Joint Hypermobility Syndrome

Hypermobility vs hypermobility syndrome

HSD and hEDS are managed similarly; the distinction mainly affects research and family counseling. Other inherited connective tissue disorders, such as Marfan syndrome and vascular or classical EDS, must be considered because they carry additional risks.

The Beighton score

A 9-point test of flexibility, one point for each:

• bending the little finger back beyond 90 degrees (each hand, 2 points)
• bending the thumb back to touch the forearm (each side, 2 points)
• hyperextending the elbow more than 10 degrees (each side, 2 points)
• hyperextending the knee more than 10 degrees (each side, 2 points)
• placing palms flat on the floor with knees straight (1 point)

A score of 5 or more in adults (6 or more in children, 4 or more after age 50) suggests generalized hypermobility. The score does not measure symptoms, and some people have hypermobility in only a few joints.

Symptoms

• joint pain, often in the knees, ankles, hips, shoulders, hands and back, worse after activity
• frequent sprains and soft tissue injuries
• subluxations (partial dislocations) and dislocations, especially of the shoulder, kneecap and fingers
• clicking or clunking joints
• muscle aches, stiffness and early tiredness of muscles
• poor balance and clumsiness
• fatigue, often significant
• flat feet

Associated symptoms

Many people also have:

• dizziness and fainting from postural orthostatic tachycardia syndrome
• digestive problems such as reflux, IBS and constipation
• bladder problems and pelvic floor weakness
• anxiety and chronic widespread pain
• soft, slightly stretchy skin and easy bruising

Causes

Hypermobility reflects the structure of collagen and other connective tissue proteins, which are partly determined by genetics. It often runs in families. Training such as dance or gymnastics can increase flexibility in specific joints. Hypermobility tends to decrease with age.

Diagnosis

There is no blood test for HSD or hEDS. A doctor, often a rheumatologist or geneticist, will:

• measure the Beighton score and examine joints, skin and posture
• ask about joint problems, pain, injuries and family history
• look for features of other conditions, including heart murmurs, eye problems and very fragile skin
• arrange genetic testing or an echocardiogram if Marfan syndrome, vascular EDS or other types are suspected

Treatment

Exercise and physical therapy

The most effective treatment is targeted strengthening to improve muscle support and joint control:

• a physical therapist experienced in hypermobility can design a graded program
• focus on core stability, proprioception (joint position sense) and balance
• low-impact activities such as swimming, cycling and Pilates are good choices
• avoid overstretching hypermobile joints

Pain and fatigue management

• pacing activity to avoid boom-and-bust cycles
• simple pain relief such as acetaminophen (paracetamol) or NSAIDs, following the label dose; opioids are generally avoided
• sleep improvement
• cognitive behavioral therapy and pain management programs for chronic pain

Practical support

• braces, taping or splints for unstable joints during flare-ups or specific activities
• supportive footwear and orthotics for flat feet
• occupational therapy for hand pain and daily tasks

Treating associated conditions

POTS, gut and bladder symptoms, and anxiety each have specific treatments.

Frequently asked questions

What is joint hypermobility syndrome?

Joint hypermobility syndrome, now usually called hypermobility spectrum disorder, is when unusually flexible joints cause symptoms such as pain, frequent sprains, dislocations and fatigue. Being flexible alone, without symptoms, is not a disorder.

What is the difference between hypermobility syndrome and EDS?

Hypermobile Ehlers-Danlos syndrome has stricter diagnostic criteria, including skin features, other body findings or family history. Hypermobility spectrum disorder describes symptomatic hypermobility that does not meet those criteria. Treatment for both is similar.

Is being double-jointed bad?

Usually not. Many flexible people never have problems, and flexibility can be an advantage in some sports. It matters only when it causes pain, instability or injuries, or is part of a wider connective tissue disorder.

What exercise is best for hypermobility?

Strengthening and joint stability exercises, guided by a physical therapist, are best. Low-impact activities such as swimming, cycling, Pilates and controlled strength training help. Avoid pushing joints to their extreme range.