Pain management in the Ehlers–Danlos syndromes

Abstract

Chronic pain in the Ehlers–Danlos syndromes (EDS) is common and may be severe. According to one study, nearly 90% of patients report some form of chronic pain. Pain, which is often one of the first symptoms to occur, may be widespread or localized to one region such as an arm or a leg. Studies on treatment modalities are few and insufficient to guide management. The following is a discussion of the evidence regarding the underlying mechanisms of pain in EDS. The causes of pain in this condition are multifactorial and include joint subluxations and dislocations, previous surgery, muscle weakness, proprioceptive disorders, and vertebral instability. Affected persons may also present with generalized body pain, fatigue, headaches, gastrointestinal pain, temporomandibular joint pain, dysmenorrhea, and vulvodynia. Pain management strategies may be focused around treating the cause of the pain (e.g., dislocation of a joint, proprioceptive disorder) and minimizing the sensation of pain. Management strategies for chronic pain in EDS includes physical therapy, medications, as well as durable medical equipment such as cushions, compressive garments, and braces. The different modalities are discussed in this paper. © 2017 Wiley Periodicals, Inc.

Urogenital and pelvic complications in the Ehlers‐Danlos syndromes and associated hypermobility spectrum disorders: A scoping review

“…Dyspareunia in the general population has been reported in 7.5% of sexually active women, with some geographic variation (3.6% in Australia, 18.6% in Brazil) and higher rates among women post‐partum (24%). One prior review of pain in EDS also noted the occurrence of vulvodynia and dyspareunia. Previous research has found that less than a third of women with pelvic pain report it to a medical provider, thus the already high rates found in this review may underrepresent this problem.…”

Joint Hypermobility among Female Patients Presenting with Chronic Myofascial Pelvic Pain

“…There are many proposed theories as to the mechanisms of pain in HSD, many of which have been drawn from other pain conditions. Available data suggest increased pain may correlate with repeat subluxations and dislocations, soft‐tissue injury, and/or low nocturnal sleep quality . Some studies suggest that patients with HSD experience hyperalgesia and central sensitization, similar to that experienced with fibromyalgia .…”

Hypermobile type Ehlers‐Danlos syndrome associated with hypogammaglobulinemia and fibromyalgia: A case‐based review on new classification, diagnosis, and multidisciplinary management

“…Nonopioid oral analgesics such as acetaminophen, nonsteroidal anti‐inflammatory (NSAID) or cyclooxygenase‐2 (COX2) inhibitor should be maximized first. Topical agents and muscle relaxants can also be helpful . Benzodiazepines should be used only cautiously for short‐term and are poor choices for long‐term use, particularly risky when coprescribed with opioids .…”

“…For patients with neuropathic pain, regiment can include a tricyclic antidepressant, serotonin‐norepinephrine reuptake inhibitor, and/or an anti‐epileptic agent. Opioids are rarely needed for the treatment of chronic musculoskeletal pain and are not recommended for neuropathic pain . Narcotics (Opioids and tramadol) should be reserved for acute pain episodes or for those who are inadequately managed after trial of all of the above approaches and should be added on to the above regimen in the lowest effective doses rather than replacing nonopioids.…”