treatment for hypermobility syndrome cover

treatment of joint hypermobility syndrome

case presentation

A 14-year-old female presents to your office with generalized fatigue, widespread joint pain and a history of multiple bilateral patellar dislocations.  After history and physical examination, you diagnose her with joint hypermobility syndrome.  What is the most appropriate approach for treatment?

A. Order an MRI for both knees
B. Counseling on diagnosis and expectations
C. Opioid medication for pain control
D. Refer to orthopedic surgery

introduction

Treatment of patients in an intensive interdisciplinary pain management program and is individualized based on the needs of each patient, physiotherapy, occupational therapy, psychology counseling, relaxation training, and medication management by a pediatric physiatrist if needed. 

Chronic pain is a major problem found in up to 90% of patients with joint hypermobility syndrome/hypermobile EDS [1]. The pain is generally complex, often generalized, and for people with JHS/hypermobile EDS often leads to poor physical, psychosocial, and overall function comparable to patients with fibromyalgia (FM) [2]. As in other chronic pain conditions, psychological symptoms are common both in EDS and JHS [3]. In a recent study on EDS where the majority had the hypermobility type, 51% of respondents reported high levels of anxiety and 20% reported high levels of depression [4]. Fatigue, sleep problems, and other subjective health complaints that influence quality of life are also more frequent in hypermobile EDS/JHS than in controls.

The management of patients with JHS can be challenging for the patient as well as the practitioner. As described by Simmonds and Keer , “patience, coupled with good communication and sensitive handling skills are required as physical problems are often longstanding and include secondary complications and psycho-social issues. [5].” There is no conclusive evidence in the literature regarding best practices for patients with JHS. However, one report highlights the importance of patient education, therapeutic exercise, and modification of work and lifestyle in the management of  JHS [6].

Thumb hyperextension in joint hypermobility syndrome JHS

Image 1: Thumb joint hyperextension in a patient with JHS.  Adopted from [21].

Physiotherapy emphasizes improvement of joint stability, proprioception, muscle strength, and overall cardiovascular fitness, because patients tend to be sedentary as a result of pain.   Physiotherapy is considered a cornerstone of treatment historically. However, reports of physiotherapy management are not always favorable. With patients reporting that physiotherapy has exacerbated symptoms and have focused on one single joint rather than treating them holistically [12-13].  Education, reassurance, closed chain strengthening and core stability exercises are recommended by experts and used frequently by physiotherapists [14-15]. While muscle strengthening and proprioceptive exercises have shown promising results for reducing pain and increasing strength in the knee, high quality intervention studies addressing mental health, widespread pain and fatigue are lacking [12]. 

Consideration of the role of exercise and the potential risks of high impact activity in deconditioned individuals has implications for exercise prescription in patients with JHS. A graded increase in activity was beneficial in the presented cases and seems appropriate for deconditioned patients with BJHS. Recently, a study by Celenay and Kaya demonstrated that a spinal stabilization program can decrease pain complaints, and improve postural stability and muscle endurance in women with JHS [7].  Vigorous activity may be relatively contraindicated in hypermobile individuals, but this restriction might be lifted once a sufficient training effect has been achieved [8]. Overall, maintenance of physical fitness is imperative for managing symptoms of JHS, especially activities that are focused on neuromusculoskeletal control; e.g., swimming, Tai Chi, pilates, yoga and dance.

Recurrent joint sprains, subluxations and low-force dislocations can occur with JHS.  These can be somewhat challenging for sports medicine providers because the imaging with radiographs and potentially magnetic resonance imaging (MRI) can be normal.  These injuries are addressed by a physiotherapist with use of supervised, progressive, motor control, strengthening and endurance exercise commencing in the normal physiological joint range and gradually moving into the patient’s hypermobile range [9]. Paced and graded activity programs establish a pattern of essential activities, including school and household tasks, before sports and recreation are added in a stepwise fashion. [10] Bracing, splinting and taping are sometimes used to assist with the child’s exercise program and functional performance, which also focuses on training balance and proprioception. Consultation with a podiatrist may assist with footwear advice and orthoses to address overpronation and improve lower limb alignment [11]. 

