In July 2019, beloved young actor Cameron Boyce died in his sleep from an entity called SUDEP, or Sudden Unexplained Death from Epilepsy. As I wrote in a prior Forbes article, few knew that Boyce had been suffering from a seizure disorder, and the entity called SUDEP was not widely recognized outside of the epilepsy community. Since the untimely death Boyce, there continues to be increased awareness about SUDEP, especially from those who have lost a loved one to such an event. For instance, the Danny Did Foundation, created by Mike and Mariann Stanton after losing their five-year-old son Danny to SUDEP, serves to advance public awareness of seizures and the risk of SUDEP. The foundation is not new, yet the death of Cameron Boyce led to the founders of “Danny Did” to lend their voice to this rarely known, devastating event. And while SUDEP is rare, many families who have a loved one with epilepsy have developed heightened awareness of this potential event after the publicized death of celebrity Cameron Boyce.
The frequency of SUDEP is estimated to be 1 in 4,500 children with epilepsy, and 1 in 1,000 adults with epilepsy. While these events may have no warning, there are known risk factors for SUDEP:
- Frequent Generalized Tonic-Clonic Seizures (GTCS)
- Not being seizure-free for 1 to 5 years
- Not taking anti-epileptic medications for seizure control
- Having a history of seizures during sleep in the past
- Males are slightly more likely than females to have SUDEP
- More common in the 20- to 40-year-old age group
After hearing of Boyce’s death, many patients and caregivers were afraid to be alone at night or leave loved ones alone at night, for fear of SUDEP. As nighttime seizures can, indeed, go unnoticed, the question of seizure detection devices in effort to quickly identify a seizure during sleep comes into question. For individuals at high risk for SUDEP, wearable devices may serve to alert those around them of an ongoing seizure during sleep. Sensors on some of the devices may be able to detect early signs of cardiac and respiratory distress, with specific alarms alerting family members of an emergency. Many of these devices are still in the early stages of development, and there will need to be some extensive follow-up to assess whether or not they do, indeed, reduce the incidence of SUDEP.
Unfortunately the jury is out as to the overall accuracy and benefit of recognition devices to identify seizure activity and potential SUDEP. And as rare as SUDEP is, the tragedy of a celebrity has brought it to the forefront of the epilepsy community. While recognition and awareness are critical, not only to those managing seizures of their own, but also to family members and caregivers, panic is never a good thing.
I connected by phone with Dr. Page Pennell, Professor of Neurology at Harvard Medical School/Brigham and Women’s Hospital and President of the American Epilepsy Society. Dr. Pennell, whose research and clinical work focus on seizure disorders, agrees that SUDEP continues to be something we don’t yet completely understand. She mentions that it is somewhat similar to Sudden Infant Death Syndrome, or SIDS, an event that occurs in young infants where they die unexpectedly without explanation in their sleep. Many families use infant monitors of varying types to assess their infants and young children during sleep. Many of these prove useful, yet many lack in accuracy. This is a similar situation in families with a child or adult family member with a seizure disorder. According to Dr. Pennell, there is one FDA-approved wearable device to detect seizures. In early 2018, the Embrace by Empatica device received FDA approval to act as a monitor to detect the highest-risk seizures (generalized tonic-clonic). It is worn like a smartwatch, and will alert a caregiver by alarm if a seizure is taking place. There are at least five to ten other wearable detection devices in use, and further study continues in hopes to identify other methods of early seizure detection
Pennell warns that these devices have their flaws. She notes that they have very high sensitivity (meaning the slightest variability in brainwaves can be detected) but very low specificity (meaning that there are many false positives in setting off alarms). This can lead to what’s known as alarm fatigue, a known entity experienced in hospital settings, where so many alarms, most of which are ‘false,’ lead to the caregiver eventually ignoring all alarms, even those that are real. This occurs from home device false alarms, as well. The other concern Dr. Pennell discusses is that these devices, including wearables and those that are placed under the mattress to be used as motion detectors, require frequent (in many cases daily) battery charging or battery changing. Last, she mentions that these alarms, even when accurate, can leave a false sense of reassurance for some, and added levels of sense of responsibility for others. In other words, if a family member does, indeed, respond to a true alarmed seizure leading to SUDEP, would they be able to revive the family member? In some cases, probably not.
But Dr. Pennell does voice some optimism going forward: “There continues to be opportunity to increase awareness” not only of SUDEP, but also of seizure disorders in general. There are several funded studies in progress to better understand risk factors and prevention of SUDEP. Risk reduction is also key, according to Pennell. Medication adherence is critical, and considering altering medications or even considering surgical intervention should be discussed with patients whose seizures are GCTC with increasing frequency and/or severity.
Cameron Boyce’s parents recently appeared on “Good Morning America” to discuss his life, legacy, and incredible short yet meaningful life that touched so many.
His mother stated “If I ever thought he could die from a seizure I never would have let him out of my sight, which would have been awful for him.” This statement is so poignant–she wanted her son, who had been seizure-free for over a year prior to his SUDEP, to live his life with as much independence and joy as she and others knew he would.