Introduction – my background with Ehlers Danlos Hypermobility.
I first heard about hypermobility in physical therapy school. We were receiving a lecture about an allegedly rare, obscure sounding condition called Hypermobile Ehlers-Danlos Syndrome, or EDS(h). I didn’t lean in too closely to the material. Like many physical culturists who enter the rehab field, I was convinced I would be spending my career working with high functioning athletes.
Fast forward 5 years, and I’m in the process of growing a high-intensity strength training facility. As a physio, I had become a bit frustrated with the process of discharging a patient only to see them recidivate 6 months later due to a lack of proper supervision or competence with safe, effective exercise. I didn’t have a Blair, Owen, or Skyler I could send my patients to when they were done with therapy. If I wanted a safe option to exist, I realized I’d have to build it.
And like many people who start “HIT” facilities, I quickly realized young athletes aren’t our primary population. Our service simply solves more problems for the time-poor, injury-averse, senior professional than the time-rich, “invincible” young footballer. And so I learned to love working with this older demographic that I found I was actually called to help.
But there was another group of people who started showing up in my studio, with whom I also never envisioned working: younger people with hypermobility disorders like Ehlers-Danlos. To my surprise, I discovered that these people are far more numerous than I’d imagined. And if you are part of the HITUNI family, or the larger community of strength professionals employing an intensity-paradigm, you are well positioned to help people with EDSh. I want to spend the rest of this article explaining the basics of EDSh, and describing how we are uniquely able to help them.
What is Ehlers-Danlos?
Ehlers-Danlos is a family of disorders involving problems with collagen, which is the primary protein that forms our connective tissues. From our blood vessels and gums, to our tendons and ligaments, collagen provides much of our bodies’ structure and stability. So it is no surprise that dysfunctional collagen synthesis could affect the cardiovascular, periodontal, and musculoskeletal systems, to name a few.
The root causes of these conditions are unclear. While there are several genetic markers that seem to predispose (but not actually *cause*) individuals to have this disorder, there is no one “SNP” (single nucleotide polymorphism) that serves as a guaranteed positive test to show whether someone does or doesn’t have EDS. And beyond genetic factors, it appears there is a relationship between EDS and Mast Cell Activation Syndromes, which are disorders causing certain cells in the immune system to malfunction and become overactive. It’s hypothesized that autoimmunity may be part of the root cause of the disorder, but this isn’t certain.
How Ehlers-Danlos progresses over time
In practice, clients with EDSh present as highly flexible when young, with a propensity towards joint injuries and dislocations. There are outliers who are male, but 90% of cases are seen in females. The cliche manifestation is of the extremely slim teenage girl whose amazing flexibility and grace grants her early success as a ballet dancer. However, with that flexibility comes joint laxity, and as she matures to adulthood, the lack of muscular support ultimately leads to wear and tear on the passive supports – the tendons and ligaments. As these structures start to degenerate, chronic joint pain can develop, and the muscle can become stiff and painful (too little, too late). With joint pain becoming chronic and debilitating, many turn to surgery before they turn 40 for procedures to stabilize joints, but complications are common.
The progression of joint issues works something like this. When someone without EDS articulates their joints, muscles recruit reflexively before the joints reach their structural limits to prevent hyperextension and excess stress on the passive connective tissues. But for reasons which are poorly understood, sufferers of EDS seem to have motor control issues surrounding the positional sense and stabilization of their joints, which is called proprioception. One possible speculation is that their joint pain or weakness manifested early enough in life that they avoided hard muscular effort from an early age, and missed out on critical neurological development as a result. Another possibility is that, since the ligaments and tendons themselves are composed of dysfunctional collagen, the nerves responsible for detecting tension in these tissues become dysfunctional.
Regardless of why, these clients have poor proprioception, which can affect balance and result in frequent falls. Further, their muscles often don’t recruit quickly enough in a reflexive manner to provide optimal stability to their joints, which results in repetitive stress on their connective tissues. If your biceps muscle doesn’t recruit automatically as your elbow nears full extension, the ligamentous capsule of your humeroulnar joint will be the only thing that stops hyperextension. And if your collagen is weak, it may tolerate that stress poorly and become not only over-stretched, but hypersensitive and chronically painful.
