1. Multiple Sclerosis An Overview for Rehabilitation Specialists 1

2. What does MS look like? Julia—a 35yo white married mother of 3 who is exhausted all the time and can’t drive because of vision problems and numbness in her feet Jackson—a 25yo African-American man who stopped working because he can’t control his bladder or remember what he read in the morning paper Maria—a 10yo Hispanic girl who falls down a lot and whose parents just told her she has MS Loretta—a 47yo white single woman who moved into a nursing home because she can no longer care for herself

3. What else does MS look like? Sam—a 45yo divorced white man who has looked and felt fine since he was diagnosed seven years ago Karen—a 24yo single white woman who is severely depressed and worried about losing her job because of her diagnosis of MS Sandra—a 30yo single mother of two who experiences severe burning pain in her legs and feet Richard—who was found on autopsy at age 76 to have MS but never knew it Jeannette—whose tremors are so severe that she cannot feed herself

4. 1396: Earliest Recorded Case of MS

5. From Sister Lidwina to the present… 1868—Jean-Martin Charcot describes the disease and finds MS plaques (scars) on autopsy. 1878—Louis Ranvier describes the myelin sheath (the primary target of MS in the central nervous system). “Multiple sclerosis is often one of the most difficult problems in clinical medicine.” (Charcot, 1894) “When more is known of the causes and…pathology of the disease… more rational methods may brighten the therapeutic prospect.” (Gowers, 1898) 1981—1 st MRI image of MS is published.

6. From Sister Lidwina to the present, cont’d 1993—The first disease-modifying agent for MS— Betaseron—is approved in the U.S. 1998—Bruce Trapp confirms that the nerve fibers themselves are irreversibly damaged early in the disease course (probably accounting for the permanent disability that can occur). 2016—Today, there are several medications approved in the U.S. for the treatment of MS and more in the pipeline. Today there are 2.3 million people worldwide

7. What MS Is: MS is considered an immune-mediated disease—perhaps autoimmune. The immune system attacks the myelin coating around the nerves in the central nervous system (CNS—brain, spinal cord, and optic nerves) and the nerve fibers themselves. Its name comes from the scarring caused by inflammatory attacks at multiple sites in the central nervous system.

8. What MS Is Not: MS is not: –Contagious –Directly inherited –Always severely disabling –Fatal—except in fairly rare instances Being diagnosed with MS is not a reason to: –Stop working –Stop doing things that one enjoys –Not have children

9. What Causes MS? Genetic Predisposition Environmental Trigger Immune Attack Loss of myelin & nerve fiber

10. What happens in MS?...cross the blood-brain barrier… …launch attack on myelin & nerve fibers... “Activated” immune cells... …to obstruct nerve signals myelinated nerve fiber

11. What happens to the myelin and nerve fibers?

12. What are possible symptoms?  Sensory changes (tingling, numbness)  Pain (neurogenic; musculoskeletal)  Spasticity  Gait, balance, and coordination problems  Speech/swallowing problems  Tremor  Fatigue (most common)  Visual problems  Bladder and/or bowel dysfunction  Sexual dysfunction  Emotional disturbances (depression, mood swings)  Cognitive difficulties (memory, attention, processing)  MS symptoms vary between individuals and are unpredictable

13. How is MS diagnosed? MS is a clinical diagnosis: –Signs and symptoms –Medical history –Laboratory tests Requires dissemination in time and space: –Space: Evidence of scarring (plaques) in at least two separate areas of the CNS –Time: Evidence that the plaques occurred at different points in time There must be no other explanation

14. What tests may be used to help confirm the diagnosis? Magnetic resonance imaging (MRI) Visual evoked potentials (VEP) Lumbar puncture

15. What is the genetic factor? The risk of getting MS is approximately: –1/750 for the general population (0.1%) –1/40 for person with a close relative with MS (3%) –1/4 for an identical twin (25%) 20% of people with MS have a blood relative with MS The risk is higher in any family in which there are several family members with the disease (aka multiplex families)

16. What is the prognosis? One hallmark of MS is its unpredictability. –Approximately 1/3 will have a very mild course –Approximately 1/3 will have a moderate course –Approximately 1/3 will become more disabled Certain characteristics predict a better outcome: –Female –Onset before age 35 –Sensory symptoms –Monofocal rather than multifocal episodes –Complete recovery following a relapse

17. Disease Types Clinically isolated syndrome (CIS) Relapsing-remitting MS (RRMS) –About 85% of people are diagnosed with RRMS Primary progressive MS (PPMS) –About 15% of people experience this course Secondary progressive –Most people diagnosed with RRMS will eventually transition to SPMS Lublin et al, 2014

18. Clinically Isolated Syndrome (CIS) A first neurologic event suggestive of demyelination Individuals with CIS are at high risk for developing clinically definite MS if the neurologic event is accompanied by multiple, clinically silent (asymptomatic) lesions on MRI typical of MS

