The SEID Criteria: How do you like your porridge?

When I think of the title of this blog post, Goldilocks comes to mind. A criteria for a disease should strike a balance between sensitivity (not leaving out those who have the illness) and specificity (not including those who have something else). It should be just right.

But, what is just right with ME/CFS? What elements are needed in that porridge and at what temperature so that it is nutritional and pleasing?

Jennie Spotila did a pretty good job of assessing the new SEID criteria and some questions it raises. So I made a comment on her blog. She particularly addresses the fear patients have expressed that the criteria will mean depressed people will mistakenly be diagnosed with SEID (systemic exertion intolerance disease, the proposed new name for ME/CFS).

SEID criteria requires three things:
  • Loss of function accompanied with fatigue
  • Unrefreshing sleep
  • Post-exertional malaise
Then it requires at least one of the following: orthostatic intolerance or cognitive dysfunctions.


But what about the Canadian Consensus Criteria and Fukuda?
The CCC is complex, takes more time and includes many features and variables. It's attempt seems to be more comprehensive of what symptoms are seen in patients. And the CCC was published in a journal that is no longer in publication and which did not have a strong reputation in mainstream medicine. And it is not evidence based considering today's evidence because it is 12 years old. Updates have been attempted, but there are two and they are still complex.

The IoM was charged with determining a criteria that is evidence based (which is a higher threshold than most of studies in our disease) and up to date and so easy a primary care doctor, or any doctor, can use it confidently.

Fukuda is even more outdated and wasn't evidence based either. It was designed for research, not clinical use. But absent a medical professional organization of certified experts creating a clinical criteria, CDC has Fukuda on its website and is featured in its physician aid materials. It has only one requirement symptom and many optional ones. A fibromyalgia person could meet the criteria; depressed people meet it and so do some MS people. Of course, the doctor is supposed to screen those out first. But that is a problem in clinical use because a person could (and very likely does) have both ME/CFS and fibromyalgia. So there's a lot of people who have both but only have the fibromyalgia diagnosis. Fukuda does not help in distinguishing the two. I had one research doctor tell me, "Doesn't every fibromyalgia patient have chronic fatigue?" Well, yes. But does that mean every fibromyalgia patient has chronic fatigue syndrome, which is what I had asked about his research cohort? I would say no. But, if CFS is defined by Fukuda, then I would venture to say all fibromyalgia folks would meet the Fukuda CFS criteria. So it doesn't distinguish between the two.

And really, isn't that what a disease criteria is supposed to do? It's supposed to tell you how to distinguish which disease a person has. So the IoM committee took the approach of trying to find the disease features that are different in this disease from others. That is why they did not include myalgia or other pain. Just how is the pain of our disease different from other pain conditions? And is it the same kind of pain in each patient? Does each patient have pain? (I didn't for the first three years of the disease.) And if a patient has our disease with pain, could it be they actually have this disease and fibromyalgia? If so, is knowing the patient has pain helpful in distinguishing which disease they have?

Too loose or too tight?
I find it very interesting that many are concerned the SEID criteria is too loose and will include depression patients. I figured out that a person could meet the SEID criteria and not meet either the Fukuda or CCC. Because more symptoms are required for both of those than is required for SEID. That would seem to indicate that those two other criteria are more narrow.

But we know a person could have fatigue, muscle pain, memory problems, headaches and joint pain and meet the Fukuda criteria, even if they don't have PEM. So it's too broad. A person could meet Fukuda and not meet the SEID criteria. Fukuda makes optional a few things that SEID requires, including PEM. That would mean the SEID criteria is more narrow.

As for the CCC, it would seem the CCC is more narrow because it requires more. But, let's see what parts of CCC are not included in SEID to see if they should be required in a criteria for this disease. But first, here are symptoms they both require:
  • Fatigue
  • Post-exertional malaise
  • Unrefreshing sleep 
  • Symptoms for six months in adults
  • Loss of function (reduced activity)
I want to point out that while fatigue is required in SEID, the emphasis is on loss of function that is accompanied by fatigue. So loss of function is the symptom that is more distinguishing, not fatigue.

Symptoms required in CCC but optional or not included in SEID criteria:
  • Pain
  • Neurocognitive dysfunction (2 types)
  • Two symptoms that would fall under either immune system dysfunction, autonomic dysfunction or neuroendocrine dysfunction.
Do these additional requirements help distinguish this from other diseases? Well, as I discussed above, the pain in our disease is different in each of us, less common and not of a nature that a doctor can tell if the pain or headache you are having is different from pain and headaches of other diseases. So requiring pain does not help in diagnosing.

The neurocognitive is required in CCC but is optional in SEID. From what I've seen and heard experts say, they don't see the neurocognitive dysfunction as much in children or adolescents with the disease. So making it a requirement would mean children with the disease won't get the diagnosis. And, in the aim of making it easy to teach and easy to be used, they opted for one set of criteria to be applied to adults and adolescents. The other required parts of SEID criteria can identify pediatric cases without having to have two different criteria depending on age. Simpler, easier, and still effective.

