Psychiatric Disorders From Ehlers–Danlos Syndrome

Nationwide population-based cohort study of psychiatric disorders in individuals with Ehlers–Danlos syndrome or hypermobility syndrome and their siblings | BMC Psychiatry | Full Text – 04 July 2016

It looks like we inherit not only chronic physical pain but also a fourfold increased risk of both anxiety and depression.

Somehow, it makes sense to me that having a body “too loose” and being physically “unstable” would also manifest as being mentally “unstable”, that along with our physical pain, we also suffer from mental pain.

Abstract

To assess the risk of psychiatric disorders in Ehlers-Danlos syndrome (EDS) and hypermobility syndrome.  

Methods

Nationwide population-based matched cohort study. EDS, hypermobility syndrome and psychiatric disorders were identified through Swedish national registries.

Individuals with EDS (n = 1,771) were matched with comparison individuals (n = 17,710).

Using conditional logistic regression, risk of

  • autism spectrum disorder (ASD),
  • bipolar disorder,
  • attention deficit hyperactivity disorder (ADHD),
  • depression,
  • attempted suicide,
  • suicide and
  • schizophrenia

were estimated. The same analyses were conducted in individuals with hypermobility syndrome (n = 10,019) and their siblings.

Results

EDS was associated with

  • ASD: risk ratio (RR) 7.4, 95 % confidence interval (95 % CI) 5.2–10.7;
  • bipolar disorder: RR 2.7, CI 1.5–4.7; ADHD: RR 5.6, CI 4.2–7.4;
  • depression: RR 3.4, 95 % CI 2.9–4.1; and
  • attempted suicide: RR 2.1, 95 % CI 1.7–2.7,
  • but not with suicide or schizophrenia.

EDS siblings were at increased risk of

  • ADHD: RR 2.1, 95 % CI 1.4–3.3;
  • depression: RR 1.5, 95 % CI 1.1–1.8; and
  • suicide attempt: RR 1.8, 95 % CI 1.4–2.3.

Similar results were observed for individuals with hypermobility syndrome and their siblings.

Conclusions

Individuals with EDS and hypermobility syndrome are at increased risks of being diagnosed with psychiatric disorders.

Though technically correct, this sentence makes it seem like we’re only at risk of being *diagnosed* with these mental disorders, not actually suffering from them.

Normally, a diagnosis would be associated with a person *having* the syndrome/symptom/disease, but here they do not acknowledge that.

These risk increases may have a genetic and/or early environmental background as suggested by evidence showing that siblings to patients have elevated risks of certain psychiatric disorders.


The above is only from the abstract, the free full text continues below:

Background

This genetic disorder is present in at least 1/5000 individualsand an important cause of joint hypermobility syndrome.

The disease is characterized by hypermobility, although symptoms and signs can be highly variable and include

  • joint complaints,
  • myalgia,
  • skin problems,
  • sleep apnea,
  • pneumothorax and
  • cardiovascular disease.

While a diagnosis of EDS can be comforting to the patient, there is no curative treatment for EDS.

Instead, physicians aim to stabilize joints and prevent complications, sometimes using surgery to resolve the condition. Physiotherapy is an integral part of patient management.

Intense pain, diagnostic delay and risk of unemployment due to musculoskeletal complications, all contribute to the emotional burden of the disorder.

Already in 1994, Lumley et al. reported a link between EDS and psychological problems. Despite the long-term awareness of psychosocial dysfunctioning in EDS, studies on psychiatric disorders in EDS or hypermobility syndrome are rare, but well summarized in a recent meta-analysis by Smith et al.

Smith et al. found a fourfold increased risk of both anxiety and depression in hypermobility syndrome, while the risk of panic disorder was even higher (odds ratio (OR) = 6.7, 95 % confidence interval (95 % CI) 2.2–20.4).

Depression has been linked to joint hypermobility (OR 4.1, 95 % CI 1.8–9.4),

Given the inconsistent and sometimes flawed data on EDS, hypermobility syndrome and psychiatric disorder, we decided to conduct a population-based matched cohort study examining ASD, bipolar disorder, ADHD, depression, suicide attempt, completed suicide and schizophrenia in patients with EDS or hypermobility syndrome.

I’ve skipped the technical details of the intervening sections. You can find them at Methods and Results.

Discussion

We found a substantially increased risk of several psychiatric disorders in addition to depression.

Since the 1990s, at least three papers have examined the association between hypermobility syndrome and EDS and anxiety. Together, they point towards a significant association between hypermobility syndrome and anxiety.

Similarly, some research, but not all, suggests a positive association with depression. However, most of these studies have been small.

The association seen in this study may depend on several mechanisms, and not necessarily the same mechanisms for all psychiatric disorders.

The disease load in hypermobility syndrome and EDS may contribute to a lower quality of life and an increased risk of depression and suicide attempt, but is less likely to explain the excess risk of other disorders such as ASD and bipolar disorder

An increased disease burden is otherwise likely to occur both before (diagnostic delay with secondary patient frustration is common in EDS) and after diagnosis (there is no curative treatment for EDS).

