Jackson Stormes:A Difficult and Amazing Journey 

Living with Dravet Syndrome and all the complications

In the Press

July 2005-VNS Implant Follow Up

Posted by [email protected] on May 17, 2011 at 5:35 PM

Severely epileptic child finds some relief By Sara Cardine

News-Sentinel Staff Writer

Last updated: Saturday, Jul 02, 2005- 06:04:18 am PDT

A renegade morning breeze rustles leaves in the trees around the backyard ofa rural Lodihome. Among its invisible furls darts a young, tow-headed boy named JacksonStormes. His gaze is set on the swing set before him.

"Go push Jack," he calls to his mother, Jennie.

When she asks where, the boy points toward a single swing and says,"There."

"Mom, come on," he continues.

She asks if he can push off one time to get the swing started. He nods andsays, "Uh-huh."

Just one year ago, Jacksonwouldn't have been able to play out in the yard for very long without fallingto the ground in the grip of a seizure.

Plagued by violent seizures that began when he was only six weeks old, hisbrain has been scarred from electrical upsets that have been known to last aslong as three hours, according to his mother Jennie.

It's been an ongoing struggle, and only one of many.

For the past three years, Jennie has been at odds with Lodi Unified'sspecial-education department to find an Individualized Education Plan (IEP)that will meet Jackson'sneeds and encourage him to grow. An extensive document, the IEP outlines aspecial-education student's needs, expectation and lays out goals for thefuture.

She has files of correspondences from the district, as well as records ofcontinued evaluations, a collection that has grown to fill several boxesthroughout her house.

According to Jennie, Lodi Unified will not provide her son special programsbecause they've determined Jackson'scapabilities will never increase.

She disputes the district's claim that Jacksonhasn't made progress over the past couple of years and cannot effectivelycommunicate ideas or simple desires.

"I can't let the school district bully him out of a future," Jenniesaid. "He's so smart -- he just can't tell them how smart he is."

District officials, bound to confidentiality laws that prohibit them fromtalking about an individual student, can not discuss the Stormes case.

Jennie admits that Jackson's seizures were asevere impediment to Jackson'sability to function. But he doesn't have them anymore, thanks to a simpledevice implanted in his chest.

The vagus nerve stimulator, or VNS, attaches to the thick Vagus nerve, locatedin the neck.

Usually, this nerve regulates blood pressure in the aorta of the heart as wellas a person's ability to taste. But when stimulated by VNS, the nerve becomes aconduit of mild, regular electrical signals, which override the impulses thatcause seizures. A magnet wand provides stronger jolts when a seizure does cropup.

"I took the magnet and swiped it over him, and it just stopped the seizureinstantly," Jennie said, adding that the nurse was shocked to see such anabrupt change in Jackson'scondition. "It was neat to see that."

Now that the threat of multiple, brain-damaging seizures has been alleviated, Jackson's mind is morecapable of concocting complex plans and performing motor skills.

Hefting a full-sized wooden shovel to his chest, Jackson scoops up a pile of pale, crustedearth mixed with gravel. With a heave, he swings the shovel above his head andwatches the gravel cascade across the corrugated tin roof of a dog shed.

A smile and small shout of glee escape him as he hears the crash of stoneagainst metal. In a few seconds, he will repeat the process -- something hewill do several times until the experience is catalogued, allowing him to moveon to another task.

Jennie explains that, just like before the surgery, Jackson's hunger for sensory stimulationstill guides most of his activities. Only now that the seizures are gone do histhoughts remain intact long enough to build on one another.

This means that his plans and schemes are more effective. For example, the highlock on the gate to the pool can be overcome with the aid of a chair orskateboard and a cooler can be used to access the swinging bar on the backyardplayground.

But on the upswing, VNS gives him the peace of mind necessary for ideas andmemories to formulate. Jennie hopes this means a greater access, not only toforbidden locks and latches, but to a normal education.

That's why she and Jackson, along with her 9-year-old daughter, Sidnee, plan tomove to the East Coast, where a school district has agreed to pay for a privateschool for Jackson.

This school will incorporate sensory-based learning into a specializedcurriculum and can offer one-on-one work with an in-class aide.

"I'm not giving up hope," she said in an earlier interview. "Iwant him to get a high school diploma."

If all goes well, the Stormes may find what they seek when they move to theirnew New Jerseyhome. Their next town of residence?

A small town aptly named Hope.

Contact reporter SaraCardine at [email protected]


 

 

 

January 2005-VNS Surgery at UC Davis

Posted by [email protected] on May 17, 2011 at 5:30 PM Comments comments (0)

Sara Cardine followed me and my mom through the medical center and surgery.