Also, I have seen some patients not recognize that they have neurocognitive dysfunction. This is discussed in "Osler's Web." So if a doctor asked, the person might say they don't have that problem. I didn't notice it until 2.5 years into my disease. I started making mistakes in my typing, making mistakes in adding up the bank deposit or in balancing my checkbook, unable to remember a phone number long enough to dial it. But when is it normal brain glitches, stress-induced or a symptom of a disease? It's hard to tell in the beginning. So making it a required symptom when many children with the disease don't have that symptom and many patients don't recognize they have it when they do could hinder making the right diagnosis.

The neuroendocrine symptoms in CCC are temperature control problems, IBS, etc. All of these are also seen in other diseases. It doesn't help in distinguishing this disease from others.

The autonomic dysfunction is optional in SEID and CCC. I had this since I was 6, decades before I developed this disease. So it is a key feature for me. And in fact, a study that showed the high prevalence of orthostatic intolerance in CFS patients helped me know for sure I have CFS, when I developed the CFS symptoms later in life. The OI have caused fainting spells every couple of years when I was a child and two episodes as an adult. However, I went for a tilt table test last fall, and it showed normal. How could that be? Was it the French onion soup (fluid and salty) I had right before? Who knows. But I know if OI was a requirement for the disease as measured by a tilt table test, I would have not met the criteria at that moment. But that doesn't mean I don't have the disease. Maybe that's why IoM committee didn't make it required.

Many wanted immune system dysfunction to be a requirement. But immune system symptoms occur in far fewer of us, that is swollen lymph nodes and sore throats. I didn't get sore throats until year three, and it was very mild. And I don't have it now. I never had swollen lymph nodes. I do have low natural killer cell function, when tested by two experts. But I didn't have it when tested by an unknowledgeable internist that I asked to run the test. I suspect she ordered the wrong test or something. This goes to show that there is either a problem in how labs run the test (some labs) or doctors don't know how to order the right test. Plus, other illnesses will show the same abnormality. So, does it help in distinguishing this disease from others? Does requiring sore throats or swollen lymph nodes in the criteria help to distinguish this disease from others when many with this disease don't have it?

So what they did include in SEID is not meant to be comprehensive, but it is meant to be easy for the doctor to use in distinguishing this disease from others. I think it's not really helpful to ask if it is too loose or too narrow. The question is whether it can aid the doctor in identifying this disease in a patient and do so within the circumstances today's doctors, including primary doctors, must practice in. While testing it in a real, multiple-clinic fashion would tell us for sure if they met their goal. It's clear that was their goal. And that's why they did not include symptoms many of us experience but are not distinguishable from symptoms of other diseases or not found in all of us.

I would imagine that some feel their symptom cluster is the one of "the disease." Yet, 60 symptoms are seen in us, but not all in each of us. For example, Laura Hillenbrand's most debilitating symptom at times has been vertigo. It is a symptom many of us, including myself, have experienced. But is it constant in all of us? So should it be in the criteria? Is there something different about our vertigo so it would help in identifying this disease instead of others? No. Yet, when we do have it, it can be very debilitating and seem like a major factor of the disease impacting us.

I know the logical argument is that the combo of symptoms distinguishes this disease. So analyzing each one separately is not understanding the power of combined symptoms being able to distinguish this disease from others. And that is true. And the SEID is a combo of symptoms that when put together, they think distinguishes this disease. It's really a matter of how many symptoms to put in the combo? Requiring or having to sift through many optional ones is complex when, if you can get to which ones are at the core and are more unusual, you can require fewer and still have it work and be easier to use. That seems to be the goal.

Why does it need to be easy to use?  Because we want doctors to use it. The CCC is not used now in clinical practice, and I've been told doctors think it's too complex. Is it an excuse? Maybe. But let's give them something more like what they want. And then maybe they'll use it. At least we remove one reason they give for resistance.

The power is in the guide.
The Institute of Medicine ME/CFS Committee clinician's guide has come out. This is really the important literature that can educate the clinicians even more than the name and criteria. And as I hoped, it has those wonderful tables for each of the symptoms. The tables include words patients use to describe each symptom and the objective tests that show a biological abnormality that has been seen in some studies that could explain the symptom (except unrefreshing sleep, which has no objective test). These tables are helpful to the clinician and informs them of the true nature of the disease. I expect the clinicians will begin to see the pattern in what the patients say about their experience, as do the experts who have been focusing on our disease do. There are also charts to show how often patients complain of each symptom compared to healthy people. The guide includes immune system abnormal findings and says these might turn out to be diagnostic biomarkers after further study.

