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DO
YOU HAVE BURNING PAIN?
- Is the pain from an injury that has not healed?
- Is the pain out of proportion to the severity of the injury?
- Has it lasted past the expected healing time?
- If your answer is yes to any of these questions, read on....
COMPLEX
REGIONAL PAIN SYNDROME (CRPS)
aka REFLEX SYMPATHETIC
DYSTROPHY (RSDS)
RSD is also known as Complex Regional Pain Syndrome (CRPS) type
1. CRPS type 2 is causalgia which is identical to type 1 except
that it is caused by a nerve lesion. Recent evidence suggests
that CRPS1 is also from nerve damage.(Please see "Evidence
of Nerve Damage in CRPS1" on WHAT'S
NEW? page.
Other names:
- causalgia
- algodystrophy
- postraumatic dystrophy
- Sudeck's atrophy
- shoulder-hand syndrome
BACKGROUND:
RSD/CRPS has existed since the American Civil War (1861-5) where
Dr. Silas Weir
Mitchell documented cases of causalgia in Civil War soldiers.
RSD/CRPS is a multi-system syndrome with diverse symptoms characterized
by chronic pain. It affects the immune system, central nervous
system (brain and spinal cord) and vascular system (hot/cold).
Recent studies (Lewis J PhD Eur J Pain 2012) have found changes
in the brain. Body perception disorder is very common. For example,
CRPS patients perceive their affected limb as 8% larger than what
it is.
Usually CRPS/RSD affects one or more extremities but it can affect
any part of the body. Due to dilation or constriction of the blood
vessels, the blood supply to the limb (hand, foot, hip, shoulder)
is affected. The nerves, skin, muscle and bone are also involved.
RSD is a debilitating disease which can impair the ability of
the limb to function or function can be lost.
How do you get RSD/CRPS?
- a soft tissue injury due to minor trauma in 65% of cases e.g.
sprain, twisted ankle etc.
- fracture
- surgery
- certain cervical spine or spinal cord disorders
- infections, stroke, heart attack, repetitive motion disorder,
or cumulative trauma e.g.. carpal tunnel.
- certain medications or venipuncture in rare cases
What are the symptoms?
- PAIN usually burning, severe, constant and in an area other
than the primary injury site
- SWELLING usually localized but can involve entire limb
- SKIN CHANGES: TEMPERATURE or COLOUR: ie. dystrophy, dryness,
tissue atrophy e.g.. can be cool, pallid or mottled; or warm,
red with increased sweating.
- LIMITED AROM (active range of motion) in the affected part
- INCREASE OF ABOVE COMPLAINTS after exercise
What other symptoms are there?
- motor dysfunction e.g. tremor, weakness, dystonia, spasms
- dystrophy e.g. muscle wasting
- limbic system dysfunction e.g.. insomnia, agitation, anxiety,
depression, poor memory or judgment
- hair and nail changes
- bone changes e.g.. osteoporosis
- joint tenderness and swelling
How is it diagnosed?
RECENT DEVELOPMENTS: After many many years
of not having a definitive test, there are new markers to test
for CRPS. From Boston, we have a skin biopsy test with a 3mm
sample which can detect distal nerve endings which are damaged.
From Haifa, Israel, we have a saliva test for high levels of
LDH (lactate dehydrogenase). There is also an albumin test.
Tests are not being done in Canada yet.
In the meantime, doctors who are qualified :
- do a thorough medical history noting sign and symptoms
- give an examination by a qualified expert physician
using the IASP criteria
- may use thermography which measures the heat emitted from
the body and senses skin temperature differences (may help)
CURRENT IASP DIAGNOSTIC CRITERIA (Moscovitz 2010)
DIAGNOSTIC
CRITERIA
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CLINICAL CRITERIA
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PAIN
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continuous pain which is disproportionate
to any inciting event
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SYMPTOMS
4 CATEGORIES: by report:
Sensory: hyperaesthesia and /or allodynia
Vasomotor: temperature and or color asymmetry
and/or change
Sudomotor: edema, and/or sweating asymmetry
and/or change
Motor/Trophic: < ROM and/or motor dysfunction,
and/or skin/hair/nail changes
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one in 3 or more of 4 categories
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SIGNS:
4 Categories by observation at the time of diagnosis: As
above
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one in 2 or more of 4 categories
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There is no other diagnosis that better explains
the signs and symptoms above.