An important point to note is that, since proprioception is poor in people with EDSh, they often crave the sense of stability that typical folks take for granted. While stretching may be uncomfortable for people like me, these clients may enjoy sitting or even sleeping in deeply stretched positions for extended periods, for the sense of stability that this can give. Unfortunately, this overstretching of the connective tissues is exactly the wrong thing to do, as it trains their nervous system to permit over-stretched muscles as normal, and over-stresses the already vulnerable connective tissues.
Other key issues experienced by many with EDS
POTS, or postural orthostatic tachycardia syndrome, is a very common comorbid condition for EDS sufferers. Possibly driven by autonomic dysfunction, POTS involves a problem of cardiac output. As a person rises from a lying or sitting position, the cardiovascular system needs to prevent the blood from pooling in the legs by reflexively constricting vessels in the legs, and increasing the output at the heart. As we’ve learned, some of the muscle-tone related reflexes are dysfunctional in EDSh clients. On top of that, low muscle mass often also shows itself in a smaller than average heart muscle. When a typical heart needs to increase output, it can BOTH increase heart rate AND increase the stroke volume, or amount of blood moved with each pump. But in clients with POTS, a weak heart can mean stroke volume remains low even with exertion. This means the heart’s only option is to pump faster, and this “tachycardia” can lead to light-headedness, nausea, and even syncope (fainting).
Finally, people with EDS(h) often have digestive issues. This may be a chicken-egg problem. Poor nutrient absorption from under-active digestive function leaves these clients chronically undernourished and fatigued. As a result, they often fail to consume adequate protein, which leads to further malabsorption as protein is required to make enzymes and stomach acid. Gut issues may be a result, or a cause, of the whole condition, given the relationship between autoimmunity, diet, and the gut microbiome.
What we can offer clients with EDSh
The good news for us is that we, as effort/intensity-based trainers, don’t need to learn any radical new techniques to help these clients. Though autoimmunity and malnutrition may be drivers in EDS (sufferers often have gastrointestinal issues and poor appetite), what we can say for sure is that stronger muscles offer more support. What these individuals lack, most often, is active stability in their joints. Thus, effective and joint-friendly strength training seems to be the best, safest, most evidence based approach to improving that.
This population is understandably highly injury conscious, and can’t tolerate traditional high force, high volume approaches. Not only are such approaches disastrous for people with poor joint stability, but the sheer amount of non-stimulating mechanical work involved in a conventional “three sets of ten” approach can leave EDS(h) clients exhausted and unable to function. As practitioners of a controlled, intensity-based exercise approach, we can offer a uniquely safe, low wear-and-tear way to provide strengthening that conventional high force, high volume exercise can not.
In addition to just increasing the size of their muscles, our approach to exercise can actually help these clients improve their ability to recruit their own muscles during daily activity, which may mitigate the wear-and-tear that results from poor neuromuscular control. My belief is that the slow tempos of our approach train the nervous system to exert more “fine-motor-control,” or precise recruitment, of the large muscles which support the major joints.
And finally, with a “slow-to-failure” model of resistance training, muscles are placed under continuous tension, which places unique demands on the heart. It is well established that any exercise where tension is continuous (either isometric or slow-to-failure) leads to special adaptations in the heart. Working against the increased peripheral resistance of contracting muscle trains the heart to beat more forcefully, instead of just faster, which may be an important factor for clients with POTS related to low heart muscle volume.
The comprehensive management of a person with EDSh, POTS, and other related conditions can be complex. But, with the right messaging around what we are accomplishing, we as high-intensity resistance training professionals can play a unique and essential role in the management of their musculoskeletal AND cardiovascular health. Our approach is uniquely safe from acute injuries, involves low-forces and reduced joint wear, and keeps exercise volumes manageable so they won’t be too exhausted to keep up with their training.
I recommend you shy away from advanced overload techniques and intensity-extenders with these clients. Keep it simple with a controlled tempo, and try to help them deeply fatigue their muscles with the lowest possible volume to optimize recovery. Some clients report less fatigue when training with a 10”/10” cadence than a 2”/4” cadence, which may be due to the reduced mechanical work performed. During their introductory period to strength training, they may not tolerate much intensity, and slightly greater frequency (2-3x/week) may be ideal to simply help them become familiar with slow, controlled resistance training.
Please don’t hesitate to reach out with questions for me through through my email at firstname.lastname@example.org.