Part 24 of our Award-Winning Series “Our Children’s Health”
By Jaimie Lazare
Seizure awareness may very well have been heightened by a series of tragic and shocking events this year. Just one day after celebrating the New Year, news headlines reported that John Travolta’s son, Jett, 16, died of a seizure while vacationing with his family in the Bahamas. Weeks later, at the Inaugural luncheon for President Obama, Senator Ted Kennedy, who is battling brain cancer, suffered from a seizure that was attributed to fatigue. And most recently, pop icon Prince revealed during an interview with PBS’s Tavis Smiley that he suffered from seizures as a child.
Despite all the media attention, there’s a lot that is still misunderstood about seizures. On March 28, 2009, the Walk for Epilepsy took place in Washington, D.C. to help raise awareness for the third most common neurological disorder in the country: epilepsy. The march demonstrated a continued need to inform people and raise awareness that seizures and epilepsy are important issues when it comes to our children’s health. According to the Epilepsy Foundation, there are 300,000 children under 14 years of age with the disorder. Contrary to the common portrayal of people with seizures—a person convulsing uncontrollably—the disorder has many different types of manifestations. And being aware of the various types helps us to be less likely to miss seizure behaviors that would have otherwise been ignored.
Shake, Rattle and Roll
Historically, people with seizures were thought to be possessed by demons or cursed by gods. Hippocrates attempted to debunk those beliefs with his book On the Sacred Disease, in which he acknowledged epilepsy as a brain disorder. Still, centuries later, people with epilepsy remain a largely misunderstood group. From ideas that those who suffer from seizures are not intellectually capable to notions that they are flat-out crazy are all wrong—and unfortunately these stigmas still exist today.
So what is epilepsy? Vijaya Atluru, M.D., chief pediatric neurologist at Winthrop University Hospital in Mineola, explains it simply: “Epilepsy is due to recurrent seizures.” And what are seizures exactly?
A seizure is a sudden burst of electrical activity that affects normal brain function. A child has to experience more than one “attack” in order to be diagnosed with an epileptic seizure disorder.
There are two types of seizures: partial and generalized seizures. These types are categorized based on whether abnormal electrical activity affects the brain partly or entirely.
Partial seizure: In this type, the brain is affected focally and is subcategorized based on whether the person is conscious during the seizure.
• Simple partial seizures do not result in a loss of consciousness. One may experience changes in his or her senses and emotions with involuntary jerking of a limb accompanied by symptoms such as tingling sensations.
• Complex partial seizures result in a loss of consciousness. During this time, the person is usually staring while engaging in some activity such as hand rubbing, chewing or twitching.
Generalized seizures: These seizures affect the entire brain, and there are four types.
• Absence seizures, also known as petit mal, result in a temporary loss of consciousness while the person is staring and their body goes through subtle movements.
• Myoclonic seizures affect the muscles causing sudden twitching or jerking movements of all limbs.
• Atonic seizures also affect the muscle, but instead of causing uncontrollable contractions, the child loses all muscle tone and suddenly collapses. This is why they’re also referred to as drop attacks.
• Tonic-clonic seizures, also called grand mal, are the most intense type, resulting in a loss of consciousness, body stiffening, uncontrollable shaking and urinary incontinence due to loss of bladder control.
Encounters of the First Kind
According to the Epilepsy Foundation, most seizure disorders begin during early childhood. This is mainly because children’s brains are immature and more susceptible to seizures.
“Neurologically impaired children” are the ones who suffer with the condition, says Winthrop’s Atluru. Factors such as head injuries, lack of oxygen during birth, or problems with the development of the brain are just some of the many risk factors for seizures in children.
Each child is affected differently, and for reasons that may or may not be clear. While some children may outgrow their seizure disorder (which was pop star Prince’s case), others may endure longer battles with epilepsy. One thing is for sure: Every parent recalls the first time they saw their child experience a seizure.
For 12-year-old Jessica Pino, of Bethpage, the seizures started after she experienced a fall in her backyard a few days shy of her 4th birthday. Though her parents did not observe any changes in Jessica’s behavior during the day, her older sister awoke to a completely different situation later that night. Jessica was found “foaming at the mouth and twitching” in her bed, recalls her mother, Marisol.
