs don’t fit the profile of the typical nursing home patient,’ said Dr. Boster. The advantage to this is the MS doctor understands the management and treatment of symptoms, and even if the person no longer benefits from taking a disease modifying therapy there may be other symptom treatments that improve the quality of life and offer palliative care.

When asked how he counsels people and their caregivers facing late stage MS, Dr. Boster says the physician must hold the difficult conversation of explaining what might happen, and then support the person in the ways they need for the quality of their life. He reflects that doctors are trained to heal people and make them better, but the reality is that is not always possible. There comes a time in late stage MS when the “goal of medical care shifts from how to help a person improve medically to how to assist the person to live to the end of their life with respect and dignity,” said Dr. Boster.

Conclusion

People might question what they did wrong to have advanced MS while so many others have a milder course of the disease, according to the NMSS. This site reminds us that there is nothing in particular we did, but it is the nature of multiple sclerosis to continue to advance, and it affects each of us differently.6 The difficult condition of late stage MS punctuates the need for accelerating the research of SPMS and PPMS. The vast majority of us will not face 8.5 or greater on the EDSS scale, but it is still important to support and understand what happens for the people in our MS community who unfortunately reach this point.

Wishing you well,

Laura

This article represents the opinions, thoughts, and experiences of the author; none of this content has been paid for by any advertiser. The MultipleSclerosis.net team does not recommend or endorse any products or treatments discussed herein. Learn more about how we maintain editorial integrity here.
View References
  1. Is multiple sclerosis contagious or fatal?. Multiple Sclerosis Foundation. 2009.
  2. Severely affected by MS. MS Society UK.
  3. Tarver M. Quantifying the Level of Disability. MS Centers of Excellence. 2009.
  4. Living with Advanced MS. National Multiple Sclerosis Society.
  5. Planning End of Life Care. MS Society UK. 2014.
  6. Living with Advanced MS. National Multiple Sclerosis Society.

Comments

View Comments (10)
  • Andrealee
    2 years ago

    My mom is at a 9 on the EDSS. She was diagnosed with MS at around 55 and is now 72. She has been in nursing home care for the past 2 years after my father was unable to care for her. She became incapable of using the bathroom and would fall frequently. Now, she is completely wheelchair bound and unable to feed herself. She has had multiple choking incidences and is a “high maintenance” resident.

    As distressing as the physical limitations have been, the most marked transformation over the past 17 years has been the mental decline. While she knows who I am, she has very little short term memory and cannot recall my visit an hour after it’s passed.

    I feel as though my “mom” died years ago and I’m left with this shell that slightly resembles her. I visit at the appropriate times, Mother’s Day, Christmas, her birthday, etc., but I feel that it’s meaningless as she has no memory of the visit. The only thing that keeps me returning is the way her face lights up when she first sees me. It doesn’t take long, however, for the lethargy/torpor to reappear and she’s gone.

    One thing I’m grateful for, however, is that the time she’s been in the nursing home being cared for by trained people has given my father the opportunity to regain his affection for her. When she was first admitted, he was very critical of the staff and felt that no one took care of her as well as he did. What he didn’t realize was his “care” was filled with resentment and anger and fear. He’s the type that never asks for help and didn’t want “strangers” in his home, so he’d become sleep deprived and depressed trying to do the job himself.

    Now I’ve been able to see the devotion and care he provides her on a daily basis. He visits the home every day and feeds her lunch. He fought to keep her on a solid diet after she choked because he knew she wouldn’t enjoy the soft diet. I’ve had a chance to see the love again that’s helped them stay married for 53 years, and while I curse the fates that have burdened her with this disease, I’m thankful I’ve witnessed the better parts of my dad as he helps her live with it.

  • TheOutlier
    2 years ago

    I am a PPMS patient in late-stage: typical story, a tingle in my hand 20 years ago is now full body spasms, intense pain, complete loss of mobility, and mental confusion UNLESS I use massive amounts of cannabis, neurontin, and ativan at which point I can hobble around the house using a walker and life is good. I’m having a late night pain jag so you guys get to suffer:

    First- if I go to a doctor or to the hospital (frequent flyer) while ‘medicated’ and without full MS symptoms, they act like I’m a med-seeking druggie and don’t acknowledge my MS, some call me med seeking. I’ve taken to going off of my meds when meeting new docs, and going ‘full twitch.’ Does it have to be this way? I had a positive spinal tap and an MRI showing massive white brain lesions and was still kicked out of the Philly “MS Clinic” and told I was just med seeking. Compassion. It’s wonderful.

    Secondly: how to I rate myself on the EDSS? Do I rate my ultra-medicated self? Do I skip my medication if this is being measured? I ask because I had to fight like a brave to try to get a powered wheelchair even though often I can’t walk more than a few feet and my arms are not strong enough to push my manual chair. Still don’t have one. Do I sound bitter? Nah. I just haven’t gone anywhere in a decade. No biggie.

    It’s not fun hearing “You look fine” when just beneath the surface are the ‘earthquake pills’ ready to kick in and massive pain in every muscle of the body. Do I have to go off of my meds to get care? BTW: it’s amazing how many tiny muscles the body has that can cause you massive pain and twitchiness. The craziest are the muscles under my shoulder blades. Feels like I’m trying to flap wings.

    Last question/comment: MS made it to my lungs a couple of months ago and from what I’m reading this is not good. I want to let anyone else out there going through this know that cannabis edibles and topical salves can help and have made me much more comfortable. As I am at end-stage I am going to try a regimen of Rick Simpson Oil, which is most often used for cancer patients, but has helped me before. I probably won’t write here much after this, my mental processes are kind of foggy and this is difficult.

  • north-star
    4 years ago

    One thing we can do is educate people about MS. I had a friend who had a virulent case of PPMS and died. Did she use less effective therapy than I did? Did she give up and let MS take over? Should she have modified her diet? What about supplements? If she had prayed harder? No, no, no and no.

    It has been surprising that people are interested and that my words can help expand their view of this hydra headed monster, MS. This is a culture that often sees disease as failure you could prevent. I think we need to be ambassadors who stick up for each other.

  • Joan
    4 years ago

    My daughter was diagnosed in 1997 (at the age of 20), although she may have had MS all her life (only a mother can say this). It must be in the family DNA because my sister, also, has it, but she was diagnosed in her mid-40s.

    I don’t need a neurologist to tell me that my daughter is a 9.0 now on the EDSS scale. What strikes me strange is that, in spite of her severe disabilities, her mind is sharp and her memory is amazing. Her last symptom and challenge is her speech which has become very difficult for her. She is 38 now, and for the past 18 years, one by one, her physical challenges have increased, in spite of years of MS drugs. She is following a particular protocol of antibiotics (this is espoused by many microbiologists who believe that the Cpn bacterium causes MS and these drugs can and will destroy it). The bacterium is called “Chlamydophila pneumoniae”, and it may take years to erase it from one’s body. My daughter believes that when she had bacterial pneumonia at age 2, she contracted this bacterium. During her childhood, many things were making her sick or in pain, but at that time, doctors didn’t believe that children could have MS. Now, they know better.

    My daughter’s spirit is very strong, and she is very hopeful. She says that it may take some years before that bacterium is destroyed. Meanwhile, she, also, takes all kinds of supplements, vitamins, and only eats organic, gluten-free foods. She has put other restrictions on her diet as well. When you can’t see, use your hands due to tremors, stand up, and have difficulty being understood when you speak, your spirit must be amazing to transcend such a reality. This is not to say that she doesn’t get panic attacks, from time to time, or experience situational depression. MS has, also, caused her a gag reflex when she eats (this is rather recent, and it’s important that she doesn’t choke, etc. which can lead to pneumonia and/or death.

  • Shannon
    4 years ago

    My mom is almost 60 and is a 9 on the scale. She is still able to communicate verbally but that has worsened quite a bit in recent years and I fear the day she may not be able to speak at all. Like your daughter Joan, my moms brain is still sharp and she has an amazing memory, especially with things from when she was much younger. I also feel there is some kind of genetic link as my great aunt had very severe MS as well.
    My mom has been in a nursing home since her late 40s due to the severe and fast progression of the disease; our last visit to a neurologist was about 10 years ago when he told us there was nothing else that could be done to help her. She’s suffered many infections over the years and now has chronic osteomyelitis as well. I didn’t realize there could be benefits to having an Ms doctor at this later stage but this article made me think that perhaps we should explore that. I hate that so many other people suffering to this extent from the disease. 10% is small but in terms of numbers of people it is a lot. It’s comforting and educational to hear from others dealing with severe ms as well, as the article said we so rarely hear these stories.

  • Bob
    4 years ago

    I have had Multiple Sclerosis since 1993. First my feet tingled, then I could feel the ground I walked on, after that I couldn’t run anymore. Now my lower back tingles, as this terrible non-feeling keeps heading up to my brain and one by one things keep not working anymore I feel it’s interesting to relate how M.S. will not kill you. I tend to disagree. It is killing me in particular just a little more every day. When I stop breathing or my heart stops beating people will say ‘Oh, it was heart failure’, but was it?
    When Christopher Reeve Died, did his heart just stop because the muscles were not getting an electrical signal or was the muscles so weak from never getting exercise that there were no muscles left. With M.S the muscle is fine there just isn’t any signal. M.S is a killer disease. I think if health community treated it as such there would be more effort to find a cure.

  • Joan
    4 years ago

    Bob, I agree with you. For too long, the medical establishment assigns an “orphan disease” category to MS. Unlike Aids, Cancer, or Diabetes, MS is relegated to the backseat for funding. It makes me angry when I read in MS journals & magazines that only 400,000 Americans suffer from MS. Each time I see this, I refute it, immediately. MS researchers have been working on remyelination for too long, now. They proved that it works in rats, so what’s taking them so long with their clinical trials, etc.? Surely, out of compassion, they can make treatments available to progressive MS patients.

  • Donna Steigleder moderator
    4 years ago

    Laura,
    Excellent summary of end-stage MS. Lynn is probably a 9.0 on the scale based on the definitions; however, I don’t consider him end-stage today. Today, he is sitting in his power chair, dictating to his assistant edits on his new book. Fortunately, his memory and cognition is as strong as ever and so he has hope and a purpose. However, recently his fatigue and his “just feeling like crap” has been a constant drain on his state of mind. Those are the tough times. His father died at 49 of progressive MS so his “sees” himself in that state whenever things get tough and it’s hard to stay hopeful. It’s just a day by day struggle. Thanks for the shout out on my articles! and thanks for your thorough review of this subject. Donna

  • Sue
    4 years ago

    I have descended to an 8 recently. 6 years ago I worked full time, no cane. I have availed myself of the most aggressive treatments and physical therapy. I sit in my power chair and worry about my “good health “. No other problems.
    Getting from the bed to the chair or out of the shower chair to the bed are dangerous challenges. Pulling up my pants with one hand could take five minutes. My brother had an even more aggressive case of m s and couldn’t hold a fork or glass. It took about 6 years for him to succumb to pneumonia. When you start falling into this hole, with no way to be pulled out, why can’t I choose to stop, legally and peacefully. The states which allow medical assisted suicide require that the patient be near death. Why should a sane person have to be bedridden and catch pneumonia?

  • Joan
    4 years ago

    You’re so right, Sue. My state of California is trying to get passed an Option-to-Die bill, but if it passes, it still would require a doctor to say that a terminally ill patient only has 6 months to live. Thes e doctors just don’t understand the slow torture of a patient with a degenerative and progressive disease.

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