Jackson Stormes:A Difficult and Amazing Journey 

Living with Dravet Syndrome and all the complications

In the Press

Jackson Stormes in the News.

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Hope Township mother, son to urge Gov. Christie to sign kids' access to medical marijuana bill

Posted by jacksonstormes@gmail.com on July 30, 2013 at 3:15 PM

Hope Township mother, son to urge Gov. Christie to sign kids' access to medical marijuana bill

 


By Andrew George | The Express-Times

on July 27, 2013 at 6:00 PM, updated July 27, 2013 at 6:03 PM

 

 

 


 

Last Tuesday night, 14-year-old Jackson Stormes suffered through 12 seizures in a Bergen County hospital.

 

The Hope Township teen and his mother, Jennie Stormes, were at Hackensack University Medical Center for a week while he received treatment for Dravet syndrome, a severe form of epilepsy that was diagnosed in 2009.

 

 

Jackson had part of his frontal lobe surgically removed to no avail, and when it comes to drugs, Jennie Stormes said, her son has endured the consequences of trial and error.

More than 50 different tries, all errors.

 

That includes a recent setback on stiripentol, an anti-epileptic drug manufactured in Europe that required special clearance from the U.S. Food and Drug Administration for Jackson to use. He showed signs of improvement on the medication but ultimately the progress was limited.

 

"I just want my son to be able to live a day without a seizure," Jennie Stormes said.

 

That's why she and her son will travel to Trenton next week to hand-deliver signed letters urging Gov. Chris Christie to sign a bill passed last month by the Assembly and Senate that would make the state's nascent medical marijuana program more accessible to sick children.

 

They will accompany another New Jersey family with a 2-year-old girl also diagnosed with Dravet.

 

Governor reluctant

 

State law currently mandates that children must obtain separate recommendations from a treating physician, a psychiatrist and a pediatrician to be granted access to the medical marijuana program. Adults are only required to receive a recommendation of a treating physician.

Though Christie has not yet made a final decision on the bill, he has shown reluctance in widening the program's reach to include kids.

 

The bill would not only reduce the recommendations for minors to one but also pave the way for state dispensaries to grow a variety of marijuana strands, some of which might be edible and more easily used by children.

Though Christie has yet to make a final call on the bill, he has shown reluctance in widening the program's reach to include children.

 

A Christie representative last week directed an Express-Times reporter to comments the governor made about the bill at an unrelated news conference July 2 in Seaside Park.

 

While the state's program is available to adults suffering from terminal or debilitating illnesses warranting the drug's use, Christie said he was "very reluctant" to arbitrarily apply the same rules to children.

 

He also took issue with the complaint by some bill advocates that requiring children to seek out three different recommendations was burdensome.

 

"I don’t understand why that’s a problem, and you know, if folks can’t get physicians to sign off on this then that tells me something," Christie said. "This is supposed to be a medically based program that is there as a last resort, not as a first resort, and so I’ll look at the bill."

 

Every state legislator from the 23rd and 24th districts, all Republicans, either abstained or voted against the bill, which is sponsored entirely by Democrats. But Jennie Stormes doesn't believe the issue has been or should be determined by partisan politics.

 

"It doesn't make sense to me because I'm speaking as a mother," she said. "I personally am speaking to the humans in front of me, not to the Republicans and the Democrats."

 

Better option

 

Despite the stigma some people attach to marijuana, Jennie Stormes said, the strand she's hoping will become available in New Jersey is a non-psychoactive one. In particular, she's lobbying for a strand that's high in cannabidiols, or CBD, rather than tetrahydrocannabinol, noting "there is more to medical marijuana than THC."

 

"Our kids aren't going to get high," she said.

 

In situations such as Jackson's, the traditional threat of marijuana as an addictive gateway drug doesn't really apply, Stormes said. Jackson is currently receiving treatment for his addiction to the slew of pharmaceuticals he's gone through in recent years, and Stormes said the double standard doesn't make sense.

 

"The potential for abuse is no different than codeine," Stormes said. "Why are we so afraid of this drug?"

