His iconic portrayal of J. Peterman on the NBC sitcom Seinfeld launched John O'Hurley, with his rich, baritone voice, into worldwide stardom. But this modern-day Renaissance man has made a name for himself in just about every entertainment medium: music, television, film, theater, and print.
Of the many roles O'Hurley plays, one is particularly close to his heart—that of epilepsy advocate. Growing up, O'Hurley experienced first-hand the effects of epilepsy on a family when his older sister Carol was diagnosed with the condition. Tragically, when she was only 17 years old, Carol died after a nighttime seizure—an event that changed the 16-year-old O'Hurley's life. (See box, “Sudden Unexpected Death in Epilepsy.”)
Sudden Unexpected Death in Epilepsy
Sudden unexpected death in epilepsy (SUDEP), which is sudden death with no obvious explanation in someone who has epilepsy, is extremely rare. Researchers are working hard to discover the roots of SUDEP and develop ways to prevent it.
At the 2013 Annual Meeting of the American Academy of Neurology (AAN) in March, James Tao, M.D., Ph.D., of the University of Chicago, presented new findings on SUDEP. He and his colleagues investigated whether sleeping facedown is a risk factor for SUDEP ( http://bit.ly/18SlODQ). Previous studies have suggested that difficulty breathing and abnormal heart rhythm may contribute to SUDEP risk ( cdc.gov/epilepsy/sudep).
If someone without epilepsy sleeps facedown and experiences disrupted breathing during the night, he or she will turn over to restore normal breathing. But if someone with epilepsy sleeps facedown and has a seizure, he or she may be unable to change body position (due to the unresponsiveness caused by the seizure), depriving themselves of oxygen. “For most people, once their airway is obstructed, they roll over,” Dr. Tao says. “People with epilepsy may not.”
For their study, Dr. Tao and colleagues completed an analysis of all of the SUDEP cases published in medical journals. They found that, of those 205 SUDEP cases in which body position was documented at the time of death, nearly 70 percent involved a person being facedown. The good news, he says, is that if sleep position is an important risk factor, then not sleeping on the stomach may help prevent SUDEP. “Turning the person so he or she can breathe normally may also save a life,” he adds. A seizure alarming device can be used to alert family members or doctors that the person is having a seizure. (More information on seizure devices can be found at epilepsy.com/epilepsy/devices_links.)
Whether or not to discuss SUDEP with epilepsy patients has been a controversial topic for neurologists. “Do I bring it up with every patient? No. But if they're at high-risk, I do,” says James Wheless, M.D., of Le Bonheur Children's Hospital in Memphis, TN. “Those patients who are still having uncontrolled convulsive seizures, especially in their sleep, are at highest risk. If, on top of that, the child, adolescent, or adult has another neurologic condition that affects their functioning (such as a prior closed head injury, meningitis, or cerebral palsy) then they are at even higher risk for SUDEP.”
To lower that risk, it is vital to find a medication or treatment strategy that controls convulsive seizures—especially during sleep, according to Dr. Wheless. “It isn't enough to just control daytime seizures,” he says, emphasizing that people with epilepsy should take their medicine regularly and as prescribed. “Make sure to have a conversation with your doctor about what to do if you miss a dose and how to make it up in a way that lowers your risk of having a convulsive seizure in your sleep,” he says. (For more Neurology Now coverage of SUDEP, go to http://bit.ly/i9I8wt.)
Epilepsy is a condition of the central nervous system characterized by seizures, which occur when nerve cell activity in the brain is disturbed. “The easiest way to describe a seizure is as an electrical storm in the brain,” says James W. Wheless, M.D., professor and chief of the department of pediatric neurology, Le Bonheur Chair in pediatric neurology at the University of Tennessee Health Science Center, director of the Le Bonheur Comprehensive Epilepsy Program at Le Bonheur Children's Hospital in Memphis, and Fellow of the American Academy of Neurology (AAN). “A seizure stimulates brain tissue, which results in certain outward symptoms—for example, if areas of the brain responsible for movement are stimulated, the person will have a convulsive seizure, in which part or all of the body jerks. A seizure also short-circuits normal brain activity, which is one reason many people are not conscious or aware of what's going on during the seizure,” he explains. (See box, “Epilepsy: The Basics.”)
