Dying with your Privacy Intact

Questions about PHIPPA and Deadly Genetic Diseases

The rigorous application of the Personal Health Information Privacy Protections Act (PHIPPA) and the presence of known disease causing genes or genetic mutations raise a host of medical/legal/ethical challenges for doctors, patients and families. In my role as a patient advocate working on behalf of families affected by inherited heart rhythm disorders; a group of diseases typically transmitted by a dominant gene and therefore affecting on average half of all children of a gene positive parent; I meet several families and physicians each year that are facing serious challenges in balancing PHIPPA and the best interests of their loved ones and patients respectively. Every year 700 young Canadians die suddenly from cardiac arrest caused by an underlying heart arrhythmia disease, for most death is the first indicator of the presence of disease. The challenge is to identify at risk individuals and get them the preventative treatment that they require before they go into cardiac arrest

This post does not have any answers it simply sets out to ask some of the questions.

Story One - A 22 year old male tests positive for Long QT Syndrome after a fainting episode at hockey practice. Follow-up testing finds his Mother is also gene positive for Long QT. His 26 year old cousin on his Mother’s side is pregnant with her first child and refuses to get tested. It is important to understand that if the female cousin and the baby both test positive there are many simple and effective options for preventing sudden death.

·        Can or should the healthcare system be able to force the mother to undergo testing to protect the unborn child?

·        Should there be exceptions to PHIPPA when minor children are known to be at risk?

The current system typically involves a letter written by a physician being sent to first degree relatives of the index patient asking them to consider being tested. If they do not respond to the request little else can be done.

·        Should Doctors and/or Genetic Counsellors be allowed to call or visit first degree relatives and explain their options and the pros and cons of undergoing testing?

Story Two - A 14 year old boy dies in his sleep. The initial autopsy fails to determine cause of death. Subsequent Molecular (genetic) autopsy determines that the cause of death was likely Brugada Syndrome. The parents are informed of the findings and it is strongly recommended that all first degree relatives undergo testing. No one in the family moves quickly to get tested. A year and a half later a 17 year old brother goes into cardiac arrest and Paramedics are unable to save him.

·        Should coroners in every province/state be required by law to complete genetic testing and inform known first degree relatives within a specific time frame, say 90-120 days ?

·        Should genetic swab kits be sent to all first degree relatives?

·        Should there be more rigorous guidelines for coroners and medical examiners following up on Sudden Unexpected Death in the Young including timelines and protocols for informing relatives?

·        Is there a way to protect healthcare professionals from disciplinary action when they pursue relatives of known gene positive patients?

Due to the constraints that PHIPPA puts on Doctor’s they often find themselves on shifting unstable ethical legal ground. When the disease they are contemplating is carried on a dominant gene there is a 50/50 chance that the knowledge they possess imparts potentially lifesaving information.

The 17 year old brother of the boy whose autopsy was positive for Brugada Syndrome died with his privacy intact.

·        Is that the intent of PHIPPA ?

Two Cost Comparison’s for Cardiac Arrest Prevention Programs

 

Brock Reuther died as a result of a sudden cardiac arrest experienced while playing volleyball at his Alberta High School in May of 2012. Since that day Brock’s mom Kim has been advocating for programs to prevent other families from experiencing the pain the Reuther’s have gone through and it has been, to say the least, an uphill battle.     http://projectbrock.com/

I was speaking with Kim this week and shared with her two comparators that I often use when speaking about preventing sudden cardiac arrest in youth. She asked if I could send them to her in writing so here they are.

The HPV (Gardasil™) Vaccine vs. ECG Screening for Heart Arrhythmia

In August of 2007 the Government of Ontario announced it would spend $39 million to provide every Grade 8 girl in the province with the vaccine for Human Papillomavirus (HPV) which is known to be a cause of many cervical cancers. That works out to a little over $400.00 per child. Cervical cancer claims the lives of 150 women of all ages in Ontario each year and is the second most common type of cancer in women between ages 22 and 44.  This is an expensive but worthwhile prevention program that will assuredly save young lives.

For many years patient advocate groups in Canada have been seeking funding and support for a pilot study into ECG screening of young teens. Many advocates would like to see the screening process include Echocardiogram as well. Published studies show that ECG alone can be over 80% effective at identifying children at-risk for sudden cardiac arrest and when Echo is added screening will identify over 90% of those at-risk.  The tests are non or minimally invasive and very inexpensive. The Ontario Health Insurance Plan billing code for an ECG to be acquired and read by a physician is $11.05. A targeted Echo is a little more expensive at $33.50.  For 10% of the cost of an HPV vaccine we could protect all young people from a far more prevalent and preventable cause of death – Sudden Cardiac Arrest.

A $250,000 grant would easily fund a pilot study that could gather, study, analyze, weigh the values and publish the results of screening tests for over 5,000 youngsters. Every year 700 young people die suddenly of cardiac causes in Canada - 200 in Ontario, 65 in British Columbia, 60 in Alberta, etcetera.   An ECG/ECHO screening program for teenagers is relatively inexpensive and assuredly would save hundreds of young Canadians from sudden death.

Note: ECG screening of elite athletes in northern Italy has reduced sudden cardiac arrest deaths in that population by 89%.

Anaphylaxis Awareness vs. Sudden Cardiac Arrest Awareness

In the spring of 2012 I gave a talk on Recognizing and Managing Cardiovascular Emergencies to 250 Human Resources professionals from across Ontario. A portion of the talk included a discussion of cardiac arrest in youth.

·        I asked for a show of hands to the question “How many of you know that you cannot send a child to school with peanut products in their lunch?”

o   Virtually every hand in the room went up.

·        I then asked “How many of you know that “fainting” is a common warning sign of a potentially lethal heart rhythm disorder?”

o   Perhaps 10 hands went up.

·        I then asked “How many kids in Ontario will die this year from peanut allergies?”

o   The answer is probably none, perhaps one and if it is a very bad year two.  Note : 2 deaths in ON since 2010

·        Finally I asked “How many young people will die in Ontario this year as a result of Sudden Cardiac Arrest?”

o   The answer is 200 – a palpable buzz went through the room.