Occupational therapy focuses on postural biomechanics, the safe completion of functional daily tasks, pacing, and development of a daily schedule. Psychological interventions include strategies to self-manage pain and stress, including relaxation techniques, and the use of coping self-statements to help in times of distress. 

Treatment table for joint hypermobility syndrome JHS 1

Figure 2.  Managment strategies for JHS.  Adopted from [16].

Medication management is often focused on correcting poor sleep latency and providing as-needed relief for breakthrough pain that did not respond to the above nonpharmacologic approaches. For musculoskeletal pain, simple analgesics and modalities such as heat, ice and compression can be used.  One can use nonsteroidal anti-inflammatory drugs (NSAIDs) with caution, particularly if there are upper gastrointestinal issues.  Opiates are generally discouraged. In patients with neuropathic or chronic pain, some providers may use agents such as duloxetine or other SNRIs [16]. 

When pain is persistent and causes distress or limits function, psychological intervention to explore stressors and develop coping strategies for chronic pain, anxiety and related fatigue is helpful. Significant pain that interferes with daily activities and is refractory to regular therapies warrants referral to a pediatric pain specialist or pediatric pain services.

Treatment table joint hypermobility syndrome JHS

Figure 3.  Management strategies for JHS continued .  Adopted form [16].

Prolotherapy has a history of success treating ligament injuries, including patients with joint hypermobility.  Some of the rationale for using Prolotherapy for patients with EDS and JHS are that it has a high safety record, is comprehensive (all or most joints can be treated at each visit), is an outpatient procedure, is cost effective (compared to surgery), pain relief is often quick, and can provide joint stabilization [18].  There are studies and a review that show prolotherapy can cause decreased pain with hypermobile TMJ dysfunction [20].  Otherwise many case studies and case series exist supporting prolotherapy and joint hypermobility [18-19].

Surgical interventions are sometimes used.  Common operations include tendon transplant or transfer, capsulorrhaphy, arthroscopic surgery and arthroplasty.  The degree of stabilization and pain reduction, overall patient satisfaction, and duration of improvement are quite variable. Unfortunately, the weakness of hyperelastic joint tissue presents a poor healing prognosis, and surgery has proven to be widely unsuccessful in the hypermobile population.  When surgery is performed, the patient and physician should cautiously anticipate some improvement but expect less than optimal results [17].

When the child attains adolescence, due to the development of resistance of periarticular tissue, some symptoms can resolve. Hence in such children, restriction of physical activity is usually not advisable unless there is the involvement of the child in strenuous activities like dance, sports, or gymnastics. This would be based on each individual case.  

Recommendations for joint hypermobility syndrome JHS Treatment

Image 4: Strategies for managing hypermobility syndromes.  Adopted from [22].

Summary

In summary, the lack of awareness of JHS may delay the diagnosis as well as delay effective care, thus exacerbating symptoms.  Optimal care requires navigation of the complex array and interplay of associated medical conditions, necessitating multidisciplinary coordinated management.  Physiotherapy can play a role in the management and has been historically used in multiple aspects of care.  Each case requires an individualized approach and expectations and counseling play important roles.  More research is needed to optimize management of JHS.

CASE CONCLUSION

Optimal care requires navigation of the complex array and interplay of associated medical conditions, necessitating multidisciplinary coordinated management.  Counseling on diagnosis and expectations with this condition can play an important role in the management of joint hypermobility syndrome.  Imaging is often normal in adolescents with this condition and surgery shows less than optimal long term outcomes.  Opioid medications should be avoided in patients with JHS.

References

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  2. Rombaut, L.; Malfait, F.; De Paepe, A.; Rimbaut, S.; Verbruggen, G.; De Wandele, I.; Calders, P. Impairment and impact of pain in female patients with Ehlers-Danlos syndrome: A comparative study with fibromyalgia and rheumatoid arthritis. Arthritis Rheum. 2011, 63, 1979–1987.
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