Yesterday I began a short series of daily posts, to mark the middle of CRPS awareness month. There is a great deal of confusion about this rare disease, so I thought it would be helpful to dispel some of it. Confusion exists about what it’s called, what it is, how to test for (or diagnose) it, how to treat it, how to live with it…
The first post explored the many names for CRPS, and explained why Complex Regional Pain Syndrome is currently its officially recognized title. Next on my list of current confusions surrounding CRPS are the questions about what it is. That’s the topic of today’s post.
My usual off the cuff explanation for CRPS is to describe it as a rare autoimmune and neuro-inflammatory disease. But that’s not how it has always been viewed by physicians and medical researchers. CRPS:
is a disease that has perplexed clinicians and basic scientists alike, not only for the refractory nature of the condition but also for its protean nature.
It can manifest in any given individual, although it is more common in females, and many of its features are difficult to explain on a pathological level.
This has led to some clinicians even declaring that the condition is psychosomatic in origin.”(1)
If you don’t have a background in medicine or healthcare, that excerpt can itself be confusing! So let’s look at some of those medical terms in plain English:
- ‘Refractory’ means a condition that’s difficult to treat. CRPS is known to often be resistant to treatment, so difficult to treat that some patients can barely be helped by modern medicine
- ‘Protean’ refers to something that changes form or shape; like a chameleon. CRPS is described that way because each patient may have a different set of the many symptoms of this disease.
- ‘Manifest’ in this context means appear or occur. What it means in this quote is that CRPS can show up in anyone; it’s not limited to any specific gender.
- ‘Psychosomatic’ is term used for conditions in which psychological, or mental, stresses negatively affect how the body functions. Frequent examples of this are how anxiety and stress can cause of worsen symptoms of a medical condition, for example hypertension or high blood pressure.
That last term, psychosomatic, has also been used throughout the history of medicine to blame patients for their own symptoms. It has led to patients being told that their symptoms are ‘all in their head’, and being treated as though they’re making up their symptoms.
To being disbelieved, dismissed, and disregarded by their physicians. If you think that only happened in the distant past, I have news for you. This is precisely what happened to me, in 2016.
That same physician I described in yesterday’s post, who made two different spelling errors while writing out one of the old names of CRPS (Reflex Sympathetic Dystrophy), refused to acknowledge that anything was wrong with me – long after I began having clinical signs and symptoms of CRPS.
I was disbelieved, dismissed, and disregarded by this physician, despite my own background in medical ethics! I’d spend a career protecting the rights of patients and medical research participants, yet I couldn’t get this one physician to listen to me or even look at my hands.
This is one of the reasons for which I raise awareness of this disease; so that other patients hopefully won’t ever have to go through this traumatic experience themselves.
So what is CRPS? We don’t really know. It’s a rare condition, so there aren’t that many of us with whom research can be done. And because each patient may present differently, may have a different set of symptoms, it’s difficult to pinpoint the overall causes of this disease; it’s pathology.
The International Association for the Study of Pain (IASP) has clearly stated that the pathology of CRPS is “unknown”.(2) Even the folks researching it don’t yet have a full grasp of what CRPS is, exactly:
In CRPS II, the pain syndrome follows a major nerve injury, but that does not explain its pathological basis.
Abnormal inflammatory responses are likely to play a role.”(2)
That last part, about abnormal inflammation, is relatively new. This is because researchers have recently identified inflammatory markers in the bodies of CRPS patients, which point to it being an autoimmune disease. One recent medical journal article states that:
Whilst CRPS was initially considered a neurological phenomenon, there is accumulating evidence that it is an immunoneurological disorder.”(3)
So it seems that I may have to update my regular off the cuff description of CRPS, from ‘a rare autoimmune and neuro-inflammatory disease’ to ‘a rare immunoneurological disease’… but I think that would add more confusion to this condition! What do you think?
I started this post with the question of what CRPS is, and the answer to that is that we don’t really know. No clear cause has been identified by researchers.
What they have found out, so far, is that this medical condition affects a patient’s immune system, nervous system, and brain. It’s suspected that there’s a genetic component to CRPS, but evidence of that hasn’t yet been found.
Thanks so much for reading, and for sharing in my patient journey. Tomorrow’s post will look at some of the confusion surrounding how to test for, or diagnose, this rare condition.
References:
(1) 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 07 Nov 2019. Web:https://jneuroinflammation.biomedcentral.com/articles/10.1186/s12974-019-1449-9
(2) 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
(3) 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/