DOCUMENT 4.1

21^st June 2018

Mr Tony

Technical Advisory Team

National Disability Service

Dear Tony, We are pleased to provide you with signed drafts of our advice to the NDIS regarding: Chronic non-cancer pain; Complex regional pain syndrome With kind regards, Dr [redacted] D Clin Physio, M Manip Physio, Grad Dip Manip Ther, B App Sc (Physio) Dr [redacted] MAPS B.A. Hons (psych) Doctor of Clinical Psychology Dr [redacted] MBBS, Dip Med (pain management), FANZCA, FFPM(ANZCA)

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Complex Regional Pain Syndrome

What is the aetiology of this condition?

Complex Regional Pain Syndrome (CRPS) is a painful, disabling and distressing condition that predominantly affects the upper or lower limb. Clinically, CRPS is characterised by pain, abnormal regulation of blood flow and sudomotor activity (sweating), oedema (swelling), active and passive movement disorders and trophic changes of the skin, hair and nails and subcutaneous tissues¹. CRPS usually follows trauma to the limb, such as a fracture, sprain, surgery or a penetrating injury². Central nervous system lesions such as spinal cord injuries and cerebro-vascular accidents and cardiac ischaemia are less frequent precipitating events¹.

The effects of CRPS can range from mild to severe and may last from months to years³. CRPS-1 is thought to occur after 1-7 % of fractures, and 2-5% of peripheral nerve lesions⁴. Fracture appears to be the most frequent initiating event³. Females are more often affected than males, with a female to male ratio ranging from 2:1 to 4:1. CRPS occurs across all age groups with a mean peak in age of 37 to 50 years¹. CRPS predominantly occurs in one limb, usually the upper limb, and is distributed evenly between the left and right side, with an incidence bilaterally of around 2%¹.

Terminology

The term CRPS was introduced in 1994⁵. Prior to this, terms such as Reflex Sympathetic Dystrophy (RSD) and Causalgia were applied. Under the 1994 terminology, RSD was re-labelled CRPS-1 and Causalgia as CRPS-2. However these terms have recently been abandoned and the single term CRPS is now preferred.

The authors note that the terms Chronic Regional Pain Syndrome and “Regional Pain Syndrome” are also frequently used to refer to persistent pain conditions. Neither of these labels refers to Complex Regional Pain Syndrome which utilises strict diagnostic criteria including signs and symptoms other than pain. In fact, there is no formal diagnostic category for either “Chronic Regional Pain Syndrome” or “Regional Pain Syndrome”.

CRPS has been defined as:

“A syndrome that usually develops after an initiating noxious event, is not limited to the distribution of a single peripheral nerve and is apparently disproportionate to the inciting event. It is”

Diagnosis

Diagnosis of CRPS has evolved since 1994. Currently CRPS is diagnosed using the following criteria (“Budapest Criteria”®).

Table 1 Budapest Diagnostic Criteria for CRPS®

Interpretation for making a clinical diagnosis:
1+ symptoms from each symptom category & 1+ signs at time of evaluation from 2+ sign categories.
i, Continuing pain, which is disproportionate to any inciting event
Symptom categories:
ii. Sensory: reports of hyper- aesthesia +/or allodynia
Vasomotor: reports of temperature asymmetry +/or skin colour changes +/or skin colour asymmetry
Sudomotor / oedema: reports of oedema +/or sweating changes +/or sweating asymmetry
Motor/Trophic: reports of decreased range of motion +/or motor dysfunction (weakness, tremor, dystonia) +/or trophic changes (hair, nail, skin)
Sign categories:
iii Sensory: evidence of hyperalgesia (to pinprick) +/or allodynia (to light touch) +/or temperature sensation +/or deep somatic pressure +/or joint movement
Vasomotor: evidence of temperature asymmetry (>1 degree Celsius) +/or skin colour changes +/or asymmetry
Sudomotor / oedema: evidence of oedema +/or sweating changes +/or sweating asymmetry
Motor/Trophic: evidence of decreased range of motion +/or motor dysfunction (weakness, tremor, dystonia) +/or trophic changes (hair, nail, skin)
iv There is no other diagnosis that better explains the signs and symptoms

Despite advances in the refinement of the diagnostic criteria, there remains limited capacity to discriminate between CRPS and the presence of other conditions with overlapping symptoms. This makes it difficult to distinguish CRPS from other neuropathic, medical and psychiatric conditions’. Figure 1 lists differential diagnoses that should be considered when making the diagnosis of CRPS.

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Figure 1 Differential diagnoses for CRPS

• Entrapment syndromes e.g. carpal tunnel
• Compartment syndrome
• Thrombosis
• Disuse or nonuse of a limb
• Costo-clavicular compression syndrome
• Lymphoedema
• Infection
• Conversion disorder
• Self harm

The difficulties with diagnosing CRPS have resulted in many radiological and laboratory investigations for patients. While these tests may assist in differential diagnosis, none of these tests has been found to offer any additional value in confirming the diagnosis of CRPS over clinical or ‘bed-side’ diagnosis alone7. This creates a burden of cost to the individual and the health system along with the risk of complications and side effects that may occur when ‘red herrings’ are treated following these investigations.

Clinical Presentation

Pain in CRPS is usually experienced in one limb, and is often described as burning. The pain may appear to be out of proportion to the precipitating event. Allodynia (pain from a stimulus that is not usually painful) and hyperalgesia (excessive pain from a stimulus that is painful) are often present throughout the limb, but do not reflect the distribution of a peripheral nerve or spinal nerve root5.

