While the traditional drugs used to treat epilepsy were once limited and carried their share of side effects, the team at The Epilepsy Center at St. Joseph’s Children’s Hospital is extremely encouraged by the vast arsenal of effective medications and other treatments now available. Photo courtesy of Getty Images

Following the recent death of Disney star Cameron Boyce from epilepsy, an expert at The Epilepsy Center at St. Joseph’s Children’s Hospital sheds light on this manageable and often misunderstood condition.

On July 6, actor Cameron Boyce, 20, well known for his roles on the popular Disney Channel shows “Descendants” and “Jessie,” died in his sleep after suffering an epileptic seizure. Though additional tests remain ongoing, many medical experts speculate that his death was likely caused by a circumstance known as “SUDEP,” or “sudden unexpected death in epilepsy.”

While numerous relatives, friends, fans and celebrities, including his fellow cast members and former first lady Michelle Obama, expressed their heartbreak over Boyce’s loss, the young star’s passage also has left many clamoring for more information about epilepsy, a condition that’s been met with mystery, confusion and stigma for centuries.

Wenlang Xia, MD, medical director of the Pediatric Epilepsy Center at St. Joseph’s Children’s Hospital

A Neurological Condition

Affecting an estimated 1 to 2 percent of the general population but seen a bit more frequently in children, “epilepsy is a neurological condition manifested in repeated seizures caused by abnormal electrical activity in the brain,” said Wenlang Xia, MD, medical director of the Pediatric Epilepsy Center at St. Joseph’s Children’s Hospital in Paterson. “It can affect all age groups and tends to be most prevalent among very young children and the elderly, but the earlier in age the onset occurs, the worse the prognosis may be.”

While many people associate epilepsy with the most obvious form of seizures — known as grand mal seizures, during which patients experience a complete loss of consciousness and violent muscle contractions — epileptic seizures can take many forms.  These include everything from myoclonic seizures, or quick jerks of the body or arms or legs, and simple partial seizures, in which patients experience disturbances to their coordination, sensation, or motor activities but don’t lose awareness, to partial complex seizures, which impair awareness. Petit mal or absence seizures, which are often seen in children ages 5 to 12 as brief staring moments, also are possible.

According to Dr. Xia, “There are as many as 20 to 30 different types of seizures that can be experienced, including twitching/tics, staring, confusion and other behaviors that many kids or parents might not even be aware of.”  Seizures can be of varying severity and duration, “but because they’re ultimately all controlled by electrical signal changes from neurons which we can actually see in the form of brain waves in our EEG lab, epilepsy used to be known as the ‘brain storm’ disease,” he said. “Because seizures can beget seizures, it’s important to catch and control them early, because they can get worse over time and increase one’s chance of dying from them if they remain uncontrolled.”

While most people with epilepsy don’t die from seizures, the Centers for Disease Control estimates that some 2,600 people die from SUDEP annually, though the circumstances of this condition remain largely unexplained. “We know that kids and particularly males who are having intractable or uncontrolled convulsive seizures are the most vulnerable to SUDEP,” noted Dr. Xia, who added that additional factors such as the presence of other neurological disorders, heart conditions, the use of drugs or alcohol, or sleep deprivation can heighten the risk of SUDEP among these patients.

The fallout of epilepsy can extend far beyond the physical, especially for pediatric patients desperate to fit in with their peers, Dr. Xia said. “Because they’re unpredictable and you don’t know when they’ll happen, epileptic seizures are socially embarrassing and can cause patients a lot of apprehension, which can lead to anxiety, depression and isolation,” he said. “There can be a huge stigma, even for kids who are doing well.”

At. St. Joseph’s Children’s Hospital, pediatric epilepsy experts have access to drug development,  epilepsy care and resources more advanced than ever. Photo courtesy of Getty Images

An Arsenal of Effective Treatments

While the traditional drugs used to treat epilepsy (including Phenobarbital, Dilantin, or Tegretol) were once limited and carried their share of side effects, Dr. Xia is extremely encouraged by the vast arsenal of effective medications and other treatments now available.

“While we don’t have a perfect solution for this condition, we do have at least a dozen new medications for patients to choose from which have proven effective in controlling seizures in 65 to 70 percent of cases,” Dr. Xia said. “For those patients whose seizures remain uncontrolled or who can’t tolerate the side effects, we also have several non-pharmacological or alternative treatments to offer, including Vagal Nerve Stimulation, through which a small chip implanted in the left side of the chest massages the cranial (vagus) nerve to control seizures,” he said. “There’s also epilepsy surgery, through which we’ll identify where seizures are coming from and surgically remove the seizure-causing area. And adherence to a high-fat, low-sugar ketogenic diet has been found to help control seizures in a number of patients as well.”

Dr. Xia shared the following top tips for those with pediatric epilepsy:

• Take it seriously – “Epileptic seizures can lead to falls, accidents and developmental delays/cognitive deficiencies if they’re uncontrolled/untreated,” he said. “And in extremely rare cases – an estimated one in 1,000 – seizures can lead to sudden unexpected death in epilepsy (SUDEP).”
• It can be well-managed – “The good news is, for the majority of pediatric cases, kids can do quite well,” Dr. Xia said. “If they take their medication as prescribed, engage in physical activity, sleep regular hours, control their diet and reduce stress, they can control their seizures, go on to perform extremely well in school and college and do all of the things normal kids do.” He also noted that SUDEP is occurring much less in children — in the range of two deaths per 10,00 children per year, and even less if they have fewer or shorter grand mal seizures.
• Take a holistic approach to treatment – Dr. Xia recommends that pediatric epilepsy patients avail themselves of the benefits of the broad spectrum of care. “At the Pediatric Epilepsy Center at St. Joseph’s Children’s Hospital, we have a full range of seasoned specialists available to treat this condition, from neurologists, epileptologists, psychiatrists, therapists/psychologists, neurosurgeons and dietitians,” he said. “Overall, we have a greater societal awareness of pediatric epilepsy today, and drug development, epilepsy care and resources are more advanced than ever before,” Dr. Xia concluded. “While it’s a neurological condition, epilepsy is fully manageable with the appropriate treatment and the vast majority of pediatric patients will be fine.”

For More Information:

St. Joseph’s Health, a world-class hospital and health care network supported by leading and renowned physicians, nurses and care teams, operates a full continuum of care, including a regional tertiary care medical center, a state-designated children’s hospital, an acute care hospital, rehabilitation and long-term facilities, and comprehensive home care. Providing advanced care for children from birth to 21 years old, The Epilepsy Center at St. Joseph’s Children’s Hospital specializes in the diagnosis, evaluation and treatment of children and infants with seizures and epilepsy. Computer-assisted, long-term video-electroencephalography (EEG) monitoring and state-of-the-art technology allows accurate diagnosis of many types of epilepsy and related conditions in children. Depending on the frequency of seizure activity, adequate data can be obtained from recordings ranging from three hours to five days.

The center also performs Ambulatory EEG’s, which allows extended recording in a patient’s home, and its specially trained Pediatric Epileptologists offer the latest personalized, comprehensive medical and surgical treatment plans that best control seizures with the fewest possible side effects for each child with epilepsy. The center’s multidisciplinary team also offers Vagal Nerve Stimulation, epilepsy surgery and a Ketogenic Nutrition Treatment Program. For additional information or to make an appointment, call (973) 754-2528 or click here.