EHLERS-DANLOS SYNDROME and SYSTEMS BIOLOGY

A couple of days ago the wonderful and inspirational artist Sia announced that she was "suffering with chronic pain, a neurological disease, ehlers danlos". She went on to offer sympathy to all those in chronic pain and, in a heartfelt message, showed solidarity to those with her illnesses, or any 'chronic illness'.

Of course, there was a media storm. Much like the time when Jameela Jamil acknowledged having Ehlers Danlos Syndrome and another autoimmune disease. But there was also a lot of confusion, and outcry.

The Ehlers-Danlos community did two things with this public statement. Firstly, they welcomed Sia with open arms into their community. Now, I already have a reservation about patient groups, specifically about groups focused on illnesses. I think that these have both value and huge consequences. I think there is both blessing and danger to find kinship and connection with those who are unwell, but perhaps this is beyond the scope of today's article. I did not see Sia asking to be invited into a group of those who shared her genetic connective tissue disorder, or her chronic pain, but I wouldn’t like to comment on how she feels about now “belonging” to one.

But secondly, and most importantly, the instant rush from the Ehlers-Danlos Syndrome (EDS) community was to issue the very formal statement that Ehlers-Danlos was a connective-tissue disorder, and not a neurological disorder. The universal comment repeated across many of the influential EDSers feeds was that Sia’s statement likely implied that she had BOTH Ehlers-Danlos and a neurological disorder - and not that she intended to imply that EDS gave her a neurological disorder, or was a neurological disorder. Of course, the press that surrounded the story of the tweet further confused the issue, and many articles have since been rewritten making it clear that EDS is "not considered a neurological disorder".

And so, instead of this being about EDS and publicity generally, there became just a lot of noise about stating that Ehlers-Danlos was solely a connective tissue issue. And this angers me deeply. Not because EDS so often comes with associated conditions (so-called comorbidities), but because to isolate EDS into being SOLELY anything is to, in my humble opinion, miss the whole entire point of what makes EDS... EDS.

It is the siloing of illnesses into one system of the body that I believe creates the lack of service and assistance for those with EDS, and (for that matter) all other conditions which are multi-system and have multiple system effects. That our professional medics cannot (and often are not allowed to) think outside their specialism is one thing. That the patients themselves are being pushed into thinking of themselves in a myopic way frustrates and upsets me deeply.

Ehlers-Danlos syndrome itself may be a condition founded on connective-tissue laxity. However, it is untrue to then imply that every other condition that often accompanies EDS is an “and” and not a consequence. I believe that it is this chronic lack of understanding about the impact and the extended consequence of connective tissue laxity that allows so many people in this “community“ to be underserved.

Ehlers Danlos IS a connective tissue disorder. But in rendering this as a condition which ONLY affects skin extensibility or joint laxity is to miss the whole point. Connective tissue is EVERYWHERE. Collagen is everywhere. Through its fundamental role in forming a foundational part of the extracellular matrix, collagen and nerves will be intimately and forever connected. Having EDS affects collagen so it WILL affect your nervous system. Whether that is deemed a 'neurological issue' or not is, as with everything in EDS, a question of severity. But having connective tissue laxity can't not impact your nervous system - and many other systems in the body besides. EDS CANNOT be pushed into a 'rheumatology' box. Any attempt to do so is what leaves patients alone, frightened, lost and feeling misunderstood.

Every single one of my Ehlers-Danlos patients, and there are many, irrespective of the extensibility of the tissues, or whether they have been diagnosed with a simultaneous dysautonomia picture, receive a primer from me about how connective tissue and the nervous system and immune system are all inter-related. This fundamental document serves as a primer on themselves, helping them to understand their own physiology. To me it doesn’t matter whether somebody has fully diagnosed “dysautonomia”, what matters is that having a connective tissue complication directly indicates that the nervous system will work differently in these individuals.

It is not about a different function, necessarily, or even a “failing”. This isn't about me suddenly trying to label everyone with EDS with any more problems - not at all. It is simply a reflection that the nervous system of those with a connective tissue weakness or laxity will be different.

I respect deeply those in the Ehlers-Danlos community who seek to truly develop understanding for the connective tissue elements of this syndrome. And yet to start laying down hard lines at where “Ehlers-Danlos proper” ends and the comorbidities begin is not something that I feel has particular value. In fact, I think it damages the pursuit of true understanding of this condition.

It is a large failing of modern medicine that comorbidities are seen as “ands” instead of consequences. In conditions such as EDS we don't need to make our lives harder by creating segregation.

All of the value from those who practice integrative, precision medicine comes from the fact that we do not separate body systems. We also do not separate mind, emotions, environment, epigenetics, historic life experiences and heritable experiences. To build a picture of someone’s health, all of these are relevant.

I do not think it is a service to anybody with a “connective tissue disorder” to label them as having ONLY a connective-tissue disorder. Not because we want to make people feel 'worse' about their fate. Instead, to facilitate those with connective tissue issues to have a real understanding that, whether symptomatic or not, their nervous system will be highly involved in their presentation, their thoughts, their emotions, and the way they interact with the world.

As such, it is a privilege and, I believe, a right, for those with connective tissue disorders to be empowered by having an understanding of the neurological differences that are present within their body. Having this awareness allows them to take care of themselves. By limiting Ehlers-Danlos syndrome to a connective-tissue disorder, it is my belief that people are left without a full understanding of their physiology.

Similarly, I am supremely cautious in my practice not to “blame” the connective-tissue disorder for everything experienced or felt. I don't like members groups (as discussed above) because I feel that life becomes about the foundation of the 'illness', which is actually just a set of symptoms to be managed at the end of the day. But to surrender and live BEYOND your 'diagnoses' (which is what I seek to do personally, and to offer my clients) first requires FULLY UNDERSTANDING your foundations of who you are and what your body deals with. It therefore isn't enough to simply say that, unless you have other formal diagnoses, the only thing that matters is your connective tissue laxity.

I believe that if those with connective tissue issues are informed early enough of the connection to the nervous system, and indeed if the professionals who take care of those with connective-tissue disorders become at least slightly aware of the interrelatedness of physiological systems, then maybe the full blown “dysautonomia” or “MCAS” issues that so many deal with may not develop because life will be lived in sync with our bodies, not irrespective of their weaknesses and sensitivities.

To take care of our nervous system is to take care of our physiology as a whole. To deny the nervous system's role in our bodies and illness, especially in connective tissue disorders, is, I believe, incredibly negligent.

Now, I do not believe that anybody extolling the fact that a connective tissue disorder and not a neurological one is saying this because they are trying to cause harm. In fact, I think they think they are doing the right thing. And yet, I see the reaction to Sia’s announcement as highly indicative of one thing. Illnesses - in particular chronic illnesses which are just sets of symptoms that get labelled as 'syndromes' - are still incredibly poorly understood. Systemic biology understanding is still not mainstream - and it needs to be. People, bodies and illnesses cannot be understood when looked at through single-system lenses.