1. Multiple Sclerosis: An Overview for Case Management Professionals Susan Raimondo Connecticut Chapter Offices in Hartford and Norwalk 860.913.2550 1.800.344.4867 www.ctfightsMS.org Susan.raimondo@nmss.org Charlene Breen Care Management Associates/ Connecticut Community Care, Inc. Toll-free: 800.654.2183 www.ctcommunitycare.org

2. What does MS look like? Julia—a 35yo white married mother who is exhausted all the time and can’t drive because of vision problems 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 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 Richard—who was found on autopsy at age 76 to have MS but never knew it

3. What is MS? Neurological disease Misguided immune cells Multiple scars Unpredictable Variable Often progressive

4. What is MS? cont’d. 2-3x women as men Usually diagnosed between 20 and 50 200 people diagnosed every week in US More common in Caucasians, especially those of northern European ancestry

5. What Causes MS? Genetic Predisposition Environmental Trigger Autoimmunity Loss of myelin & nerve fiber

6. 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). The risk of getting MS is approximately:

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

8. MS: A Timeline 1396- Earliest recorded case of MS. 1868- 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). 1981- 1 st MRI image of MS is published. 1993- The first disease-modifying agent for MS—Betaseron—is approved in the U.S. 1998- Bruce Trapp confirms that the nerve fibers are irreversibly damaged early in the disease course (probably accounting for the permanent disability that can occur). 2014- There are several medications approved for the treatment of MS and more in the pipeline.

9. How is MS diagnosed? MS is a clinical diagnosis:  Signs and symptoms  Medical history  Laboratory tests  Magnetic resonance imaging (MRI)  Visual evoked potentials (VEP)  Lumbar puncture

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

11. How is MS diagnosed? 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

12. 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 Characteristics that predict a better outcome:  Female, onset before age 35, sensory symptoms  Complete recovery following a relapse

14. An Overview of Treatment Strategies The MS treatment team includes the person with MS and a … Neurologist Urologist Nurse Physical therapist Occupational therapist Physiatrist Psychiatrist Psychotherapist Neuropsychologist Social worker/Care manager Pharmacist Primary care physician

15. What are the treatment strategies? 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 (maintain/improve function)  Psychosocial support

16. FDA-Approved Disease-Modifying Agents Aubagio (teriflunomide) * Avonex (interferon beta-1a) ** Betaseron (interferon beta-1b) ** Copaxone (glatiramer acetate) ** Extavia (interferon beta-1b) ** Gilenya (fingolimod)* Novantrone (mitoxantrone)*** Rebif (interferon beta-1a)** Tecfidera (dimethyl fumarate)* Tysabri (natalizumab)*** * oral ** by injection *** by infusion

17. What do the disease-modifying drugs do? All reduce attack frequency and severity, reduce scarring on MRI, and probably slow disease progression. These medications are not designed to:  cure the disease  make people feel better  alleviate symptoms

18. How important is early treatment? The Society’s National Clinical Advisory Board recommends that treatment be considered as soon as a diagnosis of relapsing MS has been confirmed.  Irreversible damage to axons occurs even in the earliest stages of the illness.  Treatment is most effective during early, inflammatory phase  Treatment is least effective during later, neurodegenerative phase No treatment has been approved for primary-progressive MS. As of 2010, approximately 60% of people with MS are being treated with a disease-modifying therapy.

19. MS Symptoms vs Relapses… How Are They Different? MS symptoms are chronic or ongoing indicators of MS lesion damage to certain areas of the brain and/or spinal cord MS relapses are sudden flare-ups or symptom attacks that typically last several days to several weeks Joy and Johnston, eds. Multiple Sclerosis: Current Status and Strategies for the Future. Washington, DC: National Academies Press; 2001

20. 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  H.P. Acthar® gel  Plasmapheresis Rehabilitation to restore lost function Psychosocial support

21. What are possible symptoms?  Fatigue (most common)  Visual problems  Bladder and/or bowel dysfunction  Sexual dysfunction  Emotional disturbances (depression, mood swings)  Cognitive difficulties (memory, attention, processing) (Heat can worsen many symptoms)

22. What are possible symptoms? cont’d.  Sensory changes (tingling, numbness)  Pain (neurogenic, musculoskeletal)  Spasticity  Gait, balance and coordination problems  Weakness, paralysis  Speech/swallowing problems  Tremor These are symptoms of a number of illnesses, making diagnosis difficult. (Heat can worsen many symptoms)

23. 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, hot baths/showers) 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 If a person with MS has a fever, symptoms can worsen rapidly. Important to find the source of the infection.

24. 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 Many symptoms are invisible and misunderstood.

25. Cycle of MS Symptoms: Related and Interdependent  Sleep  Fatigue Depression  Bladder & Bowel problems  Sexuality issues  Spasticity Constipation  Cognitive function

26. 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/disinterest

27. Managing MS Fatigue, cont’d. Strategies: 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

28. Managing Bladder Dysfunction 80% of people with MS experience bladder problems. Major cause of embarrassment and social isolation. Types 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

29. Managing Bladder Dysfunction, cont’d. Types, cont. Emptying dysfunction  Bladder fails to empty  risk of UTI  Sx include: urgency, frequency, nocturia, incontinence  Tx includes: ISC and anticholinergic/antimuscarinic medications

