Options in seizure management: the vagus nerve stimulator--experiences to date: Part 2: addressing challenges to VNS use and quality of life issues. (Special Report).
ISSUES OF DRUG THERAPY
The first line of treatment for seizures is usually drug therapy. Individual metabolic rates differ, breaking down drugs at varying rates, therefore requiring different dosages, and achieving different results. Drugs can be used alone (monotherapy) or in combination with other drugs (polytherapy). Although most people with epilepsy respond to a single drug the physician may try several drugs before finding the right drug or combination of drugs.
One challenge with seizure drug therapy is compliance. The individual must take medication on time, every day to maintain a steady level of the drug in the system. The success of a particular drug may depend on it being taken regularly, with no missed doses. When a treatment regimen is not followed, there is an increased chance that seizures will return.
Something else to consider is the side effect profile--this applies to all therapies, not only drugs. All medications cause side effects, whether they are perceptible or not. Some side effects (drowsiness or nausea) occur as the body adjusts to the presence of the drug, and may lessen with time. Other side effects can occur because one drug is affecting the action of another drug. There is also the chance that a medication may accumulate in the body and cause toxicity as a result. Signs of toxicity can include sluggishness, loss of coordination, slurred speech, and sometimes, an increase in seizures.
If an individual is taking multiple drugs and is either still experiencing seizures or is having adverse effects that interfere with daily functioning, the physician may consider a different approach.
When medication is not effective in controlling seizures, or if side effects are an issue, surgery may be considered. Brain surgery usually involves either removing the area of the brain that is causing the seizures, or interrupting connections between the area of the brain from which seizures originate and the rest of the brain. Although brain surgery can be performed on children and adults alike, not everyone with epilepsy can be helped with surgery. There are a number of criteria that must be met before surgery is considered. Physicians must determine: if the kind of seizures can be helped by surgery; that drugs and any other avenues have been tried and failed; that the condition may improve, and not worsen, with surgery; that surgery can be performed safely; and that the benefits of surgery outweigh the risks inherent with this type of procedure.
Risks involved with surgery are dependent on the type of surgery and the area of the brain where the surgery is performed. Side effects can range from barely noticeable to devastating, risks commonly include infection and bleeding.
The cost of surgery can vary from between $35,000 to $150,000 depending on the procedure.
MAKING TREATMENT DECISIONS
Dr. Angus A. Wilfong, assistant professor of pediatrics and neurology at Baylor College of Medicine and medical director of the Clinical Epilepsy Program at Texas Children's Hospital, in Houston, discussed his procedure for deciding on a course of treatment. "Whether it's surgery, the vagus nerve stimulator, the ketogenic diet, or drug therapy, you must remember that although all of these methods have been proven effective, it's hard to predict efficacy in an individual patient. So the efficacy issue is as big a consideration as the side effect profile of a therapy."
Cost and risk are also a part of the decision-making process. Logically, if a therapy is effective and avoids side effects, it will be cost-saving, as opposed to a therapy that is not effective, is not well tolerated, or has dangerous side effects. Dr. Wilfong explains: "Clearly, epilepsy surgery or the vagus nerve stimulator is expensive up front. But if the recipient is a good responder, it has a long-term pay off. If you can eliminate or dramatically reduce seizures, by whatever means, regardless of cost, it will be a tremendous life-long cost savings."
Dr. Paul Levisohn, assistant professor of pediatrics and neurology at the University of Colorado Health Sciences Center, and medical director of the Children's Epilepsy Center at the Children's Hospital in Denver, Colorado, has found that in people who are very resistant to medical treatment, some will eventually achieve seizure control using standard therapy with either single or combination medications, but many of them will not. "For that group," he says, "I believe we need to broaden our thinking. We can consider alternative therapies, in other words, surgery, the ketogenic diet, or the vagus nerve stimulator. The vagus nerve stimulator is not a last resort in these individuals. It is one of three options to consider after drugs have failed."
According to Dr. Wilfong, "About 20 percent of children with refractory seizures have syndromes amenable to surgery. Seventy to 80 percent are not surgery-amenable. These are the candidates for the vagus nerve stimulator."
