Addressing The Diagnostic Void Faced By Patients With Deteriorative Symptoms

[Evergreen]

Just to be clear, the diagnostic void in the title of my thread was aimed at permanent deterioraters - patients who get worse from a known trigger (such as exercise) and never recover. It is rarely mentioned in ME advocate publications and I have never met a medical professional who has believed me when I say I am permanently damaged by exercise. My oldest permanent deterioration was triggered 18 years ago.

Gotcha. For me, "permanent deteriorater" conjures up someone who is constantly getting worse, rather than someone who got worse after a trigger and never recovered from that, but I understand you, and the need to be able to describe the experience.

I think there are a few reasons why that phrase might rankle health professionals. One is "permanent" - since you don't really know if something is permanent until the end of your life, I think they'd prefer "long-term", or at least some kind of hedging like "a deterioration which, having lasted 18 years, seems to be permanent".

Another is the word "damage". The phrase "permanent damage" is usually used for structural damage e.g. to the brain or spinal cord from a traumatic injury, where, at a certain point, you can say with a great degree of certainty that the damage is permanent because you can see that something is severed or that tissue is dead. Without that structural damage, they're not going to be keen on using the term "permanent".

Yet another reason is the idea that exercise can do harm - just totally alien to most healthcare professionals. So when you put all those together, they just think you're claiming things that aren't true. But they might be more open to a narrative that explains the same thing without using words that mean different things to them e.g. "I did graded exercise therapy in 2007. My illness got significantly worse during that time and I have never regained my previous level of functioning." They might still find it hard to believe, but they're not going to dismiss it outright...as often. I found that just calmly explaining how it happened, so listing off each step down and its trigger, without saying X caused Y, just saying "I did X, then Y happened", they kind of had to accept it.

I think it would be beneficial if ME had such distinctions, the ME Association talk about ME being fluctuating with relapsing / remitting ME, there should also be a name for permanently deteriorative ME.

A label for people who deteriorated after a trigger and never returned to their previous level of health is tricky, because both people with fluctuating ME and those whose ME constantly gets worse could be in that group. People with relapsing-remitting ME couldn't be in that group because they have periods of being completely well. Though if they went from being fully recovered back to relapsing-remitting after a trigger you could argue that they qualify in the sense that the stability of their health has thus far irreparably change. So I think the label would be an add-on rather than an alternative.

It's the duration not the size of the trigger, again it's an arbitrary cut off just like some criteria say you need to have symptoms for 6 months to get a diagnosis of ME. Until medical knowledge improves, it's the best we can do.

Understood.

Something that strikes me as we talk about this is that most people's ME/CFS is triggered, e.g. by an infection, after which their health jumps down and stays down to a greater or lesser extent (putting aside those who recover). In that sense, anyone with ongoing ME/CFS is a "permanent deteriorator". So when we have a deterioration or relapse or even just a short-term flare triggered by one of the usual offenders - infection, exertion etc - it seems like the core pathology of ME/CFS is in play. Whatever it is that causes the ME/CFS process to begin, seems to happen again. And for people who don't improve from that worse state, there may be a bit of extra pathology going on. (Certainly from my experience, it's not for lack of trying to slowly build back up.)

 

[Evergreen]

I usually tell mild/moderate patients that wonder if X caused them PEM, that they should look at the very severe and see what they can’t tolerate. If it can harm someone that’s very severe, it can harm you if you cross your individual threshold.

I agree. Things that were mystifying when I was mild are very clear now that I'm severe!

 

[Chestnut tree]

The void exists for a reason imho; to keep the BPS train going. Because how can you rehabilitate patients that not only cannot exercise (because bedbound) but are also (almost) unable to communicate.

So exclude the severe, even the moderate and only treat the mild and include people who do not have ME at all, and voila treatment succes.

I think this should be priority in research and advocacy, to include this category of ME.

 

[MrMagoo]

Yes. It’s really powerful to simply acknowledge.
And the contrast shows the damage that the erasure or ignorance of very severe can do, in that constantly at every sentence of the webpage, it will give people the impression the person can do more than they can actually can.

And for mild and moderate level people (and their dr, carers etc) it’s useful for them to understand that such a deterioration is possible.

 

[JemPD]

This is really powerful. Severe and Very Severe seem to be farmed out to their own special leaflets. Including them in this way is easy to do.

I agree. I'm not as bad as some on this thread for sure, but it is still difficult when all the leaflets make it sound like I can do loads more than i can. Then i have to start explaining that the one line nod to the '25%' who seem just shunted off into a corner somewhere - thats me & then having to start all over again to try to convince them what my experience is actually like.

