CCI SURVEY – for all ME/CFS patients tested for CCI and related conditions (please answer whether tested positive or negative)

[hezza]

whether their ME/CFS onset involved an acute infectious episode (which is one of the questions of the survey).

My acute viral onset illness was dengue fever, maybe not considered a “common trigger” in western countries but there was a study out of Singapore showing a significant percentage of dengue patients develop “post viral fatigue syndrome”. But from the options given I should just select “other”?

 

[Hip]

My acute viral onset illness was dengue fever, maybe not considered a “common trigger” in western countries but there was a study out of Singapore showing a significant percentage of dengue patients develop “post viral fatigue syndrome”. But from the options given I should just select “other”?

For a dengue fever trigger, I would probably select the option "After a flu-like illness, gastrointestinal or upper respiratory infection". I believe the onset of dengue is a fever.

I know someone who developed a fatiguing long-term illness after contracting dengue.

 

[Hip]

@bombsh3ll, in your survey answers you gave, I just noticed that you do not satisfy the Canadian consensus criteria for ME/CFS. This is because in the survey you did not select "fatigue" as one of your ME/CFS symptoms. Fatigue is an obligatory symptom in the CCC, and also in all other ME/CFS criteria. Just wondering if this was an error, or whether you really do not have the fatigue symptom?

You can go back to the survey and update your answers if you answered wrongly.

 

[Hip]

@moonell, @User891, @Matt01, @rfugger, @Raemacpherson, @Josch2306

Please consider answering the CCI Survey — see the first post in this thread.

 

[Matt01]

Hi Hip,

I am planning to fill in your survey. However I have had massive twist in my journey over the last 2 weeks which may have a significant effect on my answers. I am going through numerous scans and will be seeing a surgeon here in the UK shortly. I will be in a position to explain all to the community over the next few weeks. At the moment I'm in the thick of it. Please bear with me.

 

[Hip]

@IzaL
@KatieK
@kiwiK
@NinaV
@Simone
@mattmackay76

If you guys have been tested positive (or negative) for CCI or related conditions, please answer my CCI SURVEY.

 

[Hip]

@EnigmaCo
@Catwoman8
@mxwynne
@hollyhaldan
@MissMessing
@Gigi100

If you guys have been tested positive (or negative) for CCI or related conditions, please answer my CCI SURVEY.

 

[Florianne]

Hi Hip@
No and I will not be tested. I live in Quebec and we do not have that kind of test.

 

[hezza]

For a dengue fever trigger, I would probably select the option "After a flu-like illness, gastrointestinal or upper respiratory infection".

Ok, thanks, the gastrointestinal or upper respiratory part had me stuck since dengue attacks the vessels, but yes, a flu like illness nonetheless. My appt w Dr B is not until a few more weeks so hopefully I will know something soon & can take the survey.

 

[JenB]

Thanks! I'll put No. As for the classification, my radiologist report from Prof. Smith stated:

"There is a minor degree of hindbrain herniation, where the cerebellar tonsils extend into the foramen magnum posterolaterally in a pattern characteristically seen in an individual suffering from one of the hypermobile syndromes. The appearances are not those of a classical Chiari malformation, but are typical of the hindbrain herniation seen in patients with the hypermobile Ehlers-Danlos syndrome and which was formerly, erroneously called Chiari malformation, which is a different condition."

But I haven't been able to find any further information on this.

@Hip just curious how you are handling this. Most people are still “erroneously” calling this Chiari Malformation. I think this is Prof. Smith being a little pedantic. I think it might be better to ask people, “were you diagnosed with a hindbrain herniation or Chiari Malformation?” and then ask them to enter the actual value in mm of their herniation as reported so that it’s not just “yes/no.”

For those who know them, I think measurements like CXA, Grabb-Oakes should be recorded the same way. Whenever you dichotimize variables, you are actually erasing information that could be useful.

@Joly how many mm was your herniation? @jeff_w do you have any further info on this?

 

[Hip]

For those who know them, I think measurements like CXA, Grabb-Oakes should be recorded the same way. Whenever you dichotimize variables, you are actually erasing information that could be useful.