After being rushed to the hospital, Jessica underwent many tests including an electroencephalogram, or EEG, which measures and records the electrical activity of the brain and detects patterns that are indicative of epilepsy. Though no abnormal wave patterns were detected at the time, Jessica did have a family history of seizures, so without an exact diagnosis, she was started on a drug to help control her seizures. But each seizure she experienced afterward differed in frequency and type. Her family kept records detailing the type of seizure she had and how long each one lasted. Unfortunately, despite being on medication, Jessica’s seizures weren’t improving.
It wasn’t until Marisol Pino was watching a Lifetime movie about epilepsy that she learned about a unique diet that helps certain children with epilepsy.
When Seizure Meds Don’t Work
Most people with epilepsy can either become seizure-free or experience a decrease in how often and how long their seizures last with the use of a single drug. While some may struggle to find the right medication, many doctors and parents seek non-pharmacologic options. The most promising one is the ketogenic diet.
The ketogenic diet is used to treat what’s called refractory seizures, which are seizures that are not responsive to medications, according to Dr. Atluru. Children who fall into this category usually have failed to respond to two or three medications. The chance of a fourth drug working is pretty slim and other alternatives are considered.
The ketogenic diet consists of fatty foods (i.e., butter, mayonnaise, eggs, bacon, tuna in oil). It’s measured in a 4:1 ratio, meaning for every four grams of fat, patients receive one gram of carbs and one gram of protein; so, food such as bread, pasta, fruits and vegetables are strictly limited, and the person’s total calories and fluids are also restricted. At every meal, the food has to be measured with a special scale the dietician will have the child’s parent buy.
Four years after Jessica’s first seizure, the Pino family moved to Nassau County to find better care for their then-8-year-old at Schneider Children’s Hospital in New Hyde Park. There, Jessica was given the green light for the ketogenic diet after experiencing a very dangerous type of seizure called status epilepticus, in which she experienced a prolonged seizure that was uninterrupted even after her parents gave her Diastat (a rectally administered drug used to immediately stop extended seizure episodes).
“Any seizure beyond 30 minutes…that’s a neurological medical emergency,” Atluru explains.
Trina Gill, a Pittsburgh mom, can relate to the Pino family’s ordeal. She and her husband also recall the frustrations of finding the right treatment for their daughter, Lauren. Eight-year old Lauren experienced her first seizure in December, 2006.
“She had a tonic-clonic seizure in our kitchen. She fell to the floor and convulsed for about five minutes and that was our entrance into the world of epilepsy,” says Trina. In hindsight, she believes that Lauren may have been experiencing absence seizures even before this attack.
Lauren was taken to Children’s Hospital in Pittsburgh, and to Trina’s surprise, Lauren was released with a clean bill of health. After demanding Lauren be evaluated by a neurologist, the girl was eventually given a diagnosis of benign absence epilepsy. But, as Lauren’s seizure disorder progressed, it became apparent that she was having multiple types of attacks. It took seven different drugs in three months before the Gill family demanded a 24-hour EEG, which revealed that Lauren was experiencing 300 seizures a day while on medication, and she was having five different types of seizures. Shortly afterward, Lauren was started on the ketogenic diet.
Another 24-hour EEG showed that Lauren “went from having 300 seizures to four in less than three months,” Trina says. “It was pretty dramatic and it happened pretty quickly.”
Dr. Atluru says, “These [intractable or refractory seizures] do not respond well to seizure medications, and that’s when we think about the diet.”
Understanding the Ketogenic Diet
As noted earlier, the ketogenic diet is a strict regimen that’s high in fat relative to the amount of carbohydrates and protein. Although its exact mechanism is unclear, the diet helps to control seizures in some people with epilepsy. With the child carefully monitored by a medical team, a dietician works closely with the family to assure that the right amount of fat, carbohydrates and proteins are consumed to maintain the fine balance. Though a modified version of the Atkins diet is also used, the ketogenic diet strictly restricts and measures caloric, fluid and protein consumption.