Thank you Sara for all your support and understanding.

Implanted device gives new hope to Lodi boy with severe epilepsy

By SaraCardine

News-Sentinel Staff Writer

Last updated: Saturday, Jan 29, 2005 -07:56:11 am PST

In the waiting room of the UCDavis Medical Center in Sacramento,Jennie Stormes puts her faith in a surgical procedure that could mean a normallife for her son Jackson.

 Jackson's constantcompanion, a teddy bear, comforts him while waiting for a vagus nervestimulator to be implanted at UC Davis Medical Center in Sacramento on Jan. 14. (Casey Freeman/News-Sentinel)

Only 5 years old, Jackson has struggled each day of his life with a severe case of epilepsy. His small body suffers as many as 100 seizures in a single day, and attacks can grip hisbrain in an electric surge for minutes at a time.

A cocktail of drugs coursesdaily through his tiny veins. On a normal day, he gets three doses of Phenobarbital for serious seizures, Klonopin for the smaller ones and blood pressure medications and Valium suppositories when the attacks get out ofcontrol.

The side effects of the drugs are often as strong as the symptoms they treat.In Jackson'scase, the side effects are often the only effects.

The drugs just aren't working.

Now, an implant procedure that feeds small electrical pulses to the brain andcalms down other, seizure-related pulses may be Jackson's only hope for relief.

A last option

Since 1999, Jackson'sdiagnosis has always been grim: epilepsy, mild to moderate retardation,attention deficit disorder, hyperactivity. The prognosis has been grimmerstill:

Jackson willnever talk. He will never walk. What he has can never be cured.

Jackson Stormes focuses on a bubble blown by his mom Jenniewhile they wait for him to go into surgery to implant a vagus nerve stimulator.(Casey Freeman/News-Sentinel)

But Jennie has done more thansee her son through his first steps and first words. She's watched him becomewhat she calls "my little man," someone capable of expressing hisdesires and frustrations, someone with enormous potential.

She sees herself as Jackson'sonly advocate, which is why she sought a second opinion. That decision led herto UC Davis, where pediatric neurologist Barry Tharp decided there might be onelast option for Jackson.

For the first time, Jennie says, someone else told her that her Jackson's situation wasnot hopeless.

Now, as she awaits an update from neurosurgeon James Boggan on Jackson's condition, Jennie wears a simpleblack T-shirt with a single word, spelled out in rhinestones, on its front:Hope.

Today, for her and Jackson, hope comes in the form of three letters -- VNS.

The vagus nerve stimulator is a seizure-reducing implant the size of a smallpocket watch that brings relatively few side effects. Implanted into the chest,it sends electrical currents through a major nerve in the neck to the brain.Those currents short out the electrical impulses that cause seizures.

The pulses will not hurt Jackson,Jennie says. At their worst, the feeling will be like licking a 9-volt battery.

Jackson Stormes' parents, Jennie, left, and Tim, wait for him towake up after having a vagus nerve stimulator implanted. (Casey Freeman/News-Sentinel)

The benefits, on the otherhand, could include a reduction in the number and severity of his seizures, aswell as a chance for him to get off some of the medications. No chemical sideeffects could mean increased mental function.

And, that could give Jacksona one-way ticket to a manageable lifestyle.

Falls and bruises

It's 8 a.m. and daylight is slow to come. The gift shop won't open for anothertwo hours.

Jennie's already been waiting at the hospital since 4 a.m.

A young, blonde woman who smiles through a fog of emotions, she is surroundedby a roomful of bed raggled people. They are compatriots who, like her, gave uptheir warm beds to come here and await the verdict on a loved one's conditionfrom one of many masked doctors who come from upper floors.

Jennie's every third thought is of Jackson -- his smile, his laugh -- whileupstairs, the surgeon's blade makes a fine incision on the left side of hisneck. The incision reveals the Vagus nerve.

People come and go from the hospital waiting room. Jennie stays put.

Just a short hour ago, she blew bubbles over Jackson's head in the admitting room.

"Pop, pop, pop!" heshrieked excitedly, arms waving. For a moment, he calmed down long enough towatch a single soapy orb crash delicately upon the palm of his outstretchedhand.

As Jacksonexplored the hospital room and jumped on the beds, a nurse asked Jennie about ayellowing bruise on his forehead.

He got it from falling during a seizure, Jennie answered, lifting up the leg ofher son's pajama pants to show the nurse a second purplish bruise on his shin.

The nurse adorned his wrists with hospital bracelets, one with his name andbirth date, another orange band announcing an allergy to green beans. Theyoverlapped the simple silver Medic Alert bracelet that Jackson never goes without.

