Supporting Students with Essential Tremor

1. Supporting Students with Essential Tremor 31st Annual VASN Fall Conference Breakout Session 7: 1:30 - 2:45 PM November 7, 2015 Sheraton Virginia Beach Oceanfront Hotel, Virginia Beach, VA Presenters: Dr. Claudia Testa, Peter Muller, Deborah Zeller

2. Disclosure Statement The presenters disclose the absence of personal, financial or commercial conflicts of interest relative to the content of this educational activity within the past 12 months. Peter Muller is a associated with the following: HopeNET(No More Essential Tremor) Executive Director, its Board and Medical Advisory Board Claudia Testa, MD, PhD, is associated with the following: Director, Huntington Disease Program, VCU / MCV Parkinson’s and Movement Disorders Center; VP, Tremor Research Group; Medical Advisory Boards of International Essential Tremor Foundation (IETF) and HopeNET; Founder, North American Essential Tremor Consortium; Movement Disorders Society Task Force on Tremor Deborah Zeller is associated with the following: HopeNET Medical Advisory Board; Virginia Council for Private Education; NASN; VASN; ANA; IETF

3. Learner Objectives 1. Identify at least four factors that make essential tremor (ET) relevant to the care of the school-aged population 2. Describe at least two aspects of both the phenomenology and medical management of ET in school-aged children 3. Identify at least five school nursing strategies to support students diagnosed with ET 4. State at least three potential advocacy strategies to enhance student/family support within the community

4. Essential Tremor is Health Condition That Is Relevant to the School-aged Population How are individuals with essential tremor (ET) affected by this movement disorder? • “Essential Tremor is More Than a Tremor” (IETF, 2013) • Stories from six people who live the challenges of ET • Typical motor movements associated with ET • Free DVD copies available at end of session

5. Why ET is a Public Health Challenge • Clinical syndrome that affects about 2.2% of the U.S. population (Louis & Ottman, 2014) • Limited awareness of ET as a national public health issue • Care needs r/t ET are not being addressed in at least one third of ET patients that were recently surveyed (Louis, Rohl & Rice, 2015) • Limited funding for ET research, education & healthcare • Change in ET care depends upon strong vision, leadership & advocacy

6. Public Health Opportunities To Address the Challenges of ET • Bridge knowledge gaps about ET across the lifespan • Increase public awareness among partners, stakeholders & supporters • Enhance relationships toward a common purpose, process & expected outcomes for ET answers • Educate those with ET about health-related quality of life options • Advocate for changes in policies & practices for the greater good • Leverage electronic health information systems to influence policy, funding & strategies that promote health for those with ET

7. SNs Have a Role in Supporting Students w/ ET You are trusted professionals serving on the front lines of healthcare delivery • Are typically the only healthcare professional in the school • Have care coordination, communication & collaboration competencies • Practice in the physical, cognitive, affective & behavioral domains • Serve as health educators and advocates • Have strong healthcare provider & community connections

8. Impact of ET in the School-aged Population • Mean onset age for childhood ET is 6 to 16 (Ferrara & Jankovic, 2009) & it peaks in puberty with a variable progression • Between 5 to 15% of ET cases occur during childhood (Raya, 2010) & one study found that 5.3 % of ET cases were diagnosed prior to age 20 (Louis & Ottman, 2014) • In a cohort of 317 students, 2.2% were found to have mild-to-moderate hand tremor (Louis, Garcia & Rauh, 2015) • Another study found that 50% of adults diagnosed with ET reported childhood onset of tremor (Keller & Dure, 2009) • Tremor severity may range from mild to disabling