30. 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

31. Improving Mobility 80-90% of people experience mobility impairment due to weakness, imbalance, sensory problems, or spasticity Management strategies:  Dalfampridine (Ampyra) to improve walking (speed; weakness)  Spasticity management  Exercise/gait training  Mobility aids for weakness, balance, and fatigue issues

32. Improving Mobility, cont’d. Ataxia/Tremor Less common MS symptom, but very 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)

33. 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

34. Managing Sexual Dysfunction 40-80% of people with MS  Reduced libido (behavioral/environmental strategies)  Sensory disturbances (anticonvulsant medications) Women  Reduced lubrication (gels) Men  Erectile dysfunction (pharmacotherapy; implants) Other contributory factors  Managing symptoms that interfere with sexual activity/pleasure (fatigue, spasticity, bladder dysfunction)  Managing medications to promote comfort and responsiveness (anticholinergic; antidepressants; fatigue & spasticity meds)  Feelings and attitudes – education and counseling

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

36. Speech Issues 40-50% experience speech/voice disorders  Dysarthria – impaired volume control, articulation, emphasis  Dysphonia – altered voice, pitch control, breathiness, hoarseness Speech/language assessment:  Oral peripheral examination, voice eval, communication profile Treatment: includes exercises, strategies and compensatory techniques to improve speech clarity, augmentative device or ACC, if needed

37. Swallowing Issues Dysphagia – less common symptom  Swallowing assessment, clinical history, examination, videofluoroscopy (modified barium swallow) Treatment  Exercises  Dietary modifications/positioning while eating/chewing strategies  Non-oral feeding options, if needed

38. 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

39. Cognitive Symptoms Correlates with number of lesions, 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

40. Cognitive Symptoms, cont’d. Most common problems: memory, attention/concentration, information processing Treatments: Disease-modifying therapy, donepezil Cognitive rehabilitation (primarily compensatory) Cognitive symptoms are often misunderstood.

41. Managing Depression >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 Recommended treatment: psychotherapy + medication + exercise

42. Other Affective Disorders  Bipolar disorder 10 times the rate of general population  Mood swings Rapid changes in feelings - anger, irritability, sadness - are very common  Anxiety disorder As common as depression, particularly in the early phases of the disease  Pseudobulbar affect Pathological laughing and weeping  Euphoria In progressive MS, person may be excessively happy given their situation.

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

44. What are the psychosocial challenges?  Uncertain diagnosis  Unpredictable course and outcomes  Invisible symptoms  Potential physical and/or cognitive disability  Diminished self-esteem  Impact on relationships and family systems  Uncertain financial future

45. What are essential psychosocial interventions?  Disease-related education to enhance people’s understanding of the disease, adaptive coping strategies, and available resources  Support for the ongoing grieving process as activities and roles are altered by the disease

46. What are essential psychosocial interventions? cont’d.  Help with important life transitions— diagnosis, disease progression, disability  Assessment and treatment of emotional and/or cognitive problems  Support for family members

47. What role does rehabilitation play? Rehabilitation offers 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

48. What role does rehabilitation play? cont’d. Rehabilitation offers structured, problem-focused, interdisciplinary interventions to:  Identify appropriate assistive devices and environmental modifications  Prevent injuries and unnecessary complications  Empower individual and family

49. Rehabilitation and MS: Medicare coverage for maintenance programs Jimmo Settlement - Maintenance Therapy Skilled therapy services are covered when an assessment of the patient’s condition demonstrates that skilled care is necessary for the performance of a safe and effective maintenance program to maintain the patient’s current condition or prevent or slow further deterioration.

50. 60% of people with MS have activity limitation 20-25% need long-term care services 14,000 in nursing homes 60% of nursing home residents with MS are under 60 years of age Long-term Services and Supports

51. MS-Related Stresses for Patients & Families MS is a chronic disease that many will live with for decades. The unpredictability from day to day and year to year is difficult for patients and families to handle. MS is a disease characterized by change and loss. Treatment costs and loss of income threaten patient and family well-being. With more options available and choices to make, patients and families worry about making “wrong” choices.

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

53. What can people do to feel their best? cont’d. Reach out to their support system, stay connected, avoid isolation. Become an educated consumer. Make thoughtful decisions regarding:  Disclosure  Choice of physician  Employment choices  Financial planning  Health and wellness

54. 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

55. What You Can Do Be knowledgeable about MS and its symptoms Be sensitive to the losses and sadness that people with MS experience Understand the variability of the disease Understand that people with MS want to be as independent as they possibly can Contact the National MS Society (1-800-344-4867) for information, resources and support (www.nationalmssociety.org)www.nationalmssociety.org

56. Society Resources for People with MS Chapters around the country www.nationalMSsociety.org Access to reliable information and referrals (800-344-4867) Educational programs (in-person, online) Support programs (self-help groups, peer and professional counseling) Consultation (legal, employment, insurance, long-term care) Financial assistance

57. Society Resources for Health Professionals MS Clinical Care Network www.nationalmssociety.org/ms-clinical-care-network/index.aspx Email: healthprof_info@nmss.org MS Clinical Care Connection – the Society’s quarterly enews for clinicians – provides information and resources on a range of topics related to comprehensive MS care. Comprehensive MS library/literature search services Clinical consultations with MS specialists Professional publications Professional education programs (medical, rehab, nursing, mental health) Consultation on insurance and long-term care issues

58. National MS Society, Connecticut Chapter www.ctfightsMS.org 659 Tower Avenue, First Floor Hartford, CT 06112-1269 programs@ctfightsMS.org 860.913.2550 800.344.4867