The Hypermobility Spectrum and Life as a Zebra

As a yoga instructor and former professional dancer, I’ve frequently heard people say they wish they were as flexible as I am. But the old adage holds true: the grass isn’t always greener on the other side. During my time as a physical therapist, I’ve learned that the flexibility that earned me praise in gymnastics, dance and yoga may contribute to musculoskeletal injuries, chronic pain, and symptoms of neurological, gastrointestinal, immunological and cardiopulmonary distress.

It probably seems obvious that being very stiff can limit your functioning and contribute to aches and pains. Decreased motion is referred to as hypomobility, and can occur due to a combination of genetics, disuse or aging. But what many people don’t realize is that human mobility exists on a spectrum, and being too far on either side can cause problems.

Opposite of those with limited mobility are people like me, who are hypermobile. While hypermobility can occur locally due to an injury, some of us have genetic influences that make our connective tissue more mobile generally. Excess motion can contribute to injuries like sprains and dislocations, and may require a different approach than “standard” rehabilitation, which tends to be geared toward those who are hypomobile, as hypomobility is more common than hypermobility. Hypermobility is often perceived as benign or even desirable, so it often isn’t a focus of healthcare professionals training. When hypermobile patients aren’t treated with an individualized program that meets their unique needs, they may not respond as expected, and can go on to have persistent issues that potentially worsen over time. It can be very helpful to identify hypermobility and find support for the unique deficits that come with hypermobility, like decreased proprioception (body sense) in order to prevent injury or ensure optimal healing.

Often my first indication that a patient is hypermobile is their choice of sitting position. Rather than sit in a chair in my office like the rest of my patients, they opt to sit on my treatment table in full lotus, “W” sit, or even legs straddled across the width of the table in the splits.

After noticing their choice of sitting postures, I often ask these patients if they have any “party tricks.” They usually light up with a smile and demonstrate their “double-jointedness,” or even their ability to “pop” bones out of place. Hypermobiles often discover these tricks when they are very young as their friends find such maneuvers entertaining.  They often gravitate toward activities like dance, gymnastics, or yoga because they can do them easily.

Luckily my PT’s had identified hypermobility at the age of 12 and issued a Pilates program to help help me stay strong throughout my hypermobile range of motion. When my dance career ended, I taught Pilates full-time, which allowed me to learn more about how to create support and stability for hypermobile joints by improving proprioception, motor control, strength and endurance, working first through a smaller, mid-ranges of motion where muscles can best support joint motion and progressing as needed to larger ranges of motion. In PT school, I learned more about how to distribute motion evenly across the kinetic chain to avoid over-stressing any one area, and various manual therapy techniques helped me to improve motion in specific areas that didn’t move as well, which is particularly important as we age and stiffen up (even hypermobiles stiffen with age, and Beighton Scores are known to decrease by the time we reach mid-thirties!)

If your PT suspects that your connective tissue is hypermobile, they may have you perform the following movements to determine your Beighton Score, a general screening tool for hypermobility. Feel free to follow along to self-assess, making sure not to force any excessive motion, moving only through a range that is normal and pain-free for your body:

1. Standing with legs straight, bend forward over your legs. If your palms can flatten to the floor, add 1 to your score.
2. Stretch both of your knees as far as they can go into extension (often called “locked knees.”) If your knees go beyond 180 degrees, add 1 point for each knee that can hyperextend to at least 10 degrees.
3. Straighten your elbows as far as they can go. If they go beyond 180 degrees (often called “double-jointed”) add 1 point for each elbow that hyperextends by at least 10 degrees.
4. Bend your thumb toward your forearm. If it touches, add 1 point to your score.
5. Bend your pinky backwards. (Update: due to efforts to be more consistent with grading Beighton Scores, it is advised that the hand be flat on a table and all other finger maintain contact with the table, not shown here.) Add 1 point for each pinky that can bend to at least 90 degrees relative to other fingers flat on the table.