Relative to risk factors, of particular interest is our finding of an increased risk of psychiatric disorder in unaffected EDS or hypermobility syndrome siblings. This finding suggests that it is not only the disease itself that links to psychiatric disorder but also that part of the risk increase may be due to shared genetic or early environmental influences.

Conclusion

In summary, this is the largest study to date to examine the association between EDS, hypermobility syndrome and psychiatric disorders.

A positive association between these disorders confirms earlier case reports and smaller studies.

The relative risks for several psychiatric disorders were substantial.

Because siblings without EDS or hypermobility syndrome carried an increased risk of being diagnosed with a psychiatric disorder, we suspect that these associations may be due to genetic and shared environmental factors.

4 thoughts on “Psychiatric Disorders From Ehlers–Danlos Syndrome

  1. Rachel Kirkland

    SUPER interesting. Thanks for sharing. One thought – Depression and anxiety get the most attention in discussions about psych+EDS, which is undoubtedly important. I also want to take the opportunity to highlight trauma-related disorders in this population. Anecdotally, so very many of us have substantial trauma. OCD appears to be at about 21% among EDSers (https://www.ncbi.nlm.nih.gov/pubmed/24272065). I also think there’s a more significant comorbidity with dissociative disorders than is currently recognized.

    N.B. This argument is going to have holes. I’m quite fatigued. I welcome your thoughts. I also welcome your gentle direction to resources I’ve missed. I’m sure there are some.

    First off, I would clump borderline personality disorder, dissociative identity disorder, and PTSD together as one “state” because…well…they really are in the same family in my view, and the actual dx among those three that chronically-ill folk receive seems to come down mostly to the practitioner’s training/style/feelings about a particular dx. And we know that BPD, DID, and complex PTSD are under-diagnosed to begin with, often treated as if they were bipolar disorder, MDD, ADHD, GAD, plain ol’ PTSD, what have you. (No spoons to dig all that up or sharpen my conceptualization of the PTSD piece right now, sorry.)

    I found one case report of an EDSer with BPD (https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6389018/). There’s also a Canadian study that showed some linkage (https://www.readcube.com/articles/10.1007%2Fs00296-015-3375-1?shared_access_token=GlmLCTphHrHKwWiZykF4pfe4RwlQNchNByi7wbcMAY4E3YhfYNCkOWP2YNADmTeue8t90Hes4TwsFGnQunHtJ5PFyYIzkBEoOPBLUn1QWZhw2dMY60l2oVg79Jc0HTgdd4NHmpDzGObesn2fQ-aDAf1FmrOj9-rVdFN7yLX1RyU%3D). For that one, though, I think there’s a different story in the data than what they discuss. Their numbers (p. 4 of that PDF) show PTSD at 4.7% and BPD at 3.8%, Conduct disorder is at 1.9%, which I suspect is also trauma-related in the EDS population. If I’m right about those all being in the same neighborhood, diagnostically speaking, that’s about 10% of EDSers with a trauma-related diagnosis. And that’s giving all the depression, anxiety, ADHD, and bipolar diagnoses the benefit of the doubt. I think that’s an important avenue to explore because trauma-related disorders can be so disabling.

    Liked by 1 person

    Reply
    1. Zyp Czyk Post author

      I’ve come to think that our physical laxity from EDS also applies to our mental states. Both I and my mom can have our moods pushed rapidly up or down within hours or even minutes, depending on outside circumstances.

      I was diagnosed with BPD in my 30’s and I’m embarrassed to admit, the diagnosis was right on. But I had a wonderful therapist who worked with me (successfully) for almost a decade to find a better, balanced life. Luckily this was before my chronic pain became a serious issue.

      But I think I also have some PTSD just from having my pain be minimized and invalidated when I was in great pain. When I was most desperate for medical help, I was essentially told I was crazy (that alone is enough to make anyone crazy).

      I think the genetics of EDS make us more susceptible to mental/mood disorders too. Just because doctors don’t know something is related doesn’t mean it’s not real.

      Liked by 1 person

      Reply
  2. Rachel Kirkland

    Solidarity. I wish more therapists took seriously the, for lack of a better term, medical system trauma that many of us EDSers experience. It is not insubstantial.

    Liked by 1 person

    Reply
    1. Zyp Czyk Post author

      Yes, it’s very disturbing and over time, deeply unsettling, when you are desperate for relief from your pain, but the doctors you see tell you that your pain is meaningless. How many times have we been told “there’s nothing wrong with you”.

      Like

      Reply

Other thoughts?

Fill in your details below or click an icon to log in:

WordPress.com Logo

You are commenting using your WordPress.com account. Log Out /  Change )

Google photo

You are commenting using your Google account. Log Out /  Change )

Twitter picture

You are commenting using your Twitter account. Log Out /  Change )

Facebook photo

You are commenting using your Facebook account. Log Out /  Change )

Connecting to %s

This site uses Akismet to reduce spam. Learn how your comment data is processed.