 

Stormes said the issue regarding the required recommendations is in its implementation rather than its intention. She said procuring a psychiatrist and a pediatrician along with a treating physician is costly and carries with it the potential for the process to be unnecessarily delayed.

 

For children who do not regularly see their own psychiatrist or pediatrician, asking doctors not familiar with a patient to speedily sign off on the controversial medication is an "unrealistic expectation," Stormes said.

 

"My goal is to make it appropriate," she said.

 

And though Jackson has since obtained all three recommendations needed to receive his medical marijuana card in New Jersey, Stormes said, her son hasn't been able to use it as she can't even get a call back from the state's lone operating dispensary, the Greenleaf Compassion Center in Montclair.

 

Greenleaf's website states that the center is scheduling patients in the order they received their state medical marijuana card. A February report from The Record of Bergen County indicated that the center was overrun with requests and patients were experiencing considerably long wait times.

 

A second dispensary planned for Atlantic County received state approval last month and could open in the fall.

 

Some success

 

Stormes said it's important to note that her request is not unfounded in that Jackson has used medical marijuana as a registered patient in California where he lives with his father part time, and that it's proven to be successful. While using the drug in California, she said, Jackson experienced fewer seizures.

 

"He improved so much," Stormes said of Jackson's time in California.

 

She said she fears that if Christie doesn't sign the bill into law, she might have to relocate to California or Colorado, which can also provide Jackson with access to the strand he requires, and leave New Jersey for good.

 

But that would require her to find a new job and uproot Jackson from his home, a prospect she thinks would be "detrimental" to any progress he's achieved through the years.

 

"I'm hoping that passionate care comes to New Jersey finally," Stormes said.

 

December 2011-- STP Stiripentol Medication

Posted by jacksonstormes@gmail.com on January 13, 2012 at 6:25 PM Comments comments (3)

 

Hope Township boy with severe epilepsy experiences change on new medication

Published: Monday, December 26, 2011, 4:49 AM

By Phillip Molnar | The Express-Times Express-Times Photo | MATT SMITH

Stormes spends every available moment with 12-year-old Jackson, even sleeping in his room to reduce the risk of him dying in his sleep. But that doesn't mean they've had a substantial conversation.

Jackson has a rare and severe form of epilepsy called Dravet syndrome, which causes frequent seizures that can last hours.

The condition is incurable, immune to surgery and does not respond to most medicine.

Only five weeks ago, Jackson was having up to 20 seizures a day and was so medicated he could barely function, let alone speak, his mother said.

Drug cocktails, and even having part of his brain's frontal lobe removed in 2008, made little difference.

Everything changed when Stormes, after more than a year of trying, received clearance to give Jackson a drug from France called stiripentol that the U.S. Food and Drug Administration has not approved for use.

Stormes teared up two weeks ago at her Hope Township home as she said Jackson had not had a seizure in five days and was articulating like never before.

"It's been really nice," Stormes said. "It's not constant on guard."

It is not just Jackson's mother who has noticed a difference in his capabilities.

"He actually speaks some sentences now," said Jackson's school bus driver, A. Morris Scott Jr.

Scott, who is also a Belvidere Town Council member, said Jackson used to seem "lethargic" or "drugged."

"He seems more alert," Morris said. "He wants to talk about things: 'There's a cow! There's a motorcycle! There's a truck!'"

Jackson's 16-year-old sister, Sidney Stormes, said he seems friendlier and expresses emotions like never before.

Dr. Elaine Wirrell, the director of pediatric epilepsy at the Mayo Clinic in Rochester, Minn., said those close to Jackson are seeing real improvement.

Wirrell said Dravet patients who find the right mix of medication -- such as stiripentol, which she calls very effective -- can see their life improve.

Once doctors reduce frequent or prolonged seizures, she said, "There is less risk of those seizures causing brain injury (and) causing learning problems."

Difficulty administering

Stormes said she was warned the drug could lead to severe loss of appetite, decreased metabolism and anorexia. But she said Jackson hasn't experienced those and that the drug is worth the risk.