9. ET Prevalence in School-aged Students Is Estimated at Between 1.0 to 0.5% (Kids Count, 2015;ChildStats.gov, 2014)

10. What is ET’s Impact on Students? • Multiple challenges may be experienced in school • Tremor may worsen with stress, caffeine, fatigue & exercise • Social handicaps may occur in up to 25% of young people with ET by age 20 (Bain, 2015) • Functional disability, as well as impaired QOL, can result (Chandran,2012; Elble, 2013) with up to 55% of young people diagnosed with ET reporting some functional limitation (Bain, 2015) • Chronic & often progressive condition that has a lifelong impact on at least 7 million Americans (Louis & Ottman, 2014)

11. Factors Influencing Student Health & Learning (NASN, 2015; ADAA, 2015; AACAP, 2013)

12. Medical Overview of Childhood ET Define tremor, investigate causes of ET & review treatment options, including: • Range of symptoms and differential diagnosis • Treatment approaches in the now and future paths • What drives choosing the treatment approach • Medication and non-medication approaches to tremor • Non-tremor issues and treatment approaches

13. Challenges in Tremor Treatment:Why Care About Defining Tremor? Diagnosis and treatment Effective treatments differ – finding the right one(s) for each person is key Making progress! Research and developing new treatments hinge upon how tremors are defined

14. Pathophysiology & Genetics of ET

15. Genetics & Recent Advances Collaborative efforts using new ET definitions and new technologies will change the field • Exome sequencing, whole genome sequencing • New genetic network analysis • Combining genetic data with other complex data sets

16. How Essential Tremor is Defined ET is an involuntary oscillatory movement of some body areas: • Kinetic tremor = action tremor, often also postural • Involuntary, not under direct conscious control • Visible bilateral, rhythmic & persistent tremor • Back and forth, not stuck in one posture • External tremor is not suppressible In lay terms: Nervous, shaky

17. Classic ET Manifestations & Clinical Diagnosis Primary Inclusion Features: • Kinetic tremor affecting both hands &/or arms, and/or other body regionsduring effort, not at rest or during sleep (Rana, 2010) • Some variants of ET & absence of other neurological signs Secondary Inclusion Features: • Long duration • Family history of essential tremor • Ethanol intake temporarily decreases tremor amplitude in adults & is a common response, but is not diagnostic

18. Manifestations & Clinical Diagnosis Are there non-tremor symptoms in ET? • Cerebellar signs (mild) - balance changes • Behavioral/psychiatric – anxiety, depression, harm avoidance • Dystonia in areas unaffected by tremor? • Cognitive changes? – Controversial & relevant to older adult Are there Parkinsonian features? • Mild changes in tone with cogwheel rigidity • Mild arm swing decrease – a form of bradykinesia

19. Other Conditions That Maybe Mistaken for ET Tremor May Be Induced by Other Factors: • Drugs: valproate, SSRIs, TCAs, dopamine blockers, immunosuppressants, amiodarone, lithium, nifedipine, asthma medications, stimulants (caffeine) … • Endocrine: thyroid, pregnancy… • Cerebral palsy: means FIXED deficits over time

20. Distinguishing ET from Other Conditions Enhanced physiological tremor (EPT) • Fast, low amplitude “normal” tremor • NOT possible to definitely distinguish from ET • No clear change over time Primary neurological and psychiatric tremors • Psychogenic, psychosomatiform tremor • Dystonia, focal tremors - primary writing tremor • Wilson’s disease • Stroke

21. Distinguishing ET from Psychogenic Tremor Anxiety, stress & fatigue worsen all forms of tremor • Failure to address severe anxiety or stress may impair ability to respond to tremor suppressant treatments Psychogenic/psychosomatiform movements • Can occur in isolation or in addition to ET • Are not “conscious” or “weak” • Tremor “attacks”, non-ET tremor forms • Psychogenic gait, voice breaks, non-ET voice changes • Psychiatric disease or abuse/trauma increase risk, but are NOT necessary for this diagnosis

22. Distinguishing ET from Another Clinical Diagnosis, Dystonia Co-contraction of agonist and antagonist muscles creates involuntary postures “Dystonic tremor”is a disputed label • May be very task specific • Irregular • Components of pulling Dystonia in children may have severe prognosis • Try essential tremor treatments or dystonia treatments?