The diet produces ketones: byproducts our bodies produce when we burn fat. It is an energy source for the brain. Our bodies normally use carbohydrates, which we get from foods like bread and pasta, as its energy source. But the ketogenic diet is extremely low in carbohydrates resulting in fat becoming the body’s main source of energy.
Mei-Hua Chen, a pediatric nutritionist at Schneider Children’s Hospital, says that when discussing the ketogenic diet with interested families she covers the “five Ws”: What is the ketogenic diet? Who can follow the diet? Who can prescribe or discontinue the diet? And when and where should the diet be initiated?
After a child has been admitted to the hospital to start the diet, he or she is monitored for a few days before being discharged. “Once the child is discharged, then he or she will return within two weeks,” Chen says, and then the child is periodically followed by a medical team to monitor progress.
Families must also carefully monitor children on this diet because of “cheating” or sneaking food that will disrupt the diet’s delicate balance resulting in a seizure episode. Atluru alludes to this when she says that even “half of a cookie” while on the ketogenic diet could elicit a seizure.
“I have one patient who’s 10 years old and the family has to keep the food out of reach,” says Angela Samuels, pediatric nutritionist at Morgan Stanley Children’s Hospital at New York-Presbyterian Hospital.
To minimize issues of noncompliance, Samuels suggests trying to take your child’s favorite foods to the dietician to create treats he or she will enjoy while still adhering to the guidelines of the diet. For instance, a pizza made of pepperoni, cheese and sauce without the crust. “There are recipes out there so that the child doesn’t have to feel left out,” Samuels says.
There are a lot of people taking care of children with epilepsy—when the parents are at work, the grandparents, older siblings or home-care professionals may also be involved in the child’s care. So, it’s important to assure that all caregivers understand the importance of staying on the diet. To minimize any loopholes in the diet, Samuels says, “I always try to make sure that everyone that’s taking part in the care of the child comes to the appointment to get the education also, and not just the parents.”
Though the diet offers no guarantee that a child will be seizure-free, Chen says, “Most parents [are] pleased with the improvement.” The child may experience seizures that are shorter in duration, less frequent or less intense, and there may be a decrease in the dosage or number of medications being administered. The diet works differently in each child.
Depending on the outcome of the diet, families and the medical team decide on whether to continue with it. Children who remain on the diet are followed for two years—in which they must be seizure-free—–before they can begin to be weaned off of it. Sometimes it’s decided that it’s best to keep the diet going longer than two years.
Some of the long-term effects of the diet include weight gain, constipation, dehydration, kidney stones and high cholesterol. Jessica Pino developed kidney stones during her time on the diet, but was monitored and treated. Her parents expressed the difficulties in remaining diligent (Dr. Atluru calls it “labor intensive”), but were pushed to remain strong and driven at the prospect of their daughter being seizure-free—which she has been, since 2004.
One of the major changes parents notice in their children when beginning the diet is that they become more alert and active.
“It works well if not better than medicine,” says Atluru of the diet. “These children that we use it for often tend to be sedated, lethargic, and dysfunctional, all because they are on so [many] medications.”
After the diet starts, children can begin to be weaned off their medications. As the children are taken off drugs, Atluru says, “they are more alert, their behavior improves, and they are able to function better.”
What if the Diet Isn’t For You?
For families who desire a different option when medications don’t work, there are alternative therapies.
Epilepsy surgery is an option for some people with refractory seizures. Recent studies have shown that the earlier surgery is performed, the better the outcome. When tests reveal that seizures come from well-defined areas of the brain (that won’t affect speech, language or hearing) then surgery can be considered. The results of surgery, like all epilepsy treatments, vary—there are no guarantees. Post-surgery, some children may still need medication to help prevent or minimize the frequency of future seizure episodes, while others may not.