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Criteria was developed by the International Association for the
Study of Pain (IASP) in 1994 and modified in 2004. "The criteria
are under continuous review to improve their specificity while
maintaining adequate sensitivity."
To read entire study: Moscovitz.pdf
DO YOU SUSPECT RSD?
"If the pain is out of proportion to the injury, there is
stiffness and inflammation following a seemingly minor trauma
(e.g. twisted ankle, dropping item on foot, sprain , suspect RSD.)
The pain is described as burning, shooting, stabbing or a "hot
poker". If the pain persists longer than the expected healing
time of the injury, suspect RSD."
( Hooshmand, H MD CRPS: Diagnosis and Management
Pain Digest Spring Verlager 1999).
TREATMENT IS CRUCIAL WITHIN THE 0-3 or 3-6 MONTHS
when the disease responds best. It is essential that the person
be referred to the proper specialist for treatment as soon as
possible. After 6 months you can still receive treatment however
avoid delays and see a specialist as soon as possible for best
results.
DELAY IN TREATMENT COULD MEAN A LIFETIME OF CHRONIC PAIN
for the patient. Without treatment, the disease could eventually
become resistant to treatment.
Who can get RSD/CRPS?
Anyone can get RSD, but it is more prevalent among women 3-1
to men; typically it is a women ages 40-60 who is most affected.
However, children and teenagers can have RSD as well. For them,
the prognosis is more favourable.
What can I do?
"Early diagnosis brings the best prognosis" In RSD/CRPS
the best response to treatment is within the first zero to six
months of the disease. If after reading this, you suspect that
you or a friend may have RSD, get treatment as soon as possible.
For doctor referrals in your area, please contact us.
TREATMENT
The following is a list of treatments for RSD/CRPS:
- Drug therapy: a) local or systemic corticosteroids b) muscle
relaxants c) alpha-adrenergic and beta blockers d) analgesics
e) anti-inflammatories f) tricyclics and related compounds g)
tranquillizers h) calcium channel blockers i) membrane stabilizers
j) opioids
- Blocks: a) focal b) sympathetic blockade c) intravenous regional
blocks d) epidural e)plexus catheter blocks
- Physical therapy: land PT or aqua therapy,mirror therapy.
graded motor imagery. Others are laser therapy, vitamin/mineral
supplements
- Transcutaneous electrical nerve stimulator (TENS)
- Sympathectomy: a) surgical b) chemical in rare cases (many
doctors no longer do this surgery due to complications).
- Implantable devices: a) dorsal column stimulator b) peripheral
nerve stimulator c) morphine infusion pump (intrathecal drug
delivery e.g. baclofen)
- Ketamine: a) low dose, b) 4 hour IV infusion c)ketamine coma
(currently not available in Canada)
CURRENT RECOMMENDATIONS FOR TREATMENT
UPDATE 2012: Contact PARC for current recommendations
for treatment.
2009
At the Scottsdale AZ Conference (March 27- 8), doctors discussed
the Advances in CRPS. Articles appear in our PARC PEARL. Digital
articles are available. Please contact PARC.
2006
The doctors at the Chicago CRPS/RSD 2006 Conference discussed
the following:
- Physiotherapy
is the number one treatment modality which will return function
to the limb, range of motion, flexibility and strength. It is
imperative that PT is part of the treatment program. It must
be done to help recovery.
- Aqua therapy
is also recommended in 92-93 degree water. It is moist heat
which is good for most RSD patients, it is non-weight bearing.
it supports weak muscles, and the amount of resistance can be
controlled. Warm water is also soothing.
- Psychological support is also recommended e.g.. counselling,
cognitive behaviour techniques, biofeedback, visualization,
relaxation techniques, hypnosis. Any of these modalities can
help.
- Medications that control pain are essential and will facilitate
the progress made in physiotherapy.
CONSENSUS REPORT
According to the 1998 Consensus Report compiled by Michael Stanton-Hicks
et al, "CRPS:Guidelines for Therapy" states that a three
pronged approach is best. The treatment algorithm has the following
three elements:
A) Medical Interventions: NSAIDS, Opioids, Tricyclics, Alpha
2 Agonists, Calcium channel Blockers. The next phase is blocks
(focal, sympathetic, regional,epidural and pumps.). Then Neurostimulation
(SCS, PNS) and medications.
