On November 15th I decided to mark the midway point of CRPS awareness month with a first; my first ever series of daily posts here on this blog, all touching on a single related topic. Which topic? Glad you asked! This series covers the principal areas of confusion surrounding Complex Regional Pain Syndrome.
So far we’ve talked about the confusion around what it’s called, what it is, and how to test for – or diagnose – this perplexing condition. Today we’re going to talk about how to treat it, and tomorrow’s post will be on how to live with it; that will end this five-part post series.
First off, it’s important to state, up front, that I’m not a doctor. This post should not be considered medical advice or opinion; if you need medical care, please, please, please contact a physician! My education and career have been in healthcare, in a field called biomedical ethics or bioethics. I’ve worked in hospital and clinical (medical) research environments, but am not a licenced healthcare professional.
Second, did you notice that I referred to CRPS as perplexing in the introduction? I was hoping that you’d pick up on that! That’s not only my description of CRPS, it’s how the physicians and researchers who specialize in CRPS describe it! Here are my two favourite examples, from medical journals; I’ve added emphasis to the word, showing it in bold characters:
complex regional pain syndrome (CRPS) – multiple system dysfunction, severe and often chronic pain, and disability – can be triggered by a minor injury, a fact that has fascinated scientists and perplexed clinicians for decades…
Compelling evidence implicates biological pathways that underlie aberrant inflammation, vasomotor dysfunction, and maladaptive neuroplasticity in the clinical features of CRPS.
Collectively, the evidence points to CRPS being a multifactorial disorder”.(1)
Complex regional pain syndrome (CRPS) is a disease that has perplexed clinicians and basic scientists alike…
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.”(2)
As you can see, there remains quite a lot of uncertainty – downright confusion – about CRPS even among scientific researchers and specialist physicians. It’s also an incredible confusion condition to deal with, as a patient. Trust me, I know this from my own patient journey!
When I was first diagnosed with CPRS, back in 2016, I’d expected some type of clinical or medical test to confirm the diagnosis. A blood test, a urinalysis, maybe some type of scan. As we saw in yesterday’s post, though, there are no laboratory or medical imaging tests to confirm CRPS; it’s a diagnosis of exclusion.
To diagnose a patient with CRPS, the physician must first rule out every other disease that might resemble it; to complete what’s called a differential diagnosis. Physicians disagree on how to do this, in terms of which other diseases must first be ruled out.
One of the reasons for that confusion – I call it that because in my view confusion is often the root cause of disagreement in healthcare – is that each patient with CRPS my present differently. That means that each patient with this disease may have a completely different set of the many and varied possible symptoms. This means that the sets of diseases to be ruled out might change from one patient to another.
Then, once other diseases have been ruled out, the clinician must ensure that the patient meets the Budapest Criteria(3); the list of symptoms which must be present for a person to be diagnosed with CRPS. These diagnostic criteria are published by the International Association for the Study of Pain (IASP):
an international consensus meeting was held in Budapest in 2003 to review issues related to CRPS diagnosis with the goal of recommending improvements to the IASP criteria.
The resulting proposal for modified diagnostic criteria for CRPS (the “Budapest Criteria”) was based primarily on empirically-derived criteria”(3)
I’ve met some physicians, for example at medical conferences on chronic pain, who tell me that they’ll often diagnose a patient immediately with CRPS; even before ordering any of the tests to rule out other potential conditions. They do this for several reasons.
First because they’ve seen another patient with the exact same set of symptoms; they clearly recognize the same disease. Second, because it’s very important for a person with CRPS to begin treatment quickly. Any delay can translate to worse outcomes; possibly a permanent disability, even the amputation of their arm or leg.(4)
Third, this disease is often made more challenging for patients by the fact that it’s often invisible; in many cases a person will have spent months – years, even – being told that their symptoms are ‘all in their head’. Being misdiagnosed, in effect, as I was. Patients are told that their symptoms are due to anxiety, or stress, or some other explanation that simply doesn’t explain what it is that they’re experiencing.