Movement disorders include loss of voluntary control of movements of the affected limb, slowing of movement, dystonia (long-lasting muscle spasm that can cause the limb to twist), myoclonus (brief involuntary muscle twitches) and tremor (involuntary rhythmic muscle contraction and relaxation)8. Weakness of the distal muscles of the affected limb, especially in the hand and foot, can occur2.

The hand or foot may be swollen, sweaty or dry, warmer or cooler, and may vary in colour from the non-affected side by being pink, red or purplish or white or bluish in colour9. These signs are not constant and may be triggered by sensory stimuli, environmental changes and psychological stressors.

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What is the impairment, if any?

Note: The term “impairment” has been interpreted in this context to mean a loss of, or damage to, a physical, sensory or mental function – see Mulligan and NDIA [2014] AATA 374 at [19]

CRPS may result in loss of or damage to the following functions:

• mental functions e.g. energy and drive functions, sleep, attention, memory, emotional functions, perceptual functions, higher level cognitive functions
• sensory functions e.g. pain, light touch, temperature
• neuromuscular and movement related functions e.g. mobility of joint, muscle power and tone, involuntary movements, balance and co-ordination
• functions of skin & related structures e.g. skin temperature, sweating, nail & hair growth

What medical and allied health specialties are involved in:

a) Diagnosis CRPS is often suspected by a medical doctor, occupational therapist or physiotherapist. However, the diagnosis should only be confirmed when the client’s presentation meets the Budapest diagnostic criteria®. The authors caution against the inappropriate use of the diagnostic label “CRPS” as it can create fear and stress in clients (who then undertake misguided internet-based research on the condition) or can lead to a lack of appropriate treatment for the true underlying condition e.g infection, nerve compression or conversion disorder.

b) Treatment of this condition Treatment is optimally provided in a multidisciplinary environment where medical specialists, occupational therapists, physiotherapists, psychologists, and others work together in a fluid and interactive manner®. In order to minimise chronicity, treatment of acute CRPS should be immediate and directed toward restoration of function in the affected extremity’. Because use of the affected part is painful, flexible pain reduction strategies to make the treatment tolerable to the patient must underpin the functional restoration”’. In chronic CRPS, the treatment paradigm becomes more reflective of the chronic non cancer pain (CNCP) model and the reader is invited to refer to that document for a full discussion. In chronic CRPS where there is dystonia, myoclonus and/or limb deformity, neurologists, orthotists, and podiatrists may also play an ongoing role in treating the condition and assisting the client to adapt to their impairment.

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What treatment options are clinically indicated for this condition? What are the indications and likelihood of success for each treatment? Please comment on details and dosage of any recommended treatments including frequency and duration as appropriate.

The International Association for the Study of Pain (IASP) has recommended early multidisciplinary management of individuals with CRPS, which includes the provision of physiotherapy, psychology and medical interventions. The outcome of the early management of CRPS is difficult to predict, which creates additional uncertainty and distress to those with CRPS, although there is a reasonable likelihood that the acute form of this condition will resolve or almost completely resolve in the first six to twelve months.

In its chronic stages, CRPS should be managed in a similar manner to other chronic pain diagnoses. Unfortunately, a percentage of people will continue to have CRPS and some of these will be severely affected by the condition. These people are a small minority of cases, but as NDIS clients they will require long term support including physical rehabilitation, psychological treatment, pain management training via a multidisciplinary team (refer to the CNCP document), and pain reduction therapies (medications, interventions and implanted devices). In clients experiencing dystonia, botulinum toxin injections may be appropriate. In addition there may be a need for the provision of orthotics, braces, splints and other aids, modifications to homes, cars and workplaces.

The following provides some insights into current treatment strategies for CRPS for which there is some evidence. The evidence for most therapies is low: amongst reasons for this are: the challenge of providing a suitable control group in studies; the variability and fluctuating course of the clinical manifestations of CRPS; and the difficulty in measuring changes in variables with clinical significance.

Physical therapies

Physiotherapy and occupational therapy are of the utmost importance in achieving functional recovery in CRPS. It has been recommended that physiotherapy aimed at restoring function be started as soon as possible after the onset of CRPS. An algorithm of treatment has been published which promotes a stepwise progression commencing with reduction of pain and oedema, followed by improving range of motion, then improving muscle strength, function of the whole limb, and finally function of the whole person. Graded exposure delivered in a psychologically informed manner by a physical therapist offers an appropriate approach. Graded motor imagery and mirror therapies have shown utility in certain groups of research subjects with CRPS.

Psychological therapies

Psychological therapies for people with CRPS are generally the same as those used in other pain conditions. However, there are some distinctive features of the presentation that require specific responses. It is particularly important in CRPS to teach strategies such as relaxation, mindfulness, and breathing techniques that assist the person to cope with stress and decrease sympathetic nervous system arousal. Pacing, graduated exposure, and desensitisation are likely to be necessary to overcome fear of movement and avoidance of activity. Cognitive reframing (used in Cognitive Behavioural Therapy) will be vital if a client has developed “dysmorphobia” (a pre-occupation with the appearance of the affected body part leading to avoidance including socializing or exposing the affected part). In rare cases, clients develop problems with “body integrity,” so the person has a sense that the affected body part does not belong to them or has changed in shape or size. Cognitive reframing, desensitisation and graduated exposure and de-arousal/relaxation techniques would all be appropriate in these cases. It is essential to address coexisting psychopathology such as anxiety, post traumatic stress disorder and depression, and to be aware of the impact of any personality factors.

Medications

Initially, the principal role of medication should be to assist improving function including participation in occupational therapy and physical therapy; however for clients whose symptoms remain a year or more after intensive rehabilitation, the role of medication may be largely palliation of pain. Medications may potentially be used for decades in patients with CRPS.

