2. RECOGNIZE AND RESPOND TO THE WARNING SIGNS OF PAEDIATRIC ARRHYTHMIAS PREPARED BY PACED PARENTS ADVOCATING FOR CARDIAC EDUCATION * PLEASE CLICK ON THE AUDIO ICON FOR EACH NEW SLIDE Sudden Cardiac Arrest Prevention * Audio Seminar for Teachers and Coaches

3. PACED is Parents Advocating for Cardiac Education Copyright 2015 by PACED www.paced.ca Mission To create universal top of mind awareness and appropriate systemic response to the Warning Signs of Inherited Heart Rhythm Disorders in Canadian communities Vision PACED envisions a day when Inherited Heart Rhythm Disorders (IHRD’s) are no longer a leading medical cause of paediatric mortality in Canada, the number of young people dying each year is significantly reduced and the majority of at-risk individuals are identified and receiving effective therapy or better still a cure for their disease Values To advocate, with passion and integrity on behalf of all Canadians affected by IHRD’s. To respect the voice of all stakeholders. To generate and disperse revenues ethically and wisely in pursuit of our stated goals 2

4. Dr. Andrew Krahn Copyright 2015 by PACED www.paced.ca “Our research gives us an idea of the scope of the problem – there are almost 200 young people who die suddenly every year in Ontario. A good proportion of them have unrecognized heart disease. So the question is: How can we catch this before it happens?” says Krahn. He suggests that more attention be paid to possible warning signs such as fainting. He believes that teachers, coaches and an aware public may be key to detecting risk, ensuring prevention and formal medical evaluation and therapy. “I would advocate for careful screening of people who faint, using questionnaires and education of healthcare professionals so that when warning signs present themselves, they recognize them and this information gets passed on to the right people,” he says. From an article on Dr. Krahn’s presentation at the 2012 Canadian Cardiovascular Congress http://news.bioscholar.com/2012/10/hidden-disease-sports-sudden-cardiac-arrest.html 3

5. What are IHRD’s ? A group of genetic diseases that predispose people (children) to sudden cardiac arrest Transmitted by an autosomal dominant gene: if one parent has the gene on average half of the children will acquire the gene The disease hides because there are usually no other manifestations, in every other way the child is healthy Many deaths occur between the onset of puberty and the late twenties Copyright 2015 by PACED www.paced.ca 4

6. Two Types of IHRD’s Cardiomyopathies Effect the development of heart muscle making it weak or thickened and less able to conduct electricity HCM  Hypertrophic Cardiomyopathy - a thickening of the muscle on the left side of the heart ARVC/D  Arrhythmogenic Right Ventricular Cardiomyopathy – a weakening of the muscle usually on the right side of the heart Channelopathies Effect the pathways that conduct the heart’s electrical signals Long QT Syndrome (1-14) Brugada Syndrome CPVT Short QT WPW - Wolff Parkinson White - an accessory electrical pathway not a true channelopathy. Copyright 2015 by PACED www.paced.ca 5

7. IHRD’s by the Numbers 700 young Canadians, under age 35, die each year  200 in Ontario  65 in BC 1 in 500 would be conservative estimate of the disease prevalence – Think 2 per High School !!!  About 1.5% of gene positive people die each year Nearly 50% of all patients have at least one fainting episode prior to their death  5% (1 in 20) of all fainting episodes (syncope) are a warning sign  33% (1 in 3) or more of fainting during exercise is sinister  The good news is that 95% of all faints are benign Copyright 2015 by PACED www.paced.ca 6

8. The Warning Signs Fainting (syncope) or seizure during or shortly after physical activity, especially if it happens repeatedly Fainting (syncope) or seizure resulting from emotional excitement, emotional distress, or auditory startle Family history of unexpected sudden death during physical activity or during a seizure, or any other unexplained sudden death of an otherwise healthy young person Copyright 2015 by PACED www.paced.ca 7

9. Additional Warning Signs Near fainting (syncope) or Brownouts  Dizziness, lightheadedness (New) Extreme shortness of breath  More so or different than other children Palpitations or Racing Heart Fatigue – more so than what a typical teen complains of Copyright 2015 by PACED www.paced.ca 8