And for mild and moderate level people (and their dr, carers etc) it’s useful for them to understand that such a deterioration is possible.

Absolutely. The times i see people who're wandering about - walking, driving, socialising, who really believe they have 'severe ME'. I once saw someone who was regularly competing at equestrian events - horse riding, who insisted she had it very severely 'her dr had told her so'!

Also it's so hard to convey my sense of emergency when life forces me to massively overdo it in a prolonged way. The people around me just dont get that this could be potentially the time i dont recover back to where i was the week before. They all think it's so benign "oh you'll be exhausted after that i know".

No, you dont "know". They have no idea.

It is a good point you're making Digital Drifter, everyone.
And Yann04 I love your rewordings here

I think it can be not too difficult.

Ie. if I take the CDC page. A couple example rephrasings to make it more inclusive.


Can become
People with ME/CFS often report their symptoms get worse when they are standing or sitting upright. Some people with [very] severe ME/CFS cannot stand or sit at all.


Can become

• Professional counseling. Talking about how to cope with the illness and its impact on daily life may help some people. Some patients may not have the energy to do this, and very severe patients may not be able to communicate at all.

 

[DigitalDrifter]

Lots of people eg MEA and NHS materials use the term relapse for deteriorations that last a long time

https://www.nhs.uk/conditions/chronic-fatigue-syndrome-cfs/symptoms/

PEM can happen hours or days after the triggering activity, and it can take weeks to recover.

 

[Evergreen]

https://www.nhs.uk/conditions/chronic-fatigue-syndrome-cfs/symptoms/

Yeah, so there are two relevant bits there:

[In the PEM section:] PEM can happen hours or days after the triggering activity, and it can take weeks to recover.

[In the severity section:] There may be times when your symptoms get worse. These periods are known as relapses.

If we stick with their terms (since anything else is swimming upstream), I think this would be worth creating a separate relapse/deterioration section, starting with their sentence:

Relapse and deterioration
There may be times when your symptoms get worse. These periods are known as relapses. A relapse can happen after a trigger, such as attempting to increase activity or exposure to stimuli, but it can also happen without a clearly identifiable trigger. In time, many relapses allow a partial or full return to the pre-relapse level of ME/CFS.

Other relapses last, and the person remains at a lower level of functioning long-term: their ME/CFS has deteriorated. Some people's ME/CFS gets progressively worse over time when one relapse follows another: their ME/CFS is deteriorating.

And the following could be added to the PEM section:

If PEM lasts for longer, then it is considered a relapse. See relapse and deterioration section below.

What do you think?

 

[Peter T]

I think this would be worth creating a separate relapse/deterioration section

I wonder if there needs to be a separate ‘prognosis and course of the condition’ fact sheet in addition to raising these issues under PEM.

 

[Yann04]

And the following could be added to the PEM section:
What do you think?

“relapse” has very different implications than deterioration. I don’t think it’s an adequate word to describe permanent deterioration.
And the “re” implies that the person had been at that level of deterioration before and then gotten better, so it really doesn’t capture the essence of permanent worsenings.

 

[Peter T]

“relapse” has very different implications than deterioration. I don’t think it’s an adequate word to describe permanent deterioration.
And the “re” implies that the person had been at that level of deterioration before and then gotten better, so it really doesn’t capture the essence of permanent worsenings.

For me ‘relapse’ doesn’t necessarily imply there will be subsequent remission. Further ‘relapsing and remitting’ is used to describe one course of progression in MS and is generally understood, though there are ups and downs, to indicate an overall downward trend in the disease.

 

[Yann04]

For me ‘relapse’ doesn’t necessarily imply there will be subsequent remission. Further ‘relapsing and remitting’ is used to describe one course of progression in MS and is generally understood, though there are ups and downs, to indicate an overall downward trend in the disease.

Wouldn’t remitting imply having periods with minimal symptoms and near normal functioning?

 

[Peter T]

Wouldn’t remitting imply having periods with minimal symptoms and near normal functioning?

Though it might seem the literal meaning of the words, in MS it is generally understood that there are periods of getting worse then improving but also overall there is likely to be an overall downward trend, such that each relapse is likely to be overall to a lower level of functioning than previously and each remission involves less subsequent recovery than previous remissions.

It may be in an ME context we can not be certain that our readers would understand the words in the same way that people familiar with MS would. So it maybe if we used them we would also need to clarify further.