"I always have to choose between ... bad and badder," she said of the drug's risks. Without the drug, she said, "he's dealing with the side effects of not learning, not being able to grow up and develop.

The FDA allows Jackson stiripentol despite its status as a so-called orphan drug, a promising but developmental medicine for rare diseases. His Horizon Blue Cross Blue Shield of New Jersey insurance pays for it.

But Jackson's nurse, who is with him most of each day, is prohibited from giving him the little pink pills with his other medication.

Though not approved for use by the FDA, stiripentol is benefiting Jackson, his mother says.

Atlantic Private Care Services, a division of Atlantic Health System, citing FDA regulations, says it would have to review stiripentol itself before its employee, Jackson's nurse, could administer it.

Atlantic spokeswoman Janina Hecht said the company cannot let nurses give the drug to Jackson until it goes through the process.

The company's review board takes, on average, three months to complete its analysis of a drug but would expedite the process for Jackson, Hecht said.

"It has nothing to do with what we want to do or what we don't want to do," Hecht said. "It's just standard procedure."

And before Atlantic could begin its view, Jackson's prescribing physician -- a Hoboken, N.J.-based doctor from the Northeast Regional Epilepsy Group -- would need to initiate the procedure.

The doctor declined comment, citing health privacy laws, but Jennie Stormes said he had been ignoring Atlantic because he thought the company had no right to ask for an internal review board.

"If something were to go wrong, and then it was determined ... it did not go through the (internal review) process, the nurse's license is at risk," Hecht said.

Earlier this month, the nonprofit Advocacy for Patients with Chronic Illness Inc. wrote a letter to Atlantic Health System challenging its legal analysis.

The Farmington, Conn.-based group said Atlantic was confusing stiripentol's status as an orphan drug with a personal drug.

It wrote that stiripentol's status with the FDA was "irrelevant to Jackson's use and your nurses' administration of the drug."

Jackson's doctor has since contacted Atlantic's review board and is trying to come to an understanding with the medical group, Stormes said.

While Stormes waits for an answer, she struggles to get home to give Jackson his medication three times a day while holding three part-time jobs.

A single mother, Stormes teaches notary classes, is a substitute nurse for Warren County schools and is a nurse case manager at the Homestead nursing home's Passaic and Morris county branch. She is also pursuing a master's degree in nursing.

A very frustrated Stormes said she had a hard time reasoning with Atlantic Health System and dozens of doctors.

"What's important is my son is doing better on this drug," she said.

Rare disease

Laura Cossolotto, of Centerville, Iowa, gazed through tears as her daughter, Michaela, underwent a skin biopsy in 2002.

"I watched them cut a chunk of her skin off," she recalled last week.

Doctors tried every procedure, test and drug combination possible to discover why Michaela had constant seizures.

Cossolotto spent every free minute searching the Internet for any information she could.

Finally, after 10 and a half years, Wirrell, from the Mayo Clinic, diagnosed Michaela with Dravet syndrome.

Dravet syndrome, also known as severe myoclonic epilepsy of infancy, is a seizure disorder caused by a gene mutation, according to the U.S. Centers for Disease Control and Prevention.

Those with Dravet often have seizures before the age of 1. Dravet patients do not respond well to epileptic medicines and experience wide developmental delays, Wirrell said.

"Children with Dravet, despite our best efforts at trying to control their seizures, continue to have seizures," Wirrell said.

Shortly after the diagnosis, Cossolotto began the struggle to get her daughter stiripentol.

"It's not even anywhere close to being affordable. It's more than a mortgage payment," Cossolotto said. "Most people could pay upwards of $1,000 a month."

Cossolotto even considered putting a 3-month supply on a credit card.

After four months, the determined mother convinced the FDA to allow her to get the drug and her insurance company to pay for it.

"I could tell the difference immediately," Cossolotto said.

Michaela had roughly 120 seizures a year before the medication and now has one or two a year, she said.

Dr. Ian Miller, director of neuroinformatics at Miami Children's Hospital, said there is "no question there are anecdotal examples of really dramatic improvement with this medicine."