It is fantastic that the Provincial Government and the School Boards that it funds have invested so much money, time and effort into creating universal awareness and compliance for strategies for preventing two or three peanut allergy deaths each decade. It is frustrating and nonsensical that the same Government and the same School Boards have invested virtually nothing into awareness and prevention strategies for the most common cause of death on school properties and the most common medical cause of death amongst Ontario children.

Every patient advocate probably believes that research and programming for their disease is underfunded. Those of us working to protect the lives of children at-risk for Sudden Cardiac Arrest can look around and see tens of millions of dollars being spent on prevention strategies for diseases that cause less than 1% of the suffering brought on by Heart Arrhythmia Diseases. We would never want or ask Governments to cut back that spending, we are simply asking them to listen to our story and act on our requests for similar consideration. Again this year 700 young Canadians will die suddenly of cardiac causes, many of those deaths should be prevented.

Sudden Cardiac Arrest Prevention Strategies for your League

Share in the Knowledge - not in the Tragedy

PACED, Parents Advocating for Cardiac Education, is hopeful that 2015 will be a watershed year for heart arrhythmia awareness and sudden cardiac arrest prevention in Canada. A little basic knowledge of heart arrhythmia diseases and their warning signs can go a long way to reducing the toll that sudden cardiac arrest takes on our children and families. Many of the young athletes that die each year exhibit warning signs in the days weeks or months prior to their death. PACED would like the opportunity to speak to the executive, coaches, trainers and parents of your minor sport organization and share simple strategies that can protect young athletes.

If your organization is based in southern Ontario, from London to Barrie to Oshawa to Niagara Falls, PACED will be happy to come out to an upcoming meeting and deliver a brief but comprehensive overview of Inherited Heart Rhythm Disorders that addresses the critical questions:

·        What is an Inherited Heart Rhythm Disorder (IHRD)?

o   Are there other diseases that can cause a child to experience sudden cardiac arrest?

·        What are the warning signs of an IHRD?

·      How should our organization and parents respond when warning signs are observed?

·        Is there value in a “return to play” policy for athletes that have exhibited warning signs?

·        Are there pre-participation screening tools for IHRD?

·        What is the role of genetics in the recognition and management of an IHRD?

·        There is an AED (Defibrillator) at the arena; won’t that prevent sudden cardiac arrest?

·        What happens after a positive diagnosis for an IHRD?

PACED works closely with Paediatric Cardiologists from across Canada to ensure that our message is accurate and current. The evidence based recommendations that we share are already being implemented by sport organizations across the United States and Europe. To date 13 US States have legislation passed or pending that make sudden cardiac arrest prevention programs mandatory for school and community sport organizations.

Sudden Cardiac Arrest is the leading cause of young person deaths that occur during participation is sport and play. There have been a least eight on ice deaths over the last nine years of Canadian minor hockey players. In Ontario 200 young people die suddenly of cardiac causes each year - not all of these deaths occur during participation in sport. Building communities that are more sensitive and responsive to the warning signs of potential heart arrhythmia will go a long way to reducing these numbers and the only cost is the time it takes to learn the facts.

It takes 45 minutes to acquire the tools to prevent the Sudden Cardiac Death of an Athlete

Every time a young athlete dies it is a tragedy.  When that child exhibited known warning signs of heart arrhythmia in the weeks or months prior to their death and no one picked up on those signals the tragedy is deepened. PACED is asking for 45 minutes of your group's time to help you start building the safety nets that will prevent these tragedies.

The knowledge that PACED has shared with the  Ontario sports community has already led to several children being correctly diagnosed with an IHRD and receiving the treatment that they need to lead a long and productive life. Please pick up the phone or send an e-mail inviting PACED to come and present at an upcoming league meeting.

Blake Hurst, Co-Founder of PACED may be reached at (905) 527-0462 or

info@paced.ca

 

Mandatory ECG for Paediatric Syncope - MEPS

ECG’s acquired when a child faints provide lifesaving data

Every Paramedic in Ontario, and probably where you live, is trained and equipped to take an ECG (Electrocardigram) and do basic heart rhythm interpretation. When Paramedics respond to a call for a child that has fainted it should be mandatory to get an ECG and to provide the strip to the Doctors in the ER, even if upon arrival at the scene the child has regained consciousness and “seems” fine.

Of the 700 young people that die suddenly of cardiac arrest each year in Canada about half had a fainting episode in the days, weeks, months or years prior to their death. These sentinel events provide the best opportunity to identify an underlying heart rhythm disorder and initiate treatment and prevention therapies.

Important information about what was happening to the child’s heart at the time of collapse is lost as time passes. The sooner the first or "presenting" ECG is taken the greater the understanding that Doctor’s will have of what triggered the event.  As time passes the heart returns to baseline and important clues may be lost.  

It is not essential for Paramedics to interpret these early ECG’s, they simply have to acquire the strip. Once acquired the ECG strip can be shared with the physicians in the ER or the Paediatric Cardiologists for analysis. Once acquired the ECG becomes part of the electronic call record and can be accessed weeks, months or even years later perhaps making an important contribution to a future investigation.

Studies estimate that roughly 1 in 20, about 5%, of all childhood faints are a warning sign for an underlying heart rhythm disorder. For faints occurring during or shortly after physical activity, while swimming or as a result of a loud unexpected noise (auditory startle) that number may go as high as 1 in 3, or 33%. Building communities that are more sensitive and responsive to childhood fainting can only result in young lives being saved.

There are some questions around the ability of Paramedics and the equipment they work with to collect accurate ECG’s from prepubescent patients. Two things occur to me - first, get the strip and let the physicians decide if it contains useful information – second, perform more ECG’s on children and get better at it which may also require additional training and equipment software upgrades with better paediatric programs.  As taxpayers we are paying for our Paramedics to be equipped and trained to gather ECG data, this toolkit has its greatest potential value when it is applied to our children.

Currently at least 14 states and provinces have legislation passed or pending designed to increase sensitivity to the warning signs of paediatric heart rhythm disorders in our communities. If Teachers and Coaches call 9-1-1 every time a child faints and Paramedics acquire an ECG for every child that faints and ER Doctors do a thorough work-up including event history, patient history and family history (and consults with appropriate specialists when required) for every child that faints, many young lives will be saved.

