double-jointed, ehlers danlos, hypermobile, hyper mobile, flexible

What It Means To Be Double-Jointed

What is Ehlers Danlos, the Beighton Scale, and why you should care

by Jeff S. Williams, DC, FIANM

Double-jointed. That’s what we grew up calling it. Most of us have seen one of our friends be able to do 'stupid human tricks' with their bodies. Whether it be turning their elbows the other direction, bend over and put their hands flat on the floor with straight legs, sit with their ankles behind their head, or turn their fingers in all different directions, super stretchy people can be very entertaining. 

 

It’s their connective tissue

What is it that makes them different from the rest of us? Why can some contort their bodies in all kinds of arrangements while so many of us struggle to simply bend over to try to touch our toes?

First, it is important to put the term 'double-jointed' to rest. Let's bury it right here and now. Nobody has two joints in one spot that allows more flexibility or movement in that joint. This terminology for more movement is simply incorrect.

What is really going on all comes down to connective tissue. 

What is ‘connective tissue’ exactly? It is, “A complex mixture of proteins and other substances that provide strength and elasticity to the underlying structures in your body,” according to the Mayo Clinic. The difference is not the makeup of the connective tissue. The difference is how the connective tissue is arranged. The most noticeable symptoms of connective tissue disorder are overly flexible joints, stretchy skin, chronic joint pain, and even fragile skin. The protein affected is called collagen.

Now that we have determined there is a connective tissue disorder at play, then surely it has a name. And it does indeed. It is called EHLERS-DANLOS (EDS) syndrome.  EDS is an inherited disorder running in families. It is inherited. EDS is simply the umbrella name for several subtypes of EDS. In fact, there are thirteen subtypes of EDS and each subtype is really a distinct disorder that runs in the family. 

 

How do I, as a clinician, most commonly spot EDS? 

I treat neuromusculoskeletal issues. Part of assessing a patient is to have them go through their normal ranges of motion for their neck, mid-back, and low back. It is very common for me to ask a patient to bend over to touch their toes and they just bend right over and put their hands flat on the floor. Or at least come close to doing so. 

In a case such as that, it is time to pull the Beighton Scale out of my toolbox. Unfortunately, not enough medical doctors, osteopaths, chiropractors, nurse practitioners, or physician assistance know about EDS or know the significance and importance of utilizing the Beighton Scale to assess these patients. If the issue is musculoskeletal, being able to recognize and diagnose EDS is vital to the patient's recovery. Othewise, we could be doing the exact opposite of what we should be doing to treat the patient's pain, thus making it worse. 

 

What is the Beighton Scale?

The Beighton Scale is used to quantify the severity of EDS by going through some very simple body movements to see how ‘stretchy’ a person really is. The scale then places a point value to each movement ultimately arriving at a total score by the end of the scale. The movements and point values are as follows:

  1. Being able to place your hands flat on the floor with knees and legs straight = 1 point
  2. Being able to bring your thumb in flexion to touch your wrist or arm = 1 point per thumb
  3. Being able to bring your pinky back to about 90 degrees = 1 point per pinky
  4. Being able to straighten your elbow 10 degrees beyond straight = 1 point per elbow
  5. Being able to straighten your knees 10 degrees beyond straight = 1 point per knee

That is a total of nine possible points. Most agree that a score above 4 indicates hypermobility and that the higher the score, the more hypermobile a person is, and the more they may have some interest in genetic testing with their medical professional to further evaluate and determine the exact subtype of EDS they have. 

While there are so many different subtypes, for the most part, they do not really affect your life much outside of the symptoms I mentioned. And, of course, outside of all of the party tricks you can show off by contorting your body. There is one subtype that can be particularly dangerous that I will discuss in a minute. But, the subtypes mostly just mean that you are stretchier than everyone else. Most subtypes do not shorten your life span. However, that does not mean they do not come with some minor issues. 

Two of the less severe symptoms we see most commonly are early joint degeneration and chronic pain. To demonstrate, most of us know someone that has had spinal fusion surgery. Most people don’t know this but the surgeons fuse segments of the spine because there is extra motion in the region and the thought process is, “No motion means no pain.” So they fuse or freeze the movement at that segment. Now spinal surgery comes with its own set of other issues we will not get into here but, for that very segment, they are correct. 