Epilepsy: The Basics
What is epilepsy? Epilepsy is a chronic, noncommunicable (non-infectious) neurologic condition defined by two or more unprovoked seizures. Provoked seizures result from some immediately recognizable stimulus (for example, low blood sugar in people with diabetes), while unprovoked seizures have no immediately recognizable cause. A seizure involves abnormal electrical discharges in the brain.
What are the symptoms of epilepsy? Seizure symptoms can include temporary loss of awareness or consciousness and disturbances of movement, sensation (including vision, hearing, and taste), mental function, or mood (such as anxiety and depression).
How common is epilepsy? Epilepsy affects approximately 1 percent of the general population. In the United States, an estimated 2.2 million people have epilepsy. Although people of any age can develop epilepsy, it most commonly affects the very young and the very old.
What causes epilepsy? Common causes include:
- ▸ Stroke or transient ischemic attack
- ▸ Dementia, such as caused by Alzheimer's disease
- ▸ Traumatic brain injury, including injuries during birth
- ▸ Infections, including brain abscess, meningitis, encephalitis, and Acquired Immune Deficiency Syndrome (AIDS)
- ▸ Developmental and metabolic disorders, including cerebral palsy, neurofibromatosis, tuberous sclerosis, and autism
- ▸ Brain tumor
- ▸ Abnormal blood vessels in the brain
- ▸ Genetic abnormalities
“About 30 percent to 40 percent of epilepsy is caused by genetic predisposition,” says Dr. Tao. “These patients have mutations in specific genes. Often, these are genes that control the excitability of nerve cells (neurons) in the brain.”
Some types of epilepsy run in families. If you have generalized epilepsy, your first-degree relatives (parents, siblings, and children) have about a four-fold increased risk for epilepsy. First-degree relatives of people with partial seizures have twice the risk of developing epilepsy as the general population. Although there is some increased risk, it is important to remember that the overall risk of epilepsy in other family members is still low.
Despite what happened to his sister, O'Hurley says people with epilepsy and their families must remember that “a diagnosis of epilepsy is not a death knell. Epilepsy is a very manageable disease.” Through his advocacy work, O'Hurley has worked hard to spread this message.
MAKING THE DIAGNOSIS
A diagnosis of epilepsy is based primarily on clinical symptoms, says James Tao, M.D., Ph.D., assistant professor and director of the electroencephalography (EEG) lab at the University of Chicago. Clinical symptoms are those that the patient, family, and doctor can observe—in other words, events and behaviors that are typical for seizures. An EEG, which detects any abnormalities in the electrical activity of the brain, may be conducted to confirm the clinical diagnosis. Magnetic resonance imaging (MRI) may also be used to rule out or identify any brain injury or tumor that could be causing the seizures.
Tonic-clonic seizures, which involve full-body convulsions, are easily noticed and “can be frightening to someone who has never witnessed one,” says Dr. Tao. (See box, “Seizure Types,” in Resource Central, page 45.) More subtle symptoms of epilepsy, however, may be missed by family members or even patients themselves, he says. “Those symptoms can include fainting spells, staring spells, or very brief confusion. The person having the seizure may feel something is wrong but not realize it's seizure-related. Sometimes it isn't until a person has had a full-blown, generalized, convulsive seizure, that he or she begins to suspect epilepsy,” Dr. Tao notes.
Seizures are divided into two major groups: primary generalized seizures and partial seizures. Each group contains several seizure types, often defined by whether the individual remains conscious or not (as well as the degree).
PRIMARY GENERALIZED SEIZURES usually begin with a widespread electrical discharge that involves both sides of the brain at the same time. Types of primary generalized seizures include:
- ▸ Absence seizures, which are brief episodes of staring during which awareness and responsiveness are impaired.
- ▸ Atypical absence seizures, which are periods of staring during which the individual is somewhat responsive.
- ▸ Myoclonic seizures, which are brief, shock-like jerks of a muscle or a group of muscles.
- ▸ Atonic seizures, during which muscles suddenly lose strength. For example, eyelids may droop, the head may nod, and the person may drop things and fall to the ground.
- ▸ Tonic seizures, during which muscle tone is greatly increased and the body, arms, or legs make sudden stiffening movements. People usually remain conscious during tonic seizures.
- ▸ Clonic seizures, which involve rhythmic jerking movements of the arms and legs, sometimes on both sides of the body. Tiredness or confusion does not usually follow these seizures.