If you scored any points, you have localized joint hypermobility. Those with 5 or more points (or 6+ for kids, 4+ for older adults) have generalized joint hypermobility, which was historically called benign joint hypermobility, as hypermobility can occur without any symptoms. The term Hypermobility Spectrum Disorder (HSD) is an updated term that indicates that local or general hypermobility may be symptomatic. Hypermobile Ehlers-Danlos Syndrome (hEDS) is a type of HSD that was first identified by French Drs. Ehlers and Danlos and is thought to be caused by a genetic mutation (although currently this has not been identified.) As such, diagnosis of hEDS is currently based on a mix of objective and subjective conditions, rather than genetic tests that identify other forms of EDS. You can find diagnostic criteria for hEDS here, although I like to remind folks that labels and diagnostic codes are intended to help your medical team to better create an individualized plan that meets your needs, and the difference between HSD and hEDS may not impact treatment. Clinically, HSD and hEDS are treated the same way– by addressing the individual’s complaints and reducing/managing associated risks.

Ideally, all humans will put some effort into maintaining health and wellness. In my experience, those efforts look a bit different for hypermobiles. Muscles that have to do more work to make up for lax ligaments will need to be highly conditioned in order to prevent fatigue, pain and even sensations of “stiffness.” While it might make sense for tighter bodies to stretch when they feel stiff, the sensation of stiffness is a protective mechanism, much like the sensation of pain. Perceived stiffness that is not paired with reduced range of motion doesn’t indicate a need for stretching so much as a need for other kinds of support. Being assessed by a PT who understands hypermobility can be very helpful to establish what specific areas need stretching, and which areas need improved stabilization, strength, blood flow, neurodynamics, sensory integration/proprioception, or recalibration of a sensitized system.

If you are generally hypermobile, exercise is important from a biomechanical perspective as well as neurophysiological one. Although the mechanisms are not entirely clear, hypermobiles tend to have sensitive systems that are prone to neuroimmune dysfunction and heightened pain sensitivity. Appropriate exercise helps to keep these systems running smoothly and downregulate sensitive/sensitized systems, making them less likely to cause chronic symptoms.

While hypermobility is most often considered a musculoskeletal issue, hypermobiles do have increased risk of certain medical conditions that are beyond the scope of this blog, such as gastrointestinal issues, chronic pain and chronic pelvic pain, migraines, chronic fatigue, pelvic organ prolapse, autonomic nervous system dysfunction (dysautonomia/POTS) or heart conditions like valve prolapse or aortic dissection. Therefore, it’s important to find medical professionals who understand the systemic effects of hypermobile connective tissue and can help create a proactive plan to minimize risks and improve symptoms. Finding healthcare professionals  (HCP) familiar with these issues may also reduce the harmful effects of being invalidated, misunderstood, or misdiagnosed by HCP’s who simply aren’t aware of these issues and are more likely to ignore/dismiss them or misdiagnose them as more common conditions.

In medicine, there is a saying: “If you hear hoofbeats, think horses, not zebras.” It is intended to remind medical professionals not to overcomplicate things. Hypermobile patients are often the exception to the rule, as we often present with less common issues, and sometimes medically complex conditions. As such, we are frequently referred to as “zebras.” In my own experience, being identified as a zebra was a necessary step in understanding my body’s unique needs, building my support network, and managing my symptoms. Those on the hypermobility spectrum may appear to be healthy, which makes it difficult for others who are unfamiliar with HSD to understand why they struggle with fatigue, pain, “stiffness” or malaise. I hope that sharing my story raises awareness and helps those with hypermobility-related symptoms find the support that they need.

For further information on diagnosis of Hypermobility Spectrum Disorders/Ehlers Danlos Syndromes, you may consider the 2017 EDS International Classification.

Click on these links for more information on Hypermobility Spectrum Disorder and associated symptoms.

I’d love to hear your thoughts about hypermobility! Feel free to share in the comments section below, or contact Dr. Lewan directly.