23. Changing Our Understanding of ET • Improving understanding of essential tremor causes = the best route to effectively design and use therapies • However… no need to wait! We can act on symptoms, and push research NOW • Important to think about goals of treatment: • Improve daily function? • Improve specific tasks? • Alleviate anxiety? • Wipe out tremor?

24. Pharmacologic & Therapeutic Options for ET Therapies for Children and Adults • Medication • Non-medication – cognitive/behavioral, PT, OT, SP, other holistic therapies • Cool stuff Therapies Aimed Toward Adults If Meds Ineffective • Surgery – deep brain stimulation, thalamotomy • Procedures - botulinum toxin (neck, voice > limbs)

25. Primary Pharmacologic Options Propranolol (Inderal) - Only FDA approved agent for ET • Effective beta adrenoreceptor antagonist; 1971 introduced in ET • Start with a low dose, 12.5mg to 25mg & titrate up • Propranolol LA may be better • Note that data on other beta-blockers is limited • Atenolol (Tenormin), sotolol (Betapace), nadolol (Cogard) have some evidence • Pindolol (Visken)may cause tremor Primidone (Mysoline) • GABA agnonist & antiepilepticmedication • Start LOW, 12.5mg or 25mg in the evening to avoid acute ataxia, vertigo, nausea

26. Potential Concerns w/Primary Rx Options Medication Adverse Effects Contraindications Beta Blockers Bradycardia Bradycardia Hypotension Heart block Depression Bronchial asthma General slowing CHF Insomnia DM Weight gain Nausea & vomiting Hallucinations

27. Potential Concerns w/Primary Rx Options Medication Adverse Effects Contraindications Primidone (Mysoline) Ataxia Porphyria Vertigo Phenobarb. sensitivity Nausea & vomiting Hyper-irritibility Anemia Depression Sedation

28. Secondary Pharmacologic Options for Tremor Topiramate (Topomax) • Blinded study = 24 patients cross over design • Worth the cognitive side effects? Start w/low dose Gabapentin (Neurontin) Inconclusive evidence • Only blinded study = 16 patient 3-way design Nimodipine (Nimotop) – limited evidence Benzodiazepines – Not recommended generally in kids • Alprazolam (Xanax), clonazepam (Klonoprin) & others are LA but have dependency a concern • Limited or negative data, but commonly used • May help w/ anxiety at expense of side effects • Note impact on gait!

29. Issues To Consider in Treatment Options Data quality • As of late 2005, only 2 of 159 published clinical trials used more than 50 patients Note level of efficacy • Medications can reduce tremor by 60% or more in roughly 50% of patients • Surgery excellent: balance risk/benefit Tolerability • Survey of 223 patients • 71% had tried propranolol/primidone • 56% had discontinued one or both meds

30. Tremor Support Options: Cool Stuff Assistive & Adaptive Technology • Spoon/fork/knife programmed to oscillate counter to tremor • SteadyMouse tremor dampening software & ARC anti-tremor pen • Lift Pulse App Therapeutic Devices • Wrist weights & other devices • Neuroprostheses

31. Other Treatment Options to Consider Assess for other issues that impact balance • Impaired vision • Peripheral neuropathy Potential future treatments • Need research! • DBS may improve gait – or worsen balance?? • Typical ET medications seem to have no impact on balance

32. Beyond the Mood: Tremor & Anxiety in ET Anxiety vs. tremor in ET is a growing area of research • On range of problem mainly, some on interventions • Anxiety is commonly reported in ET • Reactive? A primary problem? Factors that may be associated with worse anxiety • Not tremor amplitude! • Cognitive impairment ? Depression? So what? • Anxiety may cause social avoidance, restriction of activities, inability to experience benefit from tremor medications • Treating anxiety may lessen tremor & this is not cheating!

