Originally posted January 28, 2012; Last Updated: February 2, 2013
I have more research and updates to add to this when I can, I’ve got a lot of papers to organise into themes first though. Feel free to ask me questions in the meantime, x (April 9, 2014)
CRPS: Complex Regional Pain Syndrome. Previously known as RSD (Reflex Sympathetic Dystrophy) and Causalgia, among (many) other names!13B
Two quick but important asides: If you don’t have CRPS….
– ..take Vitamin C after injuries and operations to help to avoid developing it.(9, 12, 13 & 16)
– ..don’t use ice on an injury, or really cold compresses. (The skin mustn’t get below 10 degrees centigrade). This can initiate CRPS.7 If you do use ice, be sure not to place it directly on the skin (wrap it in a teatowel or such like), and don’t leave it more than ten minutes at a time… ten minutes on, ten minutes off. (As per the recommendation of my doctor for non-CRPS patients).
There’s good research backing these up. 🙂
Even if you do have CRPS, bear these in mind after a new injury, surgery or an invasive procedure of any kind, x
Re the interview with Paula Abdul the other year that got zapped around the internet (in May 2012)… it’s fantastic to see the explanation of how CRPS malfunctions and the message of nutrition and looking after your body is a massively important one, but one very misleading mention was that ice relieves CRPS pain. There is one subtype of CRPS for which ice relieves pain, but for all other CRPS types it is to be avoided as it can be very damaging indeed. And as healthy individuals have no way of knowing whether their mitchondria carries the predisposition to CRPS, then we all need to be aware of the possible damaging effects of using ice. Just thought I’d clarify. A lot of CRPS patients were quite rightly worried that the ice message would get taken on board generally, so I’m mentioning it here.
I’ve written this page because… none of my doctors could tell me this stuff. I’ve had to find out about CRPS for myself during the past 12 years of living with the condition. It’s a hard slog, even with my background in post-graduate research, and I’d like to save some of my fellow CRPSers out there a bit of the hassle. Information on the internet varies in relevance because research is always happening, and it changes the understanding of CRPS. There is often no way of knowing whether the information found is current, or whether it’s now outdated. Which is why I’ve listed at the bottom of the page where this info’ has come from, (mostly peer reviewed research papers), so that you can see how recent it is, and it gives you a place to start if you want to know more. Bear in mind that just because something can happen with CRPS, doesn’t mean that it will happen. I’ve just tried to cover most aspects here because each of us may be looking for different bits of CRPS info’. Think positive, love from me, x
What is CRPS?
CRPS is one of the conditions caused by autonomic dysfunction. It is a form of localised dysautonomia, and can remain localised. The genetic predisposition can become reality after the simplest of injuries, even a sprained ankle.4 It doesn’t necessarily kick in with the first injury in our lives, though. My own body waited patiently until I was 24 to surprise me!
The human body reacts to injury when the nerves send pain messages to the brain. The brain responds by inflaming the damaged area to protect it while it heals. This is all automatic; we don’t have to think about it, it just happens. And that’s one of the many jobs of the Autonomic Nervous System, or ANS for short, which runs the automatic processes inside our bodies.
The problem with CRPS is that the ANS is behaving in a faulty manner. The area can heal, but the pain messages persist, and so the area continues being inflamed. The brain is trying to do the right thing, but the messages it’s getting are not quite right.
And the trouble with long-term inflammation is that when everything gets swollen up like this, everything is getting squished. Including the blood vessels, which are trying to supply all of the tissues in the affected area. By all of the tissues I mean everything; bones, muscles, tendons, skin, nerves, eeeeverything. And lack of vital oxygen results in damage to these tissues if the condition is diagnosed too late. Which it often is, if it gets diagnosed at all.6
If the condition is diagnosed within 6-18 months and the nerves have not been permanently damaged, the effects can be shifted into remission7. However, most patients are diagnosed late6 and will require support to tame the condition, to some extent, and to find ways to live with what can’t be tamed.
There is now evidence that there are various sub-types of CRPS1B, (for example the noted differences between the patients who cannot withstand cold, and the much smaller number of patients who cannot withstand heat)6B, 2B.
