Nicole Frost is a physiotherapist working with clients experiencing complex manifestations of connective tissue disorders such as Ehlers Danlos Syndrome. Nicole has written this timely and very important piece on clinical thinking and reflection when working with people experiencing ongoing pain. Our thanks to Nicole for sharing her expertise and knowledge with us, so that we can share it with you.
“It’s all just semantics”. Isn’t it?
Depending on the context, and the source of the definition, the term “chronic pain” may be described as:
- Pain that has continued for an extended time; or more specifically
- “a pain condition [that] persists or recurs for longer than three months”; or more specifically still
- “pain that persists past normal healing time and hence lacks the acute warning function of physiological nociception” (1, 2)
Whenever we talk about chronic pain, aren’t the specifics of definition three implied? Maybe. Or is there a dangerous assumption involved in automatically considering these definitions to be interchangeable?
Not every pain state…
Understanding the mechanisms that contribute to a patient’s symptoms and disability is an important part of planning effective management strategies. Developing an effective therapeutic alliance with a patient also depends on working towards a shared understanding of the patient’s situation. The understanding that pain can persist in the absence of actual tissue damage has certainly expanded therapeutic options. But not every pain state that persists for “an extended time” or “longer than three months” “lacks the acute warning function of physiological nociception”. Oncology patients are understood to often experience pain related to the cancer or to treatment effects with the potential for ongoing nociceptive inputs among the multiple mechanisms potentially contributing to their pain experience. This is recognised with the existence of a specific subclassification of chronic pain in the ICD – 11 for “chronic cancer related pain”.
Another group of patients very commonly seeking care for chronic pain are those with inherited disorders of connective tissue falling into the category of hypermobility syndromes, such as Marfan Syndrome, Loeys Dietz and the Ehlers Danlos Syndromes (EDS). Among a wide range of multisystemic symptoms, pain is a dominant complaint in this patient population (3-5). Although the disorders are considered rare, with EDS, the most common hypermobility disorder, estimated to effect around 2% of the population (6), patients with these disorders are probably not a rarity in musculoskeletal healthcare practices and pain clinics, having been estimated to represent 30% of such patients (7). These disorders are genetically inherited conditions resulting in structural differences in the connective tissue throughout the patient’s body. These connective tissue differences predispose the patient to potential issues across multiple body systems with higher rates of disorders of gastrointestinal, urogenital, cardiovascular, neurological and possibly immunological function noted alongside a predisposition to widespread recurrent musculoskeletal injuries. Research suggests that processes of central pain sensitisation can begin very early in the life of patients with hypermobility syndromes (8) and consideration of this is essential to the most effective management. Often, however, these patients’ pain symptoms may also continue to provide the “warning function of physiological nociception” with many potential sources of ongoing nociceptive input related to the patient’s increased tissue fragility, risk of recurrent subluxations and dislocations, nerve entrapments and central neurological injury due to spinal instability, dural injury etc. An added management challenge arises from the lack of recognition of these conditions among patients and clinicians alike resulting in significant delays in obtaining a diagnosis. Patients typically face a diagnostic odyssey spanning an average of more than 10 years, as they seek treatment for a vast array of seemingly unconnected symptoms without an understanding of the underlying cause (9).
More than semantics
It’s not just semantics. “Chronic” long-term pain isn’t always “chronic pain” that “lacks the acute warning function of physiological nociception”. When managing patients with known hypermobility syndromes, or indications of a possible undiagnosed hypermobility syndrome*, ignoring the potential wide range of ongoing nociceptive sources could undermine the patient’s psychological safety, and hence the therapeutic alliance, as well as threatening their physical safety.
Nicole is a physio working with clients experiencing complex manifestations of connective tissue disorders. She facilitates the Ehlers Danlos Society Project ECHO professional development program for Australasian Allied Health Practitioners and is passionate about advocating for patients with connective tissue disorders and seeing therapy programs developed where the best science is applied together with a thorough understanding of each individual client. Nicole works at flex-ability physio in Bulli
*e.g. significantly greater than normal joint mobility, skin hyper- extensibility or fragility, easy bruising or a history of such symptoms that may be masked by age- and injury – related changes. See Malfait et al (2017) for an overview of the 13 subtypes of EDS (10)
- Treede R-D, Rief W, Barke A, Aziz Q, Bennett MI, Benoliel R, et al. A classification of chronic pain for ICD-11. PAIN. 2015;156(6):1003-7.
- Treede R-D, Rief W, Barke A, Aziz Q, Bennett MI, Benoliel R, et al. Chronic pain as a symptom or a disease: the IASP Classification of Chronic Pain for the International Classification of Diseases (ICD-11). pain. 2019;160(1):19-27.
- Syx D, DeWandele I, Rombaut L, Malfait F. Hypermobility, the Ehlers-Danlos syndromes and chronic pain. Clinical and experimental rheumatology. 2017;35 Suppl(107(5)):116-22.
- Malfait F, Colman M, Vroman R, De Wandele I, Rombaut L, Miller RE, et al., editors. Pain in the Ehlers–Danlos syndromes: Mechanisms, models, and challenges. American Journal of Medical Genetics Part C: Seminars in Medical Genetics; 2021: Wiley Online Library.
- Voermans NC, Knoop H, Bleijenberg G, van Engelen BG. Pain in Ehlers-Danlos syndrome is common, severe, and associated with functional impairment. Journal of pain and symptom management. 2010;40(3):370-8.
- Tinkle B, Castori M, Berglund B, Cohen H, Grahame R, Kazkaz H, et al. Hypermobile Ehlers–Danlos syndrome (a.k.a. Ehlers–Danlos syndrome Type III and Ehlers–Danlos syndrome hypermobility type): Clinical description and natural history. American Journal of Medical Genetics Part C: Seminars in Medical Genetics. 2017;175(1):48-69.
- Connelly E, Hakim A, Davenport S, Simmonds J. A study exploring the prevalence of Joint Hypermobility Syndrome in patients attending a Musculoskeletal Triage Clinic. Physiotherapy Practice and Research. 2015;36:43-53.
- Scheper M, Pacey V, Rombaut L, Adams R, Tofts L, Calders P, et al. Generalized hyperalgesia in children and adults diagnosed with hypermobility syndrome and Ehlers‐Danlos syndrome hypermobility type: A discriminative analysis. Arthritis care & research. 2017;69(3):421-9.
- Halverson CME, Cao S, Perkins SM, Francomano CA. Comorbidity, misdiagnoses, and the diagnostic odyssey in patients with hypermobile Ehlers-Danlos Syndrome. Genetics in Medicine Open. 2023:100812.
- Malfait F, Francomano C, Byers P, Belmont J, Berglund B, Black J, et al. The 2017 international classification of the Ehlers–Danlos syndromes. American Journal of Medical Genetics Part C: Seminars in Medical Genetics. 2017;175(1):8-26.