When a youngster has one or more fainting episodes and then dies an opportunity to save that child’s life has likely been missed. I have listened to the stories from dozens of families that have lost children to sudden cardiac arrest and far too many of those stories include anecdotes of fainting episodes that educators, sport officials and most concerning healthcare professionals have overlooked, brushed off or misdiagnosed.  Paramedics and the EMS system can play a vital role in ensuring that potentially lifesaving information is recognized and acted upon whenever our children experience a loss of consciousness faint.

Watching our Children Die on the Ice

Somebody do Something … just don’t ask me to perform CPR

For nine years I was the Public Access Defibrillation (PAD) Program Co-ordinator for a municipality with a population of just under 500,000. It was an exciting time on the frontier of AED placement in Canadian communities. In five years the number of cardiac arrest survivors in the community more than quadrupled, from 7 in 2005 to 31 in 2010, with many of those additional lives being saved by members of the public performing effective CPR and deploying an AED within minutes of patient collapse.

One life that was not saved was that of a hockey referee. He collapsed during a game that was well attended in an arena equipped with an AED. No one that witnessed the collapse knew where the AED was located or how to use it. No one was able to locate any of the arena staff that were trained to use the AED. CPR was performed but by the time Paramedics arrived it was too late for a defibrillator to be effective. In this earlier blog I share my thoughts on why this should have never happened.  

http://leadingcause.blogspot.ca/2012/12/cancel-one-game.html

A few weeks after the event the EMS Chief and me met with Executives from the Hockey and the Referee’s Associations. There were lots of emotions and lots of suggestions coming from all sides. The consensus was to move forward in a positive manner that would help ensure that the next time resuscitation needed to be performed the hockey community would be better prepared.

I lobbied hard with management to be allowed to offer “free” certification level CPR/AED training to any adult associated with the local minor hockey association – coaches, trainers, referees, officials and parents. In the end I was given the go ahead to offer six free sessions for up to 20 people per session over six consecutive Wednesday evenings in February and March. I would provide the training but the hockey association had to provide the people. The marketing and promotion, the filling of the seats, was entirely their responsibility, a condition which they readily agreed to.  The first week five adults with two teenagers in tow showed up – I had prepared 20 manikins and brought in another trainer to assist. I called the association and they promised more people the next week, four is not more than five.  After three people showed up in week three I spoke to the association and we mutually agreed to end the program. Out of the 120 seats that we had made available to the association 12 were filled.

The following winter good friends of mine that had lost a child on the ice to cardiac arrest (the AED was deployed but could not convert the heart back into a blood pumping rhythm) partnered with a local charity to donate an AED to an arena in their community. After a very nice on ice presentation ceremony that included a long and heartfelt standing ovation in memory of their beautiful son we announced that the following morning in the arena’s community room we would be offering free CPR/AED training to any and all interested parties, the session was also promoted through other means. Three people, plus the three of us attended.

The next time a coach, referee or player collapses to the ice and no one in attendance knows what to do where will the blame lie? In a public sports venue equipped with an AED it is the responsibility of every adult that regularly attends that venue as a coach, trainer, official or spectator, paid or volunteer, to acquire basic resuscitation skills. It is not the sole responsibility of the Zamboni driver to safeguard everyone’s life or to be highly visible whenever someone collapses.  

Again this week a child collapsed after taking a puck in the chest (a phenomenon called “commotio cordis” that claims the lives of about twenty young teens each year in North America) at an arena in Toronto. Thankfully arena staff and trained spectators were able to resuscitate the child. Nice job by Doug Jamieson and team. According to the CBC News story no hockey people – coaches, trainers, referees - helped with the resuscitation effort; if this is not true please let me know. 

The CBC interviewed several parents that witnessed the event and they put forward suggestions on how cardiac emergency response could be improved at Toronto arenas. The suggestions ranged from somewhat plausible to downright silly. However not one parent stepped up and suggested that they themselves and in general more people from the minor hockey community should be trained in CPR and AED use. There was lots of half-baked, buck-passing, ill-informed recommendations but not one person stepping up to take a little personal ownership for resuscitating friends or family members in cardiac arrest.

http://www.cbc.ca/news/canada/toronto/aed-defibrillator-helps-save-teen-hockey-player-s-life-1.2837497

In a related CBC story from June 2014 a senior executive from Hockey Canada explained that despite at least 8 on ice deaths in 9 years Hockey Canada is satisfied with its cardiac emergency response protocols which do not require CPR/AED training for coaches and trainers. In fact the recommendation is that if a coach or trainer suspects a player is in cardiac arrest they should seek out someone in the arena that is trained to manage the situation. This is the stated policy of the governing body of the sport that our entire nation loves so much.

http://www.cbc.ca/news/canada/nova-scotia/cardiac-arrests-kill-8-young-hockey-players-in-9-years-1.2669393

Long before the hockey referee died and every day since (it has been more than 6 years now) I have shouted from the mountaintops the importance of universal user group CPR/AED training. I believe that both minor and adult sport organizations should not be allowed to rent municipal sports venues unless the majority of their members are trained in CPR/AED and in general anyone that lives, works or plays in a building equipped with an AED should be trained.

My final two thoughts on this story:

1. When interviewed  by CBC Ali's sister used the word lucky.  A lot of forethought, planning and training on the part of The City of Toronto and the arena staff went into 13 year old Ali being "lucky" that day.
2. When you buy a Ford Escape everywhere you go you see Ford Escapes. When you take AED training everywhere you go you will notice the AED hanging on the wall. Take the training.

The Role of the Death Investigator in Preventing Sudden Cardiac Death

When a child dies of a cardiac cause seize the opportunity to protect siblings and cousins

In some jurisdictions they are called Coroners in others Medical Examiners. Regardless of job title they have the potential to significantly reduce the toll – 7,700 per year - that Inherited Heart Rhythm Disorders take on young people in provinces and states across Canada and the US.