No particular class of medications is specifically indicated for the treatment of CRPS, and the response to each class varies from patient to patient. Medications used are those used in other pain conditions. As always, use of a medication should balance actual benefit from side effects. Medications commonly used may be from these therapeutic classes: tricyclic antidepressants, selective noradrenergic reuptake inhibitors, gabapentinoids, opioids, paracetamol/non-steroidal anti-inflammatories/COX-2 inhibitors. Other types of medications including creams and gels may be used. Often, more than one class is used. Despite this, many patients with chronic CRPS will have incomplete pain relief.

Interventional Therapies

Sympathetic ganglion blockade has historically been considered an important procedure in both the diagnosis and treatment of CRPS. It would appear that only a subgroup of CRPS patients — perhaps

Implanted therapies

Implanted therapies for CRPS include forms of neurostimulation such as spinal cord stimulation and peripheral nerve stimulation, as well as intrathecal drug delivery. Implanted therapies in current clinical use with patients with CRPS include:

• Spinal cord stimulation (SCS) arises from electrodes being placed in the epidural space (the space surrounding the spinal cord) and connected to an impulse generator which sends rapid pulses of electrical energy into the spinal cord causing inhibition of pain signalling, and sometimes changing the neural control of blood flow to parts of the body.

• Peripheral nerve stimulation is indicated in the patient with pain predominating in the distribution of a peripheral nerve, such as the median or ulnar nerve. The technology is similar to SCS.

• Other forms of stimulation such as trans-cranial magnetic stimulation and deep brain stimulation have been found to be effective in selected cases, but remain mostly utilised in research rather than being clinically applicable at present.

• Implantable pumps deliver certain drugs intrathecally, i.e. to the fluid around the spinal cord and onto the spinal cord and brain, straight to the drug’s target receptors. The result is higher concentrations at the target receptors and less side effects from delivery of the drug to other parts of the body. For example, this can be particularly useful when using baclofen to treat dystonia.

5. If this condition results from an impairment, what is the likelihood that this impairment will be permanent?

Note: An impairment is, or is likely to be, permanent only if there are no known, available and appropriate evidence based treatments that would be likely to remedy (i.e. cure or substantially relieve) the impairment — see Rule 5.4 of the Rules

There is enormous variation in the prognosis for CRPS.

Many people recover in the first six to twelve months. After this time there is a diminishing chance of recovery. Once there are significant signs of joint fibrosis (a stiffening of the joints) or muscle dystonia it is the authors’ experience that it appears to be unlikely that there will be significant improvement. Again it is the authors’ experience that in these severe cases, the clinical pathway is then further deterioration over another one to two years with stabilisation occurring by the end of year two or three. At this time it would appear that any impairments will be permanent.

Are individuals suffering this condition likely to require lifelong support? If so, what types of supports are likely to be required?

Individuals suffering the severe form of CRPS are likely to require life long support, although the nature of this will vary between individuals. As stated in section 4, this support may include pain reduction and management, physical rehabilitation, psychological assistance with coping and acceptance of their changed body, pain management training via a multidisciplinary team (refer to the CNCP document) and pain reduction therapies (medications, interventions and potentially implanted devices). In clients experiencing dystonia, botulinum toxin injections may be appropriate. In addition, there will be a need for the provision of orthotics, braces, splints and other aids, modifications to homes, cars and workplaces. In the cohort of severely affected clients, these treatments may need to be provided regularly, but not frequently, e.g. botulinum toxin every 3 to 6 months, accompanied by 2 physiotherapy sessions, ketamine infusion every 3 to 6 months, an occupational therapy review every 2 years.

Would symptom management through interventions such as medication change, pain management, exercise programs etc. reduce the functional impact of the diagnosis and associated disability?

Yes: symptom management specifically aims to reduce the functional impact of the diagnosis and associated disability. However, for some people with a severe form of CRPS, pain reduction alone can be an appropriate aim, whether it reduces the functional impact or not. The following interventions aim to reduce the functional impact and associated disability:

• Reduction of dystonia through botulinum toxin injections and the use of splinting to prevent contractures.
• Medication management.
• Multidisciplinary pain management (refer to the CNCP document).

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• Exercise programs designed and supervised by a physiotherapist over a period of 8 to 12 weeks in gymnasiums or hydrotherapy pools.
• Hydrotherapy pools also allow for the whole person to exercise while the affected limb is gently supported by the buoyancy of the water, the water temperature and pressure on the limb can exert a therapeutic advantage greater than that of gym-based exercise.
• The general principle should be that clients are instructed in land-based and water-based exercises in the expectation that they continue them without supervision but having reviews at intervals of say 6 months to 2 years to confirm that the exercises are being continued appropriately or so that minor changes can be made.

8. How prevalent is the incidence of CRPS being diagnosed as a stand-alone condition as opposed to being diagnosed as part of comorbidity?

By definition CRPS “usually develops after an initiating noxious event”®, such as a fracture, a soft tissue injury, or some form of surgery. If diagnosis is made promptly then the inciting event is often clear and the CRPS can be seen to have occurred in relation to this. However, when diagnosis occurs late in the presentation, the inciting event may no longer be contributing to the presentation, for example a fracture or soft tissue injury may have healed.

9. Any other comments? A small subset of people with CRPS develop an extremely disabling and painful condition, so severe that they may ask for the limb to be amputated. These requests can be misinterpreted as a sign of mental illness. It is essential that people making these requests are taken seriously and are given access to a multidisciplinary service skilled in the management of CRPS and able to offer assessment by an entire team including a Pain Specialist (Fellow of the Faculty of Pain Medicine) and a Psychiatrist where deemed necessary.