- ▸ Tonic-clonic seizures, also known as grand mal seizures, involve the entire body and are what most people think of when they hear the word “seizure.” People often lose consciousness during tonic-clonic seizures.
PARTIAL SEIZURES begin with an electrical discharge in one limited area of the brain. They can have many different causes, including traumatic brain injury (TBI), brain infection, stroke, tumor, or changes in the way an area of the brain was formed before birth. Types of partial seizure include:
- ▸ Simple partial seizures are localized to one area on one side of the brain, but may spread from there; consciousness remains intact.
- ▸ Complex partial seizures can begin in any lobe of the brain, but cause altered awareness due to spreading of seizure activity.
- ▸ Secondarily generalized seizures begin in one part of the brain, and then spread to involve both sides of the brain with associated loss of consciousness.
O'Hurley's sister had experienced a series of early fainting spells when she was in the second grade. However, fainting can happen frequently for other reasons, such as low blood pressure or skipping breakfast. “We didn't immediately know it was anything more than that,” O'Hurley says. Finally, in eighth grade, after Carol had experienced several more fainting spells, she was diagnosed with epilepsy.
Their father, a surgeon, and mother, a nurse, made sure their five children were well-informed about Carol's condition. They were “very careful to explain epilepsy to all of us,” O'Hurley recalls. He witnessed several of his sister's seizures, and admits that they were extremely upsetting at first. “But then I realized it was my job to help her rather than be reactive to her condition,” he says. During the 1960s, Carol had a normal life, O'Hurley says, including an active social life. “When she dated boys, my father would sit them down and say, ‘Here's the situation with my daughter. If you want to be with her, this is what might happen,’” he recalls.
But in 1970, the night after O'Hurley's sister Carol completed the school year, she experienced a seizure while lying face-down in bed. She did not wake up the next morning. “She was 17, I was 16; we were thick as thieves back then. Extraordinarily close,” he recalls. Her death caused an enormous shift in the family. “It was just a deep wound—something was missing and it would never be there again. It was incredibly hard on my parents,” says O'Hurley.
TURNING TRAGEDY TO ADVOCACY
Even as O'Hurley began to gain recognition as an actor and performer, he never lost sight of the pain of losing his sister to neurologic disease. “It's something that has always stayed with me. As I've grown in my entertainment career, I have always felt that if I could lend a voice to educating others about epilepsy, I would,” he tells Neurology Now.
During the time when he was starring in Seinfeld, O'Hurley remembers that there was already mounting public interest in neurologic disease. The actor decided to use his celebrity to honor his sister's memory. He would advocate on behalf of people with epilepsy and their families by teaching the public about epilepsy.
Most recently, O'Hurley teamed up with the AAN and the American Brain Foundation to create a patient-education video on epilepsy ( aan.com/view/PatientVideos). In the video, O'Hurley, neurologists, and people with epilepsy discuss how their lives have been affected by the condition. “It's a general information project that's being used not only in the medical and professional community, but also for people out there who may be experiencing epilepsy in their family for the first time. It's for patients as well as parents and caregivers. I think it's a wonderful piece of education,” he says.
O'Hurley has also participated in a partnership initiative with several chapters of the American Epilepsy Society and funded a day camp in San Diego for children with epilepsy. The camp, he says, “allows children with epilepsy to talk to one another and share their experiences so that they don't feel alone.” O'Hurley also worked on a nationwide education campaign, visiting cities with a medical professional and appearing on radio and television to teach the public about epilepsy. One of the key messages was combatting stigma.
“Stigma continues to be an enormous problem, especially for a child who is going through school,” O'Hurley says. “Children can be cruel and candid.” His own sister was lucky, he remembers. She “was such a popular person that I don't think [her epilepsy] made anyone think differently of her.” Being raised in a medical family, he suggests, was “helpful for all of us in that we all were able to realize and understand that epilepsy was just something she happened to have”—that it didn't define her and it was also nothing shameful.
“As caregivers, I think it's important to make sure that the people around the person with the disorder know exactly what might happen,” O'Hurley says. For example, he says, a child's teacher should know and be able to educate other students in the class about epilepsy. “The manifestations of a grand mal seizure can be traumatic for the people that witness them. I think that's one reason children with epilepsy grow up feeling a sense of stigma,” O'Hurley speculates.