10. Responding to the Warning Signs Copyright 2015 by PACED www.paced.ca 9-1-1 for all faints but especially those occurring during or shortly after physical activity  An ECG acquired on scene by Paramedics may hold many diagnostic answers Immediate visit to the Family Physician or walk-in clinic for all other fainting episodes or secondary warning signs. Provide parents/guardians with information about IHRD’s, sudden cardiac arrest and the warning signs. No return to play until medical clearance has been obtained 9

11. Review #1 Copyright 2015 by PACED www.paced.ca 1. Every year in Canada ______ young people, under age 35, die as a result of sudden cardiac arrest. A. 200 B. 65 C. 700 D. 11 2. What percentage of young cardiac arrest victims had at least one fainting episode in the weeks, months or years prior to their death? A. 24 % B. 48 % C. 96 % 3. Faints associated with _______ are the most concerning as a risk factor for sudden cardiac arrest. A. Flu symptoms B. Auditory startle (unexpected loud noise) C. Physical Activity D. Lack of sleep E. B and C 4. When a (young) person faints during or shortly after physical activity ______ A. 911 should be called B. They should be given a 30 minute rest period before return to activity C. Parents should be provided with information about fainting and heart arrhythmia D. You should wait 15 minutes to see if they “seem fine” before calling 911 E. A and C 10

12. Diagnosing an IHRD Post syncope physicians will perform any or all of: An Event History  Gather eyewitness accounts A Patient History A Family History  Parents have homework An ECG  Stress ECG  Holter Monitor Echocardiogram (ultrasound) Cardiac MRI Tilt Table Testing Genetic Testing Copyright 2015 by PACED www.paced.ca 11

13. Treating IHRD’s There are a number of effective treatment options for patients diagnosed with IHRD’s:  Pharmacological Therapy – β Blockers  Surgical Options – Catheter Ablation  Implantable Devices – Pacemakers and ICD’s  Lifestyle Modification – No intense physical activity Once identified patients living with an IHRD should enjoy a long, healthy and productive life Copyright 2015 by PACED www.paced.ca 12

14. Testing First Degree Relatives Dr. Joel Kirsh (Sick Kids, Toronto) estimates his clinic identifies between 4 and 5 additional gene positive First Degree Relatives for every index patient Finding one patient must begin a search for all affected family members The policies being recommended have a significant multiplier effect Copyright 2015 by PACED www.paced.ca 13

15. Pre-Participation Screening Questionnaire Copyright 2015 by PACED www.paced.ca Patient History Questions 1. Has this child ever fainted during or shortly after physical activity? 2. Has this child ever experienced extreme shortness of breath, fatigue or “brown outs” during physical activity? 3. Has this child ever fainted as a result of emotional distress or excitement? 4. Has this child ever fainted from auditory startle: an alarm clock, a door slamming or unexpected noise? 5. Has this child ever fainted from any cause? 6. Has this child ever sustained an injury as a result of fainting? 7. Have any of this child’s faints involved seizure like activity? 8. Has this child ever been diagnosed with a seizure disorder such as epilepsy? Family History Questions 1. Is there history of unexplained early death on either side (maternal/paternal) of this child’s family? a. More than one early death in the family? b. Unexplained death of family members under age 50? c. Unexplained death of family members under age 35? d. Any deaths occurring during or after intense physical activity? Running, swimming, cycling, soccer, hockey. e. Deaths of undetermined origin or “presumed” cardiac origin f. Are there any SIDS deaths (Sudden Infant Death Syndrome) in the family? g. Are there any deaths attributed to seizure disorder or epilepsy? 2. Is there any member of this child’s family that has a history of unexplained fainting or seizures? 14

16. A Five Point Cardiac Arrest Prevention Strategy 1. 30 Minute Arrhythmia Awareness Training  mandatory for all Teachers, Coaches and CPR/AED trained staff 2. Pre-Participation Screening Questionnaire  to be completed by parents/guardians at the time of enrollment in a new school or a new sport 3. Mandatory 9-1-1 Calling for all Faints  at least for all faints occurring during or shortly after physical activity 4. Mandatory Notification of Parents/Guardians of all Faints  including providing them with information about IHRD’s 5. Mandatory Medical Clearance for Return to Play  the most tragic deaths are those where fainting is ignored and the child is allowed to continue to participate in physical activity Copyright 2015 by PACED www.paced.ca 15