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References:

• Baron, R. Complex regional pain syndromes. In Mc Mahon S and Koltzenburg M. (Eds). Wall and Melzacks Textbook of Pain. 5th edition. Philadelphia: Elsevier/Churchill Livingstone; 2005.
• Harden, R., Baron, R., Janig, W. (Eds.). Complex Regional Pain Syndrome. Progress in Pain Research and Management. Seattle, IASP Press; 2001.
• de Mos, M., de Bruijn, A., Huygen, F., Dieleman, J., Stricker, B., Sturkenboom, M. The incidence of complex regional pain syndrome: A population based study. Pain, 2007; 129:12-20.
• Oerlemans, H., Cup, E., de Boo, T., Goris, R., Oostendorp, R. The Radboud skills questionnaire: construction and reliability in patients with reflex sympathetic dystrophy of one upper extremity. Disability and Rehabilitation, 2000; 20:233-245.
• Merskey, H. and Bogduk, N. (Eds.). Classification of Chronic Pain, Descriptions of Chronic Pain Syndromes and Definitions of Pain Terms. Seattle, IASP Press; 1994.
• Harden, R. Objectification of the diagnostic criteria for CRPS. Pain Medicine, 2010; 11:1212-1215.
• Perez, R., Zollinger, P., Dijkstra, P., Thomassen-Hilgersom, I., Zuurmond, W., Rosenbrand, K., Geertzen, J. and the CRPS 1 task force. Evidence based guidelines for complex regional pain syndrome type 1. BMC Neurology, 2010; 10:20.
• van Hilten, J. Movement disorders in Complex Regional Pain Syndrome. Pain Medicine, 2010; 11:1274-1277.
• Sandroni, P., Wilson, P. Factor III: Sudomotor changes and edema - Pathophysiology and measurement. In Wilson P., Stanton-Hicks M., Harden R. (Eds.). CRPS: Current Diagnosis and Therapy. Seattle: IASP Press; 2005. 32: 107-118.
• Stanton-Hicks, M., Burton, A., Bruéhl, S., Carr, D., Harden, N., Hassenbusch, S., Lubenow, T., Oakley, J., Racz, G., Raj, P., Rauck, R. and AR, R. An updated interdisciplinary clinical pathway for CRPS: Report of an expert panel. Pain Practice, 2002; 2:1-16.
• de Jong, J., Vlaeyen, J., Onghena, P., Cuypers, C., den Hollander, M., and Ruijgrok, J. Reduction of pain related fear in Complex Regional Pain Syndrome type one: The application of graded exposure in vivo. Pain Practice, 2005; 116:264-275.
• Singh, G., Willen, S., Boswell, M., Janta, J., and Chelimsky, T. The value of interdisciplinary pain management in Complex Regional Pain Syndrome type one: A prospective outcome study. Pain Physician, 2004; 7:203-209.
• Daly, A., and Bialocerkowski, A. Does evidence support physiotherapy management of adult Complex Regional Pain Syndrome Type One? A systematic review. European Journal of Pain, 2009; 13:339-353.
• Brugger, P., and Lenggenhager, B. (2014) The bodily self and its disorders: neurological, psychological and social aspects. Current opinion in neurology. 27 (6): 644-52
• Oaklander, A. Evidence based pharmacotherapy for CRPS and related conditions. In Wilson P., Stanton-Hicks M., Harden R. (Eds.). CRPS: Current Diagnosis and Therapy. Seattle: IASP Press; 2005. 32: 181-200. Burton et al., 2005
• Burton, A., Lubenow, T., and Raj, P. Traditional interventional therapies. In Wilson P., Stanton-Hicks M., Harden R. (Eds.). CRPS: Current Diagnosis and Therapy. Seattle: IASP Press; 2005. 32: 217-233.
• Janig, W., and Baron, R. Complex regional pain syndrome: mystery explained? Lancet, Neurology, 2003; 2:687-697.

Appendix A: Disability and Early Intervention sections of the NDIS Act 2013

Section 24: Disability requirements

(1) A person meets the disability requirements if: (a) the person has a disability that is attributable to one or more intellectual, cognitive, neurological, sensory or physical impairments or to one or more impairments attributable to a psychiatric condition; and (b) the impairment or impairments are, or are likely to be, permanent; and (c) the impairment or impairments result in substantially reduced functional capacity to undertake, or psychosocial functioning in undertaking, one or more of the following activities: (i) communication; (ii) social interaction; (iii) learning; (iv) mobility; (v) self care; (vi) self management; and (d) the impairment or impairments affect the person’s capacity for social and economic participation; and

Section 25. Early intervention requirements

(e) the person is likely to require support under the National Disability Insurance Scheme for the person’s lifetime.

(2) For the purposes of subsection (1), an impairment or impairments that vary in intensity may be permanent, and the person is likely to require support under the National Disability Insurance Scheme for the person’s lifetime, despite the variation.

Section 25. Early intervention requirements

(1) A person meets the early intervention requirements if: (a) the person:

(i) has one or more identified intellectual, cognitive, neurological, sensory or physical impairments that are, or are likely to be, permanent; or (ii) has one or more identified impairments that are attributable to a psychiatric condition and are, or are likely to be, permanent; or (iii) is a child who has developmental delay; and (b) the CEO is satisfied that provision of early intervention supports for the person is likely to benefit the person by reducing the person’s future needs for supports in relation to disability; and (c) the CEO is satisfied that provision of early intervention supports for the person is likely to benefit the person by: (i) mitigating or alleviating the impact of the person’s impairment upon the functional capacity of the person to undertake communication, social interaction, learning, mobility, self care or self management; or (ii) preventing the deterioration of such functional capacity; or (iii) improving such functional capacity; or (iv) strengthening the sustainability of informal supports available to the person, including through building the capacity of the person’s carer.