These particular physicians with whom I’ve spoken are well aware of this, and don’t want to leave their patients without a diagnosis once they suspect CRPS. They understand how important it is for a person – after having one’s symptoms dismissed, disregarded, and ignored – to finally have their experience validated by a medical professional. To know that are believed, to feel that they’ve been heard.
Finally, these clinicians will sometimes immediate diagnose CRPS – the first time they meet a patient – because they’re familiar with the Budapest Criteria; which was discussed in yesterday’s post. To account for all of the variation in symptoms of CRPS, this list takes an approach including “At least 1 symptom reported in at least 3 of the following categories…”(5) and “At least 1 sign at time of evaluation in at least 2 of the following categories…”(5)
So let’s assume that an individual now has a clear diagnosis of CRPS. What’s next? Treatment, of course! Here again, we encounter confusion. As a reminder, because I’m neither a physician nor a nurse practitioner none of what I write should be viewed as recommendations about anyone else’s medical care.
As much confusion as there is around diagnosing CRPS, what exactly this chameleon disease is, there’s just as much confusion around treatment. Each of the different symptoms and clinical signs require different treatments, all for the same patient. But there’s no consensus, or agreement, among clinicians and specialists on the best ways to treat these symptoms.
A large part of the reason for that is that there is a lack of clinical evidence, or medical research, providing that specific treatments are effective for Complex Regional Pain Syndrome. Because it’s a rare condition, and patients have different sets of symptoms, each person with CRPS has – in some ways – their own particular version of the disease.
For these reasons, and others, “clinical trials that demonstrate effective therapies are difficult to perform”.(5)
There is very little good evidence in the literature to guide treatment of CRPS.
Early recognition and a multidisciplinary approach to management seems important in obtaining a good outcome.
Treatments aimed at pain reduction and rehabilitation of limb function form the mainstay of therapy.
Comorbidities, such as depression and anxiety, should be treated concurrently.”(6)
Without clinical trials to show that a particular treatment is effective, treating CRPS is often a kind of ‘hit-or-miss’ adventure. This is one of the reasons for which it’s so important that CRPS be treated in a multidisciplinary team; including, of course, the patient in any treatment decisions!
The pain management unit (PMU) with which I’m a patient, at a university hospital centre, takes a truly multidisciplinary approach all pain conditions; particularly CRPS. Members of this PMU include dentists (for dental pain conditions), nurses, physical therapists, physicians (within specialties such as anesthesiology, family medicine, rheumatology, and others), psychologists, and social workers.(7)
This range of healthcare professionals is generally needed in CRPS, and other chronic pain conditions, because it can truly affect your entire life. My CRPS is in my right hand and arm, but some days it feels as though the disease has taken over my life. Late last year, I developed mild cognitive impairment (MPI) due to this disease; I had to step away from my dream job, from my career. For a disease that technically only affects my hand and arm!
Because my pain management team understood how difficult that would be for me, stepping away from a job that I adored, they recommended that I meet with one of the psychologists who specializes in chronic pain patients – right there at the PMU clinic! These consultations were very important, in terms of helping me deal with my own disease-related grief; grief over losing so much of my life, of my sense of self to CRPS.
That psychologist suggested that I participate in group psychology sessions, specifically developed and tailored for chronic pain patients, again within the PMU clinic. Although none of the other people within these group sessions had CRPS, because it’s a rare disease, it was helpful to talk about other people’s experiences with a variety of pain conditions. Some of our feelings, such as being in limbo in our lives, were common to many of us.
I’ve repeated several times in this post that I’m not a doctor, so you shouldn’t consider anything here to be medical advice. So I’m not going to write about medications for CRPS; this isn’t the place for that. And rather than writing about possible treatments for CRPS myself, I’m going to refer to a public webpage from the University of Alberta(8).
Some cases can be reversed if CRPS is treated within the first six months…
The major treatment of CRPS is mobilization of the affected limb.
The natural response to pain is to not use the limb but this can actually make things worse.
Treatments with medications and other adjuncts may help with the mobilization.
For the upper limb, scrubbing is a useful type of mobilization, for the lower limb walking is useful.
Desensitization therapy is also useful.
In this therapy your limb is exposed to a variety of touches.