19. Lublin et al, 2014

23. An Overview of Treatment Strategies

24. Who is on the MS “Treatment Team”? Neurologist Urologist Nurse Physiatrist Physical therapist Occupational therapist Speech/language pathologist Psychiatrist Psychotherapist Neuropsychologist Social worker/Care manager Pharmacist Primary care physician

25. What are the treatment strategies? Gone are the “Diagnose and Adios” days of MS care Management of MS falls into five general categories: –Treatment of relapses (aka exacerbations, flare-ups, attacks—that last at least 24 hours) –Symptom management –Disease modification –Rehabilitation (to maintain/improve function) –Psychosocial support

26. How are relapses treated? Not all relapses require treatment –Mild, sensory sx are allowed to resolve on their own. –Sx that interfere with function (e.g., visual or walking problems) are usually treated 3-5 day course of IV methylprednisolone—with/without an oral taper of prednisone –High-dose oral steroids used by some neurologists Rehabilitation to restore lost function Psychosocial support

27. How is the disease course treated? Thirteen disease-modifying therapies are FDA-approved for relapsing forms of MS: –glatiramer acetate (Copaxone®; Glatopa™ - generic equivalent) [inj.] –interferon beta-1a (Avonex®, Plegridy™, Rebif®) [inj.] –interferon beta-1b (Betaseron® and Extavia®) [inj.] –dimethyl fumarate (Tecfidera®) [oral] –fingolimod (Gileny®) [oral] –teriflunomide (Aubagio®) [oral] –alemtuzumab (Lemtrada®) [inf] –mitoxantrone [inf] –natalizumab (Tysabri®) [inf]

28. What do the disease-modifying drugs do? All reduce attack frequency and severity, reduce scarring on MRI, and probably slow disease progression. These medications do not: –Cure the disease –Make people feel better –Alleviate symptoms

29. How important is early treatment? The Society’s National Medical Advisory Committee recommends that treatment be considered as soon as a dx of relapsing MS has been confirmed. –Irreversible damage to axons occurs even in the earliest stages of the illness. –Tx is most effective during early, inflammatory phase –Tx is least effective during later, neurodegenerative phase No treatment has been approved for primary-progressive MS. Approximately 60% of PwMS are on Tx

30. How are MS symptoms managed? Symptom management continues throughout the disease course Effective symptom management involves a combination of medication, rehabilitation strategies, emotional support—and good coordination of care Virtually every medication used to treat MS symptoms is used off-label

31. What role does rehabilitation play? Structured, problem-focused, interdisciplinary interventions to: –Enhance/maintain function, comfort, safety, and independence over the course of the disease –Educate for self-management and behavior change –Identify appropriate assistive devices and environmental modifications –Prevent injuries and unnecessary complications –Empower individual and family

32. Managing MS Fatigue > 80% of people with MS experience fatigue; many identify it as their most disabling symptom Along with cognitive dysfunction, fatigue is the most common cause of early departure from the workforce MS fatigue is easily misunderstood by family members and employers as laziness or disinterest MS fatigue is multi-determined

33. Managing MS Fatigue, cont’d Identify/address contributory factors –Disrupted sleep; muscle fatigue; disability-related fatigue; depression; medications Develop comprehensive treatment plan –Energy conservation: planning/prioritizing; mobility aids; environmental modifications –Exercise regimen –Medications: amantadine; modafinil; armodafinil; psychostimulants

34. A Word about Temperature Sensitivity 70-80% experience heat sensitivity 20% experience cold sensitivity Slight elevations in core body temperature (related to ambient temperature, exercise, fever) can cause temporary worsening of MS symptoms—a pseudoexacerbation Cooling strategies (A/C, scarves, vests, cold liquids, cool showers) can help maintain core body temperature

35. Managing Visual Impairments Nystagmus: Jerky eye movement World is “wiggling” Optic Neuritis – inflammation of the optic nerve can cause: Blurred vision Dimming of colors Pain when eye is moved Blind spots Loss of contrast sensitivity

36. Managing Bladder Dysfunction > 75% of people with MS will experience bladder problems. Bladder dysfunction is a major cause of morbidity, embarrassment, and social isolation.

37. Managing Bladder Dysfunction Storage dysfunction –Small, spastic bladder in which small quantity of urine triggers the urge to void –Sx include: urgency, frequency, incontinence, nocturia –Tx includes: anticiholinergic/antimuscarinic medication Emptying dysfunction –Bladder fails to empty  risk of UTI –Sx include: urgency, frequency, nocturia, incontinence –Tx includes: ISC and anticholinergic/antimuscarinic medications

38. Managing Bowel Problems Experienced by 50% of people with MS –Constipation—most common - Loose stool (related to impaction) –Bowel incontinence—least common Managed best with regular bowel routine –Adequate fluid/fiber intake –Exercise –OTC products as needed –Anticholinergic medications added to manage incontinence

39. Managing Spasticity Experienced by 40-60% of people with MS (more common in the lower extremities) Management strategies: –Stretching –Oral medication (baclofen, tizanidine, clonazapam, gabapentin, cyproheptidine, dantrolene, dopaminergic agonists) –Baclofen pump –Botox injections; nerve blocks; surgery Some spasticity is useful to counteract weakness