Note: In certain circumstances, a person with a degenerative condition could meet the early intervention requirements and therefore become a participant.

(2) The CEO is taken to be satisfied as mentioned in paragraphs (1)(b) and (c) if one or more of the person’s impairments are prescribed by the National Disability Insurance Scheme rules for the purposes of this subsection.

(3) Despite subsections (1) and (2), the person does not meet the early intervention requirements if the CEO is satisfied that early intervention support for the person is not most appropriately funded or provided through the National Disability Insurance Scheme, and is more appropriately funded or provided through other general systems of service delivery or support services offered by a person, agency or body, or through systems of service delivery or support services offered: (a) as part of a universal service obligation; or (b) in accordance with reasonable adjustments required under a law dealing with discrimination on the basis of disability.

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DOCUMENT 4.2

MD FRACP

Professor of Medicine

Consultant Infectious Diseases Physician

Provider No: s47F - personal privacy

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Appointments:

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Report in response to a request for information and advice regarding chronic fatigue syndrome.

Preamble: The request (31.1.2018) sought information and advice regarding chronic fatigue syndrome, however it should be noted firstly that this diagnosis is syndromal - that is it is a syndrome recognized via a characteristic set of symptoms and careful exclusion of alternative medical and psychiatric explanations for those symptoms (individually or as a whole) via medical and psychiatric history, physical examination and laboratory investigation)1. This sort of assessment contrasts with diagnoses made on the basis of a test, such as pneumonia recognized by chest Xray. Secondly, as a consequence of this syndromal diagnosis the label of chronic fatigue syndrome is recognized to overlap with other syndromal diagnoses, such as fibromyalgia (in which pain rather than fatigue is the dominant feature).2 In practice, this means that individual patients may be given both diagnoses in relation to the same set of symptoms. Thirdly, a diagnosis of chronic fatigue syndrome may be replaced in some circumstances with an alternative label (for the same condition) when the prolonged illness follows from a well-characterised initiating event. This includes post viral fatigue syndrome or post-infective fatigue syndrome when the triggering event was an acute infection (such as glandular fever)3. The label chronic fatigue syndrome is referred to in the UK as myalgic encephalomyelitis (ME). The symptom set and diagnostic approach for chronic fatigue syndrome is closely analogous to the diagnosis of post cancer fatigue, which is applied when survivors of cancer have completed surgery and adjunctive treatments such as chemotherapy and radiotherapy, are free of cancer recurrence, but have a disabling chronic fatigue state4. Finally, there are various diagnostic criteria that have been proposed for the chronic fatigue syndrome, placing emphasis on slightly different elements of the illness, but the most widely accepted and recommended criteria5, are those usually termed the ‘international diagnostic criteria’ which were formulated by an international expert group convened by the Centers for Disease Control in the USA1.

What is the aetiology of this condition? Chronic fatigue syndrome is a condition characterised by prolonged (greater than 6 months), unexplained and disabling fatigue, which is accompanied by neurocognitive difficulties, like impairments in short-term memory and concentration, as well as the complaint of unrefreshing sleep. In addition, constitutional symptoms are typical including muscle pain (myalgia), joint pain (arthralgia), recurrent sore throat, headache, and tender lymph nodes in the neck (i.e cervical lymph nodes)1. The fatigue state is characterised by a sustained worsening of symptoms after

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MD FRACP

Professor of Medicine

Consultant Infectious Diseases Physician

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Appointments:

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relatively minor physical or cognitive tasks – often termed ‘post-exertional malaise’ (after physical activity) or ‘brain fog’ (after cognitive activity). The diagnostic criteria also require careful exclusion of alternative medical and psychiatric explanations for the symptoms by a full history, complete physical examination, and laboratory investigations. The minimum requirement for the investigations include: a full blood count, markers of inflammation (ESR or CRP), renal, liver and thyroid function tests, as well as a random blood sugar, and a urinalysis.

In terms of aetiology, the only scientifically-recognised trigger for the onset of a chronic fatigue syndrome is an acute infection3, 6. The mechanism of disease remains unknown.

2. What is the impairment, if any? Note: The term

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practitioners are not confident in making the diagnosis – despite the expert recommendations that they should be able to do so5. In my opinion, in cases being considered by the NDIS, it may be reasonable to enforce an expectation that the diagnoses be affirmed by both a specialist physician and psychiatrist.

Despite more than 100 randomised controlled trials having been completed, there is no evidence-based curative treatment for chronic fatigue syndrome3, 6, 7. There are a myriad of proposed cures found on the internet, which have no credible evidence-base.

4. What treatment options are clinically indicated for this condition? What are the indications and likelihood of success for each treatment? Please comment on details and dosage of any recommended treatments including frequency and duration as appropriate. The only evidence-based management approaches which reduce symptom severity and impairment, include cognitive behavioural therapy (CBT) 8, 9, graded exercise therapy (GET)10 and cognitive remediation11, 12. There are Australian data from an integrated service providing these interventions which support their effectiveness, but recognize that the benefits are incomplete (that is not everyone gains improvement), the magnitude is relatively modest, and may not be sustained13.