The vagus nerve stimulator (VNS) is the most recent development in seizure management. As an implantable device that requires minor surgery, the risks are the same as those for any type of surgery, mainly infection. The side effects, once the generator is activated, may include hoarseness and a tickling in the throat when the device is in the active cycle.
VNS can stop or reduce the frequency of seizures on its own, or if additional stimulation is needed, the VNS magnet can be used. The magnet is one of the most important features of VNS. It is a component of the VNS system, and can be worn on the wrist or clipped to the belt. Magnets can be given to caregivers, teachers, and anyone else who has contact with the individual.
When placed or "swiped" over the area of the chest where the VNS generator is implanted, the magnet activates the system to deliver an additional pulse to the vagus nerve, which can either stop or shorten a seizure, and reduce the time it takes to recover afterward--the "post ictal" stage. This gives people control over the device and how it operates. Individuals can use the magnet if they are able to sense that a seizure is about to occur. Caregivers or friends can be shown how to use the magnet for the individual in the event of a seizure.
The magnet can give people with epilepsy control over their seizures. Dr. Wilfong illustrates: "For someone who has warning signs (called an aura), that a seizure is about to occur, all he or she knows is that a seizure is about to start, that he or she will fall down, have a convulsion, and possibly lose bladder control. Imagine how terrible it must be during those few seconds between the aura and the seizure. What must those seconds feel like? Now, imagine you have the VNS. You feel an aura, you reach into your pocket take out the magnet, pass it in front of your chest, and 75 percent of the time, the seizure stops. For the first time you have something you can do to control epilepsy, which before was always in control. It is so empowering."
There are advantages to VNS in all populations
* Proven efficacy
* Positive effect on quality of life
* 100% compliance
* No cognitive side effects
* No medication interactions
* No systemic neurotoxic side effects
If for some reason VNS is not working, it can be removed or the generator can be deactivated. It isn't irreversible.
WHY ARE SOME PHYSICIANS HESITANT TO USE VNS?
Many physicians are not quick to accept innovative therapies. They often have their own proven treatment methods and may not always be receptive to something completely new. Dr. Wilfong observes, "People tend to be suspicious of new things, and sometimes the attitude is, 'even though the published scientific studies have shown the medication or procedure is effective, that doesn't mean it's going to be effective for my practice.'"
Regarding new therapies, there is always the question: Does it really work? In the recent past, the neurology community has weathered negative experiences surrounding new treatments--specifically, the approval of new drugs for epilepsy that either were ineffective, or had to be removed from the market because of severe, potentially fatal side effects. This may have contributed to the "once-bitten twice-shy" attitude many physicians now have toward new treatments.
Unlike cardiologists, who have long had access to pacemakers and heart valves, devices have not traditionally been part of neurology's therapeutic arsenal, until the development of VNS. The fact that VNS uses computer-based technology has been another barrier to its applied use.
VNS involves surgery and that, according to Dr. Wilfong, "turns a lot of physicians off, even though it's straightforward, incredibly low-risk surgery."
Dr. Syed Hosain, assistant professor of pediatrics and neurology at Weill Cornell Medical College in New York City and director of the Pediatric Epilepsy Program, agrees. "Most people think the VNS surgery is invasive, which I think is a misconception. The surgery is simple and does not take more than an hour. At our institution it is an outpatient procedure, and is very well tolerated even by very young children."
The surgery involved with VNS poses additional challenges. Dr. Wilfong explains, "If someone is on a drug and they don't like it, or if they are having an allergic reaction, you stop the drug, and that's it. If the VNS doesn't work, or the person doesn't like it, it's still in their body--if they want it removed, they have to have more surgery."
Dr. Wilfong talked about his introduction to VNS: "The reason why I was interested in exploring VNS was that I had a lot of patients who had severe epilepsy, whose seizures weren't controlled with drugs and who were not candidates for surgery. They had tried the ketogentic diet, many medications, and at that point I was open to considering any kind of therapy that might benefit these children."
With time, Dr. Wilfong observed that children who had been having hundreds of seizures a day were showing dramatic improvement with VNS. He says, "It didn't take long for me to realize that this was going to be a safe, effective therapy."
Dr. Levisohn's first experience with VNS was when he was working with an adult epileptologist who was involved in the initial VNS studies. The first experience with VNS was not positive, Dr. Levisohn recalls. "The patient did not like it and asked for it to be removed."