Most medical practitioners do not know enough, or even anything, about this condition condition simply because it is a tertiary level specialist condition that covers multiple disciplines, and as the symptoms are medically confusing, diagnosis is often delayed.7 This means that no matter how many times a patient goes back to the doctors’ surgery about their symptoms, they can still experience being falsely advised that their symptoms are psychological. For example that they’re depressed, or being told that it’s ‘all in their head’, and the medical practitioners don’t even realise that they’re missing anything vital at all7, 13. Research has found no psychological difference between CRPS patients and control groups1A. Yes, us CRPSers do often experience depression at some point along the line, but it is caused by living with CRPS, not the other way around.15, 4AA, 7C
It can take many referrals to various specialists picked almost at random before the patient sees someone who makes a connection and gets them re-referred to someone else who’ll finally get it right. It took me six years from injury to diagnosis, which is a little over average, but it happens.
Experts still don’t fully understand CRPS. But every new piece of research gets added to existing knowledge and theories to help form a better picture.
There used to be a concept of three stages of CRPS development 2. But it turned out that these stages do not have to follow each other, a patient can stick at a stage2. They do not have to happen in this order2. And there is also no consistent time scale between stages, some people have gone from stage 1 to 2 in a fortnight. So even when the experts try to pin the condition down it still has its own rules. Or lack of them! So these stages were discarded2, 1B, but you’ll still find information about them dotted about online, which is misleading when you’re trying to find information that’s actually up-to-date.
CRPS is currently viewed as involving interactions between the immune system, the ANS and the Central Nervous System,10 (or CNS for short, which is involved in external actions such as how we move our body).
There are a load of symptoms that go hand in hand with CRPS and these have been used to compile and amend a diagnosis reference. The kind of CRPS symptoms that can be experienced in the affected parts of the body are5:
- Skin temperature, skin colour changes
- Pain being caused by things that you wouldn’t ever expect to cause pain, the lightest touch can cause pain in some patients (allodynia/hyperalgesia)
- Muscle contractions causing unusual movements and postures (dystonia)
- Fluid build-up causing swelling (edema)
- Lower bone density as they become more porous (osteoporosis)
- Changes in hair/nails/skin
- Sweating changes, sometimes sweating varies from one side of the body to the other
- Reduced response to stimulation of nerves/body areas, responses can vary from one half of the body to the other (hemi-body hypoethesia)
- Oddness of the Central Nervous System (CNS). The CNS is made up of the brain and spinal cord which use the information from the wider spread ANS to control and co-ordinate what we do. The CNS can do some odd things when it’s dependent on a faulty ANS for it’s information.
- Avoiding using the part of the body which hurts, which then causes additional problems like the muscles starting to waste away through lack of use (atrophy)
As the condition develops the pain becomes continuous, it is truly relentless. There are brief periods where it may not be as severe, but considering that the pain experienced is off the scale of previous personal experience for most patients, the lesser pain moments are still excruciating by ‘normal’ standards. No wonder the natural response is to stop using whichever part of our body is affected.
The McGill Pain Scale shows the level of pain CRPS patients are dealing with. You can see the placement of CRPS in the pic’ below.1 CRPS is consistently and reliably shown as having higher pain levels than childbirth and digit amputation, for example.
The McGill Pain Scale is not just a number on a scale. It is compiled from lots of data which includes numerical scores, but also descriptive words patients used to answer various questions about the pain experience which were then assessed for additional aspects such as consistency. It has been researched very thoroughly over the years and has withstood all tests into reliability, so it’s a really useful tool to get our heads around pains that we may not have experienced personally, and for putting our own pain into some kind of perspective for others.
After looking at this pain scale it might be good to pause and remember that this pain is relentless. It is also very important to understand that CRPS patients become very good at covering up, and even functioning through, their pain simply because they have to function around it permanently. Research into chronic pain has found that those who experience higher pain levels as part of their condition are less likely to report those pain levels even though they are just as painful2A.
So the best way to find out how much pain a CRPS patient is in is to ask them. Trying to assess their pain visually, and even physically, is very unreliable as their responses to continuous familiar pains are often very low key when compared to the responses of non-CRPS patients dealing with a matched scale pain level.
Patients reporting their CRPS pain are to be taken very seriously.
Some CRPS info’:
- It can spread. It doesn’t always, but it can. And if it does, it does it weirdly. This is because it spreads through the ANS, (which is wired up representing a grid form), rather than from one body part to another connected body part. So yes, it really can spread straight from the right knee to the left knee, without having to go via any other body parts.14
- It’s can spread simply as a result of a new injury in an unaffected area. In my case, as the CRPS was already throughout both legs, it then spread to a new injury in one of my hips very quickly.14
- For this reason anything invasive should be avoided. So… no operations unless absolutely necessary.7
- Suicide and suicidal thoughts are all part of living with a condition that causes such high levels of pain and, at its worst, reduces ‘living’ to ‘existence’. On the plus side, those that come through it are deemed to be some of the strongest and most determined of people17 and get a blummin’ huge cheer from me for hanging in there.