"Seizure Disorder," the tag reads.

Jennie remembers the nurses in attendance telling her he seemed happier thanmost kids do before surgery.

"That's just Jack," she replied with a knowing smile.

Judgments the worst

Now in the waiting room, Jennie imagines what could be if all goes well withthe VNS implant. The fog in Jack's mind will clear, giving him a chance tolearn and maybe go to school.

He can manage without all the drugs and cast off their nasty side effects.

She will be able play with him in the park without people staring at him whenhe yells or bangs on things.

She will be able take him out in public without someone whispering, "Shedoesn't know how to control her kids."

The hardest part of caring for her son with special needs, Jennie says, isdealing with the opinions of others.

"I get a lot of gawking, a lot of staring, but the judgments are what killme the most," Jennie says as her eyes flicker behind a film of tears."I'm so tired of hearing (that) I need to go to a parenting class."

Upstairs, a wire connected to a small, round implant is fed from the incisionin Jackson'sneck to a second opening in his chest, above and to the left of his heart.

Through the first incision, Boggan will attach the wire to the Vagus nerve, athick branching cord that leads to the brain. Usually, the nerve controls thepressure of blood traveling through the heart's aorta and a person's ability totaste.

When the surgical scars have healed and device is activated, it can be operatedwith a special magnet to send waves of electricity to the brain that interferewith the disturbance caused by a seizure.

Jennie does not know how long it will take the device to start working afterit's been turned on. Some friends have told her their children reactedpositively within months; for others it took years to kick in. Even if it takesthat long, she thinks, it will have been worth today's effort.

"It's been a rough five years," she says, looking back at her timeraising a child with Jackson'scondition. Heaving a big sigh, she shifts her weight in the waiting room loveseat.

At any cost

Jennie's fight to keep Jacksonalive has cost her the life she once knew. Before he was born, she was themother of a healthy 4-year-old girl, Sidnee. She had a husband, Tim, and a jobas the officer manager of a Stocktonmedical imaging company.

She gave birth to Jackson Robert James Stormes on June 3, 1999, and thougheverything seemed normal, she felt an undying need to stay close to him. Shehad returned to her job part time when he was six weeks old, but lasted onlytwo weeks before she gave her final notice.

"I couldn't do it -- I couldn't not be with my baby," she says.

While she was finishing out her time at work, Jackson had his first seizure. It began withan eye twitch that quickly traveled throughout the muscles in his face.

The hospital later told her and Tim that babies often experience seizuresbrought on by fever.

But in the following months, baby Jacksonwould have more frequent and more serious seizures, until at 1- year-old, hewould be diagnosed with epilepsy by a doctor who'd say to Jennie, "There'snothing we can do for him."

In the following years, Jackson'scondition would prove to be too much of a strain on her marriage. She says shecared so much about Jacksonthat it hurt to think Tim was afraid of being around him. Arguments with Timwould become fights, fights would eventually lead them to divorce.

With the diagnosis of epilepsy, Jacksonjoined 2.5 million Americans who are living with the disorder, which causesneurons, the spark plugs of the brain, to act abnormally and interrupt normalcommunication between the body and the brain. The result of themiscommunication is seizures ranging from slight eye flutters to full-bodyconvulsions.

Prolonged seizures, lasting longer than 5-10 minutes, can damage the brain andimpede mental development in children. Jackson'slongest seizure to date lasted 15 minutes, Jennie said.

Since he was 2 years old, Jackson was enrolledin special classes organized through the Lodi Unified School District, butJennie pulled him out when she realized his condition might be too specializedfor a teacher who has many kids to handle. Now, she teaches him on her ownusing a program developed for autistic kids.

He's made great improvements in communicating with others. But each seizure Jackson has threatens toundo the progress he's made. VNS may be his only chance to keep brain damage atbay.

Nothing to lose

Downstairs, at 9 a.m., Jennie's lonely vigil is interrupted by Tim's arrival.

They have been courteous with one another since their divorce last March,though she feels Tim has distanced himself from Jackson -- out of fear or frustration, Jennie cannot say.

At the time of Jackson's surgery, Tim declined to comment for this story.

After a period of silence, Tim goes over to a nearby coffee kiosk and comesback with a drink for Jennie. She sips at his conciliatory gesture, a mochawith whipped cream, and fixes her gaze back on the hallway. Tim sits near her,but not by her, expressionless.

It's nearly an hour past the time Boggan told her the surgery would befinished. She looks for him to come from the elevators by the gift shop. Anolder woman unlocks the shop and begins to set things up for the start of theday.

Boggan does not come.