The Coroner’s responsibility begins with correctly identifying the cause of death.  For the first degree relatives of a child that died of sudden cardiac arrest caused by an underlying genetic heart rhythm disease the words “unknown” or “undetermined” or “possible cardiac” on the death certificate is too often their own death sentence. (this will sound like hyperbole …. until it happens to your family). Inherited structural diseases, the cardiomyopathies such as HCM and ARVC can normally be detected through routine examine due to the extensive remodelling of the hearts structure. Inherited electrical diseases such as Long QT and Brugada Syndromes are more challenging as the telltale electrical signature of the disease is now switched off.  Often molecular genetic testing is required to determine cause. There are currently at least 15 genes and many more mutations associated with the various heart rhythm diseases and with the intensity of research in the field that number grows almost monthly. When on autopsy a clear phenotypical cause of death does not present the coroner must use DNA testing to seek a genotype that suggests a likely cause.

Coroners and ME’s should be collecting and storing genetic material, blood and tissue, for all young person deaths - under the age of 35 would be ideal, 18 is too low. Every province and state should have a DNA Bank where death investigators can safely store and easily access genetic material. Collecting and testing DNA samples from infants where the death was classified as SIDS (Sudden Infant Death Syndrome) is essential. Published research estimates up to 30% of all SIDS death are caused by an arrhythmia gene.

Every time a young person dies of “cardiac” or “presumed cardiac cause” both sides, the ME's Office and the Family (with guidance from the family physician) should be pursuing DNA testing.  Typically if the family does not ask, the ME will not initiate genetic testing.  Typically if healthcare professionals do not present DNA testing as an option the family is unaware of its availability and unaware of its lifesaving potential for gene positive first degree relatives. This is why death investigators and family physicians should be simultaneously informing parents of their options.  If it takes two or three or ten years for the family to recognize the importance of genetic testing the genetic material should be readily available when the call comes.  Note:  The family physician may wish to call upon the services of a genetic counsellor to help the family understand the importance and significance of genetic testing and the implications of all of the possible results – positive, negative, undetermined or any number of shades of grey.

When the testing is complete and the results are positive for a known genetic association with a heart rhythm disease the real work begins, notification and testing of all first degree relatives.  Most primary heart rhythm disease are transmitted by an autosomal dominant gene – if one parent carries the gene on average half of the children will acquire the gene, with no gender bias.  Step one therefore is determine which parent carries the gene.  Note: I have worked with a family where one parent was positive for Long QT and the other for ARVC, rare but possible.  Step two is to test siblings and then aunts, uncles, cousins and grandparents on the affected side of the family. It is common to identify four, five or more affected first degree relatives for every index patient.  This is the silver lining; this is the gift that the deceased has given to his or her family.  From one child’s death comes the ability to identify many at-risk relatives and provide them with the appropriate prophylaxis for sudden death.  
 

Step two is not without challenges. Identifying and locating all of the potentially affected family members may be difficult, convincing each of them that they should get tested for a genetic disease that they have never heard of  may be near impossible.    “Catcallamungowhatapolywhatacardia….. You think I might have this wack-a-doodle disease and should get tested?”    At this point all of the stakeholders – the Parents of the deceased, the Family Physician, the Paediatric Cardiologist/Electrophysiologist, the Genetic Counsellor, the Coroner’s Office must work together to protect the living.

Often this process moves into areas where the moral/ethical issues around personal health information privacy come into play.  Navigating these waters can be tricky, rules must be followed and boundaries respected. On a very personal note I believe that when a child’s life hangs in the balance it is best to err on the side of protecting the child.  I hate it when children die with their parents privacy still intact.

The other critical challenge in this process is speed. The Family and the Coroner’s Office must move quickly to pinpoint the cause of death and then to locate and test the first degree relatives. It is difficult to say exactly what the appropriate time frame is for this often complex process to play out but I would submit that if seventeen and eighteen year old cousins die just over one year apart the system has failed both families. Anecdotally, I hear some version of this story far too often.

In every jurisdiction in North America there is significant room for improvement in the way death investigations of young people dying from cardiac causes are handled and followed-up.  If you are an advocate for cardiac arrest prevention in youth please be sure to include improving protocols for death investigations on your must-do list when speaking with provincial or state bureaucrats and legislators.  If you are a family that has lost a child to sudden cardiac death and are unclear what triggered the event push the healthcare and death investigation systems for answers.

The Arrhythmia Train

The Arrhythmia Train is pulling out of the station.  In my talk at Canadian Cardiovascular Congress (CCC) in Vancouver I spoke about the growing awareness of Inherited Heart Rhythm Disorders that I have been observing in many communities and organizations.  My observation has been confirmed over the past few weeks as there has been a flood of arrhythmia news both in the mainstream media and in the places where only an arrhythmia geek like me would look.

The folks at CHEO (Children’s Hospital of Eastern Ontario, Ottawa) have put genetic heart rhythm disorders, especially Long QT Syndrome, front and centre. CHEO has launched a legal challenge to the concept of patenting a gene or gene sequence.  A positive outcome to the case will benefit all Canadian families seeking diagnosis of a genetic disease.

http://ottawa.ctvnews.ca/cheo-launches-lawsuit-against-owners-of-gene-patent-1.2084430

In conjunction with the CHEO story CTV ran the story of the Dines Family from Ottawa. Their story is similar to many of the stories we hear from families affected by an arrhythmia gene. The elements of family history and misdiagnosis in the 20^th century are common themes.  The fact that a definitive diagnosis was made immediately based on a simple, inexpensive, non-invasive ECG is also noteworthy.

http://www.ctvnews.ca/health/diagnosis-of-genetic-heart-rhythm-disorder-life-changing-for-families-1.2089409

A colleague from Victoria BC sent me information about Sports Cardiology BC and the ECG screening clinics that they are hosting in that province.  At the end of November they will be hosting a clinic in Victoria for competitive athletes between the ages of 12 and 35. To the best of my knowledge, please correct me if I’m wrong, this is the first and only group to hold ECG screening clinics for Canadian youth.  While the merit of ECG screening is debated in the cardiology community PACED sees this as a very good thing.

http://www.sportscardiologybc.org/news/sports-cardiology-b-c-is-coming-to-victoria/

In a related story Dr. Vicotria Vetter the highly respected paediatric cardiologist from Children’s Hospital of Philadelphia (CHOP) came out firmly in support of ECG screening for all youth.

