PACED – Parents Advocating for Cardiac Education
PACED (Parents Advocating for Cardiac Education) is group of individuals and families affected by Inherited Heart Rhythm Disorders (IHRD) advocating for greater awareness and identification of these diseases. PACED calls upon a number of cardiologists and electrophysiologists to advise us on how best to direct our efforts. To date those efforts have been focused in two areas;
- Putting on seminars in our communities to educate; primary care physicians, educators, sport officials on understanding these diseases with a focus on recognizing the warning signs and responding to them.
- Developing and advocating for Bill 81, The Inherited Heart Rhythm Disorder Awareness Act, 2012. This legislation unanimously passed second reading in the Ontario Legislature and unfortunately died on the order paper when the provincial parliament was prorogued in October 2012. MPP Christine Elliot-PC will be re-introducing the bill in the Fall 2014 session. When enacted the bill will the first in Canada to address awareness of IHRD’s. To date six US States have passed similar legislation and at least six more have laws in the works.
In his paper presented at the 2012 Canadian Cardiovascular Congress Dr. Andrew Krahn showed that in Ontario in 2008 almost 200 young people, under age 40, died suddenly from cardiac arrest resulting from an underlying Inherited Heart Rhythm Disorder (IHRD). These numbers are consistent with the reported incidence of sudden unexplained death amongst otherwise healthy young people from other countries and jurisdictions around the world. These numbers suggest that taken together the group of heart arrhythmias known as IHRD’s may be the leading medical cause of death in the paediatric population in developed countries.
The challenge in reducing the toll that these diseases take is that in most cases the patient is otherwise healthy making identifying at risk patients difficult. For roughly half of the young people that die from an IHRD related cardiac arrest the first indicator of the disease is death. Of the other half many present with warning signs in the weeks or months prior to their death. The most obvious warning sign is fainting (syncope) others include; palpations, racing heart, extreme shortness of breath, brown outs, and dizziness; with any of these episodes being triggered by physical activity, emotional distress, excitement, auditory startle or no obvious trigger.
Two strategies for identifying at risk individuals and protecting them from cardiac arrest are;
- Raising awareness of the warning signs of IHRD’s and encouraging parents, educators and minor sport officials to be diligent in following-up on syncope and other warning signs with a knowledgeable physician. This approach can be very effective for diagnosing disease in the half of the affected population that exhibit warning signs.
- Screening programs which include a pre-participation screening questionnaire and a resting ECG. This option is the most cost effective method for identifying patients in the half of the affected population that present with no obvious symptoms of disease.
Note: Most IHRD’s are heritable diseases transmitted by an autosomal dominant gene. Therefore the yield from any awareness or screening programs must be far greater than the total number of index patients identified. Dr. Joel Kirsh, Sick Kids, Toronto, suggests that he typically identifies five or six first degree relatives in addition to the index patient.
Once diagnosed, most patients with an IHRD can expect to live a long and productive life. Excellent prophylaxis from lethal tachyarrhythmia can be provided by implantable devices, pharmaceuticals, surgical procedures, lifestyle modification or a combination of the above.
The Awareness Campaign
Over a decade of working to raise awareness of IHRD’s we have identified three key target audiences that need to hear and act on the awareness message:
- Primary Care Physicians. Much of the current understanding of IHRD’s has come about in this century with the first genes connected to Long QT being identified in 1995-96. Awareness campaigns are designed to have parents take their children to see a Family or ER physician anytime warning signs are observed. It is therefore critical to the success of a campaign that these key partners are equipped with the latest knowledge and tools for diagnosing and managing these patients
- Community Leaders in Education and Sport. The efficacy of an awareness campaign is contingent upon post syncope patients being seen by a physician. School Board Directors of Education and Superintendents and Minor Sport Organization Executives and Directors have the authority to mandate medical follow-up for all syncope and other warning signs. They also have the authority to implement and enforce return to play policies for post syncopal children. In our experience most are willing to do so once they understand what is at stake.
- Parents, Teachers and Coaches. Everyone that spends time with young people needs to know the warning sign and be prepared to either get the patient to a physician or advise a parent or guardian of the importance of doing so.
There is a vast array of communication options available to assist in spreading the IHRD awareness message to the target audiences. It may seem a little old fashioned, but bringing target audiences together for a brief (one to three hour) seminar with information delivered by Electrophysiologists and other subject matter experts and with ample time for dialogue, is still highly effective. This information could be supported and enhanced by development of teaching videos and other on-line tools.
Stakeholder organizations could provide links to this information on their respective websites and direct members with questions or concerns to the online material. Another longstanding vision of PACED is funding for a traditional electronic media (television and radio) public service announcement (PSA) campaign.
The messaging in the awareness campaign will be designed to initially steer at risk children to their family physician and ultimately to the regional centre of excellence for paediatric cardiology where a comprehensive work-up and definitive diagnosis will be completed. A few of the measurable outcomes of this initiative will include:
- An increase in the number of patients being appropriately referred to the regional centre of excellence.
- A decrease in the number of patients being inappropriately referred.
- An increase in the diagnosis of IHRD’s in the regions paediatric population
- A decrease in incidence of sudden cardiac arrest in the paediatric population
A Canadian Pilot in ECG Screening
There is a worldwide movement toward ECG screening for teenagers, especially competitive athletes, for the purpose of identifying underlying heart rhythm disorders including both cardiomyopathies and channelopathies. To date we are unaware of any ECG screening projects conducted in Canada. It would be instructive to complete a pilot that includes enough subjects to give the study horsepower, a minimum of 1,000 and perhaps even double that number. From the very beginning we wish to state that we would want no more than one third of study participants to be competitive athletes. Genetic diseases do not recognize athletic ability and will visit any child without discrimination.
The most referenced screening program was completed in northern Italy and attributes an 89% decrease in sudden cardiac arrest deaths amongst competitive athletes in the region to a comprehensive screening program. A more recent and equally compelling study from Switzerland was presented at the 2012 ESC Congress in Munich. The study found 1:250 athletes screened ultimately received a diagnosis of a potentially lethal heart arrhythmia http://www.medicalnewstoday.com/releases/249534.php
In order to simplify the inclusion process we propose that study be conducted with subjects that have attained the age of majority. A university would be an ideal place to complete the study. The magnitude and the endpoints for a screening program would be determined by funding levels and in-kind contributions. The barrier that we have encountered when proposing ECG screening programs in Canada has been finding qualified Cardiologists willing and/or able to read the ECG’s. Many screening programs in the US and UK have cardiologists that donate their time to the program.