- About 75-80% of CRPSers are women10AA & 2AA and most cases start in an injured extremity.
- Neurological effects of CRPS, (including long term cognitive and mood changes),8 are often incorrectly treated as they are not recognised as part of the condition. New research will hopefully help to combat this lack of awareness, as it has been recently shown that neuropsychological deficits are present in 65% of CRPS patients. These deficits have been noted in the executive functions, (for example planning, organising, self-awareness, self-regulation and initiation of action), word recall (lexical memory) and conscious memory of events (declarative memory).7B
- Sleep is understandably affected by the constant pain and makes additional impacts on health.11
- Strangely, painless CRPS can exist. It is very rare, but basically presents with all the other symptoms, just without the pain.3
- Ice-packs are bad for the majority of CRPS forms and can even be part of the development of the condition. The ANS is made up of the sympathetic (response to external events) and parasympathetic (balancing these effects). A temperature drop to 10 degrees centigrade shuts down the parasympathetic system, whereas a further drop to 0 degrees is required to shut down the sympathetic, so when an ice-pack reduces the temperature to 0-10 degrees, only the sympathetic system is running and the experience becomes one of continuous burning pain because one is not there to balance out the other.7
- It can affect the Central Nervous System (CNS), too. Apparent in outward signs such as tremors15 and spreading to a new area without a new injury, owing to some rewiring of the brain that occurs.14 Research has even shown that children with CRPS have significant changes in the wiring of the CNS: the brain responds to stimuli differently in affected CRPS areas of the body than it does in unaffected areas, even after recovery7A. And in adults, research has demonstrated that structural cortical and subcortical reorganisation is directly associated with chronic pain.9A,10A As a result of investigating such sensory and motor abnomalities in CRPS, research back in 1998 discovered that dysfunction of the medulla is common regarding skilled movement of fingers, toes etc (corticospinal function), and pain signals from the body (spinothalamic function) and face (trigeminothalamic function)13A. The medulla, part of the CNS, is involved in control of autonomic (automatic) functions and demonstrates another tangible link between CRPS and general dysautonomia. More recent research has demonstrated further evidence of cortical reorganisation is CRPS, pointing out that it is incorrect to think of this condition as a peripheral disorder8A.
Other related conditions can develop (known as co-morbidities). As CRPS is based on having a faulty ANS, and because the ANS runs all automatic processes in your body, any other automatic processes are also at risk of developing wobbly functioning if a trigger event is sufficient enough to set it off. See also info’ on Dysautonomia (autonomic dysfunction). For example:
– Fainting, and near-fainting symptoms, are common in CRPS , especially if the lower limbs are affected. Autonomic dysregulation in the lower limbs can result in impaired regulation of blood vessel size (the vasoconstriction function becomes compromised) which then results in blood pooling and lower blood pressure, causing a higher heart rate, and fainting is the body’s way of resolving the issue12A.
– CRPS patients can have greater intestinal permeability4A, with outcomes such as developments of food allergies.
Doctors need to be made aware that various autonomic issues can crop up, and the concept of general dysautonomias is good to keep in mind to enable appropriate referral to a specialist who has the crucial experience required to treat this complex and medically esoteric condition.
Multi-disciplinary Pain Management teams have improved their ways of working with CRPS patients over the years. With better understanding of this condition, (and of the patient’s experience of it), comes improved support, and improved outcomes.
- Physiotherapy for muscles that have wasted (atrophied) through lack of use owing to high pain levels.
- Teaching about the ANS and how controlling the amount of activity with the affected areas can be used to retrain the ANS so that the pain signals reduce from the amplified levels to more relevant levels.
- Psychological support regarding understanding the processes, what are our best coping mechanisms etc. Brilliant when with a qualified Clinical Psychologist as they are trained to encourage and assist us and also have knowledge of the condition, (or if they don’t they’ll go away and find out). Very refreshing! The new psych’ approach also focuses on accepting the condition so that we can move on to focusing on what we can do to live with it, rather than not living our lives whilst waiting for a magical cure to be discovered.
- External treatments, such as TENS machines (which administer minor electrical pulses) or application of warmth through baths, not-too-hot water bottles etc. (NOT ice, this can make it worse).
