Transcript Neuromuscular Disorders and Pain

American Academy of Physical
Medicine and Rehabilitation 2015
Myalgia, Neuralgia, and Arthralgia:
Neuromuscular Disorders and Pain
Impact of pain on function and quality of life
across the spectrum of neuromuscular
disorders (NMDs)
• Slides: Gregory T Carter, MD, MS
• Medical Director, St Luke’s Rehabilitation
Institute, Spokane, WA
• Speaker: Erik Ensrud, MD
• Director, Neuromuscular Center,
Boston VA HCS
• No disclosures or conflicts of interest from
either
Objectives
• -Review the frequency of pain in patients
with various forms of NMDs
• -Outline the impact of pain on function
• -Discuss novel strategies for the treatment
of pain in patients with NMDs
Defining the “issues”
• Quality of life is a vague
and ethereal entity,
something that many
people talk about, but
which nobody very clearly
knows what to do about.
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Campbell, in
The Quality of American
Life
What is Quality of Life (QoL)?
• “An individuals perception of their position
in life in the context of the culture and value
systems in which they live, in relation to
their goals, expectations, standards, and
concerns” WHO, 2001
Why do We Measure QoL?
• physicians consider their patients more
impaired than the patients themselves
• same for families and caregivers
• gives the clinician valuable information that
can help in making the best choices in
patient care.
Dimensions of QoL
Dimensions that are most
determining quality of life are:
important
• Physical and material well-being
• Material well-being and financial security
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Health and personal safety
Relations with other people
Relations with spouse
Having and rearing children
in
Dimensions of QoL
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Relations with parents, siblings, or other relatives
Relations with friends
Social, community, civic activities
Helping and encouraging others
Participating in local and governmental affairs
Personal development, fulfillment
Intellectual development
Dimensions of QoL
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Understanding and planning
Occupational role career
Creativity and personal expression
Recreation
Socializing with others
Passive and observational recreational activities
Participating in active recreation
Comment
• These dimensions are NO different for
people with neuromuscular disorders or
other disabling conditions
How Do We Measure QoL?
• Use the right tools
• Know how to interpret
the information
• Use controls
Examples
• Medical Outcomes Study Short Form 36
(SF 36)
• Nottingham health Profile
• Sickness Impact Profile
• World Health Organization Quality of Life
instrument
For NMD patients these tools can
• monitor their health status and the impact of
domains like PAIN on QoL
• assess effectiveness of rehabilitation services
• justify allocation of limited social and healthcare
resources
• tailor management to the needs of the patient
• serve as outcome measures in trials
QoL is impacted by painfactors that impact this are:
• Severity and distribution of weakness
• The relative suddeness of loss and tempo of
progression (rapid versus slow)
• Presence of sensory impairment
• Presence of comorbidity: speech and
swallowing problems, labored or restricted
breathing, cognitive issues
Pain and QoL now well studies
in the major NMDs
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Amyotrophic lateral sclerosis
Duchene/Becker (DMD/BMD
Myotonic (DM1/DM2)
Limb Girdle (LGMD):
Facioscapulohumeral (FSHD)
Charcot Marie Tooth disease (CMT)
Spinal Muscular Atrophy (SMA)
Brief Literature Review
• Growing body of evidence showing that
chronic pain is common in most forms of
NMD and does negatively impact QoL
• Treatment paradigms not well studied
• Long terms outcomes not well studied
Hanisch F, et al. Characteristics of pain in
amyotrophic lateral sclerosis. Brain Behav 2015
5(3):e00296
• Pain is an often underestimated and
neglected symptom in amyotrophic lateral
sclerosis (ALS).
• Pain reported in up to 78% of ALS
patients,79% of DM2 patients, versus 54%
of controls (P<0.05).
• ALS patients reported moderate to severe
pain
Hanisch F, et al. Characteristics of pain in
amyotrophic lateral sclerosis. Brain Behav 2015
5(3):e00296
• There was no correlation between the
duration of the disease and the severity of
pain
• Movement-induced cramps were reported in
63% of ALS patients,
• pain is a frequent symptom and negatively
impacts quality of life at every stage of
ALS.
Jensen MP, et al. Chronic pain in persons with
neuromuscular disorders. Arch Phys Med Rehabil
2005; 86(6):1155-1163
• Pain is a common problem in NMD
• There are important differences between different
NMD groups on the nature and scope of pain and
its impact
• Seventy-three percent of broad based sample
reported pain, with 27% of these reporting that
this pain was severe
• Patients with ALS and DM1 reported the greatest
pain interference
Abresch RT, et al. Assessment of pain and health-related
quality of life in slowly progressive neuromuscular disease.
Am J Hosp Palliat Care 2002; 19(1):39-48
• descriptive, non-experimental survey of a total of 1,432
subjects with slowly progressive NMDs
• frequency and severity of pain reported in slowly
progressive NMDs was significantly greater than levels of
pain reported by the general US population and was
comparable to pain reported by subjects with osteoarthritis
and chronic low back pain
• adult SMA had least frequency and severity of pain
reported
Abresch RT, et al. Assessment of pain and health-related
quality of life in slowly progressive neuromuscular disease.
Am J Hosp Palliat Care 2002; 19(1):39-48
• Frequency and severity of musculoskeletal type pain
reported in NMD was significantly greater than levels of
pain reported by the general United States population and
was comparable to pain reported by subjects with
osteoarthritis and chronic low back pain.
• In NMD, like the general population, there is a significant
correlation between pain and depression. This creates
fatigue, sleep disturbance, loss of vitality, and decreased
social interactions.
Carter GT, et al. Neuropathic Pain in Charcot Marie Tooth
disease. Arch Phys Med Rehabil 1998; 79:1560-4
• 617 CMT subjects
• 440 (71%) reported pain. with the most severe pain sites
noted as low back (70%), knees (53%), ankles (50%), toes
(46%), and feet (44%).
• Most common pain descriptors included hot, dull, and
deep.
• Neuropathic pain is a significant problem for many people
with CMT. The frequency and intensity of pain reported in
CMT is comparable in many ways to PHN, CRPS-1, DN
Jensen MP et al. Chronic pain in persons with myotonic and
facioscapulohumeral muscular dystrophy. Arch Phys Med
Rehabil 2008; 89(2):320-328
• subjects with FSHD (82%) and DM1 (64%)
reported pain.
• the most frequently reported pain sites for both
diagnostic groups were lower back (66% DM1,
74% FSHD) and legs (60% DM1, 72% FSHD).
• mobility limitations and use assistive devices (eg,
wheelchair, cane) correlated to more pain severity
• pain is common in persons with FSHD and DM1
Engel JM, et al. Pain in youths with neuromuscular
disease. Am J Hosp Palliat Med 2009; 26(5):405-412
• Study included youth with DMD, BMD,
DM1, LGMD, FSHD, C-MD
• 55% of the youths reported having chronic
pain.
• Pain in the legs was most commonly
reported
• 83% reported using pain medications.
In rapidly progressive disorders
• Assess and treat pain aggressively - Treat patient
with the goal of relieving the symptoms
• Make sure your goals match the patient’s goals
and expectations
• Use treatment paradigms that allow patient to stay
engaged and alert
• Consider multi-modality treatment including nonpharmacological options
In slowly progressive NMD
• Pain is likely underreported and may go
unnoticed by
clinicians
• Yet can create
tremendous disability
Strategies for the treatment of
pain in patients with NMDs
• Correcting biomechanics: address neck
flexor weakness, toe walking, lordotic
posturing, etc
• Make sure there is proper fitting equipment
• Treat co-morbidity: depression,
psychosocial issues
Ordering Therapy Modalities
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Stretching/bracing
Pool therapy for exercise
Adaptive devices
Lifts, bed, cushions
Weight control and good Nutrition
Educational assistance: IEP
Appropriate and timely surgical interventions
Family/psychosocial issues
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are parents or siblings depressed?
Is the child adapting in school?
Is the adult being accommodated at work?
REMEMBER THE ADA!!!
Future directions: CannabinoidBased medicines
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alleviates pain and spasticity
Improves appetite
improves sleep/mood state
Dries up oral secretions
Strong anti-oxidant and neuroprotective
Low toxicity, well-tolerated, no LD50
Ethical Considerations
• Patients should direct their care. Clinicians
provide information and options and be
supportive, not directive
• Living with any NMD should be relatively
pain-free
• Thank you for attending