Are you “Double Jointed”? You may want to read this…

 1. Can you now (or could you ever) place your hands flat on the floor without bending your knees?

2. Can you now (or could you ever) bend your thumb to touch your forearm?

3. As a child did you amuse your friends by contorting your body into strange shapes OR could you do the splits?

4. As a child or teenager did your shoulder or kneecap dislocate on more than one occasion?

5. Do you consider yourself double-jointed?

Endorsement of two or more questions suggests generalised joint hypermobility.

These are the 5 questions outlined in the new “Self-Reported Five-Part Questionnaire for Joint Hypermobility”.  My favorite part about it is that it very politely asks a very real question that happens in the clinic:  “Were you the ‘check out my cool trick’ person at parties?”

You know the person- maybe it was you.  Your thumb touches your forearm, you can dislocate your shoulder at will, your skin is really, really stretchy.   Remember when you put your feet behind your head and walked on your hands?  Everyone loves that trick.   

We always called it “double jointed”.  It was just a passing phrase to describe those freakishly flexible folks. 

Just in case there are any false assumptions out there: There is no such thing as “double joints”.  It’s the same joint as everyone else, the difference is that that person (your) collagen is extra stretchy; all the ligaments and tendons that are supposed to be restricting joint movement simply give way. 

The gold standard for assessing Joint hypermobility is the Beighton scale.  A score >6 indicates “Generalized Joint Hypermobility”, now identified as Hypermobility Spectrum Disorder (HSD).  Bear in mind that an individual may score higher on this scale if they have a background in dance, gymnastics, etc.  It does not necessarily mean that there is a pathology- although certainly, those sports will attract people who are excessively flexible.  

For more information, check out the Ehlers-Danlos Society.

 In other words, the Beighton scale is not the final say in diagnosis; rather, it opens the door to further medical examination.  In order to land on the Spectrum, there is a cluster of symptoms and genetic diagnoses that will point to a collagen production dysfunction. Ehlers Danlos Syndrome falls at the far end of this spectrum, requiring specific criteria in order to be diagnosed.

Collagen is not isolated to your skin.  Collagen is a protein that creates the binding in connective tissues. It’s what makes youthful skin so firm and smooth and it’s what gives Jell-o that wiggly jiggle.   Collagen is also responsible for anchoring your internal organs and the elasticity of your cardiovascular system (think blood pressure). 

 For individuals on the Hypermobility Disorder Spectrum, there is a very real chance that they experience more than REEEAAAALLY flexible joints.  Given collagen’s roll in, well, every single system that makes up the human anatomical experience; its dysfunction can create disruption in blood flow, digestion, immunity, respiration, hormone regulation, and sleep (to name a few).  While severity and predictability vary immensely (remember: it’s a spectrum disorder), the course of HSD has a general trend which is currently being proposed by the Ehlers-Danlos Society as 3 phases:

1. Hypermobility (Childhood/Adolescence):

   •  Contortionism and “Party tricks”

   • Delayed motor development

   • Poor coordination

   • Fatigue 

   • GI Problems

   • Intermittent pain- most likely associated with injuries such as sprains/dislocations.  

2. Pain (Early adulthood,20’s-30’s)

   • Development of recurrent joint, muscle and tendon injuries or pain.

   • Peripheral neurogenic (nerve) pain

   • Sleep disorders 

   • Urogenital problems

   • Headache

   • Worsening fatigue

   • GI Disorders

   • Orthostatic intolerance (dizziness with standing up)

3. Stiffness (Later adulthood 40+)

   1. “Negative” Beighton Score (<5)

   2. Fibromyalgia or generalized chronic pain

   3. Disabling fatigue

   4. Central sensitization

   5. Multiple visceral problems- oftentimes with difficulty achieving a solid diagnosis.  

 This list is not comprehensive, nor does it do justice to the complex interplay of the symptoms.  Let’s take this example:

  After an hour of walking around the grocery store, you wait patiently in line to check out. Even though it was a leisurely stroll through the store, you feel worn out and your knees are starting to hurt.  As you stand there, you grow increasingly anxious but you have no idea why.  It feels like your heart is thumping faster than it should and your gut feels a little dicey. 

 In a healthy system, the body is able to counteract the affects of gravity on the blood pooling in the legs.  Veins have just the right resiliency to keep blood moving up to the heart.  In a system where veins and arteries are just a little bit extra stretchy, there isn’t quite enough pressure to efficiently push the blood up to the heart. 

 What to do?  Your body, being the self-sufficient system it is, will work to elevate blood pressure some other way- namely through dumping adrenaline into the system.  That kicks up the heart rate and creates vasoconstriction. 

 Adrenaline also makes you feel like you are being chased by a tiger.   So as you stand in the grocery store on locked out knees (aching from being so unstable) and fatigued (from having to work just that much hard to walk on a wobbly body), your body is tapping into the Fight or Flight (Sympathetic) Nervous System in order to keep you from passing out.  

You may have always known about your hypermobile joints. If you didn’t know,however, that there is a physiologic driver that affects your entire system, what impact will these unpredictable ebbs and flows of fatigue, pain, and anxiety have on your mental health? Relationships? Quality of life?

The future of hEDS and HSD is becoming increasingly interdisciplinary. Physicians from every field will have to coordinate care due to the complex interplay. Physical therapy continues to be the cornerstone for treatment: Thoughtfully planned and (very) carefully progressed strength training has to be combined with education in pain management and energy conservation. This is the domain of a great PT.

The key is to start with recognition.

In Part II, we’ll start talking more specifically about how exercise makes a difference for HSD.

If you think that you or a family member may fall on the spectrum, please contact your health care provider as well as set up a physical therapy appointment.

For more resources go to Ehlers-Danlos.com.