“Epilepsy still carries a tremendous amount of stigma,” Dr. Wheless adds. “If you talk to people with epilepsy—even if it is well-controlled—most of them are convinced that people can tell they have epilepsy just by looking at them.”
How can stigma be reduced? As with any disease, Dr. Wheless says, more people need to understand the real facts. “The only way to debunk them is to get the facts out there,” he says. “For example, most people still think you can swallow your tongue during a seizure. That's physiologically impossible—it's attached to the back of your throat!”
Advocates like O'Hurley can go a long way in debunking these myths. “Celebrity spokespeople for epilepsy are extremely hard to find,” Dr. Wheless says. “There's enough stigma with the disease that people don't want others to know or even think they have it.”
Since the 1960s, when Carol O'Hurley was first diagnosed with epilepsy, tremendous progress has been made in treatment and research for the condition. “So many drugs are available now that my sister did not have access to in 1970—and so many other therapeutic options.” Specifically, O'Hurley tells Neurology Now, the “haunting” side effect profiles have greatly improved.
The number of available antiepileptic drugs (AEDs) has increased from a handful in the early 1980s to more than 20 today, according to Dr. Tao. “The new generation of AEDs are not necessarily more effective than the old medications (phenytoin, phenobarbital, carbamazepine, valproic acid), but they are a lot more tolerable in terms of side effects,” says Dr. Tao. With new choices, far more opportunity exists for patients to find the right medication. Unfortunately, none of them so far are disease-modifying treatments. In other words, they control the symptoms of epilepsy but do not cure it. In addition, in approximately 30 percent of patients, seizures cannot be adequately controlled with medications. For these people, surgery can be an effective option.
Dr. Wheless explains that four different approaches for controlling seizures are available: drug therapy, which is the most commonly used treatment; dietary therapy, such as the ketogenic diet, which is primarily used in young children because it can be very difficult to maintain (for a full description, see http://bit.ly/e3EehH); neurostimulation devices, such as a vagus nerve stimulator (see http://bit.ly/12LeWHb); and epilepsy surgery to remove or disconnect the area of the brain causing the seizures (see http://bit.ly/1abih2k). Each of these therapies has its own unique set of risks and the benefits, so it is important to discuss the options carefully with a neurologist.
Through his experience with his sister and his work as a patient advocate, O'Hurley has learned that people with epilepsy must educate themselves about the condition and follow doctor's orders—for example, by taking medication as prescribed. “If the medications you're taking don't control your seizures or if the side effects are too severe, you have to go back to the doctor and work together,” he says.
A RENAISSANCE MAN
As O'Hurley continues to take on new and exciting projects, starring in Seinfeld is still his favorite performance to date. “It's rare that you get to play a role that was so…iconic—the little gem that the character Peterman was. He was an example of some of the best writing at the best time on television, and I miss it dearly.”
In 2005, O'Hurley and his dance partner made it to the ultimate round of the very first season of Dancing with the Stars. “We were all very innocent, just trying not to fall over,” he recalls, admitting that he had never really danced before appearing on the show. “There was no need for me to unlearn anything—I just didn't know anything!”
Among his numerous accomplishments, O'Hurley became a crowd favorite as host of Family Feud (2006 to 2010). He continues to perform recurring roles in Broadway productions Chicago and Spamalot. Most recently, O'Hurley cofounded a renewable energy company, Energy-Inc, which converts waste into usable energy.
O'Hurley makes efforts to keep “as many plates spinning” at one time as possible—and as an advocate, father, actor, Broadway performer, host, golfer, author, composer, business man, and motivational speaker, it's safe to say he is achieving his goal. Many of the formative influences in his life, he says, had to do with “the power of positive thinking, motivation, and personal responsibility”—all of which O'Hurley has channeled into his career and his efforts for epilepsy awareness.
FOR MORE INFORMATION
- ▸ Epilepsy Foundation: epilepsyfoundation.org
- ▸ Epilepsy Therapy Project: epilepsy.com
- ▸ Mayo Clinic: mayoclinic.com/health/epilepsy/DS00342
- ▸ National Institutes of Health: ncbi.nlm.nih.gov/pubmedhealth/PMH0001714
- ▸ National Institute of Neurological Disorders and Stroke: ninds.nih.gov/disorders/epilepsy/epilepsy.htm
- ▸ SUDEP Aware: www.sudepaware.org