- Pharmacology. Prescribed painkillers are common. A lot of people have injections as well. There are side effects and long-term issues to be weighed up for each individual. Although if the physio’ knowledge is employed over time to reduce ANS amplifications of pain signals, this can be reduced to ‘over the counter’ painkillers when needed. (It’s taken years for me to get to this stage, but it’s well worth it).
- There’s always research into other treatments, and sometimes there are new trial treatments available, but they are often offered in a country we don’t happen to live in and can be really expensive. Some have scientific back-up, some are part of research projects and some are dangerous, but patients have turned to them because they are desperate. They don’t fall under general treatment techniques from the pain management team, and it’s not something I know masses about because I try to use as few med’s as possible.
(There’s one reference missing as I am still searching for the magazine with the relevant publication in it. I suspect it has prematurely been tidied away into the loft! When I come across it again I shall update the missing reference for you, x)
1. Australian Reflex Sympathetic Dystrophy Syndrome Support Group, The (2006), in http://www.ozrsd.org/forum/kb.php?mode=article&k=4
1A. Beerthuizen, A., Stronks, D.L., Huyen, F.J. et al (2011) ‘The association between psychological factors and the development of complex regional pain syndrome type 1 (CRPS1) – A prospective multicenter study’ in European Journal of Pain, Vol. 15, Issue 9, pp 971-975
1B. Bruehl, S. et al (2002) ‘Complex regional pain syndrome: are there distinct subtypes and sequential stages of the syndrome?’ in Pain, Issue 95, pp119-124
2. Carden, E., ‘Reflex Sympathetic Dystrophy Complex Regional Pain Syndrome (CRPS) Recognition and Management for the Physician’, http://ww1.prweb.com/prfiles/2008/05/22/968434/0_carden_crps_physician.pdf
2A. Cook, I.J., van Eeden, A. & Collins, S.M. (1987) ‘Patients with irritable bowel syndrome have greater pain tolerance than normal subjects’ in Gastroenterology, Vol. 93, Issue 4, pp 727-833
2AA. de Mos, M., de Brujin, A.G.J., Huygen, F.J.P.M. et al (2006) ‘The incidence of complex regional pain syndrome: a population-based study’ in Pain, doi:10.1016/j.pain.2006.09.008
2B. Eberle, T, Doganci, B, Kramer, H.H. et al (2009) ‘Warm and cold complex regional pain syndromes’ in Neurology, Issue 72, pp505-512
3. Eisenberg, E. and Melamed, E., (2003) ‘Can Complex Regional Pain Syndrome be Painless?’, Pain, Vol. 106, Issue 3, pp 263-267
4. Fields, R.D., (2009) in ‘New Culprits in Chronic Pain’, Scientific American’, Vol 301, Issue 5, pp50-57
4AA. Glick RJ, Chopra P, Richardi T. Rethinking the Psychogenic Model of Complex Regional Pain Syndrome: Somatoform Disorders and Complex Regional Pain Syndrome. Anesth Pain. 2012;2(2):xxx. DOI: 10.5812/aapm.7282
4A. Goebel, A., Buhner, S., Schedel, H. et al (2008) ‘Altered intestinal permeability in patients with primary fibromyalgia and in patients with complex regional pain syndrome’ in Rheumatology, Vol. 47, pp 1223-1227
5. Harden, M.D. & Bruehl, S. (2007) in ‘Proposed New Diagnostic Criteria for Complex Regional Pain Syndrome’, Pain Medicine’, Vol 8, Issue 4, pp326-331 [This reference has only been used regarding the list of CRPS symptoms, and it gives me a good excuse to tell you that Harden has been discredited for faking results. There are a few researchers out there like this, the way the US health system works is not conducive to some less moral researchers sticking to a code of ethics. http://www.rsdfoundation.org/en/harden.htm. Also to be avoided for faking results is Reuben, S.S. http://online.wsj.com/article/SB123672510903888207.html)
6. Hooshmand, H., (1999) ‘Complex Regional Pain Syndrome, Reflex Sympathetic Dystrophy, Diagnosis and Therapy – A Review of 824 Patients’, Pain Digest, Vol. 9, pp 1-24
