Dopamine Deficiency and Ehlers-Danlos Syndrome

Cross Pollination of Ideas Across the Silos of Different Diagnostic Labels

Introduction

This follows on from a series started in:

Symptoms of Stress <-> Symptoms of Chronic Illness

There, I made the case that chronic stress is the primary root cause of most chronic illnesses, but that we all get different “disease labels” based on the best fit matching of our own unique basket of symptoms to specific diagnostic patterns:

“…note also, that this is definitely not saying that everyone will get all of these symptoms… chronic stress manifests very differently in each of us, resulting in our own personalized basket of symptoms “picked” from these lists… the diagnosis we eventually get from the doctors then depends on how closely the contents of our unique basket matches to a specific collection of symptoms which has been given a disease label, almost via a flowchart…”,

We continued this series in:

Fibromyalgia as a Dopamine Deficiency Condition

There, I made the additional case that we can learn much more by considering the commonalities between the disease labels, than by separating them and treating them like silos.

“The new lesson is that we can learn much more by considering the similarities and commonalities between various chronic disease diagnostic labels, rather than the emphasis on the differences. In my view, there is much to be gained by sharing the wisdoms and strategies gleaned across the different [artificially] siloed community boundaries. Indeed, I believe that this artificial silo-ing, mainly due to the ultra-specialized nature of our current medical systems, has done more harm than good. Thus, I advocate for knowledge synthesis and generalization above separation, specialization, and reductionism, and for much more cross-fertilization, and cross-pollination, between the different disease labels. Especially important is the co-sharing of solutions and wisdoms by members of the different communities themselves with each other.”

In the quoted article, I illustrated this point by considering how much the fibromyalgia and Parkinson’s communities have to learn from each other.

In this article, we continue this theme by looking at Ehlers-Danlos Syndrome (EDS).

Ehlers-Danlos Syndrome

EDS is perhaps one of the common diagnosis labels of chronic conditions that is thought to have the most inherited genetic contributions. According to the NHS:

“Ehlers-Danlos syndromes (EDS) are a group of rare inherited conditions that affect connective tissue [fascia], the main symptoms include:

• an increased range of joint movement (joint hypermobility);

• stretchy skin;

• fragile skin that breaks or bruises easily”.

So at first glance, being a “genetic disorder” with very peculiar physical symptoms, it seems that EDS provides the exception to our rule that most chronic diseases have the same root cause, and that that common cause is chronic stress.

Lets first look at the inherited generic component. Again, according to the NHS:

“currently, there are no tests to confirm whether someone has hEDS [hypermobile EDS]… rhe diagnosis is made based on a person's medical history and a physical examination.”

So like most other chronic illnesses, most EDS is diagnosed by “flowchart”, based on trying to fit an individual’s unique basket of symptoms to a specific collection of symptoms of a diagnostic disease label. In fact EDS is a prime example of the medical system trying to reduce symptoms to diagnostic labels, and then creating more labels when cases occur which don’t fit with existing ones: there are currently 13 types of EDS label!!!

Genetic testing is available for some of the rarer types of EDS via a blood test, but the genetic test only finds about 50% of cases.

To my mind, these considerations means there is a lot more to the root causes than genetics alone.

Commonalities with Other Diagnostic Labels

Now let us consider the commonalities with other chronic diseases. This is illustrated by the fact that, despite the “obvious” peculiar physical symptoms, EDS presents so similarly to other chronic conditions that:

“people may initially be misdiagnosed with hypochondria, depression, or chronic fatigue syndrome^”,

and

“it typically takes 10+ years for an accurate diagnosis, leaving individuals exposed to a decade of additional ramifications”.

Considering some of the other common features of EDS beyond the obvious ones, a very good article on this on this is “Ehlers-Danlos, Proprioception, Dystonia, Dysautonomy, L-Dopa and Oxgenotherapy’s Efficacy”, from which we glean that EDS manifests with symptoms very similar to movement disorders and chronic conditions often related to dopamine deficiency. The authors discuss three principle commonalities.

The first is dyseregulation of proprioception, the sense of where body parts and how they are moving.

“Due to changes in tissue texture, bodily sensations from internal or external sensors fail or are distorted and misleading. This dysproprioception is the key to understanding the mechanism of postural and motor manifestations of Ehlers-Danlos syndrome, but also pain from hypersensoriality.

The second is dystonia. Various types of dystonia are chronic illness diagnostic labels in their right, and also feature large under other labels, such as Parkinson’s and fibromyalgia.

“… involuntary movements or muscles twitches... contractions can be observed in the face, shoulders, neck, limbs, sometimes triggered by a sensation of pain… resting tremor or jerky movements. These signs are sometimes discrete, but they have an important meaning: it is a symptom of a more diffuse motor disorder, often invisible, as an iceberg of which we only see the tip. Deep muscle contractions are at the origin of pain, sometimes evolving by seizures and can turn into violent contractions of the extremities with difficulty in stretching out the fingers especially during writing or using a computer keyboard ('writer’s cramp')."

Not being able to write is one of the cardinal early signs of Parkinson’s cluster of symptoms. The authors even directly make the link to Parkinson’s:

“The probable mechanism of this Parkinsonian syndrome is, most likely, one of the manifestations of proprioceptive disorder. Centers controlling automatic motility receiving inappropriate signals have this type of response.”