http://www.pharmiweb.com/PressReleases/pressrel.asp?ROW_ID=102909#.VFzsq410zec

More positive news (with a very sad and tragic etiology) out of BC is the $1.7 million out-of-court settlement paid by a Vancouver School Board to the family of child with Long QT. The board failed to prevent the cardiac arrest which left the Grade 5 girl with significant physical and neurological deficits. These preventable tragedies should not happen however lawsuits encourage the type of due diligence that can prevent future tragedies. Ontario law firm Miller Thomson picked up on the story and discusses the implications in their client newsletter.  Our thoughts and prayers are with Bezawit and her family.

http://www.millerthomson.com/en/publications/newsletters/education-law-newsletter/september-2014/keeping-abreast-of-the-standard-of-care

Perhaps the most exciting news to come out of CCC is that the BRIDGE project at Canadian Cardiovascular Society (CCS) has invited a SUDY (Sudden Unexpected Death in Youth) working  group to join the project. This invitation from CCS represents invaluable recognition for Inherited Heart Rhythm Disorders, the Electrophysiologists that treat them and the families affected by them. To learn more about the BRIDGE project visit the website.

http://bridge.ccs.ca/index.php/en/

Closer to home PACED is very pleased to announce that a new and improved version of Bill 81, The Inherited Heart Rhythm Disorder Awareness Act, 2012 is in the works and may be receiving First Reading at Queen’s Park prior to the Christmas Break.  Amongst other things  bill require that 911 be called for all children that faint during physical activity at school or while participating in minor sport and that they be physician cleared for return to play. It will also require educators and coaches to receive regular training on recognizing and responding to the warning signs.

http://www.ontla.on.ca/web/bills/bills_detail.do?locale=en&BillID=2629&isCurrent=false&ParlSessionID=40%3A1

Here are seven links to recent news stories that are directly related to creating greater awareness and heightened sensitivity to the warning signs and prevention strategies for Inherited Heart Rhythm Disorders and sudden cardiac arrest in youth. The Arrhythmia train is picking up passengers and momentum.

Where we aren't at Preventing Sudden Cardiac Arrest in Youth

Despite at least 8 on ice deaths in 9 years Hockey Canada’s Head of Safety, Todd Jackson told CBC that "We are at a point where we are delivering something we are very comfortable with"  when asked if his organization was doing enough to prepare for and respond to cardiac arrest.

In Ontario, OPHEA, the organization charged with setting guidelines for safe and healthy schools has given Arrhythmia Diseases a place of prominence in their   Sample Information Letter to Parents/Guardians and Medical Information Form   and provides a brief overview of Heart Arrhythmia Diseases  as Appendix “M” in their guidelines. Unfortunately Boards, Principals and Teachers are not mandated to follow these guidelines.  In my significant personal experience I have found Educators in Ontario to be largely unaware of all aspects of arrhythmia disease including common warning signs.

Legislatively no Canadian province has passed a bill targeted at preventing Sudden Cardiac Arrest in Youth, the #1 killer of young people at schools and at community sports venues. (At the time of writing a Private Members Bill is being drafted in Ontario. Thank you Christine Elliot and Dr. Kirsh) In the United States there are at least six states with legislation passed and six or more with legislation pending.

In this century alone at least 10,500 young Canadians have died suddenly of cardiac causes.  For the entirety of this century organizations such as The Canadian SADS Foundation (Sudden Arrhythmia Death Syndromes) have been promoting the warning signs of Heart Arrhythmia.  Paediatric Electrophysiologists  (physicians that specialize in treating electrical diseases of the heart) have provided the scientific rationale for the warning signs and endorsed the widespread dissemination of them.  Sadly awareness and prevention initiatives have gained zero traction in this country.  If the road to an effective national prevention strategy for sudden cardiac arrest in youth is 100 miles long Canadians have taken no more than one or two steps since the 1990’s. The investments (mostly non-monetary) required to reduce the 700 young person deaths each year have simply not been made, rarely even discussed.

In contrast, I sat at my desk, on a cold and drizzly October afternoon in 2009, and watched parents drag their children a kilometer or more down a busy roadway to line up outside in the rain for up to two hours to receive a vaccine for a strain of flu (H1N1) that despite its advanced billing ended up being relatively benign.  Health Canada estimates of the potential for between 2,000 and 8,000 flu related deaths in Canada that year vastly overestimated the reality of the 428 H1N1 deaths, mostly older adults with co-morbidities, reported in Health Canada’s final report on the pandemic.  The point I want to make is that with little to no evidence to back their play healthcare officials used their legal and moral authority to mobilize resources, spend significant public monies often by accessing emergency funds, require healthcare workers to put in overtime, mandate lower levels of government to respond immediately to demands for human resources and facility space.  In all it was an impressive, expensive, coordinated, lightning quick response, that while probably unnecessary, demonstrated what the various levels of Government in Canada are capable of when they set their collective minds to a task.  The 62 page final report from Ontario’s Chief Medical Officer of Health is at least 60 more pages than any government official working in healthcare has ever written on prevention strategies for the heart arrhythmia diseases that claim the lives of 700 otherwise healthy young people each and every year.

On October 27, 2014 I will be attending the first ever National Strategy for the Prevention of Sudden Death in the Young  meeting at the Canadian Cardiovascular Congress in Vancouver. (Thank you Dr. Sanitani and Dr. Krahn) A small group of EP’s and other stakeholders will hopefully begin to delineate some critical pathways and kick start some meaningful prevention activities across the country. This meeting coupled with the proposed Private Members Bill in Ontario is as much positive activity as we have ever seen. In my next blog I will report on the discussions at the Vancouver meeting  as well as work we are doing with Ontario School Boards and Hamilton area Family Health teams.  By October 2015 perhaps we can be a mile or more into the 100 mile journey.

Epinephrine Auto Injectors in Restaurants

Not a well thought out proposal

My hometown Hamilton, Ontario has been receiving continent wide attention for  a unique proposal put forward at city council to make it mandatory for restaurants and shopping mall food courts to be equipped with epinephrine auto injectors.  On its face it may seem like a good idea but when you break down the numbers the chance of a Hamilton family benefitting from this program are about the same as their chance of winning a 6/49 lottery scheme. At the same time restaurant owners will be required to throw $280.00 in the trash each year.  There are proven anaphylaxis prevention strategies that could be implemented with $150,000 that would be far more effective at preventing life threatening events.