An expert on Dravet, he said that in one extreme example, a patient was stuck in the intensive care unit of a hospital because of seizures so severe the patient needed a medically induced coma to be treated.

Once on a drug cocktail that included stiripentol, the patient left the hospital and was seizure-free for more than a year, he said.

"It is really the only medication that has been proven to work for this specific type of epilepsy," he said.

Cossolotto, who is now president of Dravet.org, said what makes Dravet so difficult is that a diagnosis is sometimes only clinical -- meaning even a negative gene test does not rule out the illness.

Roughly 20 percent of patients with the diagnostic criteria of the syndrome test negative for a mutated gene, according to a 2002 report from the Centre for Genetics Education in Australia.

Wirrell is concerned the syndrome is under-diagnosed.

"I think things are getting better, but it is still a diagnosis, that in many cases, we are making too late," she said. "We are making it years after a child has years of uncontrolled seizures before it is really recognized."

Wirrell said roughly 5 percent of children with early onset epilepsy may have the syndrome but there has never been a careful population study.

Advocates put the Dravet population at very high numbers. Medical professionals are hesitant to give an estimate.

Also making the diagnosis difficult: Dravet symptoms vary from patient to patient. Michaela, now 15, has a much less severe case than Jackson. She talks on the phone to her friends and has a Facebook page, her mom said.

Jackson's home life

 "What is important is my son is doing better on this drug," Jennie Stormes said.

 Two weeks ago, the Stormes had a surprisingly normal Friday night.

Sidney Stormes brushed her hair in a mirror before a friend's sweet-16 party, Jennie Stormes prepared a meal, and Jackson enjoyed one of of his favorite drinks, decaffeinated coffee.

"He just likes whatever he sees other people drinking," Sidney Stormes said as she motioned for Jackson to give her a high five.

For fun, Jackson engages stares underneath the kitchen table, watches "Barney & Friends" and wears a Michigan Wolverines football helmet given to him by a nurse.

A faux Christmas tree and a television lit the Stormes' living room while Jackson, wearing a Spider-Man sweater, seemed fascinated by a photographer's camera.

Although it was the kind of evening most families take for granted, the normalcy of the situation has been a major life change for the family.

Jackson started having seizures at 4 months old.

Yet, considering doctors said Jackson would never walk or talk, Jennie Stormes is grateful.

"It's Jack!" Jackson said, smiling at his mom.

"It's mom!" they said in unison, before Jackson swung his head back down to stare at an iPad playing an episode of "Barney."

"He's a big ham," his mother said.


© 2012 lehighvalleylive.com. All rights reserved.

September 2008-Make A Wish

Posted by jacksonstormes@gmail.com on May 17, 2011 at 5:36 PM Comments comments (2)

Make a wish come true for boy who had brain surgery

Published: Wednesday, September 03, 2008, 5:14 PM    

Updated: Wednesday, September 03, 2008, 5:16 PM By Craig Turpin

Follow Share0 Share Email Print BLAIRSTOWN -- Tears well up in the eyes of Jen Stormes as she talks about the progress her son, Jackson, has made since having major brain surgery in April.Jackson, age 9, was diagnosed with severe brain-related complications just four-and-a-half months after he was born. As a result, Jackson has had to contend with epilepsy and multiple disabilities. Something no mother wants to hear. But the entire process has made Stormes strong. And Jackson's most recent progress is something Stormes is more than happy to talk about.

"You can't even tell he had the surgery," said Stormes. "He's just completely improved."

The family moved from California two years ago after doctors there determined there was no more help for Jackson.

"He is complicated," said Stormes. "He has tried many different drugs."

In all, Jackson was taking some 35 medications for his treatment and all failed to work.

But Stormes believed that he could be helped. So she packed up and moved to New Jersey in the hopes of getting more treatment from doctors here.

"Shortly after we moved here he got worse," said Stormes.

But she kept faith and began taking Jackson to doctors here in New Jersey, who conducted testing trying to determine the best option for surgery on Jackson.

"They took out his right frontal lobe," said Stormes.

On April 22 at the Children's Hospital in Philadelphia, Jackson's surgery began. The two step process took three days to complete, but Stormes said he's back home and doing fine now.