The third is dysautonomia, or dysregulation of the Nervous System

“Dysautonomia proceeds from the same causes: malfunctioning sensors informing about blood pressure, heart rate, temperature regulation (sweating, cold feet, coldness ...). It also affects the digestive tract: swallowing difficulties, gastro-esophageal reflux, bloating, and constipation sometimes alternating with diarrhea. It finds its explanation in the anarchy of information sent by the sensors involved in the digestive system’s physiological control. Postural orthostatic tachycardia syndrome (POTS) is one of the frequent manifestations in Ehlers-Danlos syndrome associating, at the time of verticalization or movement (rotation) of the body: vertigo, tachycardia, sensation of malaise and "weakness" of the lower limbs with, sometimes, fall. The role of blood pressure is crucial here.”

These are all also common symptoms in other movement disorders, and other chronic conditions, especially those which are associated with low dopamine levels.

Connection with Chronic Stress

Turning now to the question of whether EDS is stress related, and indeed, whether chronic stress is causal of many of the symptoms, a quick google search of “EDS stress” brings up a plethora of articles making the connection.

A really good article on this is “Top 5 Ehlers-Danlos Hypermobility Considerations: Stress & Anxiety.”

“In those with hypermobility, [stressful] events can have far-reaching effects with amplified symptoms thereby creating a frustrating cycle of pain, fatigue, anxiety, POTs, insomnia, and digestive upset (to unfortunately name just a few).”

Interestingly, the authors pose a few leading questions, for folks suffering with EDS to ask themselves, in order to self-assess that stress is indeed causing/worsening their symptoms, including:

• can you identify any patterns- timing, environment, people, etc-  regarding any of your repeated bodily manifestations?

• do certain environments or situations increase HR/breathing/temperature or make you dizzy?

• does muscular tension or an increase in pain occur when wearing certain clothes or shoes?

• what habits or life events tend to take place in the days prior to symptom onset?

• are symptoms markedly worse when you haven't slept?

This is interesting, as they are similar to the questions that my therapist

Lilian Sjøberg

gets her clients to ask themselves. Indeed, the “O” in the HOPE of her

The HOPE shortcut

method stands for “Observe symptoms”.

Perhaps the most profound and important learning from the above article is that:

“a known correlation has been found between hypermobility and higher levels of adrenaline… greater adrenaline production predisposes people to anxiety and enhances overall sensitivity to stress thereby perpetuating this cycle of symptoms... whether emotional, physical, or environmental, the impact of stress is felt more intensely in people with hEDS & HSD: this internal, and very real effect, is akin to feeling deafened by music that others can barely hear.”

This is so important, because a necessary consequence of high adrenaline is low dopamine, since adrenaline is created from dopamine, so if we are always producing high levels of adrenaline in our bodies and brains, then we are also constantly using up our dopamine supplies. Thus folks with high adrenaline may appear as if they are unable to produce their own dopamine, while in reality it is that they are constantly converting their own dopamine into the stress chemical, so dopamine can never build up to healthy baseline levels.

Here is Lilian explaining more about this link, and its vital importance in disease management:

Whether the heightened adrenaline is an effect of the disease, or is more causal as a result of chronic stress, to my mind, this firmly places EDS also in the “dopamine deficiency” class of chronic conditions.

“Does this higher surge of adrenaline and over-sensitivity mean you're doomed to a persistent onslaught of pain, anxiety, and fatigue? Not at all. We are all in control of our environments to varying degrees and genetic predispositions are quite simply that: something we are predisposed to but can better manage through awareness, understanding, and choices. By targeting the root causes of our anxiety – too much adrenalin – we can change the environment inside the brain.”

Also, dopamine [and lack thereof] is known to have a profound effect on fascia/connective tissue. Anyone with Parkinson’s will experience how the extreme stiffness and rigidity in the fascia just melts away with dopamine supplementation, only to return as the exogenous dopamine supply runs out. Also, as covered in the previous article on fibromyalgia, dopamine has an analgesic role on chronic pain. So dopamine deficiency goes along way to explain many of the symptoms of EDS.

Indeed, this was confirmed by the authors of the above cited article on dystonia and EDS:

“Advised by a neurologist familiar with Parkinson's disease, we started to us… L-Dopa [the dopamine precursor which is still the mainstay treatment for PD] in two doses, an extended-release tablet in the morning successfully. Adverse effects are rare, especially digestive”.

I was also pointed by a reader to the existence of an “EDS and Dopa Responsive Dystonia” facebook group, who are self-experimenting with this idea.

However, as covered by Lilian in the video above, external dopamine supplementation is still not the long term solution, because of the same trap that folks with a Parkinson’s diagnosis get stuck in: needing ever increasing amounts of exogenous dopamine, and hence getting ever increasing side-effects.

Instead, the goal is to start self-generating enough of our own endogenous supplies of dopamine again, via stress reduction, and thereby preventing ourselves turning our own internal dopamine supplies into adrenaline.

See my post, and accompanying video,

The Antidote to Suffering in the Modern World

for my “master list” of ways to go about this.

Also consider taking the online course Lilian and I have created “Emotional Trauma, Fascia And Breathing”, which, according to the above, should be just as a relevant to folks with an EDS diagnosis as to those with other chronic disease labels, as well as to better health and wellbeing in general.