 

[Evergreen]

I wonder if there needs to be a separate ‘prognosis and course of the condition’ fact sheet in addition to raising these issues under PEM.

Yes but what on earth would it say?! I don't think we have a clue because no-one has followed us properly over time. There are a few studies showing that at any given time about 60% say their illness is fluctuating, about 10% relapsing-remitting (where they have periods they feel healthy), about 15% constantly getting worse and the remainder constantly getting better. But that doesn't tell us much about how things pan out over the long term. I think the MEA always says most people improve over time, but I don't know what a proper study would say.

 

[Evergreen]

“relapse” has very different implications than deterioration. I don’t think it’s an adequate word to describe permanent deterioration.

Yeah, it's tricky, because I'm trying to start from their terminology, to come up with something that would have a hope of being accepted. I didn't want to use the term "deterioration" in the "PEM" section, because I felt that minimised it more, and it was better to point to the relapse and deterioration section and do it there, but maybe that was the wrong choice. Would this be better:

If PEM lasts for longer than days or weeks, then it is considered a relapse. If the post-exertional worsening does not abate long-term, it is called a deterioration. See relapse and deterioration section below.

And the “re” implies that the person had been at that level of deterioration before and then gotten better, so it really doesn’t capture the essence of permanent worsenings.

Well the term I'm proposing using for long-term worsenings is deterioration, but maybe that's not clear from the draft above? Maybe someone can propose wording that works better.

What's tricky is that in my experience, the only difference between PEM and a relapse and a deterioration is whether it lifts or not and how long that takes. When I was in my 10 years of deterioration, I still had some episodes of PEM that lifted. I'm not sure I could say I had relapses that lifted because the deteriorations came every 3-6 months.

It's a bit like the difference between post-viral fatigue syndrome and ME/CFS. If post-viral fatigue syndrome lasts, then it's ME/CFS. Anyone with post-infectious ME/CFS could be said to have post-infectious fatigue syndrome instead, but at a certain point it gets a bit daft to have the trigger in the name. Now, the idea is to diagnose ME/CFS at 3 months, which means there's going to a be a lot of people recovering, because plenty of people have post-viral fatigue that lifts over the course of a year or two.

 

[Evergreen]

The 2021 NICE guidelines say this:

Tell people with ME/CFS that:
• they are likely to be having a flare-up if they experience a worsening of their symptoms beyond their normal day-to-day variation, which lasts a few days
• a relapse is when there is a sustained and marked exacerbation of ME/CFS symptoms lasting longer than a flare-up and needing substantial and sustained adjustment of energy management.

And I think there's wisdom in advising that anything lasting more than a few days requires adjustment of what you're doing.

The NICE guidelines define PEM and relapse as follows:

Post-exertional malaise
The worsening of symptoms that can follow minimal cognitive, physical, emotional or social activity, or activity that could previously be tolerated. Symptoms can typically worsen 12 to 48 hours after activity and last for days or even weeks, sometimes leading to a relapse. Post-exertional malaise may also be referred to as post-exertional symptom exacerbation.

Relapse
A sustained and marked exacerbation of symptoms lasting longer than a flare-up and needing a substantial and sustained adjustment to the person's energy management. It may not be clear in the early stages of a symptom exacerbation whether it is a flare-up or a relapse. Relapses can lead to a long-term reduction in the person's energy limits. [my underlining]

I agree with them there, during my 10 years of deterioration, there was no way of telling at the beginning how long any particular worsening was going to last - ie whether I was at the beginning of a step down that would last.

It's good that they acknowledge that last bit re long-term reduction, but it's not just in energy limits. I would prefer "long-term reduction in the person's functioning".

 

[Peter T]

What's tricky is that in my experience, the only difference between PEM and a relapse and a deterioration is whether it lifts or not and how long that takes. When I was in my 10 years of deterioration, I still had some episodes of PEM that lifted. I'm not sure I could say I had relapses that lifted because the deteriorations came every 3-6 months.

I would say that PEM necessarily involves some deterioration but deterioration can occur independently of PEM, such as following a new viral infection.

In my mind I see two cycles of functioning, one relating to shorter term variation within individuals’ current activity range and the other longer term in what I regard as the underlying ME. My hypothetical underlying ME that determines the current activity ceiling and activity floor below which you can avoid triggering PEM has subjective meaning for me but is unevidenced.

It is confusing that too much PEM can worsen the underlying condition, but it can also change with infections and for reasons that are not currently obvious.

 

[Evergreen]

I would say that PEM necessarily involves some deterioration but deterioration can occur independently of PEM, such as following a new viral infection.

