Interview with Dr. Pradeep Chopra regarding the psychological and psychiatric aspects of the Ehlers-Danlos syndromes and hypermobility spectrum disorders

Introduction

EDS expert and pain specialist Dr. Pradeep Chopra contacted Chronic Pain Partners to speak about a recently published review on the psychological and psychiatric aspects of Ehlers-Danlos syndrome. Dr. Chopra has been treating complex pain patients with a focus on people with Ehlers-Danlos syndrome for decades and is a passionate advocate for EDSers across the globe. Today, I discuss some of the review’s main statements with Dr. Chopra.

Summary

Karina Sturm:

Hi Dr. Chopra, and thanks for talking to me today. You contacted Chronic Pain Partners to share your opinion on this recently published review: Bulbena‐Cabré, A., Baeza‐Velasco, C., Rosado‐Figuerola, S. and Bulbena, A., 2021, December. Updates on the psychological and psychiatric aspects of the Ehlers–Danlos syndromes and hypermobility spectrum disorders. In American Journal of Medical Genetics Part C: Seminars in Medical Genetics (Vol. 187, No. 4, pp. 482-490). Hoboken, USA: John Wiley & Sons, Inc. Can you give me a brief summary of your opinion on this paper?

Dr. Pradeep Chopra:

The concern I am having is that this paper addresses the psychological and psychiatric aspects of EDS/HSD, but I am questioning whether there is really a relationship between EDS and mental health conditions. And also, if mental health conditions occur in patients with EDS, are those different compared to the non-EDS population? Are mental health conditions actually more common in EDS patients than compared to non-EDS patients? I am in doubt of this. And I am concerned this paper will be used to the disadvantage of people with EDS.

Key points of the Paper

Karina Sturm:

Ok, let’s start and talk a bit about some of the key points the authors make. However, first, I want to give our readers a short overview of the academic publication process. There are different kinds of papers one can publish. For instance, scientists can do experiments and publish the results of their research, or they do a survey and publish the responses. Those are considered research articles. But scientists can also summarize all the literature on a specific topic with no actual research involved, which would be a review. And then, there are opinion pieces that are only based on the authors’ own views. All those papers get published in academic journals – some of those journals are better than others. Usually, academic papers are peer-reviewed. This means that other researchers read and comment on the paper and decide whether it should be published or not.

The paper we are talking about today is a review article that summarizes the  current knowledge on EDS and psychiatric conditions. To accomplish this, the authors did a literature search. They used words like “EDS” and “anxiety” as search terms in specific search engines for medical and scientific literature (for example, PubMed), and then they summarized what they found in those publications. In 2017, the authors published a review on the same subject, and now, they are updating this previous review.  Now, let’s talk about some of the statements in the paper.

The authors state, “New research has confirmed that anxiety disorders are very common in patients with EDS (Bulbena-Cabré & Bulbena, 2018).” The authors talk about the connection between EDS and anxiety (and depression later on), especially in the context of chronic pain. The occurrence of anxiety with chronic pain has been a fact widely recognized in the medical world, right? It’s common knowledge that chronic pain may lead to depression and anxiety. Can you explain to our readers why you think people with EDS may develop signs of anxiety and/or depression?

Dr. Pradeep Chopra:

First, let’s talk about this new research mentioned in the paper. I looked at the paper they cited, and there is no new research, unfortunately. Additionally, both papers, the new one and the one they are referring to, are opinion pieces and not reviews. It’s not facts. So now, let’s talk about anxiety in people with EDS. In my opinion, there are two kinds of anxiety in EDS: 1. Normal anxiety: this anxiety develops, for instance, when a person has a constantly subluxing knee or elbow, and this person is in pain, and their quality of life is reduced. This causes some anxiety. That’s normal. 2. The symptoms of dysautonomia can mimic anxiety but have an organic cause, namely a spike in the heart rate and adrenaline. Some comorbid conditions of EDS, like dysautonomia, can be confused with mental health conditions, but their origin is organic.