5. If this condition results from an impairment, what is the likelihood that this impairment will be permanent? Note: An impairment is, or is likely to be, permanent only if there are no known, available and appropriate evidence based treatments that would be likely to remedy (i.e. cure or substantially relieve) the impairment – see Rule 5.4 of the Rules. Given that there is no evidence for any curative intervention (as above), the key issue regarding permanence of impairment due to chronic fatigue syndrome relates to the natural history of the condition. When followed prospectively from acute infections such as glandular fever, the great majority of individuals recover without intervention over weeks to months, but approximately 10% will meet diagnostic criteria for chronic fatigue syndrome at six months14. When the chronic fatigue syndrome has been present in a stable, non-improving pattern, despite evidence-based management (as above) for 5 years, the Australian expert guidelines indicate that the condition should be regarded as permanent for medico-legal purposes.5 In this context, the only additional consideration relates to the severity of the impairment. As described above, chronic fatigue syndrome is an entirely subjective illness (that is there are no abnormal findings on history, examination or laboratory investigation), yet it is clear that the level

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of disability associated with chronic fatigue syndrome is commonly comparable to or greater than conditions such as rheumatoid arthritis 15. A small subset of all patients do suffer from both severely disabling and very prolonged (greater than 5 years) chronic fatigue syndrome – these patients may be housebound or even bed-bound as a result of the illness and despite best available evidence-based management.

6. Are individuals suffering this condition likely to require lifelong support? If so, what types of supports are likely to be required? If the above criteria are met (i.e prolonged and severe disability attributable to chronic fatigue syndrome), the patients typically require practical support to maintain independent living (assistance with shopping, cooking, cleaning) and travel (to/from medical appointments). This would rarely include the need for assistance with personal hygiene. The support may include items for practical support such as computers adapted for bed-based use.

7. Would symptom management through interventions such as medication, pain management, exercise programs etc. reduce the functional impact of the diagnosis and associated disability? All simple interventions such as symptom-relieving medication for pain relief or sleep-promotion should already be in place as part of standard management. I have referred to the evidence base for graded exercise therapy above.

8. How prevalent is the being diagnosed as a stand-alone condition as opposed to being diagnosed as part of co-morbidity? At least 50% of all patients with a chronic fatigue syndrome will develop co-morbid mood disorder (major depression, anxiety) during the course of the illness. This should be recognized and managed on its merits (if present) and the mood disorder will respond to interventions such as anti-depressant medication comparable to ‘stand alone’ major depression. It should be noted that there are multiple studies demonstrating the lack of efficacy of anti-depressant medication in patients with ‘stand alone’ chronic fatigue syndrome.

References

1. Fukuda K, Straus SE, Hickie I, Sharpe MC, Dobbins JG, Komaroff A. The chronic fatigue syndrome: a comprehensive approach to its definition and study. International Chronic Fatigue Syndrome Study Group. Annals of Internal Medicine. 1994; 121(12): 953-9.

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Source Page 56

References and Citations

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• Consultant Infectious Diseases Physician

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1. Goldenberg DL, Simms RW, Geiger A, Komaroff AL. High frequency of fibromyalgia in patients with chronic fatigue seen in a primary care practice. Arthritis Rheum. 1990; 33(3): 381-7.
2. Afari N, Buchwald D. Chronic fatigue syndrome: a review. American Journal of Psychiatry. 2003; 160(2): 221-36.
3. Bennett B, Goldstein D, Friedlander M, Hickie I, Lloyd A. The experience of cancer-related fatigue and chronic fatigue syndrome: a qualitative and comparative study. J Pain Symptom Manage. 2007; 34(2): 126-35.
4. Royal Australasian College of Physicians Working Group. Chronic fatigue syndrome - Clinical practice guidelines 2002. Med J Aust. 2002; 176: S17-55.
5. Prins JB, van der Meer JWM, Bleijenberg G. Chronic fatigue syndrome. Lancet. 2006; 367(9507): 346-55.
6. Smith ME, Haney E, McDonagh M, Pappas M, Daeges M, Wasson N, et al. Treatment of Myalgic Encephalomyelitis/Chronic Fatigue Syndrome: A Systematic Review for a National Institutes of Health Pathways to Prevention Workshop. Ann Intern Med. 2015; 162(12): 841-50.
7. Price JR, Mitchell E, Tidy E, Hunot V. Cognitive behaviour therapy for chronic fatigue syndrome in adults. Cochrane Database Syst Rev. 2008; (3): CD001027.
8. Reid S, Chalder T, Cleare A, Hotopf M, Wessely S. Chronic fatigue syndrome. BMJ Clin Evid. 2011;
9. Larun L, Brurberg KG, Odgaard-Jensen J, Price JR. Exercise therapy for chronic fatigue syndrome. Cochrane Database Syst Rev. 2017; 4: CD003200.
10. Cvejic E, Birch RC, Vollmer-Conna U. Cognitive Dysfunction in Chronic Fatigue Syndrome: a Review of Recent Evidence. Curr Rheumatol Rep. 2016; 18(5): 24.
11. McBride RL, Horsfield S, Sandler CX, Cassar J, Casson S, Cvejic E, et al. Cognitive remediation training improves performance in patients with chronic fatigue syndrome. Psychiatry Res. 2017; 257: 400-5.
12. Sandler CX, Hamilton BA, Horsfield SL, Bennett BK, Vollmer-Conna U, Tzarimas C, et al. Outcomes and predictors of response from an optimised, multidisciplinary intervention for chronic fatigue states. Intern Med J. 2016; 46(12): 1421-9.
13. Hickie I, Davenport T, Wakefield D, Vollmer-Conna U, Cameron B, Vernon SD, et al. Post-infective and chronic fatigue syndromes precipitated by viral and non-viral pathogens: prospective cohort study. Bmj. 2006; 333(7568): 575.
14. Joustra ML, Janssens KA, Bultmann U, Rosmalen JG. Functional limitations in functional somatic syndromes and well-defined medical diseases. Results from the general population cohort LifeLines. J Psychosom Res. 2015; 79(2): 94-9.