33. Beyond the Mood: Tremor & Anxiety in ET Potential treatments • Need research! • Propranolol may lessen situational anxiety New treatments in the now • Evaluation by neuropsychologist, psychologist, or psychiatrist • Effective treatments differ – find the right one(s) for the student • Many medication and non-medication options, but use caution with benzodiazepines as they are not the answer • Exercise! • Assess for other issues that can impact mood, for example, life stressors, panic disorder

34. Beyond the Mood: Depression in ET Growing area of research • On range of problem, on etiology, some on interventions Depression vs. tremor in ET • Depression is commonly reported in ET - Reactive? A primary problem? • Potential role for the cerebellum in both Factors that may be associated with worse depression • Not tremor amplitude! • Cognitive impairment? Younger age? So what? • Depression affects perceived health status in ET – at any level of tremor severity • Depression worsens cognition & can manifest as real symptoms in the body

35. Beyond the Mood: Depression in ET Potential treatments • Needs research! • Propranolol and benzodiazepines may worsen depression New treatments in the now • Evaluation by neuropsychologist, psychologist, or psychiatrist • Effective treatments differ – find the right one(s) for the student • Many medication and non-medication options! • Exercise! • Assess for other issues that can impact mood, such as untreated hypothyroidism, bipolar disorder

36. Tremor: New Understanding • Tremors come in many forms • Essential tremor is the most common movement disorder • Disability may be more about non-tremor symptoms than the tremor itself • There are many options for symptom control of the tremor and associated symptoms

37. Taking Action! • Investigate the student’s tremor and non-tremor symptoms • Think about clinical picture, symptoms, change over time • Observe effects of treatments • Ask if the tremor label makes sense over time? • Are non-tremor symptoms ET or something else? • Explore pros and cons of potential treatments • What symptom(s) will the treatment address? • Does the risk / benefit equation make sense for the student? • Second opinions are not insults • Interdisciplinary, holistic approach • Consider larger overall goals of treatment

38. Healthy Learner Model for Student Chronic Condition Management(Erickson, Splett, Mullett & Heiman, 2006)

39. ET & Disability: Behavioral Conditioning Model (Lundervold & Poppen, 2004) Emotional Distress Behavior • Social anxiety • Fear, worry • Social isolation • Depression Awareness of the Motor Dysfunction Dysfunctional Rule Governed-Behavior • I am different • Everyone stares at me • I can’t control my tremor • My life is worthless Tremor

40. SN Strategies To Support Students with ET • Assess student health status to identify needs • Refer students appropriately • Establish plans to help students manage diagnosed ET • Teach and counsel about health and ET care (Louis, Rohl & Rice, 2015) • Coordinate care delivery • Evaluate student response to health interventions • Identify learningproblems that impact full participation

41. Universal Strategies to Increase Protective Factors (CDC, 2009) • Advance universal protective factors that lead to positive student health and educational outcomes • Serve as guide for academic team to take supportive actions • Involve decision-making processes that facilitate student, family, and community engagement in school life • Give students academic, emotional & social skills in school • Use classroom management & teaching methods to foster a positive learning environment • Promote a climate of trust and support

42. Consult on Potential Academic Interventions • Individualized Education Plan (13 defined disabilities under IDEA, Galemore & Sheetz, 2015) • Section 504 Plan (Those with disability are protected from discrimination) • Related school based health services: • Therapies: OT, PT, SP • Adaptive PE • Vocational & educational counseling (Gillespie, 1991)

43. Sample ET Learning Accommodations (Floyd, 2015;Peters, 2015; Prokesch, 2010) • Avoid grading for neatness in handwriting, keyboarding • Use weighted pencils, Dr. Grip™ pens, wrist weights, sturdy straws • Access to school provided iPad/computer & speech recognition software, such as Dragon • Provide class outlines to limit need for note taking • Use non-standardized answer sheets, “x” for correct response • Assign science lab partner to handle beakers, sharp tools • Utilize a tape recorder, other assistive technology • Schedule study hall at end of school day when fatigue sets in