17. Review #2 Copyright 2015 by PACED www.paced.ca 1. Event History is an important part of diagnosis. Eyewitnesses should be asked about: A. The presence of seizure like activity B. The minutes leading up to the event C. Whether the patient was able to use their hands and arms to break the fall D. All of the above 2. Family History often holds the key to diagnosis. Parents should be asked about A. The unexpected or sudden death of family members under age 35 B. Only those deaths that have occurred in the last 20 years C. Any crib deaths or SIDS (Sudden Infant Death Syndrome) deaths in the family D. Only deaths in the patients maternal family E. A and C 3. Most children have a definitive diagnosis after two simple, non-invasive tests A. Genetic Blood Test and Cardiac MRI B. Tilt Table Testing and Stress ECG C. ECG and Echocardiogram D. Holter Monitor and Cardiac MRI 4. Controlling heart rate and rhythm to prevent cardiac arrest often requires A. Taking one Beta Blocker each day B. Wearing a Pacemaker or ICD C. Open Heart Surgery D. Lifestyle modification E. Any combination of A, B and D 16

18. State or Organization Awareness Education Pre Par QRemove from Play / 911 Return to PlayInform Parents Pennsylvania HB1610 - 2012 Mandatory for Coaches. Annual Yes. Signed by parents annually Yes. Mandatory after any syncope Yes. Cleared by licenced HCP Yes. Website and handouts New Jersey S2367 (39-0) Mandatory for Coaches. Annual Confirm receipt of pamphlet Yes. Mandatory for any syncope Yes. Cleared by licenced HCP Mandatory prior to participation Maryland HB0427 (136-0) Yes. Mandatory for all Coaches Confirm receipt of information Yes. Mandatory after any syncope Yes. Cleared by licenced HCP Mandatory prior to participation Indiana HB1178 ( Yes. Mandatory for all Coaches Confirm receipt of information Yes. Mandatory after any syncope Yes. Cleared by licenced HCP Yes. Parents receive info sheet California Inter - scholastic Fed. Yes. Mandatory for all Coaches Yes. Signed by parents annually Yes. Mandatory after any syncope Yes. Cleared by licenced HCP Yes. Parents receive info sheet Ontario Private Members Bill Yes. Mandatory for all Coaches and Teachers annually Strongly recommended Yes, Mandatory after syncope Yes, Cleared by licenced HCP Yes. Prior to participation and post syncope Laws and Regulations for SCA Prevention http://www.simonsfund.org/sudden-cardiac-arrest-legislation-by-state/ Copyright 2015 by PACED www.paced.ca 17

19. http://www.millerthomson.com/en/publications/newsletters/education-law-newsletter/september-2014/keeping-abreast-of- the-standard-of-care?utm_source=Mondaq&utm_medium=syndication&utm_campaign=View-Original September 2014 Keeping Abreast of the Standard of Care: Automated External Defibrillators in Schools Gillian Tuck KutarnaGillian Tuck Kutarna, Guelph In April of 2009, grade 5 student Bezawit Chanyalew suffered a cardiac arrest during gym class at her Vancouver school. Before emergency workers could arrive, she suffered brain damage due to a lack of oxygen, leaving her with severe cognitive and physical disabilities. The School Board recently settled her claim out of court for $1.7 million. The allegations of negligence levelled against the Board raised some interesting questions about the standard of care to which school boards may be held. Two days prior to the incident, the school Bezawit attended received a fax stating that she had a congenital heart condition known as “Long QT Syndrome”, putting her at increased risk of seizures and cardiac arrest, especially if she engaged in physical exertion. Bezawit was running in a 100 metre relay when she collapsed. The Statement of Claim filed against the Board alleged that the Board’s school was negligent in two ways: first, that after receiving the fax, the school should have immediately developed a protocol to minimize Bezawit’s risk of injury; and second, that the school should have obtained all the information necessary to ensure Bezawit was safely able to participate in physical education class. Copyright 2015 by PACED www.paced.ca 18

20. Submit your Questions to Blake at info@paced.cainfo@paced.ca Answers Review #1 1.C, 2.B, 3.E, 4.E Review #2 1.D, 2.E, 3.A, 4.E Copyright 2015 by PACED www.paced.ca 19

21. Thank you for Learning the Warning Signs Parents Advocating for Cardiac Education - PACED Blake Hurst, Co-Founder 29 Mericourt Rd. Hamilton ON L8S 2N5 905 527-0462 info@paced.ca www.paced.ca @rhythmichearts Copyright 2015 by PACED www.paced.ca 20