B) Physical Therapy:
It is essential that early intervention occur here. Progress is
contingent upon the degree of pain and successful treatment of
pain. "It is critical to progress slowly and within patient
defined limits....adequate and liberal analgesia should be used
to facilitate these steps.". Current research indicates that
mirror therapy and graded motor imagery are effective.
Steps include gentle reactivation: contrast baths, desensitization,
flexibility, edema control, peripheral E-Stim, isometric strengthening,
and most importantly diagnosis and treatment of secondary myofascial
pain. Gentle ROM (range of motion exercises), stress loading,
isotonic strengthening, general aerobic conditioning, postural
normalization and balanced use can also be done. Next is ergonomics,
movement therapies, normalization of use and vocational and functional
rehabilitation. Any of these methods can be used to facilitate
progress in an individual with RSD/CRPS.
C) Psychological Interventions: Various methods include counselling
(expectations, motivation, control, family, daily journal) behaviour
therapy, relaxation techniques, imagery, hypnosis and cognitive
behaviour therapy (CBT). Any of these methods can be used. Methods
A, B, and C are designed to work simultaneously in order to facilitate
the return of function and good pain control for the patient.
( Source: Stanton-Hicks M et al "Consensus
Report: CRPS: Guidelines for Therapy" Clin Jour Pain 1998:
14; 155-66.)
UPDATE 2004: While this is an old study, the
findings are still relevant today.
The revised therapeutic algorithm for CRPS with emphasis on therapeutic
options in response to patient's clinical progress in the rehabilitation
pathway".(1)
Abstract: The goal of treatment in patients with complex regional
pain syndrome (CRPS) is to improve function, relieve pain and
achieve remission. Current guidelines recommend interdisciplinary
management, emphasizing 3 core treatment elements: pain management,
rehabilitation, and psychological therapy. Although the best therapeutic
regimen or the ideal progression through these has not yet been
established,
increasing evidence suggest that some cases are refractory
to conservative measures and require flexible application of the
various treatments as well as earlier consideration of such interventions
such as a spinal cord stimulator(SCS). While
existing treatment guidelines have attempted to address the comprehensive
management of CRPS, all fail to provide for contingent management
in response to a sudden change in the patient’s medical
status.
This paper reviews the current pathophysiology as it is known,
the purported treatments, and provides a modified clinical pathway
(guideline) that attempts to expand the scope of previous guidelines.(2)
An Updated Interdisciplinary Clinical Pathway for CRPS: Report
of an Expert Panel World Institute of Pain Pain Practice Vol.
2 No.1; 2002 1-16.
PROBLEMS
If treatment is delayed, many RSD/CRPS patients can face the
following:
- a lifetime of chronic pain.
- not being believed or misdiagnosed
- receiving inappropriate treatments
- dealing with doctors who are inexperienced in diagnosing and
treating RSD/CRPS
Unfortunately, these situations are all too common. Most of the
time, reliable information about RSD/CRPS is the key to being
believed and diagnosed properly. Receiving inappropriate treatment
would not happen if the patient and doctor were better informed.
Unnecessary surgery to "correct RSD" would also not
occur.
As the patient, it is possible to take on the responsibility
of informing the doctor. Perhaps then, some of these problems
could be avoided. Giving the doctor as much information as you
can so that he can better treat you will certainly promote a healthy
doctor-patient relationship. Working together with your doctor
is a win-win situation.
WAYS TO HELP YOUR DOCTOR
- Prioritize a list of your complaints. Whatever
is bothering you the most, list it first. e.g.. be specific
about where your pain is. e.g.. Before and after the visit,
record pain levels throughout the day to help determine the
overall pattern of how the disease affects you.
- Keep a journal of your medications. e.g.
effective and non-effective medications, side effects, etc.
- Be sure to take someone with you. Take notes
so that you are clear as to what transpired. Another point of
view is often helpful.
- Prepare a list of questions. Your spouse/friend
can record the answers.
- Keep a record of your past treatments and
medications. Record how they affected you.