If we extrapolate that idea to every joint in the body, then we can say that stretchy EDS folks have extra movement in almost every joint of their body. That can mean a bit of extra wear throughout the years and can most definitely mean some extra chronic joint pain due to the increased motion in the joint itself. 

 

Vascular EDS

Beyond the mostly harmless EDS patients, there is a subtype that is more concerning. It is the vascular type of EDS. It is the subtype that clinicians should be especially on the lookout for. It is exceedingly rare happening in only 1 in 250,000 people. It weakens the blood vessels and makes organs more like to develop a tear. Obviously, the vascular subtype of EDS needs to be monitored by a medical professional. 

This more severe type of EDS is obviously more of a concern and more impactful than simply being able to bend more than other people or developing some chronic pain. 

 

Treatment of EDS

The treatment of hypermobile Ehlers-Danlos syndrome depends on the signs and symptoms present in each person. For example, chiropractors that specialize in orthopedics and exercise rehabilitation or physical therapists are often recommended to strengthen muscles and improve joint stability. Assistive devices such as braces, wheelchairs, or scooters may be necessary depending on the severity of joint instability.

Chiropractors are well-known for spinal manipulative therapy (SMT), otherwise known as ‘popping bones’. Chiropractors use SMT to increase movement in joints. If a patient has EDS, they already have excess movement in most of their joints. For this reason, consistently using SMT on an EDS patient is counterintuitive. As with anything in life, there are exceptions to the rule and SMT can be useful in an EDS patient without a doubt. Mostly when it is used sparingly. However, as a general rule, EDS patients do not typically need to be seen for spinal manipulation several times per week for a long course of treatment. A treatment schedule of SMT like that can work in the reverse for and Ehlers Danlos patient and actually worsen the condition. 

However, more and more evidence-based chiropractors are utilizing exercise rehabilitation in their offices. Exercise rehabilitation is simply the chiropractic profession’s term to describe physical therapy without using the term since the term is ‘owned’ by the physical therapy profession. 

Now that we are clear on the terminology, it is important to understand that joints with extra movement in them benefit the most from strengthening and supportive activities. Activities such as weight training for example. Weight training can build stiffness, support, and strength in joints throughout the body. When a patient can ‘stiffen’ the joints through activities like this, they can decrease the movement in each joint. As the movement is decreased, so is the wear and so is the chronic pain. 

At the end of the day, as a chiropractor in Amarillo, TX, I can only tell you where you score on the Beighton Scale and I can educate you on Ehlers Danlos syndrome. To really explore EDS and a specific subtype, a patient needs to consult with a geneticist because EDS is a genetic disorder and they are most able to classify the subtype and do the necessary testing that might be needed. 

For the most part, EDS isn’t going to affect your life or your livelihood. This isn’t meant to scare. It is only meant to educate on a topic most simply don’t know much about. Education is how we should proceed and how we improve our patients’ lives. With the right diagnosis and the right treatment, EDS patients do very well and can live their lives fairly unaffected. 

 

To learn more visit www.ehlers-danlos.com or the Mayo Clinic’s website on EDS. 

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Dr. Jeff Williams, DC, FIANM is a Fellowship-trained Neuromusculoskeletal specialist and chiropractor in Amarillo, TX. As an Amarillo chiropractor, Dr. Williams treats chronic pain, disc pain, low back pain, neck pain, whiplash injuries, and more. Dr. Williams is also the host of The Chiropractic Forward Podcast. Through the podcast, Dr. Williams teaches fellow chiropractors and advocates weekly for evidence-based, patient-centered practice through current and relevant research. If you have any questions for Dr. Williams, feel free to email at [email protected]

Learn more about Dr. Williams and his practice at https://www.amarillochiropractor.com. Dr. Williams's full-time Amarillo chiropractic practice is Creek Stone Integrated Care at 3501 SW 45th St., Ste. T, Amarillo, TX 79109