But, I wish the guide had a few tips on how to easily differentiate SEID from other conditions that at first glance may present with same or similar symptoms. A table that has these and then lists primary symptoms for each could easily show the distinctions. Many of those can be eliminated through tests for those conditions. But, as in depression, it's more the nature of the symptoms and other symptoms that help to differentiate it from SEID. A few key questions could help the doctor distinguish the two conditions. But, I still mostly fear doctors continuing to diagnose SEID folks with depression than I do depressed people being given a SEID diagnosis. SEID is not to be diagnosed until symptoms have been experienced for six months. Most clinicians will first look at easily treated conditions (including depression) as a possibility in those six months.

I know the argument that if you list some, you will miss others. But, there are a few diseases that are more commonly mistaken for our disease. I would love to see a guide on distinguishing SEID from those. One reason is not just to help diagnose, but it will also be helpful in educating about our disease by making distinctions as well as showing similarities. It could be done in a table and take no more than two pages, maybe one.

I wish they would have explained a bit more thoroughly about the time between "exertion" and the increase in symptoms. Experts and patients know it is sometimes a full two days between the two. So the correlation is not easily discerned, especially early in the disease.

Please rename PEM.
I don't understand much of the fear and criticism of the SEID criteria because from soooo many, I heard that PEM should be required; it is what defines our disease. So it's in there, as a requirement, just as patients said. But many still aren't happy.

One last thing about the criteria: I hate the term "post-exertional malaise." I sent in a comment to the IoM committee saying that. I wish they had taken this fresh-start opportunity to get that terminology right. They didn't and didn't explain why.

First, "post" only means afterward. It connects by sequence and does not indicate cause or timing.
"Exertional" is misleading because the trigger for the "crash" or "collapse," as we colloquially call it, can be activity that most people think of as ordinary, even sedentary, such as sitting at a desk for an hour, emptying the dishwasher, etc. "Exertion" is associated with lifting weights or running a marathon or opening a new jar. The trigger for the crash for us is not just exertion like that; it's normal activity. Plus, other stressors, such as an infection, psychological or emotional stress, surgery, an accident, and etc. create the same crash.
"Malaise" is no where near describing the headache, mental fog, fatigue, pain in lower limbs, hot and cold flashes and more that come from the activity or stressor.

I would prefer "activity or stressor-induced sickness symptoms." ASISS. I use a general term of "sickness" symptoms because people understand the experience of feeling sick. It's like coming down or having the flu, except without the respiratory part. But all the rest is like we are sick with something. And this would be more clear to doctors too. "Malaise" can include sickness symptoms, but it's more vague and can include just not having motivation, being slow. It's not specific enough to describe what we have, which is like we're sick with some illness, an infection.

The question could be asked, "When you have a day of activity, within a day or two, how would you describe your symptoms? Like you are coming down, getting sick with something?" (I know. It's too leading, but something like that can help the doctor find out if they have this disease feature.) "Post-exertional malaise" is such a vague term that it doesn't convey hardly anything in the words themselves. UGH! Since communications is my profession, this bugs me.

If the IoM Committee is considering tweaks, I'd like to say the temperature of the porridge is good. It describes my condition. It's not too hot (narrow) or too cold (broad). It's just right, temperature-wise. And, it is pleasing to digest, that is it will be easier for doctors to use. But the ingredient of PEM needs to be improved. It's the wrong color. It's misleading and confusing. This is an opportunity to use clear language to describe the most important and distinguishable symptom. Vague terms will not be as effective to the goal. And the side dishes, as in the clinician's guide, could use some additions. It's almost right, but could use a little more flavor.

Comments

  1. "Was it the French onion soup" ;) so typical for this disease, having to figure out/explain/guess éverything yourself

    and am stealing your Golidlocks comparison ifyoudontmind

    ReplyDelete
  2. Go ahead, take it, use it.

    When I was a child and told about my blood pressure thing, I understood that it was only triggered by a painful or stressful event. So, if I stumped my toe, my body reacted abnormally and blood pressure dropped and I fainted. I could not feel any other symptoms in normal every day activity. So it seemed I had normal blood pressure until some stressor hit. The doctor told me when that happens to lie down quickly to get blood back to my brain, hopefully preventing me from fainting.

    But, I now know it affects me in more subtle ways every day. And I unconsciously do things to compensate, such as folding my legs when I sit in a chair, etc. I remember now the doctor saying way back then I would learn to control it unconsciously. I now know what he meant.

    But even though it is constant, I think there are times it's worse than others. I had that normal tilt table test, but 2 years ago I tried to attend an event and had to keep my head on my lap to keep from fainting almost constantly for the 45-minute program. So, like other abnormalities, I think it can go up and down in us.

    And that I just figured out in last six months.

    ReplyDelete
  3. This comment has been removed by the author.

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  4. now I'm srsly curious what I said that was so awful that it couldn't even be on the net ;) (cleaning up my old bookmarks..)

    ReplyDelete

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