Multidisciplinary Chronic Pain Management Strategies in Patients with Ehlers–Danlos Syndromes

Abstract

Ehlers–Danlos syndromes (EDS) are a group of disorders characterized by abnormal connective tissue affecting several organ systems. Patients with the hypermobile type of EDS (hEDS) commonly experience chronic pain which can present as musculoskeletal pain, fibromyalgia, neuropathic pain or abdominal pain. The effective management of chronic pain in hEDS patients is a challenge. This study reviews two cases of chronic pain in hEDS patients and the multimodal treatment regimen used along with peripheral nerve stimulation for shoulder and knee pains, never before reported in hEDS patients. Since hEDS associated chronic pain is multifactorial in origin, treatment requires a multidisciplinary approach which includes physical therapy, psychotherapy, pharmacotherapy and interventional pain procedures such as trigger point injections, peripheral nerve block, radiofrequency ablation and peripheral nerve stimulation.

Plain language summary

Ehlers–Danlos Syndromes (EDS) are a group of disorders that affects the connective tissues that supports skin, muscles and organs. Patients with the hypermobile subtype of EDS (hEDS) often experience chronic pain of multiple locations including the muscles, joints, nerves, abdomen, head or generalized pain. Treating chronic pain in patients with hEDS is challenging. In this study, we review the treatment of chronic pain in two patients with hEDS using multiple therapies including the novel use of peripheral nerve stimulation in this patient population. Chronic pain in hEDS patients is caused by multiple different mechanisms and therefore, the treatment of this pain requires multiple different therapeutic interventions such as medications, physical therapy, psychotherapy and minimally invasive procedures such as peripheral nerve stimulation.

Keywords::

• chronic pain
• Ehlers–Danlos syndromes
• joint hypermobility
• multidisciplinary therapy
• pain management
• peripheral nerve stimulation

Author contributions

PA Gullapalli was responsible for literature review, data analysis, drafting and revision of the manuscript. S Javed was responsible for study conception, acquisition of data and revision of the manuscript.

Financial & competing interests disclosure

The authors have no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties.

No writing assistance was utilized in the production of this manuscript.

Informed consent disclosure

The authors state that they have obtained verbal and written informed consent from the patients for the inclusion of their medical and treatment history within this case report.