But due to his life threatening illness, Make-a-Wish Foundation chose Jackson's wish to grant.

Make-a-Wish determines eligibility for granting a wish based on four criteria. First there is a referral by a medical professional, parent, or the children themselves. Children from age 2 to 18 who have not received another wish from another wish granting organization are eligible. Once that is determined, the child's medical condition is looked at to see if they truly have a life threatening medical condition. A Make-a-Wish Foundation team member is then sent out to discuss what wish may be the best option for them. Finally, the wish is then granted.

Stormes said the children are asked to choose two wishes, although only one gets granted. Jackson's wish for a swim spa was denied by the Make-a-Wish Foundation due to the cost, which is around $30,000.

"It's amazing the healing properties in the water," said Stormes.

She said Jackson wanted a swim spa because he loves the water.

"Water is the only place Jackson can be equal with his peers," said Stormes.

Stormes said Make-a-Wish grants the wishes of children, but in her situation, with an old in-ground pool in her backyard, Jen still has a problem.

"Even if they grant that wish, I still need to clear the ground," said Stormes.

But the pool is beyond repair for use, so instead the foundation is granting Jackson's second wish. However, in order to make room for the new play set that the foundation has granted, the land needs to be filled and level. But Make-a-Wish doesn't pay for preparing the land. They will only pay for the play set. Jen still needs help removing an abandoned pool in her backyard so that Jackson can have a place to play.

"We still need an electrical upgrade, someone to clear out the ground, put fill dirt in and compaction," said Stormes.

The single mother of two -- Jackson and his 12-year-old-daughter Sydney -- runs her own business, "Academy for Notaries," as a notary. She offers notary services both private and to colleges throughout the state.

"I transplanted what I did there (California) to here," said Stormes. "Everything I have goes into taking care of Jackson."

Stormes said with a child suffering seizures all the time and Jackson going to therapy some nine times a week in nearby Phillipsburg, she couldn't go out to work like most others. So running her own business was her only option. But the future looks bright now for Jackson.

"He's back to school now," said Stormes.


July 2005-VNS Implant Follow Up

Posted by jacksonstormes@gmail.com on May 17, 2011 at 5:35 PM Comments comments (0)

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 sarac@lodinews.com.


 

 

 

January 2005-VNS Surgery at UC Davis

Posted by jacksonstormes@gmail.com 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 sarac@lodinews.com.

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.

 


 

 

January 2004-Riding Out the Stormes

Posted by jacksonstormes@gmail.com on May 17, 2011 at 5:25 PM Comments comments (0)

This article from the Lodi News Sentinal waswritten by Alex Lazo.  He received the 2004 Journalist of the Year Award from the Epilepsy Foundation of America.

Thank you Alex for sharing with others the reality of our world.

Riding Out the Stormes

Couple hopes for normal life for their epileptic son

By Alejandro Lazo

News-Sentinel Staff Writer

Jackson Stormes, 4, plays  at the top of a slide at Legion Park. 
Stormes suffers  from epilepsy and has experienced more than 100 minor seizures a day. 
(Jennifer M. Howell/News-Sentinel)

It's a crisp afternoon and 4-year-old Jackson Stormes is attacking Lodi's Legion Park playground like a young lion.

His lungs belt out a roar as he climbs onto the jungle gym here and jumps wildly up and down on the middle of the play suspension bridge, frightening a smaller girl away from it.

"Jackson, play nice," yells his mother, Jennie Stormes, from a nearby park bench.

Jackson Stormes later climbs his way to the top of the jungle gym again, this time approaching the slide. But now he starts to slow down. His eyes begin to flutter, like a butterfly shaking its wings. The boy hesitates and stops. Stormes changes his mind and slowly backs down the ladder again.

When he reaches the last rung, he lets go and falls into the playground bark below. He begins convulsing. Every muscle in his body is rigid yet trembles.

A woman nearby starts screaming for help.

Jennie Stormes bolts off the bench to the spot where her son has fallen. She sweeps him up in her arms and breaks into a dead run to where her car is parked on the street.