Prior to Sabrina’s Law (Jan 1, 2006) paediatric death triggered by food allergies was very rare in Ontario, less than 1 per year from 1986-2000. Since Sabrina there were no reported deaths in the province from 2004 until the tragic 2013 event in Burlington, that’s one in 10 years across the entire province, population 13.5 million. That equates to about one event every 250 years in a city of  half a million residents.  The majority of children that are anaphylactic to a known allergen are identified and their parents are responsible for insuring that they are protected from both known triggers and lethal reactions when they occur.  The chance that a potentially lethal anaphylactic event involving a child will occur in a Hamilton restaurant in the next 10 years, and the only available epinephrine is the auto-injector supplied by the restaurant are slim and none, and Slim is out of town.  The chance that one of these auto-injectors will be used to save a child’s life during a two year trial period is microscopic.

The financial burden on restaurant owners is not insignificant. Auto-injectors cost $140.00, each restaurant will need to purchase two, a 15mg dose for smaller patients and a 30 mg does for larger patients. Staff must be trained and program integrity must be maintained. Every year the two injectors must be safely disposed of and then replaced at a cost of $280.00. In the current economic climate it hardly seems fair to ask a small businessman to burn at least three hundred dollars a year on a program that has no evidence base to support its potential efficacy.  If the city gives the restaurateurs a break and picks up the $150,000 annual tab for throwing this medicine in the trash, the cost will be borne by ratepayers.

An interesting sidebar to this discussion is the retail price of epinephrine auto-injectors. When a dose of epinephrine is drawn up at a hospital or in the back of an ambulance it costs taxpayers less than 50 cents, which  includes the medicine, the syringe and the needle. The pharmaceutical companies that sell epinephrine on the retail market are taking advantage of parent’s vulnerability and marking the product up 14,000 percent.  Auto-injectors could be sold for $20.00 and the Pharma’s would still make money. It begs the question, who would benefit most from this policy?

If the cost of the program is $150,000 per year, as suggested by a local Councillor on national radio, what alternative strategies could be funded with that money? Are there strategies that would provide greater protection from severe allergic reactions for Hamilton children?  Is it possible to fund auto-injectors for low income families?  Is it possible to require restaurants to provide diners with a list of ingredients that they use that are on the list of known food allergens?  Is it possible to require restaurants, especially those with outdoor seating, to take steps to reduce the presence of stinging insects – wasps, hornets, bees?  Is it possible to invest in free anaphylaxis awareness education for parents and caregivers, including seminars and online training tools?  Is it possible to reduce wait times for children that have had a mild to moderate reaction to an allergen to see a specialist and get tested? 

All five of these suggestions could be funded with $150,000 per year with money left over and I’m sure that people far smarter than me could come up with a list of five even better suggestions on how to prevent and respond to anaphylactic reactions.

A STEMI Protocol for Muskoka and Cottage Country

Ontario’s 15 cardiac catheterization labs and the interventional cardiology teams that staff them represent a significant investment in achieving the best possible outcomes for ST-Elevated Myocardial Infarction (STEMI) patients. Paramedics are equipped and trained to recognize STEMI heart attacks and transport patients to the regional cath lab with a goal of achieving the international gold standard time of 90 minutes door-to-balloon (DTB) – that is 90 minutes from when the Paramedics make patient contact to when the Cardiologist restores blood flow to the affected part of the heart by inflating the balloon at the tip of the catheter. Patients that are treated at a cath lab within the 90 minute window enjoy significantly improved short, medium and long term outcomes for both return to a good quality of life and survival.

The vast majority of Ontarian’s, as many as 12 of 13.5 million residents, live within the 90 minute catchment of a regional cath lab when transported by land ambulance. One notable exception is the tens of thousands of people that migrate to Muskoka and cottage country during the summer months, especially on weekends. By design cottages are remote places and access is complicated by kilometres of twisting gravel roads and in many instances the need to cross water. Achieving 90 minute DTB times using land ambulance is not possible from most cottages. The cath labs at South Lake in Newmarket and Health Sciences North in Sudbury are tantalizingly close but not quite attainable. Even the proposed cath lab at Royal Victoria in Barrie will lie outside the 90 minute window for most of cottage country. The new cath lab at Peterborough is now accessible from many cottages in the Kawartha Lakes and Haliburton regions, but certainly not all.

It is the right time to consider the viability of having an air ambulance dedicated to Muskoka and cottage country, perhaps only as a May to September program, perhaps only from Friday to Monday. The appropriate first step is a review of EMS call volumes for the past several years, looking at calls – cardiac, trauma and other – where air ambulance was requested or where the patient may have benefited from air ambulance. If the data suggests that call volumes warrant a dedicated helicopter for Muskoka a deployment strategy should be developed. This may involve having a helicopter based out of Muskoka Airport in Bracebridge during peak volume periods.

The high cost of building and maintaining Ontario’s world class STEMI response program has been met. The cost of providing access to the program for tens of thousands of people that have a permanent residence within the 90 minute window but spend a significant number of days each year cottaging or vacationing on the periphery is incremental.  A dedicated air ambulance for Muskoka, tasked with getting STEMI patients to the cath lab within the 90 minute window, should be viewed as a value added proposition, not an expense. It was the tax dollars of the people in cottage country that in no small part funded the development of the Province’s STEMI program, it seems only fair that a relatively few additional dollars be invested to provide them with year round access.

The true value of an air ambulance access program will be realized when patients that would have been left debilitated (or dead) and facing months of expensive rehab are now coming home within days of their MI with little or no loss of heart function, little or no death of heart muscle and enjoying a rapid return to a lifestyle that in many cases is better than what they were experiencing pre-heart attack.  The cost saving to the healthcare system when these outcomes are achieved is significant.