I was originally thinking about recording in the survey the exact numerical values of parameters like CXA, Grabb-Oakes, BAI, etc. But in the end I decided against it, because:

(a) People may not enter this measurement data correctly (especially as Dr G uses the comma for the decimal separator, which confuses some people).

(b) Some neurosurgeons conceivably may not provide the actual measurement value, but may merely state that a certain parameter like the CXA is pathological. So I wanted to provide a simple response options, where you just need to select whether your CXA, Grabb etc is: normal, borderline and pathological.

(c) On his reports, Dr G provides the CXA, Grabb, BAI and BDI values in flexion, neutral and extension, so that's 12 values you would need to enter. Plus the values for translational BAI, translational BDI, dynamic BDI, dens over Chamberlain and cerebellar tonsil ectopia making a total of 17 values for respondents to type in. I thought that might be too tedious, and might put people off answering the survey.

(d) I am not sure that the precise values of these measurements really tells us much. What's more important is whether the values are pathological or not.



@JenB Iif you really think it is important, I can add 17 additional questions to the survey asking about these 17 brain measurements; but I think it may be a bit overkill, and might put people off answering the survey.

@valentinelynx, @Mere72, what do you guys think of the idea of adding 17 additional questions to the survey, which ask respondents to enter the exact numerical values for their CXA, Grabb, BAI, etc measurements in flexion, neutral and extension?

 

[Joly]

@Joly how many mm was your herniation?

3.5mm.

 

[Mere72]

@Mere72, what do you guys think of the idea of adding 17 additional questions to the survey, which ask respondents to enter the exact numerical values for their CXA, Grabb, BAI, etc measurements in flexion, neutral and extension?

Here are my thoughts on the survey. I feel like we need to define what our goals are for this data, and we might have to hash it out a bit. Please correct me if I'm wrong, but I sense that @Hip, you are looking at this through a deductive reasoning lens, working off of the premise that there is a subset that we can identify that will help us target/ encourage certain PWME to get screened. It seems that @JenB is seeing it through an inductive reasoning lens, and wants to collect data that can then be shaped, or conclusions drawn, with the end purpose being having this info to back up our ideas and to give to researchers etc.

I think all of this info is valuable. But in my opinion it is premature for us to try to draw any conclusions as to which ME patients might be candidates for structural issues. While there are numerous blocks to everyone obtaining testing, my opinion as it stands for now is that I would like to see all patients screened, and I hope the work we are doing will help with that end goal. But I think we are going to have to let the data speak first.

In terms of adding 17 more questions to the survey, I think we need to consider a) what our goals are for this data, and b) ensuring ppl will take the time/ effort to fill it in. The length of it has been mentioned by one person in the FB group. Others have said they will fill it in when they have more energy/ time. I am concerned that this survey will get cumbersome if you keep adding big pieces to it. But I'm also not sure if length really is a deterrent, based off of one comment. Maybe we can open up a discussion about it here. I'm wondering if having a separate survey for measurements might be worth considering.

 

[Hip]

I sense that @Hip, you are looking at this through a deductive reasoning lens, working off of the premise that there is a subset that we can identify that will help us target/ encourage certain PWME to get screened.

Yes, I had some specific questions in mind which I hoped the survey would answer, and used those to help shape the survey.

But I am very aware that the survey is long (it actually contains over 5000 words, which is 20 minutes reading time), and that some of its questions, especially the CXA, Grabb, etc measurements questions and possibly the viral blood test questions, demand a bit of effort to answer, as they may require you to check your medical records and reports.

So while we can all agree that more data is better, I think this needs to be balanced with the need to keep the survey as short and as simple as we can make it.

 

[JenB]

My one comment is that the question re: Chiari v. Hindbrain herniation needs to be made more clear, and that it’s not about the 5mm cutoff (Prof. Smith defined someone’s 8.8mm herniation as “not Chiari”).

I’d also be uncomfortable not collecting the data that someone has a 3.5mm herniation as that can still be significant depending on what it is actually doing to the brainstem and/or CSF flow.

My recommendation would be to ask:

• Have you been diagnosed with a Chiari Malformation or a herniation of the cerebellar tonsils (“hindbrain herniation”)?
• If so, what is the length in mm of your herniation?