This is an issue I have with the paper: It doesn’t look at symptoms and conditions associated with EDS, such as MCAS, dysautonomia, spinal issues and others. So this paper should also look at how many of their patients with anxiety actually  have dysautonomia and not anxiety.

Another problem I have with this particular statement is that the paper only looks at pathological anxiety but ignores anxiety as a normal consequence of chronic pain and dislocations. From my experience, treating 800 patients with EDS, I can say that patients may feel some anxiety related to their chronic pain, but that’s not significant.

Karina Sturm:

I read the publication the authors mentioned in the previous quote above (Bulbena-Cabré, A. and Bulbena, A., 2018. Anxiety and joint hypermobility: an unexpected association. Current Psychiatry, 17(4), pp.15-21.), which suggests as “most accepted biologic hypotheses” – a very strong term to use – for the association between joint hypermobility and anxiety: “genetic risks, interoceptive sensitivity, somatosensory amplification, emotion processing variances, autonomic nervous system dysfunction.” In the following paragraph, they immediately point out that their first point, genetic risks, is controversial because research could not replicate the genetic findings, which feels confusing because it is stated as the “most accepted biological hypothesis.” What’s your opinion on this?

Dr. Pradeep Chopra:

Well, you should be able to replicate findings. For instance, if you say, “the hair of a person who wears glasses turn green on Tuesdays,” then somebody in France or Germany or anywhere else should be able to replicate this finding. If you can’t replicate it, it probably  is not true.

Karina Sturm:

The authors also say: “Baeza-Velasco, Sinibaldi, and Castori (2018) also studied low and high anxious people with hEDS and found those with high anxious hEDS showed greater levels of pain catastrophizing, somatosensory amplification (SSA) as well as poor social functioning and general health.” The quoted study seems to be yet another review article. And while it might be referring to the actual study somewhere in the review, I’d prefer you explain to me what they mean with this statement.

Dr. Pradeep Chopra:

This paper was published in 2018, and I am not sure where they got the terminology low and high anxious from. I have seen many patients, but I wouldn’t divide them into low or high anxiety because there are periods a person might have high anxiety or a period where they might have low anxiety, but it’s not the nature of EDS to cause high or low anxiety.

Now, let’s talk about pain catastrophizing. This expression means that people are building up their pain to more than it actually is, being “dramatic” about their pain. When a patient comes into my office, I tell them about my three rules, of which the first one is that I need them to complain about their pain. Usually, my patients look very puzzled when I say this, and they’ll ask, “You really want to hear about my pain?” Because most people with EDS do not talk about their pain; they downplay their pain. There are many reasons for this. One is that many of them were born with pain. Secondly, they have been treated poorly every time they complain about their pain, so they just don’t anymore. So saying people with EDS catastrophize their pain is absolutely wrong. It’s actually the opposite: Patients with EDS play down their pain.

Now, coming to somatosensory amplification, which is another word for central sensitization. That doesn’t usually apply to EDS either because that’s something people develop after an injury. It means that the pain won’t go away even though the injury is healed.

The next point is “poor social functioning,” which is also not because of mental health issues. It’s a consequence of debilitating symptoms like lightheadedness, dizziness, heart racing and many more. It’s not that they don’t want to go out. They do. But if they do, their conditions get worse. So “poor social functioning” is not a consequence of anxiety. It’s a consequence of physical symptoms and pain.

Karina Sturm:

One sentence in particular that I didn’t quite understand was related to research on autism spectrum disorders and EDS. The paper mentions a study comparing people with autism with and without EDS. The mentioned “study” is not a peer-reviewed publication. It’s a pre-print of 2019 where they used a survey and invited participants via Reddit, and I could not find it in a journal. (Casanova, E.L., Sharp, J.L., Edelson, S.M., Kelly, D.P., Sokhadze, E.M. and Casanova, M.F., 2020. Immune, autonomic, and endocrine dysregulation in autism and Ehlers-Danlos syndrome/hypermobility spectrum disorders versus unaffected controls. Journal for ReAttach Therapy and Developmental Diversities, 2(2), pp.82-95.) Nevertheless, this publication states the group with EDS also had more autoimmune disorders and pain associated with hormones like pain during menstruation. The authors then went on to conclude that this would “reinforce the etiological relationship between ASD and GJH.” I don’t understand this conclusion. Can you explain this?