Leading Cause Prevention Strategies, along with many other CPR and First Aid training agencies and local EMS services, is dedicated to providing Cottager’s with the knowledge and tools to achieve the best possible outcomes for cardiovascular emergencies. The first step in achieving good outcomes is early recognition and management of the warning signs and symptoms of heart attack, stroke and cardiac arrest. Even if first responders restore a blood pumping rhythm to a cardiac arrest patient with early CPR and early Defibrillation patient outcome is compromised if there is no early access to advanced care. Even if first responders recognize the signs of heart attack and Call 911 immediately, patient outcome is compromised if there is a delay in restoring blood flow to the affected part of the heart. The work that we do helping Lake Associations and cottage families build cardiac safe environments will be lost if patients do not have rapid access to advanced care and cardiac cath labs.

Mandatory Arrhythmia Awareness in all CPR Classes

This year, like every year, more than 7,700 people age 35 and under will die suddenly of cardiac causes in the US and Canada. Roughly half of them will experience fainting episodes or exhibit other warning signs in the months, weeks and days prior to their death. If these sentinel events are recognized and acted upon a great number of at risk young people will not die.  Several of the diseases that underlie paediatric Sudden Cardiac Arrest are genetic, with a dominant gene transmission pattern, and therefore many asymptomatic first degree relatives might also be saved.

All properly taught certification level CPR training includes a discussion on Heart Attack and Stroke warning sign recognition and management. It is time to include a discussion on recognizing and responding to the warning signs of paediatric heart arrhythmia diseases in every CPR class with extra attention being given to the discussion for classes being taught to educators, coaches and any adults that are responsible for the well-being of children.

The basic warning signs of inherited heart rhythm disorders are as follows:

·        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

Other warning signs include; brown outs (near fainting); a strong sensation of palpitation or racing heart; extreme shortness of breath (new) with exertion - more so than other children.

The recommended response to all loss of consciousness fainting is to Call 911, even if the child quickly regains consciousness and “seems fine”. For both fainting and all other warning signs parents should be advised of the event, provided with information (a pamphlet or a link to a web page) on heart rhythm disorders and encouraged to consult a physician that is trained to recognize paediatric arrhythmia. 

Every CPR Training Agency, both not-for-profit and private, should be required by state or provincial law to include a discussion of the warning signs of Paediatric Inherited Heart Rhythm Disorders in every class.  Special attention should be given to the topic when the audience is educators, coaches or any other group that works directly with young people.  Already eight states have passed or are considering legislation that will make awareness training of paediatric heart rhythm disorders mandatory for coaches, sport officials and physical education teachers. We need every state and province to mandate this training as a required component of every CPR class.

The evidence to support the inclusion of Arrhythmia Awareness in every CPR class is abundant und unequivocal. The International Liaison Committee on Resuscitation (ILCOR) was asked to consider a recommendation to include Arrhythmia Awareness in all Basic Life Support training classes as part of the 2010 BLS Guideline Recommendations.  They chose a softer position regarding the follow-up by coroners of all potentially cardiac related deaths of young people. Even if ILCOR chooses to ignore the evidence state and provincial legislators can still choose to act in the best interest of at-risk young people.

The Untapped Resuscitation Goldmine is in the pre-EMS Phase

Scientists love rigor - controlled environments, carefully defined processes and known variables (is that an oxymoron?).  Much of the research relating to the resuscitation of cardiac arrest patients in the out-of-hospital setting is focused on therapies delivered by healthcare professionals beginning with Paramedics and then moving through the system to the ER and the CCU.  Patients that receive effective therapies – quality chest compressions and defibrillation – prior to EMS arrival are at least six times more likely to survive (31.7% vs. 5.2%) than EMS attended patients and yet this group remains very small representing less than 6% of all cardiac arrest victims. Common sense dictates that it is time for a resource re-allocation that shifts the focus from what happens to the patient after EMS makes contact to what happens to the patient prior to EMS contact.

http://www.sca-aware.org/sca-news/aha-releases-latest-statistics-on-out-of-hospital-cardiac-arrest

The pre-EMS world is a little messy and unpredictable.  Although we know that there will be a next event it is impossible to know when and where it will occur. It involves the great unwashed masses and there is no way to control or predict how any individual or group will respond when faced with a friend or family member in cardiac arrest (or is there?). It can be nearly impossible to collect reliable data about how the event unfolded prior to EMS arrival.  In consecutive post event investigations responders to the first incident told me with confidence that they shocked the patient three times - the ECG download showed no shocks, in the second event bystanders said with conviction “No, we didn’t shock him at all” - the download indicated two shocks.  Scientists are not happy working in this chaos and as a result little research is focused on this most critical stage of resuscitation.

Out-of-hospital cardiac arrest is one of the most studied medical phenomenons in the world and researchers have access to high horsepower data sets that often include more than 10,000 SCA events.   Every time large data sets are analyzed the pure gold, survival to hospital discharge, is always found in the Bystander Initiated Response cohort. Therefore it almost defies logic that that preponderance of research dollars is spent on studies that are seeking to find out such things as which pharmacological therapy or which cooling strategy initiated by Paramedics is the most effective.

More research dollars should be dedicated to finding ways to build communities that have high rates of Bystander CPR and high rates of Bystander Delivered Defibrillation. The critical question has to be, how can we increase the percentage of patients that have ROSC prior to EMS contact?  How can we take what is proven to be the most effective therapy, bystander response, and ensure that it happens significantly more often? I will share some ideas in upcoming blogs.

In addition to research, scientists and the organizations that fund them also need to come out of their offices and laboratories to meet with politicians, senior bureaucrats, leaders in education and sport and explain to them the simple truth - Improving the historically dismal survival rates for out-of-hospital cardiac arrest is entirely dependent on the community’s willingness to take ownership of the issue. Mandatory placement of AED’s in broad spectrum of venues and mandatory CPR training for broad spectrum of citizens is the simple and inexpensive solution to  an enormous problem that isn’t nearly as vexing as scientists would have us believe.

Why is simple, inexpensive and effective not the pathway of choice ?

Achieving the Best Possible Outcomes for Cardiovascular Emergencies - Part 3 of 3

What could be accomplished with Two Dollars?

