To mark the halfway point of November, CRPS awareness month, I began a short series of posts to help dispel some of the confusion that clouds any discussion of this rare disease. These posts are focusing on five specific questions that tend to occur in any of my conversations about Complex Regional Pain Syndrome; what it’s called, what it is, how to test for (or diagnose) it, how to treat it, and how to live with it.
The focus of today’s post will be on the third question, the diagnosis of this rare autoimmune and neuro-inflammatory disease. It might surprise you, in today’s age of genetic and medical testing, that there are no medical tests that would provide a clear diagnosis of CRPS. Not yet, at any rate.
An article in a medical journal last year, entitled Complex Regional Pain Syndromes Treatment and Management, clearly stated that:
No specific diagnostic tests identify the presence of CRPS and no objective guidelines verify its existence.
The current criteria for diagnosing CRPS are based mainly on physical examinations and a careful analysis of patient history”.(1)
In short, nothing has changed in the realm of CRPS diagnosis since this other medical journal article was published, in 2017:
There is currently no gold standard for the diagnosis and treatment of CRPS.
History and physical examination are the cornerstones for appropriate diagnosis and management.(2)
To be perfectly clear, I’m not a nurse, physician, or other type of licensed healthcare professional. My field is biomedical ethics, or bioethics, and I’m not qualified to diagnose or treat anyone – not even myself. For medical advice or opinions, go see a doctor!
For that reason, I’m not going to delve into what a doctor would be looking for – in a patient’s medical history, clinical signs, and symptoms – to make a diagnosis of CRPS. That doesn’t mean, though, that I have nothing to say on this topic! Come on, you must know me better than that by now; I’m rarely without words ‘-)
Can you recall what it was about CRPS that I wanted to try to dispel in this little series of posts, for CRPS awareness month? That’s right, I wanted to work through some of the confusion surrounding this disease. Well, let’s talk about some of the reasons for which diagnosing CPRS can be confusing…
First off, it’s important to specify that Complex Regional Pain Syndrome isn’t only confusing to patients and their loved ones. It has also been confusing doctors and researchers for hundreds of years now! Even medical journals say that CRPS “is a disease that has perplexed clinicians and basic scientists alike”.(3)
This is particularly unfortunate for CRPS patients, because: “An early diagnosis is mandatory for therapeutic success and functional outcome.”(4) My diagnosis was delayed for three months, because one specialist physician refused to even consider that something was wrong with my right hand and arm, after a simple fracture.
That delay may well have made the difference for me, between the likely-permanent disease that I have now and the possibility of it resolving; resolving is another word for it just going away. Yes, you read that correctly. Had I been diagnosed with CRPS when my symptoms first presented, and begun treatment immediately, there’s a good chance that my CRPS would have simply gone away after a few months.
I now have to live with that knowledge, each and every day. That’s what pushes me to raise awareness of this condition, and to advocate for other patients… so that no one else will have to live with knowing that a few weeks or months could have prevented their CRPS from becoming entrenched; from it changing into a life-long and life-changing disease.
The International Association for the Study of Pain (IASP) provides guidance on the diagnosis of CRPS to physicians, a list of symptoms named the Budapest Criteria(1). This very detailed information isn’t necessarily available on the public portions of the IASP’s website, or other public information websites.
I’ve been able to access it on a medical website, to which I have access because of my work in bioethics. This particular website, from which I’ve pulled the following quote, bears a warning that “This site is intended for healthcare professionals”(1):
A clinical diagnosis of CRPS can be made when the following criteria are met:
A. Continuing pain that is disproportionate to any inciting event
B. At least 1 symptom reported in at least 3 of the following categories:
i Sensory: Hyperesthesia or allodynia
ii Vasomotor: Temperature asymmetry, skin color changes, skin color asymmetry
iii Sudomotor/edema: Edema, sweating changes, or sweating asymmetry
iv Motor/trophic: Decreased range of motion, motor dysfunction (eg, weakness, tremor, dystonia), or trophic changes (eg, hair, nail, skin)
C. At least 1 sign at time of evaluation in at least 2 of the following categories:
i Sensory: Evidence of hyperalgesia (to pinprick), allodynia (to light touch, temperature sensation, deep somatic pressure, or joint movement)
ii Vasomotor: Evidence of temperature asymmetry (>1°C), skin color changes or asymmetry
iii Sudomotor/edema: Evidence of edema, sweating changes, or sweating asymmetry
iv Motor/trophic: Evidence of decreased range of motion, motor dysfunction (eg, weakness, tremor, dystonia), or trophic changes (eg, hair, nail, skin)
D. No other diagnosis better explaining the signs and symptoms”(1)
As you can see, that information is clearly intended for physicians! Here’s a sample of what the IASP does provide on its public pages, for the diagnosis of Complex Regional Pain Syndrome:
“The onset of symptoms usually occurs within one month…
The pain is frequently described as burning and continuous and is exacerbated by movement, mechanical or thermal stimulation, or stress.
The intensity of pain may fluctuate over time”(5)
So with CRPS we have different patients who might have different symptoms, and those symptoms change over time; within each individual patient. Confusing, isn’t it?
After the exclusion of other causes, CRPS is characterised by a typical clinical constellation of pain, sensory, autonomic, motor, or trophic symptoms which can no longer be explained by the initial trauma.
