In medicine, we have a saying, “When you hear hoofbeats, think horses, not zebras”. In essence, it means that the more common, straightforward, and expected answer (or diagnosis) is OFTEN the right one. However, as we all know, OFTEN and ALWAYS are not the same. One of the trickier “zebras” of health care are hypermobility spectrum disorders (HDS), including Ehlers-Danlos Syndrome (EDS). In this specific demographic, sometimes the hoofbeats really are a zebra!
- Do you feel like your joints are unstable or move more than they should?
- Did you always ace the mobility tests in PE classes or sports warm-ups while everyone else seemed to struggle? Were you “double-jointed” as a kid?
- Do you have frequent injuries from seemingly benign movements, like ankle or wrist sprains?
You may have a hypermobility disorder.
Ehlers-Danlos Syndrome
Ehlers-Danlos Syndrome (EDS) (https://www.ehlers-danlos.com) is defined as a group of connective tissue disorders; it typically has a genetic component and can impact all systems of the body. Some of the more common types of EDS are vascular (vEDS), classical (cEDS), and hypermobile (hEDS). The most common form, and what we treat most often, is the hypermobile sub-type.
Hypermobile Ehlers-Danlos syndrome (hEDS) (https://www.ehlers-danlos.com/heds/) is mainly associated with global joint hypermobility, joint instability, and chronic pain. It accounts for ~90% of all EDS cases, impacting > 1 in 3000-5000 people. Like other complex multi-systemic issues, EDS is not always readily diagnosed, and individuals are often not educated on the underlying condition. Unlike other types of EDS, hEDS does not yet have a distinct genetic marker or test available, further complicating diagnosis. EDS has an average time to diagnosis of 10-12 years!
Hypermobile Spectrum Disorder
In contrast to hEDS, the term hypermobile spectrum disorder (HSD) indicates joint laxity or hypermobility and can encompass a much broader array of individuals and symptom-presentations. It does not have the same diagnostic criteria as hEDS, and is commonly used to capture individuals who do not qualify for an EDS diagnosis.
To-may-to, To-mah-to
There is no conclusive evidence of HDS and hEDS being the same or distinctly separate conditions. What we do know is that hypermobility syndromes exist on a spectrum, and at the end of the day, treatment is catered to individual needs rather than based solely on diagnosis.
How do I know if I’m hypermobile?
It is important to know that hypermobility can exist without any underlying pathology. Some individuals may demonstrate more flexibility (like many dancers or gymnasts) without any symptoms. Simply put, hypermobility just means that an individual’s joints can move more than normal.
A quick and easy screen you can do to check for hypermobility is called the Beighton Scale. It involves five different movement patterns, 4 of which are performed on both sides of the body, for nine possible points. Please don’t push any of these positions into pain!
- Elbow hyperextension > 10 degrees (2 points – 1 point per side): Reach your arm straight out and lock out your elbow. Does the arm seem to bend backward (beyond straight)?
- Pinky finger > 90 degrees extension (2 points – 1 point per side): Pulling your little finger straight back, can it bend to 90 degrees or further?
- Thumb to forearm (2 points – 1 point per side): Can you pull your thumb forward and down to touch the underside of your forearm?
- Knee hyperextension > 10 deg (2 points – 1 point per side): Stand up straight and lock out your knees. Do they bend backward beyond straight?
- Palms to floor w/ knees straight (1 point): Keeping your knees straight, bend straight down and reach for the floor. Do both palms rest fully on the ground?
Of the nine possible points, a score > or = to 5/9 in adults, 6/9 in children/pre-adolescents, and 4/9 in individuals over 50 is considered POSITIVE for hypermobility.
More than just hypermobile…
The underlying cause of hypermobility in HDS/hEDS is a connective tissue abnormality. This can also present as skin hyperextensibility and abnormal scarring.
Beyond hypermobility, these individuals often experience other symptoms, including chronic fatigue, headaches, gastrointestinal problems, and autonomic dysfunction. (Our autonomic nervous system is responsible for involuntary bodily functions, including heart rate, blood pressure, and digestion.)
Other common complaints in this population can include:
- Bloating or constipation
- Light-headedness or poor balance
- Decreased proprioception or body awareness
- Increased nerve pain
- Pelvic floor dysfunction
These conditions can even impact heart valves and teeth/palate development!
What can we do?
The most common complaints we see in physical therapy are pain, increased frequency of injuries, feelings of weakness or instability, and lack of body awareness. We also work with individuals with autonomic dysfunction, including POTS (postural tachycardia syndrome). First and foremost, physical therapy interventions focus on patient awareness and education. Many who are new to the world of HDS have no idea that so many of these vastly different symptoms are related and “normal” within this diagnosis. While there is no cure for HDS, numerous strategies exist to reduce symptoms and improve quality of life. After a thorough evaluation, we discuss goals and devise a plan TOGETHER to help manage symptoms, promote safety and longevity, and return to activity. Our goal is to help keep you Active For Life!
If Pain Is Preventing You From Being Active – WE CAN HELP!
For a thorough evaluation and individualized treatment, contact our Sherwood or Bethany clinic today and be seen in less than 48 hours.