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MD FRACP

Professor of Medicine

Consultant Infectious Diseases Physician

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Professor of Medicine

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s47F - personal privacy

Professor s47F - personal privacy MB BS PhD (Medicine) FAFRM (RACP) Consultant Physician in Rehabilitation Medicine

18 January 2018

Ms Wendy s47F - pers Acting Director Technical Advisory Team- Operational Guidance NDIA

Dear Ms s47F - perso

Request for expert information and advice - fibromyalgia

Thank you for your request, dated 22 December 2017, that I provide information and advice regarding fibromyalgia to the National Disability Insurance Agency (NDIA). This is a revised report that has been prepared after consideration of additional materials.

I note the “issues to consider” that you outlined. These, and my responses, are shown below.

This report is based on a review of the scientific literature. I conducted a systematic review and meta-synthesis to update the systematic review of Clauw (2014). The database, Medline, was searched using the terms “fibromyalgia” and “meta-analysis” from 2014 to current. This was to bring the search strategy of Clauw (2014) up to date.

My responses are:

1. What is the aetiology of fibromyalgia?

Fibromyalgia is a condition of generalized body pain without a known cause or cure (Fitzcharles et al 2013). Its aetiology is therefore unknown. It is hypothesised that certain people are more likely to experience chronic widespread pain. This is due to a combination of environmental and genetic influences (Clauw et al 2014). It is definitely more prevalent in women and men in a ratio of 2:1 (Clauw 2014).

2. What is the impairment, if any?

You have advised that

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damage (IASP 2017). The World Health Organisation’s International Classification of Functioning, Disability and Health (ICF) defines generalized pain in similar terms “b2800 Generalized pain - Sensation of unpleasant feeling indicating potential or actual damage to some body structure felt all over, or throughout the body.” (WHO 2017).

Whether long standing generalized pain is seen as an impairment is open to conjecture. Taking the definition of impairment listed in the Mulligan decision there needs to be a loss or damage to a sensory function. It is not possible to detect damage to the body in people with fibromyalgia (Fitzcharles et al 2013). On this basis there is not loss or damage and thus there is no impairment.

The IASP and ICF definitions emphasise pain as a subjective experience that is associated with potential or actual tissue damage. Pain is classified as an abnormality of body function in the ICF (and thus is an impairment in the terms of that classification) but, in the context of the NDIS, it cannot be seen as an impairment given the requirement for actual tissue damage because this is not present in fibromyalgia.

While pain is the most important symptom in fibromyalgia, others “such as fatigue, nonrefreshed sleep, mood disturbance and cognitive impairment are common, but not universal” (Macfarlane et al 2017). These are also symptoms and are not associated with actual tissue damage.

3. What medical and allied health specialties are involved in: a) Diagnosis; and b) Treatment of this condition?

The recent clinical guidelines (Macfarlane et al 2017, Fitzcharles et al 2013) suggest that a definitive diagnosis of fibromyalgia, based on standardized criteria (Clauw 2014) should be made in primary medical care with a minimum of investigations. The most effective treatment is exercise, and health professionals involved with provision of exercise programs can be involved. Other allied health specialties may be involved in treatment, particularly psychologists if there are associated mental health conditions (Macfarlane et al 2017).

4. What treatment options are clinically indicated for this condition? What are the indications and likelihood of success for each treatment? Please comment on details and dosage of any recommended treatments including frequency and duration as appropriate.

There are a variety of treatment options that are available. None of the Guidelines provide a measure of “likelihood of success of treatment” because this will be highly dependent on the group of people with fibromyalgia who are treated. Some groups of people with fibromyalgia, for example those assessed in primary medical care, will have substantially better responses to treatment than others, for example people assessed and treated by medical specialists or in specific settings, for example pain clinics.

The strength of evidence of effectiveness (GRADE) is the best approach to assessing the effectiveness of treatment. Using this method the recommendations are:

There is strong evidence of effectiveness for aerobic or strengthening exercise in the treatment of fibromyalgia. Weak evidence supports the use of non pharmacological treatments which are cognitive behavioural therapy, multicomponent therapies, defined physical therapies (hydrotherapy and acupuncture), meditative movement therapies (qigong, yoga, tai chi) and mindfulness-based stress

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reduction. Weak evidence also supports the use of pharmacological treatments which are amitriptyline (in low dose), duloxetine, tramadol and pregabalin (MacFarlane et al 2017).

If this condition results from an impairment, what is the likelihood that this impairment will be permanent?

It is noted that impairment is, or is likely to be, permanent only if there are no known, available and appropriate evidence based treatments that would be likely to remedy (i.e. cure or substantially relieve) the impairment – as shown in Rule 5.4 of the Rules.

Fibromyalgia cannot be regarded as permanent because it is responsive to treatment (see above) and is likely to vary in intensity at different times due to a variety of factors.

A well conducted study has shown that most people continue to experience symptoms over a long period but approximately 25% improved in the long term (Walitt et al 2011). However, severity worsened in about 40%. Thus there is considerable variability in prognosis and responses to treatment. It should be noted that this study was conducted in patients of American rheumatologists and may represent a cohort of people with more severe fibromyalgia than those treated in primary healthcare settings.

Fibromyalgia cannot be considered permanent in the same way as many health conditions, because the underlying impairment is pain and this varies considerably over time and in response to treatments and life situations.

Are individuals suffering this condition likely to require lifelong support? If so, what types of supports are likely to be required?