- For new patients, bring your test results,
scans, x-rays etc. Make a brief summary of your medical history
thus far.
OPQRST
If you are experiencing pain and are having difficulty describing
it, this is a device that doctors use to diagnose pain. It could
help prepare you for your next doctor’s visit. Answers should
be as clear and brief as possible:
• ONSET: When did the pain start?
• PROVOKES: What makes it better and/or worse?
• QUALITY: What does the pain feel like? e.g. burning, throbbing
• RADIATES: Does the pain travel or stay in one place?
• SITE:Where do you feel the pain?
• TIMING: When do you get the pain? (e.g. morning, evening,)
and how long does it last?
PAIN DIARY
Keep a pain diary with recorded pain levels three times per day.
Divide the seven days into three parts and record levels by assigning
number to your pain:
WHY DO WE GET CRPS/RSD?
This urgent question remains in the minds of researchers. Dr
Anne Louise Oaklander has offered a rational explanation.
"RSD/CRPS THE END OF THE BEGINNING"
Oaklander AL MD PhD Pain 139 (2008) 239-40
" 'De Mos et al. have identified the clinical links
to CRPS and validated findings that implicate inflammation, vasodysregulation
and axonal injury in CRPS pathogenesis.'
The challenge now is the learn how these interact, because each
can lead to the other, like the chicken and the egg. For instance
distal axons are exquisitely vulnerable to energy deprivation,
so vasodysregulation (blood flow problems) or inflammation can
trigger axonopathy (nerve damage). "My working hypothesis
is that although specific patients may enter the CRPS rotary from
different points, malfunctioning small fiber axons are central."
OTHER THEORIES
There are several theories as to why we get CRPS. Most of the
current theories cannot fully explain all the processes going
on in the body e.g.. signs and symptoms vary from patient to patient
and can be inflammatory e.g. pain, edema, skin and temperature
changes and neurological e.g. hyperesthesia, hyperpathy or motor
dysfunction..
The psychosocial theory states that various predisposing factors
such as emotional instability, nervousness, depression, anxiety
and life events contribute to a person developing RSD. There is
no evidence thus far, in the literature to support this theory.
Inactivity has also been suggested to contribute to edema and
muscle atrophy in RSD. In chronic RSD, atrophy and edema are present.
However, inactivity cannot explain the skin discoloration, altered
temperature or pain. Quite popular even still, is the sympathetic
theory which states that an hyperactive sympathetic nervous system
is responsible for RSD. Since sweating and vasomotor instability
are present and a sympathectomy reduces pain, this theory was
thought to hold water. However, it did not explain why some sympathectomies
did not work. None fully explained the enigma of RSD. The causalgia
theory stated that the burning sensations in RSD were caused by
a nerve injury. However, not all RSD had an nerve injury as the
cause. In many cases, fracture, or soft tissue injury was the
trigger for RSD and no definable nerve lesion could be found.
Based on Sudeck's exaggerated inflammatory response theory, the
signs and symptoms of inflammation and acute RSD have similarities:
"rubor (redness), calor (warmth), dolor (pain), tumor (swelling)
and functio laesa (limited function)". Based on Sudeck's
theory Dr. Veldman, Dr
L van der Laan and numerous other Dutch doctors have done
extensive studies with RSD/CRPS patients in Holland. They hypothesize
that after an injury or surgery there is an exaggerated inflammatory
reaction process which begins in the body. Based on a 1993 study
of 829 patients over 8 years, Dr
Veldman found that the most common signs and symptoms of pain
(92%), swelling (86%), difference in skin temperature (98%), difference
in skin colour (97%), limited active range of motion (90%), increase
of complaints after exercise (89%) were inflammatory in nature.
There is evidence that free radicals are also involved in this
inflammatory process since they can damage tissue or membranes
in the body. Further evidence, is that free radical scavengers
like DMSO (dimethyl sulfoxide),
N-Acetyl-cysteine,or IV Mannitol are used successfully in
treatment of early cases in Holland.
Inflammation, blood flow problems, nerve damage and maladaptive
plasticity (brain changes i.e. grey matter atrophy) are thought
to be the puzzle pieces in CRPS. Yet the question onf why we get
CRPS, remains unknown.
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