It is important to realize that while pain normally means there is ongoing damage, in CRPS if you have increased pain due to activity, you have not damaged anything (and may be helping yourself).
Relaxation, hypnosis and biofeedback may be useful.
Unfortunately the results of amputation are poor with the usual result that you would be left with a painful stump which might in fact be worse than the pain you already suffer…
Sympathetic blocks are frequently used in the treatment of CRPS alone or in combination with other treatments.
The purpose of the sympathetic block is to reduce your pain to enhance mobilization.
Not everybody with CRPS will respond to sympathetic blocks and whether or not you respond to a block does not affect your diagnosis.
Not all experts in CRPS believe in the value of sympathetic blocks.
Surgical or permanent sympathectomy is usually not effective.(8)
Note the use of terms such as “may be useful”, “Not all experts agree”, “Not everybody with CRPS will respond”. These are all signs of the confusion that still surrounds the treatment of this rare condition. When I’ve discussed medications for CRPS with physicians, it’s clear that there’s even more confusion and disagree among experts with regards to which medications to prescribe.
This brings me to the best summary I’ve ever seen for the treatment of CRPS, although it’s for the treatment of chronic pain conditions in general. This specific quote was used by a neuroscience researcher, at a discussion forum on chronic pain earlier this year, called Play the Pain.
This line has struck with me, ever since, and makes a perfect closing for my five-part blog series on the confusion surrounding CRPS:
Chronic pain therapy is a bit of an art, a bit of voodoo medicine, and a bit of science.”(9)
References
(1) Clinical features and pathophysiology of complex regional pain syndrome. (Review Article.) Johan Marinus, G Lorimer Moseley, Frank Birklein, Ralf Baron, Christian Maihöfner, Wade S Kingery, et al. The Lancet Neurology. 2011(10):7; 637-648. 01 July 2011. doi: 10.1016/S1474-4422(11)70106-5. Accessed 15 Nov 2019. Web:
https://www.thelancet.com/journals/laneur/article/PIIS1474-4422(11)70106-5/fulltext#secd13577181e290
(2) 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
(3) Harden RN, Bruehl
S, Perez RS, et al. Validation of proposed diagnostic criteria (the
“Budapest Criteria”) for Complex Regional Pain Syndrome. Pain.
2010;150(2):268–274. Online 20 May 2010. doi:10.1016/j.pain.2010.04.030. Accessed
15 Nov 2019. Web:
https://insights.ovid.com/article/00006396-201008000-00014
(4) Schrier E,
Geertzen JHB, Scheper J, Dijkstra PU (2019) Psychosocial factors associated
with poor outcomes after amputation for complex regional pain syndrome type-I.
PLoS ONE. 2019:14(3). doi.org/10.1371/journal.pone.0213589. Online 13 March 2019.
Accessed 18 Nov 2019. Web:
https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0213589
(5) 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
(6) Raneem Albazaz, Raneem Albazaz, Yew Toh Wong, and Shervanthi Homer-Vanniasinkam. Complex Regional Pain Syndrome: A Review. Annals of Vascular Surgery. Mar-Apr 2008. 22(2);297-306. doi.org/10.1016/j.avsg.2007.10.006. Accessed 17 Nov 2019. Web:
https://www.annalsofvascularsurgery.com/article/S0890-5096(08)00015-0/fulltext
(7) Alan Edwards Pain Management Unit. About us: The Team: Clinical Team. McGill University. Online. Accessed 15 Nov 2019. Web:
https://www.mcgill.ca/paincentre/about-us/team/clinical-team
(8) University of Alberta: Multidisciplinary Pain Clinic. About Chronic Pain: Complex Regional Pain Syndrome. Online. Accessed 15 Nov 2019. Web:
https://www.ualberta.ca/medicine/institutes-centres-groups/multidisciplinary-pain-clinic/about-chronic-pain/complex-regional-pain-syndrome
(9) Yoram Shir, quoted by Christophe Tanguay-Sabourin. Presentation: NeuroScientific Boundaries, at Play the Pain, 01 Oct 2019. Montreal, Canada.