Other children and parents just stand and stare.

When Stormes gets to her car, she lays her son down on the grass and unlocks the door. From inside, she pulls out a black bag with an oxygen tank and affixes a tube to the boy's nose. She quickly turns the small tank's handle.

His contractions slow. His chest begins to heave deeply. The seizure ends.

Stormes rubs her son's chest and talks to him soothingly. He rests.

Jackson Stormes has an uncontrollable case of epilepsy. The boy had his first seizure when he was 4 months old. His parents have tried countless medications and therapies, but nothing seems to work.

Finding a place for her son in the public schools has been particularly trying for Jennie Stormes, but she and her husband have hopes that their son will one day lead a normal life.

The answer to when and how just hasn't come yet.

A rambunctious life

Watching Jackson Stormes play on a jungle gym, he seems like a rambunctious but otherwise normal blond and blue-eyed 4-year-old. He loves to play ball and swim in his backyard pool over the spring and summer. He plays catch with his dad. He can catch a football from 10 feet away, his father, Tim Stormes, said proudly.

For Christmas, he was given a new bike with training wheels. He rides it like it's connected to him, his mother says.

Yet Jackson Stormes can have dramatic convulsive seizures nearly every day or every other day. These seizures are known as generalized tonic-clonic seizures, but are also known by the older name of grand mal seizures.

These tonic-clonic seizures are the type that most people imagine when they think of epilepsy, although there are several different types of seizures, as well as different kinds of epilepsy. Stormes has a severe case of generalized epilepsy, says Dr. Donald Olson, an epileptologist at the Lucile Packard Children's Hospital at Stanford University. The young boy's type of epilepsy involves his entire brain and makes treatment by surgery difficult, Olson said.

Stormes has many different types of seizures. Not all of them are the tonic-clonic type that make him convulse severely. In fact, he can have hundreds a day, the most typical kind are myoclonic seizures which send jerks and twitches of varying degrees through the muscles in his upper body.

But each tonic-clonic seizure that Stormes endures is potentially fatal, and leaves him unconscious and utterly spent. Some of these seizures have lasted as long as an hour. They are frightful to watch. They scare even his doctors.

No drug or combination of drugs has yet been able to control the boy's seizures. Doctors have tried more than 20 different medications or combinations of medications, his mother said.

While many young children with epilepsy often respond to medication, Stormes may fall into that minority (10 to 15 percent of all cases) whose seizures can't be controlled by drugs, Olson said.

Jennie Stormes holds the hands of her son, Jackson, 4, as he comes out of a seizure which was brought about due to his epilepsy after playing at Legion Park in Lodi. (Jennifer M. Howell/News-Sentinel)

Olson admits that the chances of finding a medication that will control Stormes' severe seizures appears slim -- but everybody just has to keep on trying.

"Although there are many seizure medications, there are only three or four basic kinds," Olson said in a telephone interview.

Seizures are typically the result of abnormal excitation or inhibition of the brain. Anti-epileptic medications work by either reducing stimulus to the brain or increasing that activity.

Either, "too many brain cells are on or too many are off," Olson said. "Medicines work by restoring the balance. It's like a teeter-totter: You can push down on one side or the other" to restore the balance in the brain.

Sensation and hardship

When Stormes had his first seizure it lasted 10 minutes and, like many more to follow, ended in a trip to the Lodi Memorial Hospital's emergency room.

On that first trip, he was given oxygen by a doctor and sent home. Just a week later, he had another seizure. He was then prescribed an anti-epileptic drug called Phenobarbital, one of the first of these kinds of medications. Since then, doctors have prescribed drug after drug. All of them have had varied side effects, including lethargy and educational delays. None of them have controlled his epilepsy.

When Stormes was younger, he would pick up toy cars and drop them on different parts of the ground, searching for the loudest crashing sound they could make. He would do it over and over. It was the same thing with his mother's kitchen plates, which she has since replaced with plastic ones.