These symptoms spread…
If CRPS is not improved in the acute phase and becomes chronic, the visible symptoms change throughout because of the changing pathophysiology; the pain, however, remains.”(6)
My case is a good illustration of that, because my disease-related cognitive issues cropped up well over three years after my original symptoms began. You can read about MCI, mild cognitive impairment, here.
Overall, CRPS is considered to be a diagnosis of exclusion; before confirming that a patient has CRPS, all other possible diagnoses must first be eliminated. This processed is called a differential diagnosis.
Because CRPS can present with such a diverse range of clinical signs and symptoms, there are a number of other conditions that must be eliminated to confirm the diagnosis:
the differential diagnosis includes other neuropathic conditions, as well as a host of metabolic, systemic, vascular, and rheumatological disorders.
Excluding other treatable but serious causes is indicated even in cases that present with the classic signs and symptoms.”(1)
I’m not sure whether I’ve helped dispel any of the confusion around the diagnosis of CRPS, but I hope that I’ve explained why there’s still significant confusion surrounding this particular question! Sadly, this confusion often means delays for patients, in obtaining appropriate care, time during which those people are left suffering. Complex Regional Pain Syndrome:
is widely considered to be the single worst source of pain – ranking higher on pain scales than childbirth, cancer and amputation.
Yet the condition is rare and can be difficult to recognize.
As a result, patients with CRPS… according to the National Institutes of Health – can search for an average of approximately four years before being properly diagnosed with the condition”.(7)
Even though I waited – and fought for – a diagnosis during a period of almost three months, there have been patients who were left without treatment for much longer than that. Once again, my outlook is that my situation ‘could be so much worse’.
There is plenty that I’m thankful for, on a daily basis, in my own patient journey. The continuing support of family and friends, that’s so very important; thank you all so much! On the healthcare side, my family physician and physical therapist have been – and continue to be – extremely supportive and helpful.
Last, but definitely not least, is the multi-disciplinary pain management unit at a university hospital. The team there has been absolutely fantastic, refusing to give up on me at any point during the three-plus years that they’ve been treating me. Not only their medical staff, but also their psychology team and others; all specialized in the management of chronic pain. Thank you all so very much!
And you, my faithful readers… what can I say? It’s always a true pleasure to hear from you, to get your feedback on what I write and what I’m doing. I’m still saddened by the fact that I’ve had to disable the comments feature on the blog, because of all the obscene spam that was being posted from what seems to be Chinese and Russian sources. Please keep reaching out via Instagram and Twitter!
You make this worthwhile – you have my heartfelt thanks ’-)
Keep an eye out for more orange this month, the awareness colour for CRPS!
References
(1) Gaurav Gupta and Arthur Carminucci. Edited by Stephen A Berman. Complex Regional Pain Syndromes Treatment and Management. Medscape. Online update 20 Jun 2018. Accessed 17 Nov 2019. Web:
https://emedicine.medscape.com/article/1145318-treatment#d5
(2) Krishna D Bharwani, Maaike Dirckx, Frank JPM Huygen. Complex regional pain syndrome: diagnosis and treatment. BJA Education. 2017: 17(8):262-268. August 2017. doi.org/10.1093/bjaed/mkx007. Web:
https://academic.oup.com/bjaed/article/17/8/262/3957912
(3) Russo, M.A., Fiore, N.T., Vreden, C. et al. Expansion and activation of distinct central memory T lymphocyte subsets in complex regional pain syndrome. J Neuroinflammation. 2019;16(63). Online 18 Mar 2019. doi: 10.1186/s12974-019-1449-9. Accessed 17 Nov 2019. Web:
https://jneuroinflammation.biomedcentral.com/articles/10.1186/s12974-019-1449-9
(4) Iolascon G, de Sire A, Moretti A, Gimigliano F. Complex regional pain syndrome (CRPS) type I: historical perspective and critical issues. Clin Cases Miner Bone Metab. 2015;12(Suppl 1):4–10. Online 07 Apr 2016. doi:10.11138/ccmbm/2015.12.3s.004. Accessed 07 Nov 2019. Web:
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4832406/
(5) International Association for the Study of Pain (IASP). Classification of Chronic Pain, Second Edition (Revised). Section Ai Relatively Generalized Syndromes. 2012 update. Online. Accessed 07 Nov 2019. Web:
https://www.iasp-pain.org/PublicationsNews/Content.aspx?ItemNumber=1673
(6) Frank Birklein, Violeta Dimova. Complex regional pain syndrome-up-to-date. PAIN Reports; PAIN Clinical Updates. Nov/Dec 2017. Volume 2, Issue 6, page e624. doi: 10.1097/PR9.0000000000000624. Online. Accessed 17 Nov 2019.
https://journals.lww.com/painrpts/Fulltext/2017/12000/Complex_regional_pain_syndrome_up_to_date.8.aspx?WT.mc_id=HPxADx20100319xMP
(7) CRPS rank higher on pain scales than childbirth, cancer, amputation. Medical Life Sciences News. News-Medical.Net. Online 25 Mar 2016. Accessed 17 Nov 2019. Web:
https://www.news-medical.net/news/20160325/CRPS-rank-higher-on-pain-scales-than-childbirth-cancer-amputation.aspx