6B. Hooshmand. H. (2000) ‘Ice versus heat’ http://www.rsdrx.com/rsdpuz4.0/puz_102.htm
7. Hooshmand, H., (2007) ‘What is RSD, What is Sympathetic Anyway?’, http://rsdrx.com/what_is_rsd.htm
7A. Lebel, A., Becerra, L., Wallin, D. et al (2008) in ‘fMRI reveals distinct CNS processing during symptomatic and recovered complex regional pain syndrome in children’, Brain, Vol. 131
7B. Libon, D.J., Scwartzman, R.J., Eppig, J. et al (2010) in ‘Neuropsychological deficits associated with Complex Regional Pain Syndrome, Journal of the International Neuropsychological Society
7C. Lohnberg, J.A. & Altmaier, E.M. (2012) ‘A review of psychosocial factors in complex regional pain syndrome’ in Journal of Clinical Psychology, DOI 10, 1007/s10880-012-9322-3
8. Marinus, J. et al, (2011) in ‘Clinical Features and Pathophysiology of Complex Regional Pain Syndrome’, The Lancet Neurology’, Vol 10, Issue 7, pp637-648
8A. McCabe, C.S., Haigh, R.S., Halligan, P.W. et al (2003) ‘Referred sensations in patients with Complex Regional Pain Syndrome Type 1’ in Rheumatology, Vol. 42
9. Pleiner, J. et al. (2008) ‘Intra-arterial vitamin C prevents endothelial dysfunction caused by ischemia-reperfusion’, Atheroscelorsis, Vol. 197, Issue 1, pp 383-391
9A. Ramos-Remus, C. & Duran-Barragan, S. (2010) in ‘Neurological Involvement in Rheumatoid Arthritis’, Current Immunology Reviews, Vol. 6, Issue 3
10. Rooij, A.M., (2010) in ‘Genetic and Epidemiological Aspects of Complex Regional Pain Syndrome’, Doctoral Thesis,LeidenUniversity (take the ‘chapter 4′ summary with a pinch of salt, as I’ve mentioned earlier, it’s been shown that their are no psych’ differences between control groups & CRPS groups. So if you rpint this paper out, perhaps also print off the more recent paper by Beerthuizen et al, listed here above as reference ‘1A’)
10AA. Sandroni, P., Benrud-Larson, L.M., McClelland, R.L. & Low, P.A. (2003) ‘Complex regional pain syndrome type I: incidence and prevalence in Olmsted County, a population based study’ in Pain, Vol. 103, Issue 1, pp 199-207
10A. Schmidt-Wilcke, T., Leinisch, E., Gänßbauer, S., et al (2006) in ‘Affective components and intensity of pain correlate with structural differences in gray matter in chronic back pain patients’, Pain, Vol.125, Issue 1, pp89-97
11. Schwartzman, R.J., et al. (2009) in ‘The Natural History of Complex Regional Pain Syndrome’, Clinical Journal of Pain, Vol. 25, Issue 4, pp. 273-280
12. Smith, H.S. (2010) ‘The Role of Genomic Oxidative-Reductive Balance as Predictor of Complex Regional Pain Syndrome Development: A Novel Theory’, Pain Physician, Vol. 13, pp79-90
12A. Smith, J.A., Karalis, D.G., Rosso, A.L. et al (2011) ‘Syncope in Complex Regional Pain Syndrome’ in Clinical Cardiology, Vol. 34, Issue 4, pp 222-225
13. Stevermer, J.J. (2008) in ‘Give vitamin C to avert lingering pain after fracture’, Priority Updates from the Research Literature from the Family Physicians Inquiries Network
13A. Thimneur, M., Sood, P., Kravitz, E. et al (1998) ‘Central Nervous System Abnormalities in Complex Regional Pain Syndrome (CRPS): Clinical and Quantitative Evidence of Medullary Dysfunction’ in Clinical Journal of Pain, Vol. 14, Issue 3, pp256-267
13B. Todorova, J. (2012) ‘Complex regional pain syndrome acceptance and the alternative denominations in the medical literature’ in Medical Principles and Practice, DOI: 10.1159/000343905
14. van Rijn et al, (2011) ‘Spreading of Complex Regional Pain Syndrome: Not a Random Process’, Journal of Neural Transmission, Vol.118, Issue 9, pp 1301-1309
15. Wasner, G., et al, (2003) in ‘Complex Regional Pain Syndrome’ Spinal Cord, Vol. 41, pp 61-75
16. Zollinger, Paul.E., (2010) ‘Upper and Lower Limb Orthopaedics: Prevention of CRPS & Other Traumatology’, Open Orthop, Vol. 4, Issue 61. Published online 2010 February 17
17. T’was in one of the British Psychological Society mag’s I have, I just haven’t been able to find the original article, yet!