He would also bang his head against things, hit himself, and put a toy with a high-pitched beep up to his ear and hold down the beep button. His parents don't know if this behavior is a part of his epilepsy or whether its a side affect of medications or another symptom of his abnormal brain patterns.

But his brain is constantly seeking sensation.

"Whether it's a swing or a slide, he seeks everything with texture," Jennie Stormes said. "He'll run his fingers across a cheese grater. It gives me the chills."

Jackson Stormes has undergone physical, occupational, speech and behavioral therapy from organizations like the Easter Seals, Valley Mount Regional Center and United Cerebral Palsy.

From this therapy, Jennie Stormes has learned how to help feed her son's need for sensation, and how to live with his epilepsy better. She wraps him up in a blanket and tickles him. She lets him swim in the backyard pool with a life jacket on.

When he was 11 months old, workers from the Valley Mount Center said he was testing at an 8-month level. That gap has widened. When he was 2, he was at 12-15 months. Now, at 4, he's still testing at 15-18 months in speech and 2 years in some other areas. The delay is mostly due to his abnormal brain patterns, Olson said. His delays are also from the side effects of all the medications he has taken, although Olson attributes the preponderance of his lack of development to his disease.

However, his mother blames the medications, but says she has to place her faith in her doctors.

Olson says that it's not unusual for parents to blame the drugs.

"I'm extremely frustrated. I don't know how to help him," Jennie Stormes said. "It's been really hard. His educational delays keep getting wider and wider."

Finding a place for Jackson

Jackson Stormes began his education in a preschool program with the Valley Mount Center. He went on to a federal Early Start preschool program at the Dorothy Mahin Elementary School in Lodi when he was 2. While at Mahin, Stormes was not inhibited by his epilepsy because he was on a special diet called the ketogenic diet -- a strict fat- and protein-based diet that controlled his seizures.

But eventually, that diet failed as well. He would break it too often and the seizures would return. Every time he broke the diet, he would have to go without food. The boy lost 10 pounds during his last month on it, Jennie Stormes said.

Once he was back on medications, Jackson Stormes was enrolled at a Communicational Handicapped class within the Lodi Unified School District at Lawrence Elementary School. Today, he remains at Lawrence and his teacher is Sara Rouse, a speech therapist there. But school district officials have told Jennie Stormes that he is falling behind, and that he needs to be placed in a class for children with more severe disabilities.

Jackson Stormes is late in learning how to speak. He communicates more with hand gestures and sign language than with words. He is very hyper, running up the walls, and has trouble socializing normally with his classroom peers, his mother says.

But Jennie Stormes has resisted moving her son to a class with other children who have severe learning disabilities. Despite his epilepsy, she believes her son can earn a high school diploma and then move on to study at a university one day. If Lodi Unified doesn't have a program that meets his needs, they are required to create one. She contends that there still isn't a place for him within the school system next year.

"They're baby-sitting him," Stormes said. "I want them to give him an education."

Getting the district's side of the story isn't easy, as confidentiality laws limit officials from speaking about an individual student's case. Catherine Conrado, in charge of the Special Education Local Plan Area for the LUSD, could only reply to News-Sentinel inquiries by saying she couldn't discuss Jackson Stormes' individual case.

However, school districts are required by the federal Individuals with Disabilities Education Act to provide what is known as a free and appropriate education, Conrado said. This law states that an Individualized Education Plan must be created to meet Stormes' special needs. While the boy does have such a plan with the district, his mother says it's inadequate for him.

The district is able to meet Stormes' needs, Conrado said, but declined to elaborate due to the constraints the privacy law places upon her. At times, parents do disagree with the school district though, Conrado added.

Hope for the future

To deal with the sudden seizures, the long emergency room waits and car trips to Stanford, both of Jackson Stormes' parents have had to change their lives significantly. Jennie Stormes now works her own hours as a notary public after quitting her job as an accountant for a doctor's office.

Tim Stormes is a police officer who works for the Stockton Unified School District. He is forever thankful to an understanding chief who has allowed him to take off in emergencies and has been very supportive throughout his son's illness.

The couple also have an 8-year-old daughter, Sidnee, who has had to learn about her brother's condition at her own young age. