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
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.
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.
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?”
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.|
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.
Planning 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.
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.
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,