In 1996, Dr. Levisohn began hearing more about VNS and applied the treatment protocol that he uses with medications: "I picked a couple of the really difficult patients for whom I felt this was a reasonable alternative." The children had significant developmental issues, with neurobehavioral problems. Dr. Levisohn observed that neither of them had tolerated medications well, and taking multiple drugs resulted in deterioration in their already difficult behavior. "They needed something different that what was readily available. So we implanted them with the VNS and both these kids did very nicely. They didn't become seizure-free, but were better managed with a single drug each, and with no behavioral difficulties."
Dr. Levisohn says, "Since then, my thinking has evolved to the concept of aiming at a single drug plus VNS as a reasonable adjunctive therapy."
A PHYSICIAN'S PERSONAL EXPERIENCE WITH VNS
Dr. Keith Holan is an internist in Ohio, who had the VNS implanted in February 2000, when he was in his fifties.
He began having seizures as a teenager after having meningitis that required neurosurgery. He had been on many different medications through the years. In 1999, Dr. Holan's seizures were no longer controlled with drugs and it was at this time that he first heard about VNS.
After surgery, when the generator was turned on, Dr. Holan said that it took a while for the device to have an effect. "Initially, the seizures were controlled only slightly better than prior to the surgery, but since July 2000, I've been seizure free, which is obviously a lot better than I had ever done with any medication."
He remembers, "Before surgery, I was having a seizure every two to three weeks. I had mostly complex partial seizures, but once in a while, I was having generalized seizures where I lost consiousness. That particular part of my seizure disorder was under control for a number of years. When I started having generalized seizures again, I felt the problem was getting out of control and that was what led me to seek a neurologist and get VNS."
Unlike many people with the VNS implant, Dr. Holan has no sensations or feelings when the generator switches on. He says, "My generator goes on every seven seconds and stops for 12 seconds. In between, if I choose to activate it, or feel a seizure coming on, I use the magnet. I have only used the magnet a couple dozen times, during the first few months after surgery. Once or twice it completely aborted a seizure."
Dr. Holan says that the VNS has had a big impact on his quality of life, and on his life in general. Unable to practice medicine when his seizure disorder was not controlled, he is ready to start seeing patients again.
SUCCESS AND ACCEPTANCE
VNS has great potential both in seizure control and quality of life issues. It is a new and unique treatment method, and its success will be the best advocate for its acceptance by the mainstream medical community. Dr. Levisohn states, "As with any new therapy, new ideas get accepted when the conservative thought leaders finally take on the method and adopt their practice to it and use it."
In Part 3, the long-term safety and efficacy of VNS will be explored, as well as its use as a treatment for depression in individuals without seizure disorders, which is currently the focus of a double-blind, multicenter study in the US.
TIPS ON HANDLING THE MAGNET
* The magnet should be kept at least 10 inches away from credit cards, televisions, computers, computer disks, microwave ovens, or other magnets.
* The magnet can break if it hits a hard surface, and care should be taken not to drop it.
* The magnet should be carried with the person who had the VNS implant. Family members should be shown how to use it.
RELATED ARTICLE: Forced to fight.
Dominique Bacci, 7, lives in Florida, with her parents, Roy and Kelly, and her brother, Justin, 4. Dominique began having seizures (about 12 a week) at 2 years of age, and had undergone two brain surgeries, the ketogenic diet, and nearly every medication, all with no effect.
She was scheduled to have the VNS implanted last July (2000). Everything was in order, when the Baccis were told that Medicaid funding for VNS had been vetoed by the Governor.
Kelly remembers, "I was told I would have to wait until next year. Dominique had been suffering for five years. She was losing skills, and her IQ was dropping. She missed 70 percent of school the previous year because of seizures."
She contacted the St. Petersburg Times, their local newspaper, which had run several stories on epilepsy in children. The newspaper printed a story about how the state wouldn't cover seizure treatment with VNS. The response from the general public was tremendous, with people writing the newspaper and governor's office.
Kelly, who had still not gotten an answer to why funding for VNS had been cut, e-mailed the Governor one last time. She set a deadline for Friday of that week to hear from the Governor's office before she contacted the television networks.
That Friday, Kelly got a call from the Governor's office saying that Dominique's VNS would be covered and that she could go ahead and schedule the surgery.
