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.

• 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 important in determining quality of life are:

• Physical and material well-being• Material well-being and financial security • Health and personal safety• Relations with other people• Relations with spouse • Having and rearing children

Dimensions of QoL

• 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

• 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 pain- factors 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

• 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

• 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

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 non-pharmacological options

In slowly progressive NMD

• Pain is likely under-reported 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

• 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• are parents or siblings depressed?

• Is the child adapting in school?

• Is the adult being accommodated at work?

• REMEMBER THE ADA!!!

Future directions: Cannabinoid-Based medicines

• 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