Dr. Pradeep Chopra:

In preparation for this interview, I looked at all the studies about EDS and autism. When researching a relationship, you have to compare the incidence of autism with the incidence of autism and joint hypermobility to see if there really is any difference. And I found no difference in the statistics. People with autism have a lower motor tone. That’s a known fact, but that’s not similar to joint hypermobility. So in my professional opinion, I can not see a relation at this moment. And as for the autoimmune conditions, those are well known to affect people with EDS, but this has nothing to do with autism and also does not reinforce any relationship between EDS and autism.

Karina Sturm:

And yet another conclusion I couldn’t follow was the connection between EDS and eating disorders. The paper says, “[…] proposed a model of eating disorders suggesting that several intra- and extra-articular features such as GI problems, temporomandibular joint disorders, and food intolerance could contribute to developing and maintaining disturbed eating patterns and significant weight loss in JHS/hEDS.” Now, the term “eating disorder” is defined as “any of a range of psychological disorders characterized by abnormal or disturbed eating habits (such as anorexia nervosa).” This is what Google, in collaboration with Oxford Dictionary, comes up with as a definition. So how are weight issues that are caused by TMJ or GI problems classified as eating disorders? The same counts for sleep disorders where they mention obstructive sleep apnea as the main cause, which is, as far as I know, a very common physical cause for sleep disorders in EDS. Can you explain this to me?

Dr. Pradeep Chopra:

Yes, those were extremely confusing paragraphs, and I am surprised the editors of the paper did not catch that. The problem is that this whole paper looks through a psychiatric lens. They are talking about people with EDS having eating disorders, which are psychiatric conditions, but then they say people with EDS have GI issues and TMJ – all physical conditions. To the layperson, this is very confusing. Because on the one hand, they are saying people with EDS have a psychological problem, namely an eating disorder, and at the same time, they are stating the eating disorder is caused by physical comorbidities of EDS. So they don’t clearly state that eating disorders and the EDS comorbidities, for instance, TMJ, are different conditions. Personally, I have not seen one EDS person with an eating disorder. However, patients with EDS can have more than 20 conditions affecting the GI system. For me, to prove that a person has an eating disorder would mean excluding all of these GI conditions. My concern is that because of this paper, when EDS people with GI issues go to a GI doctor now, they will have their GI issues dismissed as eating disorder. As a consequence, they might not receive appropriate treatment.

Karina Sturm:

In my understanding, a review usually puts different findings from original sources in context, right? So when writing a review, one has to be really careful they do not confuse facts with interpretation and speculation. Now, looking at this paper, would you consider this a literature review? Do you think it is clear what the original research is versus what is the authors’ interpretation?

Dr. Pradeep Chopra:

In my view, this paper is labeled as a review, but it seems as if it mainly consists of opinions. In general, a lot of poor-quality papers get published every day, and it is very hard for any reader to clearly differentiate between opinion and review or good versus bad papers.

Consequences of the Paper

Karina Sturm:

As kind of a new idea, the paper suggests the “Neuroconnective Phenotype,” which they have been mentioning in previous papers as well. It’s basically an assessment tool consisting of five areas: Sensory sensitivity, corporal signs, psychological and psychopathological dimensions, behavioral dimensions and somatic illness. The focus of this “tool” seems to be very much on psychiatric symptoms. At the end of the review, they state that such new tools not only would “help patients to understand their illness better, but it offers new ground upon which to explore and potentially uncover the biological basis of these disorders.” Can you explain what this means and what you fear the consequences of this paper could be for patients?