44. Initial Encounter with Student c/o “Shaking” S:Student states that she feels upset, “hurt” about the teacher’s remarks that her work is too messy and she takes too much time to write out answers. If student goes back to class everyone will stare at her. The shaking problem has been present for a long time. The child says she can’t help it and she is doing her best. O: Child has trembling hands with action, like the example seen on the DVD. She is crying, tearful. Temp is normal, BP/P/RR are slightly elevated. There is no prior history of problems r/t the child’s health, growth or development. How might you open the conversation with this student to gather more information in the assessment process?

45. Possible Approaches in Initial Encounter • What brought you to the health office today? • Has anything worked before? • Do you want to try that now? • Are there others on the team that can help us problem solve? • Talk about a non-school issue to relieve the anxiety of the moment • Provide safe place for student to discuss tremor and relieve tension, anxiety

46. Nursing Intake Questions for a Child w/ Tremor (Sprague-McRae et al, 2014; Edelman et al, 2014) • History: General health? Conditions? Accidents? Absences from school/class? Important things you do to keep healthy? Current health concerns? What do you think has caused this? What actions have you taken since it started? Has it helped? What things are most important to your health? History of allergies, medications, OTCs, substances, CAMs, treatments? Responses? Healthcare providers? Family history of similar/other neurological conditions? Traditional, cultural practices, taboos? • Nutrition & Metabolism: Does child eat at school? Where? Daily food & fluid intake? Appetite? Patterns? Food access? Weight changes? • Elimination: Bowel & urinary elimination patterns? Odors?

47. 4. Activity & Exercise: Energy for required activities? Exercise patterns, types, leisure activities? Perceived ability to perform ADLs? Tremor movement type, location, onset, patterns, associated symptoms? Worsening & improving factors? Movement awareness? Things that reduce or eliminate tremor? 5.Sleep & Rest: Sleep routine? Current patterns? Sleep problems including limb movements? Rest & relaxation periods? Rested for activity? 6. Cognitive-perceptual: Hearing? Vision? Glasses? Discomfort or pain? Memory? Easiest way to learn? Ease in making important decisions? Present level of understanding health condition? Ability to use information? Change readiness? Academicperformance level? Recent changes? Prior academic testing? Identified learning disabilities? IEP or 504 Plan?

48. 7. Self-perception/self-concept: Describe self? How does student feel about him/her self? Changes in body appearance? Changes in things student can do? What things that make child feel fearful? Angry? Annoyed? Worried? Sad? Not able to control things? Concern about things (tremor) interfering with activities? What works? Vocal tremor? Speech pattern? Posture? Nervous? 8. Role relationships: Family structure? Relationships? Difficulty handling problems? Changes? Friends? Social groups? Do things generally go well at school/home? Feel part of community? Problems with other children? How is child managing? Family financial concerns? Behavior changes? 9. Sexuality reproduction: as appropriate to the student’s age & situation

49. 10. Coping-stress tolerance: Big life changes in last year or two? Who is most helpful in talking things over? Tense or relaxed most of the time? When tense, what helps? What do you do/or use to relax? If so what? If there are big problems in your life? How do you handle them? Avoidance or withdrawal? Most of the time, are these ways successful? 11. Value-belief pattern: Do you generally get things you want from life? Important plans for the future? Are religious beliefs and practices important to you? Does might this factor into choices or decisions when health challenges arise? Physical assessment: Ability to write, feed, dress, do hygiene & grooming

50. Student Health Referral Process • Consult your organization’s health services referral guidelines • Initiate referral to PCP for (a) management of student’s acute and (b) chronic medical conditions and (c) need determination for movement or other specialist services • Educate parent/student on the movement specialist’s role • Instruct parent in insurance preauthorization & referral procedures • Refer the parent of the student back to other specialists for worsening medical problems and disabilities • Track referral results in student health record