A specific type of surgery known as vagus nerve stimulation (VNS) helps control intractable seizures by sending electrical impulses to the brain through the vagus nerve—which carries sensory information about the body to the central nervous system—through a battery-operated device that delivers short bursts of electrical impulses. It is believed that by stimulating the vagus nerve, electrical energy is discharged toward the brain, resulting in an interruption to abnormal seizure activity. This helps to reduce seizures by 30 to 40 percent. The surgery doesn’t involve the brain; rather, the device is implanted under the skin of the chest like a pacemaker. Wires attached to the device run underneath the skin to the left vagus nerve in the neck.
Lauren Gill underwent VNS surgery because her second EEG revealed that she was still experiencing “background activity.” She was still having difficulties in school and had developed autistic-like behaviors. All of these stopped when she started VNS.
“It was extremely dramatic and extremely fast and all those autistic characteristics, they were all gone,” says her mother. “She was reading again, she was counting and saying her ABCs and functioning and paying attention in the classroom.”
Lauren, like others with epilepsy, experiences auras, which is a sensation or motor phenomenon that foretells the coming of a seizure. Those people can then prepare for the seizure or activate their VNS device if they have one.
But there’s more to the device than controlling seizures. There’s a psychological aspect to it, as well—the device provides a feeling of independence and control.
Though Lauren’s last EEG in December still showed signs of seizure activity, she has come a long way from 300 seizures a day, and her parents are hopeful that her next test will come back normal.
Promoting Epilepsy Awareness
As a community educator, Janet Romeo of the Epilepsy Foundation of Long Island says, “One of the catchphrases that people use is, epilepsy is ‘not who I am, it’s what I have.’” And that motto rang true for a group of local parents who founded the organization many years ago.
In 1953, the Epilepsy Foundation of Long Island (EFLI) was organized by parents whose children were diagnosed with the disorder. They realized that there was so much misinformation circulating about epilepsy—not to mention the stigmas and myths—that the foundation would be an opportunity to create greater awareness about the disorder so that affected children wouldn’t have to feel the need to hide their condition.
Romeo says that epilepsy is “a unique condition, in that it has this added difficulty of having had the stigma attached to it and people wanting to hide it in the shadows.”
And 55 years later, the EFLI has become one of the leaders in educating the public and bringing greater awareness to the community about seizures.
Through the Foundation’s Office of Development and Community Relations, Romeo helps to educate children throughout the school system about epilepsy, because they are going to be exposed to children with the condition. It’s very likely that they may see a classmate experiencing a seizure in class. It’s important that they understand what’s going on and what to do, such as remain calm, time the seizure if possible, lay the person on a flat surface and turn his head to the side, put something soft under his head, do not restrain the person, call an ambulance, don’t attempt CPR, do not leave the person alone.
“Do not stick anything in the mouth, including the fingers. They won’t swallow their tongue,” Dr. Atluru says of what not to do.
One epilepsy awareness school program is called “Kids on the Block,” a puppet show performed in elementary schools. “We gear it toward the third grade, but it doesn’t have to be limited to the third grade,” Romeo offers. “In the skit, one of the puppets, Ricky, has a grand mal seizure and his friend Joanne is with him and she doesn’t know what to do. And when he wakes up from his seizure, he goes on to explain what she should do the next time he experiences a seizure. At the end of the performance, Ricky, the puppet, stays in character and answers audience questions.”
Other programs include “Take Charge of the Facts,” which is usually done in middle and high schools. Romeo goes into the health classes as a guest speaker, where she shows a video made by a group of teens with epilepsy demonstrating the different types of seizures, First Aid responses, and social issues faced by people with seizure disorders. The teen video focuses on the idea that epilepsy sufferers don’t want to be treated differently.
There’s also a program to help school staff recognize and learn how to respond to children experiencing seizures. Teachers and school staff are introduced to the psychosocial aspects of epilepsy and how it affects children. Last, but not least, an educational workshop is held for nurses twice a year—one in Nassau County and the other in Suffolk.
Though epilepsy affects each person differently, it undeniably changes every life it touches. With support, tailored treatment and early intervention, people with epilepsy can begin to seize back their lives.
“Lauren loves life,” says her mother. She wakes up every morning and says, ‘Today is a gift from God.’”