This response, however, did not address the other families in Florida who were waiting to get VNS for their children. Kelly says she's no hero, but adds, "I had a lot of people behind me who had fought for this with me, whose children were waiting for the same surgery. What about all of the other kids? There are a lot of people in the state who need VNS." Three days later a bill was passed allotting government money for VNS.
Dominique got the implant in September 2000, at age 6. Kelly says that before VNS, all Dominique would do was lay in bed. She never interacted, never talked, and wouldn't look at people. Now, says Kelly, "The changes in her personality are amazing. Before VNS she had about 12 seizures a week. Now, she has about six per month."
In response to professionals who, where VNS is concerned, have expressed more interest in numbers than quality of life, Kelly is vehement: "Quality of life matters to parents who don't want to see their children suffer anymore. I didn't want to see my daughter laying in bed and never going outside to play with the kids across the street because she didn't feel well. If we still have to deal with some of the seizures but we see our kids having a life--getting up and playing, learning, even able to remember someone they met the day before, it's not just the numbers. My daughter feels empowered and she can do things better. She has control over something she never had control over before."
RELATED ARTICLE: What does it take to convince physicians?
When he was 3 years old, Brady Thompson, of Utah, began having severe seizures--as many as 500 each day. His physicians tried every medication in every combination, to no avail.
They wanted to perform a corpus callosotomy, where the bundle of nerve fibers connecting the brain's hemispheres are partially or completely severed. Brady's parents, Lori and Craig, had just learned about VNS, and wanted to try implantation first.
The doctors in Utah were extremely reluctant to try VNS, and the Thompsons had to fight to get them to perform the procedure. "Even on the morning of the surgery," Lori remembers, "They were still trying to get us to change our minds."
Brady's seizures were so severe and out of control, and he was suffering so terribly that his parents were prepared to take him off all of his medication, regardless of the consequences. Lori said, "It would take him over 12 tries to get one spoonful of cereal from a bowl to his mouth because his seizures wouldn't stop long enough."
Brady underwent VNS surgery at the age of 7. During the first week after the VNS generator was turned on the number of seizures decreased to 70 in one week. In addition, Lori says, "With the magnet I could abort any seizure immediately. It could be right in the middle of a grand mal, I would use the magnet and it would instantly stop--he would come out of it. There was no post ictal time."
Brady's doctors did not understand why it worked. When Brady had the VNS surgery, Lori recalls, the physicians' attitudes were: "We'll let you have this done because your child is close to death anyway, but it isn't going to work."
On the other hand, she notes, "If the corpus callosotomy didn't work, I couldn't say 'I'm sorry, let's put it back the way it was.' With VNS, if it didn't work, it could be taken out or turned off."
Brady, who is now 9, has no memory of the four years during which he was having seizures. He has just completed third grade and is finishing a second grade level of work. Lori says, "He's excited about life, he has energy and wants to do everything he can. I think it's because of what he went through."
Before VNS, Lori and Craig were told that they would have to think about putting Brady in full head gear and a wheelchair permanently. Instead, Brady is a typical, active boy. He plays basketball and football and enjoys skiing.
"Our reduction in seizures is 99 percent," said Lori. "He might have seizures, but with the magnet they stop instantly. He used to have drop attacks, focal seizures, grand mals, and complex partials. Now we have maybe a 1- to 2-second-long grand mal every four months. The last one he had was six months ago (in January 2001)."
The effectiveness of VNS has increased with time, notes Lori. "With any medication we tried, we'd have a two-week honeymoon period and then the drug would stop working." She adds, "The magnet has also given us control over his seizures, where with drugs we had no control. You couldn't put more medication in him to get the seizures to stop--you'd have to worry about overdosing. The magnet doesn't hurt him. He has a magnet at school, and we trained his teachers to use it."
Strangely, the physicians' remain unconvinced, despite many success stories like Brady's. In Utah, parents still have to fight to get VNS for their child. According to Lori, "The doctors have had success with every child they have implanted, but they still aren't sold on it."
"My only regret," Lori says, "is that we couldn't have done it sooner. I wish we didn't have to waste so much time."
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|Publication:||The Exceptional Parent|
|Date:||Sep 1, 2001|
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