Dr. Pradeep Chopra:

The term “neuroconnective phenotype” was actually invented by the authors and does not really describe much. But that’s not what concerns me the most. I think this whole publication will be misinterpreted. I fear it will be used against people with EDS. I am concerned physicians, disability insurance people and many others will use it to belittle EDS symptoms. I fear people with EDS will no longer be believed. Some lawyers and physicians will not look at actual evidence like we just did. And the problems we already have, for example, children being taken away from their parents with wrong child abuse allegations, will be worsened by this. So, in summary, I am concerned this will be a horror show for patients because with this paper, when they see a doctor, that doctor might already have opened up his mind and think the patient has a psychological problem and, as a consequence, refuses to treat their physical symptoms, which is dangerous and harmful.

Karina Sturm:

What would you like to happen in terms of research on EDS and mental health?

Dr. Pradeep Chopra:

So for one, psychiatrists are more than welcome to publish papers, but in general, papers on complex multisystemic conditions such as EDS should be written in collaboration with other specialists to get more than one perspective. So what I am saying is: Of course, people with chronic conditions, including EDS, could potentially also have psychiatric conditions, but you need a multidisciplinary team to diagnose any mental health condition in EDS because you have to conclusively prove that there is no other explanation for the patient’s symptoms. And in patients with EDS, there often are many other explanations.

Clarification & Advice

Karina Sturm:

It’s not new that complex multisystemic conditions without clear origin have been categorized as psychosomatic. This happens to people with ME/CFS, EDS, and now also with Long-COVID. Hopefully, we will know one of the causative genes for hEDS soon, but until then, can you reiterate why it is clear to you that hEDS has a physical origin (not a psychological)?

Dr. Pradeep Chopra:

Connective tissue is called connective tissue because it connects everything in the body. Everything in the human body is, to some part, made up of connective tissue. So EDS affects every single inch of the body: from skin, teeth, gums, heart, lungs, GI system, bladder, spine, hips, knees, to ankles – every joint. And on top of that, people with EDS are prone to developing other comorbid conditions, like mast cell activation, which is a multisystemic condition itself. And there are many other comorbid conditions as well.

Karina Sturm:

Why do you think some physicians are so quick to diagnose us with mental health conditions?

Dr. Pradeep Chopra:

Good question. I know that’s a common problem. Personally, I don’t expect every physician to know about EDS. Just like I don’t know much about some other conditions, they might not know much about EDS. However, if somebody came into my office with unexplained symptoms, I would research those. And if the patient comes in and says, “I have Ehlers-Danlos syndrome,” and in case I didn’t know what it was, I would look it up, read about it, and try to understand it, so that I can not only help this patient but also the next one that comes in. But some physicians don’t want to get involved in something they are not comfortable with. In the US, one of the main issues is the health system and how it is set up. It’s not made for medically complex cases. I might be a lousy physician, but I simply can’t treat any medical problem in only 15 minutes unless you come in with an actual cut on your hand. But maybe that might even take longer than 15 minutes. So I don’t really blame the physicians but the system.

Karina Sturm:

What can or should patients do now?

Dr. Pradeep Chopra: If you have been diagnosed with EDS, establish yourself with an EDS expert in your area so that you have a well-documented assessment and management plan. If you don’t have anyone in your area, talk to your general practitioner or primary care physician to have them on your side. You can say, “Look, I don’t expect you to treat all of my issues, but it would be great if you would understand my conditions.” At the end of the day, finding the right person to treat you is key. On the way, try to educate as many physicians as you can.

For more information, listen to the Hypermobility Hour Podcast’s series of interview with Dr. Chopra. Episode 1 can be found here:

Hypermobility​ HHH  PODCAST with  Dr. Pradeep Chopra: Overview on EDS and Pain (Part 1)

This interview was officially published as part of our Chronic Pain Partner newsletter and also on the organization’s website: https://www.chronicpainpartners.com/18085-2/