Fibromyalgia is a form of chronic pain (that is pain that is present for more than three months of the previous six months). Chronic pain affects about 20% of the Australian population (Blyth et al 2001). Clearly almost all people with chronic pain do not require lifelong support.

Specifically, people with fibromyalgia do not require lifelong support and, indeed provision of this support is likely to be harmful to them. Fitzcharles et al (2013) summarise a fundamental component of assisting people with fibromyalgia as “the requirement that the health community focus toward maintaining and improving function of patients, with a call to temper the culture of disablement and medicalization that has become evident in recent years”. While pain may interfere with daily activities to some extent the presence of pain is not a reason to avoid specific activities.

Would symptom management through interventions such as medication change, pain management, exercise programs etc. reduce the functional impact of the diagnosis and associated disability?

These are all treatments that have some evidence of effectiveness. Therefore they are likely to reduce the functional impact of fibromyalgia.

Contemporary clinical guidelines (Macfarlane et al 2017) recommend an approach of assessment and diagnosis in the setting of primary healthcare. If the diagnosis is established, patient education and provision of an information sheet is recommended. If that is insufficient, physical therapy with “individualized graded physical exercise” is the next step. If further treatment is required, the

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Recommendation is for further assessment and treatment.

If psychological symptoms are prominent, psychological therapies are recommended. If there is severe pain or sleep disturbance, pharmacological therapy is recommended. If there is severe disability, a “multimodal rehabilitation program” is recommended (Macfarlane et al 2017). Thus, there is a well-established treatment guideline that is applicable in the condition and is likely to be effective in a significant proportion of people with fibromyalgia.

How prevalent is the incidence of fibromyalgia being diagnosed as a stand-alone condition as opposed to being diagnosed as part of comorbidity?

Fibromyalgia is frequently associated with other health conditions and adverse social circumstances and past adverse life events. Ten to 30% of people with fibromyalgia will have other rheumatological conditions, and people with fibromyalgia are more likely to have psychiatric disorders, including depression, anxiety, obsessive-compulsive disorder, and posttraumatic stress disorder (Clauw et al 2014). These data suggest that about 20% to 40% of people with fibromyalgia will have another health condition that is relevant for how the fibromyalgia will be approached.

Other comments

There are clear risks for people with fibromyalgia in becoming involved in processes involving proving that their disability is sufficiently severe to meet requirements for disability eligibility (Hadler 1996). Hadler discusses the “vortex of disability determination,” which, in his opinion, is clearly detrimental to the person with fibromyalgia or chronic pain more broadly.

Schweiger et al (2017) provide a worldwide review of trends in disability claims in fibromyalgia. They include Australia and note that the Australian ICD-10-AM code for FMS is M79.7. They note that fibromyalgia “is not fully recognized worldwide, and patients often do not receive the treatment and disability benefits planned for other chronic diseases” (Schweiger et al 2017). However, their review concentrates on work disability claims and does not deal specifically with schemes such as the Australian National Disability Insurance Scheme.

I have noted Appendix A which sets out the disability requirements.

Yours sincerely, s47F - personal privacy 847F - personal privacy

References

1. Blyth FM, March LM, Brnabic AJ, Jorm LR, Williamson M, Cousins MJ. Chronic pain in Australia: a prevalence study. Pain. 2001 Jan;89(2-3):127-34. PubMed PMID: 11166468.

2. Clauw DJ. Fibromyalgia: a clinical review. JAMA. 2014 Apr 16;311(15):1547-55. doi: 10.1001/jama.2014.3266. Review. PubMed PMID: 24737367.

3. Fitzcharles MA, Ste-Marie PA, Goldenberg DL, Pereira JX, Abbey S, Choiniere M, Ko G, Moulin DE, Panopalis P, Proulx J, Shir Y. Canadian Pain Society and Canadian Rheumatology Association

Recommendations for Rational Care of Persons with Fibromyalgia: A Summary Report

J Rheumatol. 2013 Aug;40(8):1388-93. doi:10.3899/jrheum.130127. Epub 2013 Jul 1. PubMed PMID: 23818709.

References

4. Hadler NM. If you have to prove you are ill, you can’t get well: the object lesson of fibromyalgia. Spine (Phila Pa 1976). 1996;21(20):2397-2400.

5. IASP. https://www.iasp-pain.org/Taxonomy, accessed 18 January 2018

6. Macfarlane GJ, Kronisch C, Dean LE, Atzeni F, Häuser W, Fluß E, Choy E, Kosek E, Amris K, Branco J, Dincer F, Leino-Arjas P, Longley K, McCarthy GM, Makri S, Perrot S, Sarzi-Puttini P, Taylor A, Jones GT. EULAR revised recommendations for the management of fibromyalgia. Ann Rheum Dis. 2017 Feb;76(2):318-328. doi: 10.1136/annrheumdis-2016-209724. Epub 2016 Jul 4. Review. PubMed PMID: 27377815.

7. Schweiger V, Del Balzo G, Raniero D, De Leo D, Martini A, Sarzi-Puttini P, Polati E. Current trends in disability claims due to fibromyalgia syndrome. Clin Exp Rheumatol. 2017 May-Jun;35 Suppl 105(3):119-126. Epub 2017 Jun 29. Review. PubMed PMID: 28681709.

8. Walitt B, Fitzcharles MA, Hassett AL, Katz RS, Häuser W, Wolfe F. The longitudinal outcome of fibromyalgia: a study of 1555 patients. J Rheumatol. 2011 Oct;38(10):2238-46. doi: 10.3899/jrheum.110026. Epub 2011 Jul 15. PubMed PMID: 21765102.

9. WHO. http://apps.who.int/classifications/icfbrowser, accessed 18 January 2018