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 ?