40. Managing Primary Sexual Dysfunction 40-80% of men and women with MS –Reduced libido (behavioral/environmental strategies) –Sensory disturbances (anticonvulsant medications) –Anorgasmia (body-mapping exercises) Women –Reduced lubrication (gels) Men –Erectile dysfunction (pharmacotherapy; implants)

41. Managing Secondary/Tertiary Sexual Dysfunction Secondary dysfunction (other contributory factors) –Managing MS symptoms that interfere with sexual activity/pleasure (fatigue, spasticity, bladder dysfunction) –Managing medications to promote sexual comfort and responsiveness (anticholinergics; antidepressants; fatigue and spasticity meds) Tertiary dysfunction (feeling; attitudes) Education; counseling

42. Managing Cognitive Dysfunction Occurs in up to 65% of people with MS Ranges from relatively mild to quite severe Correlates with lesion #, lesion area, and brain atrophy Can occur at any time in the course of the disease Can occur with any disease course Being in an exacerbation is a risk factor for cognitive dysfunction Most common problems: memory; attention/concentration; information processing Treatments: –Disease-modifying therapy to reduce relapses –Cognitive rehabilitation (primarily compensatory)

43. Managing Depression Depression is one of the most common symptoms of MS (resulting from immune system changes, neurologic changes and psychosocial stressors) >50% of people with MS will experience a major depressive episode Suicide in MS is 7x higher than in the general population –Greatest risk factor for suicide in MS is depression. Depression is under-recognized, under- diagnosed and under- treated in MS Depression can impact cognitive function Recommended treatment: psychotherapy + medication + exercise

44. Managing Pain 75% of people with MS experience pain Neuropathic (central) pain –Paroxysmal pain (trigeminal neuralgia; headache) Anticonvulsants –Continuous pain (dysesthesias) Tricicyclics; anticonvulsants Nociceptive (secondary) pain –Musculoskeletal pain  Physical therapy; NSAIDs –Spasticity—As described previously

45. Managing Speech Problems 40-50% experience speech/voice disorders –Dysarthria – impaired volume control, articulation, emphasis –Dysphonia – altered voice quality, pitch control, breathiness, hoarseness Speech/language assessment: –Oral peripheral examination –Voice evaluation –Communication profile Treatment: –Exercises –Strategies and compensatory techniques to improve speech clarity –Augmentative device or ACC, if needed

46. Managing Swallowing Problems (Dysphagia) One of the less common MS symptoms Swallowing assessment –Clinical history –Examination –Videofluoroscopy (modified barium swallow) Treatment –Exercises –Dietary modifications/positioning while eating/chewing strategies –Non-oral feeding options, if needed

47. Managing Ataxia/Tremor One of the less common MS symptoms Potentially severely disabling No effective treatments at this time –Medications that may be tried: propranolol; primidone; acetazolamide; buspirone; clonazepam –Occupational therapy Weighting; assistive devices –Thalamic surgery for tremor (generally poor results)

48. Serious Complications Urosepsis Aspiration pneumonia Pulmonary dysfunction Skin breakdown Untreated depression Osteoporosis

49. What can people do to feel their best? Balance activity with rest. Talk with their rehabilitation professional about the right type/amount of exercise for them. Eat a balanced low-fat, high-fiber diet. Avoid heat if they are heat-sensitive. Drink plenty of fluids to maintain bladder health and avoid constipation. Follow the standard preventive health measures recommended for their age group

50. What else can people do to feel their best? Reach out to their support system; no one needs to be alone in coping with MS. Stay connected with others; avoid isolation. Become an educated consumer. Make thoughtful decisions regarding: –Disclosure –Choice of physician –Employment choices –Financial planning –Health and wellness

51. So what do we know about MS? MS is a chronic, unpredictable disease The cause is still unknown MS affects each person differently; symptoms vary widely MS is not fatal, contagious, directly inherited, or always disabling Early diagnosis and treatment are important –Significant, irreversible damage can occur early on –Available treatments reduce the number of relapses and may slow progression Treatment includes: attack management, symptom management, disease modification, rehab, emotional support

52. Society Resources for People with MS Nationwide network of chapters around the country Web site (www.nationalMSsociety.org) Access to information, referrals, support (1-800-344-4867) Educational programs (in-person, online) Support programs (self-help groups, peer and professional counseling, friendly visitors) Consultation (legal, employment, insurance, long-term care) Financial assistance

53. Society Resources for Healthcare Professionals Professional Resource Center Website: www.nationalMSsociety.org/PRCwww.nationalMSsociety.org/PRC Email: healthprof_info@nmss.org –Clinical consultations with MS specialists –Literature search services –Professional publications –Quarterly e-newsletter for professionals –Professional education programs (medical, rehab, nursing, mental health) –Consultation on insurance and long-term care issues –Free app for your smart phone