In Ontario roughly 20% of the population is age 55 or older. In a typical LHIN (Local Health Integration Network) with a population of 1,000,000 this represents about 200,000 people. A budget line based on $2.00 per age 55+ resident dedicated to raising awareness amongst older adults of how to recognize and respond to cardiovascular emergencies would represent an annual expenditure of $400,000 of the best money ever spent on healthcare.  Provincially that’s about $6.4 million out of the $49 billion healthcare budget.

Managing the care and rehabilitation of patients that have experienced Heart Attack and Stroke represents a multi-million dollar cost to any healthcare system. Due to the extremely high cost of treating patients that have experienced the worst possible outcomes every healthcare jurisdiction invests heavily in developing facilities and teams of specialists that are tasked with achieving the best possible outcomes. The tools and the techniques that these interventional teams use are at the leading edge of medical technology representing the culmination of billions of dollars of worldwide research and development costs. The programs are so expensive and highly specialized that in Ontario only one in eleven (15 of 165) hospitals are set-up to perform the cardiac procedures.

What I find fascinating is that the same billions of dollars of research has shown conclusively that the independent variable with the strongest association to good outcomes for Heart Attack and Stroke victims is time, specifically the time from symptom onset to definitive treatment. In-hospital improvements to programs may result in incremental improvements in time to treatment but the potential for quantum improvement lies entirely in the pre-hospital realm and specifically with the lay public - the patients, their families, coworkers and friends.

The gold standard treatment times of 90 minutes for Heart Attack (STEMI) and 180 minutes for Stroke will only be achieved consistently when the majority of the adult population is knowledgeable around recognizing and responding to observed symptoms. Every time the gold standard time to treatment is achieved the healthcare systems saves tens perhaps even hundreds of thousands of dollars. We should also pause to consider the human cost of missing the 90 or 180 minute window.

Every month I teach CPR to between 50 and 100 older adults across Ontario and we typically spend up to an hour discussing Heart Attack and Stroke recognition and response.  The vast majority of the adults I meet are somewhat aware of the symptoms, totally unaware of how to respond when symptoms are observed, unaware of the importance of a timely response and most importantly unaware of the life altering difference in patient (and family) outcomes for those that are treated within the gold standard timeframes.  Especially with adult learners explaining the why of things, in this case significantly better patient outcomes,  is the most effective way to deliver the message.

If every LHIN in Ontario hired one fulltime Health Promoter, with an appropriate program budget, dedicated to raising awareness amongst older adults of how to recognize and respond to cardiovascular emergencies measuring the impact of their efforts, in both economic and human terms, would likely be the best news story that many LHIN’s would have to report on each year.  Through stakeholder networking to create opportunities for workshops and presentations and effective use of both traditional and social media a little money could go a long way to disseminating the “time is of the essence” message.  Realizing the economic benefit of increasing the number of Heart Attack and Stroke patients that arrive at Ontario’s world class facilities within the target time from symptom onset is some of the lowest hanging fruit in the healthcare system

Note: The task of educating the public on Heart Attack and Stroke Recognition and Response is entirely separate and distinct from promoting lifestyles that contribute to good cardiovascular health.  Programs aimed at lifestyle modification will have little or no effect on “symptom onset to definitive therapy times” for patients experiencing a cardiovascular emergency.

Achieving the Best Possible Outcomes for Cardiovascular Emergencies - Part 2 of 3

What does the best possible outcome for Heart Attack or Stroke look like?

I was at The Cottage Life Show in Toronto last weekend, promoting CPR and AED’s (Automated External Defibrillator) for the family cottage.  A couple stopped by the booth to ask a few questions. Both were healthy and happy and I would have never guessed that less than a year ago he had suffered a significant myocardial infarction. The reason he looked and felt so good – immediately upon the onset of symptoms 911 was called and he was taken directly to the cardiac catheterization lab at Sunnybrook Hospital and emergency angioplasty was performed well within the 90 minute target time.

Over the past six years I have met many patients that have been treated within the 90 minute door to balloon time (DTB) window that is the target for all STEMI (ST Elevated Myocardial Infarction) programs and it would be impossible to tell that any of them had recently had a significant MI.  Most patients that make the 90 minute DTB window have suffered little or no loss of heart function and little or no permanent damage to the heart muscle. Return to a good quality of life often takes only a few weeks and return to work often occurs even sooner than many patients would have hoped for.  Many STEMI patients have told me that they “feel better now than they have in a couple of years.”

Last summer I met a neighbour walking his dog.  As he approached I didn’t even notice his slight limp and wouldn’t have guessed he had recently suffered a stroke unless he had told me. His story was similar to the stories of most stroke patients that are home within a few weeks with little or no noticeable deficit – both he and his wife instantly recognized what was happening and wasted no time in calling 9-1-1.  The Paramedics transported him directly to the Regional Stroke Centre where he received thrombolytic therapy within two hours of the onset of symptoms.

These two real world examples are what I would define as “the best possible outcome” for Heart Attack and Stroke. After a short stay in hospital both patients were home, physically and neurologically intact, enjoying a very good quality of life and quite willing to buy green bananas.  

What does it mean for patients, families and taxpayers when the best possible outcome is achieved?

For spouses, children, grandchildren, friends and co-workers to have a person they care about come through an acute cardiovascular event with little or no permanent deficit and able to return quickly to a pre-event quality of life is priceless.

For our overburdened healthcare system the price of not consistently achieving the best possible outcome for heart attack and stroke is one that we can no longer afford to pay. When Heart Attack and Stroke are allowed to run their course and visit the maximum damage upon the patient the result is lengthy hospital stays including many days or weeks in intensive care, the costliest level of care.  Many patients are unable to go directly home but must go to a rehabilitation facility where they re-acquire simple skills needed to manage daily living. Return to anything like their pre-event quality of life, including return to work, may take months or years or may never happen.  Many patients will have an intimate and costly relationship with the healthcare system for the rest of their life. 

The investment required to educate older adults (and for that matter everyone) how to recognize and respond to significant cardiovascular emergencies is miniscule by comparison. In a world where communication and knowledge sharing is omnipotent and omnipresent it astounds me how few of the adults that attend my classes have even a rudimentary knowledge of heart attack and stroke recognition and management. I do know that for two dollars for every adult over age 50 we could go a long way toward providing each of them with the knowledge and tools to achieve the best possible outcome for Heart Attack and Stroke.