What is the management plan for hypermobility spectrum disorder?

Management of Hypermobility Spectrum Disorder

The management of hypermobility spectrum disorder (HSD) should focus on therapeutic exercise, motor function training, and psychological support to improve pain, function, and quality of life.

Assessment and Diagnosis

Before implementing management strategies, proper assessment is crucial:

• Use validated tools like the Brighton Criteria or the 5-point questionnaire by Hakim and Grahame to confirm diagnosis 1
• Assess for joint hypermobility, skin findings, joint pain, and recurrent dislocations 2
• Evaluate for common comorbidities: anxiety disorders, chronic pain, fatigue, orthostatic intolerance, functional gastrointestinal disorders, and pelvic/bladder dysfunction 2

Core Management Strategies

1. Physical Therapy Interventions

Physical therapy is central to HSD management with evidence supporting:

• Therapeutic exercise:

  • Strengthening exercises focusing on proximal hip girdle and quadriceps muscles 3
  • Low-impact aerobic activities to improve cardiovascular fitness and reduce fatigue 3
  • Regular individualized daily exercise regimen that includes both strengthening and aerobic components 3

• Motor function training:

  • Proprioceptive training to improve joint position sense
  • Coordination exercises to address deficits 4
  • Balance training to prevent falls

• Manual therapy considerations:

  • Manual lymphatic drainage (MLD) for patients with puffy hands to improve hand function 3
  • Caution with aggressive joint mobilization that could exacerbate hypermobility

2. Occupational Therapy Interventions

• Address activities of daily living and participation restrictions 3
• Provide adaptive equipment and assistive technology as needed 3
• Consider ergonomic modifications at home/work 3

3. Psychological Support

Psychological interventions show benefit for patients with HSD:

• Cognitive behavioral therapy (CBT) to address pain catastrophizing, anxiety, and depression 5
• Pain management strategies including relaxation techniques and mindfulness 3
• Group therapy and psychoeducational programs to improve quality of life 3

4. Education and Self-Management

• Provide information about the nature of HSD, its causes, consequences, and prognosis 3
• Develop individualized management plans with short-term and long-term goals 3
• Teach pacing strategies ("small amounts often") to prevent symptom exacerbation 3
• Link exercise regimens to daily activities to promote adherence 3

Specific Symptom Management

For Pain Management:

• Graded exercise approach starting with levels within the individual's capability 3
• Sensory grounding techniques for acute pain episodes 3
• Avoid positions that promote prolonged positioning of joints at end range 3

For Fatigue:

• Energy conservation techniques
• Pacing of activities throughout the day
• Regular aerobic exercise has been shown to reduce fatigue 3

For Functional Movement Issues:

• Address optimal postural alignment at rest and during activities 3
• Encourage even distribution of weight in sitting, transfers, standing and walking 3
• Grade activities to increase time that affected limbs are used within functional activities 3

Cautions and Considerations

• Avoid:

  • Aggressive stretching that could increase joint laxity
  • Splinting may prevent restoration of normal movement and could exacerbate symptoms 3
  • High-impact activities that may increase joint stress

• Special considerations:

  • Progress is often slow and may be hampered by physical and emotional setbacks 1
  • Regular reassessment is necessary to adjust interventions based on response

Multidisciplinary Approach

Given the multi-systemic nature of HSD, coordination between healthcare providers is essential:

• Primary care physician to oversee overall care and manage comorbidities 2
• Physical and occupational therapists for movement and function
• Psychologist for mental health support
• Pain management specialist when needed

Monitoring and Follow-up

• Regular follow-up appointments to assess progress and adjust interventions
• Monitor for new symptoms or complications
• Reassess functional goals and adjust as needed

The evidence strongly suggests that a combination of physical therapy interventions (particularly therapeutic exercise and motor function training) alongside psychological support offers the most effective approach to managing HSD, with the goal of improving function, reducing pain, and enhancing quality of life 4, 5.