In my mind I see two cycles of functioning, one relating to shorter term variation within individuals’ current activity range and the other longer term in what I regard as the underlying ME. My hypothetical underlying ME that determines the current activity ceiling and activity floor below which you can avoid triggering PEM has subjective meaning for me but is unevidenced.

It is confusing that too much PEM can worsen the underlying condition, but it can also change with infections and for reasons that are not currently obvious.

Yes, I'm with you on that notion of "underlying ME". And yes, there are triggers other than exertion for those changes to the underlying condition - noise being a big one for @DigitalDrifter and me, for example.

 

[Evergreen]

There are a few studies showing that at any given time about 60% say their illness is fluctuating, about 10% relapsing-remitting (where they have periods they feel healthy), about 15% constantly getting worse and the remainder constantly getting better. But that doesn't tell us much about how things pan out over the long term.

Thought it would be helpful to have a record here of the detail of those studies. I put line breaks in to make these quotes a bit easier to read:

Bretherick et al. 2023 (DecodeME)
https://pubmed.ncbi.nlm.nih.gov/37881452/

Most participants (58.0%) indicated that their ME/CFS is “Fluctuating (my symptoms vary day to day but don’t go away)”,
12.7% describe their symptoms as “Relapsing and remitting (good periods with no symptoms alternating with symptomatically bad periods)” and
15.3% indicate their symptoms are “Getting worse”, similar to previous research15.

Chu et al. 2019
https://www.frontiersin.org/journals/pediatrics/articles/10.3389/fped.2019.00012/full

For the overwhelming majority of patients (96%, n = 141), their illness did not improve with time although different patterns of illness were seen:
14% of subjects believed their illness was constantly worsening;
7%, relapsing-remitting (all symptoms might disappear for a time only to return);
8%, persisting with little change;
59%, fluctuating (symptoms could change in severity but were always present) and
7%, “other” pattern, although the most common response here was analogous to the “fluctuating” pattern with some symptoms worsening while others improved over time.
Thirteen percent of subjects reported that they experienced remissions (i.e., no symptoms) of more than a month during their years of illness.
The median duration of remission was 7 months with the range being from 1.5 to 240 months.

Stoothoff et al. 2017
https://symbiosisonlinepublishing.c...stics/biosensors-biomarkers-diagnostics13.php

Most the sample (59.7%), described the course of their illness as Fluctuating, with symptoms periodically alternating between getting better and getting worse but never disappearing completely.
Constantly Getting Worse comprised the second largest group, representing 15.9% of the sample.
Similarly, 14.1% of the sample described their illness as Persisting, which was characterized by having no changes in symptomatology.
The Relapsing and Remitting group represented 8.5% of the total sample, and was defined by having “good” periods with no symptoms alternating with symptomatically “bad” periods.
The remaining 1.9% of the sample described their illness as Constantly Getting Better.

 

[Yann04]

Thought it would be helpful to have a record here of the detail of those studies. I put line breaks in to make these quotes a bit easier to read:

Bretherick et al. 2023 (DecodeME)
https://pubmed.ncbi.nlm.nih.gov/37881452/


Chu et al. 2019
https://www.frontiersin.org/journals/pediatrics/articles/10.3389/fped.2019.00012/full


Stoothoff et al. 2017
https://symbiosisonlinepublishing.c...stics/biosensors-biomarkers-diagnostics13.php

Worth noting that all these will have selection biases leaving out yhe most severe, who are also probably more likey to say they have a deteriorative pattern.

 

[Kitty]

Wouldn’t remitting imply having periods with minimal symptoms and near normal functioning?

No, not always.

Take MS for instance. The relapsing/remitting group includes people who're on a downward trajectory, where every relapse is a step down the ladder. Some may have minimal progression for years, then suddenly have a big flare that bumps them down three or four rungs. It's very unpredictable, but it's a different pattern from people with continual deterioration.

Remitting doesn't always imply normal function. It can mean significantly impaired, but less so than in an active flare (a friend with MS is currently in remission, but she still needs care support). It's partly about treatment/medication needs, where people can manage on maintenance therapy in remissions, but may have additional treatment or care needs in relapses. It's an elastic definition because it's used in all kinds of conditions that have periods of relative quiescence interspersed with more active ones.

One of the things we need to know about ME/CFS is what these patterns are like over whole lifetimes, and how many patterns there are. At the moment nobody is following people long term, the way they do in the hospital rheumatology department that treats my psoriatic arthritis. They develop huge experience of disease patterns and courses because they follow people all the way from initial onset.