There are plenty of success stories from people living with VNS implants. Inthe past decade, some 30,000 people worldwide have undergone the surgery.

The procedure has been approved by the Food and Drug Administration only forpeople over the age of 12, though about 30 percent of VNS patients, like Jackson, are under 12years old.

Jennie, who waited three years for a doctor to recommend Jackson for the device, knew right away thatshe wanted to try it. After all, she says, what do they have to lose?

Traffic in and around the waiting room picks up. The morning sun breaks throughthe clouds and is making its way through the sliding glass doors. The mochawith whipped cream is getting cold. It's nearly 10 a.m.

A tall thin man dressed head to toe in medical scrubs gets off the elevator andheads toward the waiting room. Jennie's pulse quickens. It's Boggan.

She and Tim rise to meet him. The doctor is smiling. He explains thateverything went well and that Jacksonwill be in post-op shortly.

For the first time in the morning, Tim smiles.

Healing the mind

In the dimly lit recovery room, anesthesia has reduced Jackson to a shadow of his former self.Disrobed from the flannel sheath of a hospital pajama shirt, his chest is apost-operative road map. Highways of intravenous tubes and pulse monitors snakeacross his body, carefully avoiding plateaus of gauze-covered sutures.

Even in an anesthetic stupor, the 5-year-old understands that he must take hissix morning pills. He chews the Phenobarbital and Klonopin tablets like stalecandies as they turn to a chalky paste in his cheek.

A little grumpy, but none the worse for the wear, Jackson's heavy-lidded eyesfinally close. As his parents lean over the rails of his bed, Jennie reachesout to caress her son's hair. Tim's face is full of restrained emotion.

The scars from Jackson'ssurgery will heal, leaving behind no more evidence of that day than two thinlines of skin tissue and a small, raised disk over his heart.

The skin will be the first thing to repair itself. If Jennie's hope isfulfilled, the healing of her Jackson'smind is soon to follow.

Contact reporter Sara Cardineat [email protected]

Vagus nerve stimulation at a glance

Vagus nerve stimulationtherapy involves the surgical implantation of a device into the chest thatsends intermittent electrical impulses to the brain that override otherimpulses associated with seizures.

The procedure, which takes about one hour to perform, has been shown to greatlyreduce the occurrence and severity of seizures. In some cases, it allowspatients to reduce epilepsy medication, which can lead to improvements in moodand mental capacity.

An implant the size of a small pocket watch is implanted into the patient'schest and sutured into the tissue above and to the left of the heart. Attachedto the device are two spiral electrodes that coil around the left vagus nervein the neck.

On the end of each electrode is a small tab that transmits electricity from theimplant to the nerve. From the nerve, the electricity travels directly to thevein.

The typical voltage of the stimulator is about three to four volts, though itmay go as high as 12 volts.

When a patient has a serious seizure, the VNS device can be activated to send acharge to the brain by a handheld magnet. If the patient is a child, magnetsare given to caregivers.

VNS therapy is becoming a preferred treatment for people with epilepsy whoseseizures are not generally reduced by antiepileptic drugs. Approved by the Foodand Drug Administration in 1997 for patients over 12 years of age, VNS therapyis being used by over 30,000 people world-wide. About 30 percent of thosepatients, like Jackson Stormes, are under age 12.

Source: Cyberonics, Inc.

 -- News-Sentinel staff

Epilepsy at a glance

Epilepsy is an illnesscharacterized by recurrent seizures. Seizures are sudden attacks or convulsionscaused by involuntary bursts of electricity in the brain. Symptoms includetwitching of the fingers, unrecognizable speech and even unconsciousness.

Facts:

• About 2. 5 million Americans have epilepsy.

• Of all Americans diagnosed with epilepsy, 30 percent are children under theage of 18.

• Each year, more than 181,000 new cases of seizures and epilepsy are reported.

• Today, more than 30 percent of epilepsy patients have pharmacoresistantepilepsy, which means seizures cannot be adequately controlled by drugs.

• In about 70 percent of new epilepsy cases, there is no known cause.

Non-VNS treatments:

Medications, including the sedative Phenobarbital and Valium. Each can haveserious side effects, such as drowsiness, weight gain and confusion. In somecases, a ketogenic diet is recommended. This is a diet that is high in fat, lowin carbohydrates and protein and is typically prescribed for children.

Brain surgery and brain tissue removal is an option when the epilepsy isthought to be contained within a certain area of the brain and can be removedsafely.

Intracerebral infusion involves drilling holes in the patient's skull andstimulating the brain itself through pulses sent by wires inserted into thecranial holes.

-- Source: Cyberonics,Inc.