And no, not everyone will know, but they can just leave it blank if they don’t. Missing data is OK.

I hope @jeff_w chimes in as he may be able to say more.

 

[JenB]

@bombsh3ll, in your survey answers you gave, I just noticed that you do not satisfy the Canadian consensus criteria for ME/CFS. This is because in the survey you did not select "fatigue" as one of your ME/CFS symptoms. Fatigue is an obligatory symptom in the CCC, and also in all other ME/CFS criteria. Just wondering if this was an error, or whether you really do not have the fatigue symptom?

You can go back to the survey and update your answers if you answered wrongly.

I don’t think it’s an ICC requirement

 

[Hip]

My one comment is that the question re: Chiari v. Hindbrain herniation needs to be made more clear, and that it’s not about the 5mm cutoff (Prof. Smith defined someone’s 8.8mm herniation as “not Chiari”).

That's strange. Perhaps we are missing an important understanding here.


Some statements of interest about Chiari:

Type 1 Chiari malformation (CM-I) has been historically defined by cerebellar tonsillar position (TP) greater than 3–5 mm below the foramen magnum. Source: here.

In one study, all patients with greater than 12 mm of protrusion were symptomatic for Chiari, whereas approximately 30% of those whose protrusion measured between 5 and 10 mm remained without symptoms. Source: here.

So it seems that some people can have a tonsil ectopia/hernia of up to 10 mm, but still not get Chiari symptoms.

It could be that Prof Smith only labels it Chiari when (a) the cerebellar tonsil ectopia (hindbrain herniation) is greater than some threshold value such as ≥ 5 mm, and when (b) the symptoms of Chiari are present? Maybe if the symptoms are not present, then he does not diagnose Chiari?

My recommendation would be to ask:

• Have you been diagnosed with a Chiari Malformation or a herniation of the cerebellar tonsils (“hindbrain herniation”)?
• If so, what is the length in mm of your herniation?

OK, I will add a new question on the mm length of tonsil herniation/ectopia.

How do the following two questions sound?
Question 1:

Were you diagnosed with Chiari malformation?

This is the original question in the survey. Answer options are:
— None of the medical professionals who viewed my scans diagnosed me positive for Chiari
— Diagnosed positive for Chiari by at least one medical professional

Question 2:

Enter your cerebellar tonsil ectopia (hindbrain herniation)

Enter the distance in mm which your cerebellar tonsils protrude out of the base of your skull (the foramen magnum). This protrusion is called the cerebellar tonsil ectopia, or the hindbrain herniation. It is potentially pathological if 5 mm or greater. If your cerebellar tonsils do not protrude (no significant cerebellar tonsillar ectopia), please enter zero.

Dr Gilete on his reports uses phrases like "cerebellar tonsillar ectopia: 4 mm" and "no significant cerebellar tonsillar ectopia"). Whereas the Prof Smith reports refer to "hindbrain herniation".

 

[Hip]

I don’t think it’s an ICC requirement

Wow, I've never noticed that before! Very interesting, but also a bit strange, given that all other ME/CFS definitions I am aware of have made fatigue an obligatory symptom.

 

[JenB]

I think I know why Prof. Smith says this. It’s because the original Chiari malformation had to do with a congenital skull malformation that is different from the reasons why people w/ EDS get Chiari, but he’s being a bit pedantic IMHO. Most of the medical profession still calls this and all types of Chiari, Chiari. However, given how many people are having MRIs at Medserena, it’s a good idea to use all variations of language.

@Joly, if you did not know the difference between Chiari v. hindbrain herniation v. tonsillar ectopic, would the above question be clear to you?

I think I might suggest for the second question, Hip, something like “Whether or not you were diagnosed with a Chiari malformation, if you had a cerebellar tonsillar ectopic (hindbrain herniation), please enter the length below.”

 

[Hip]

I think I might suggest for the second question, Hip, something like “Whether or not you were diagnosed with a Chiari malformation, if you had a cerebellar tonsillar ectopic (hindbrain herniation), please enter the length below.”

OK.

This question has now been added in the "Craniocervical Instability and Chiari Measurements" section of the survey.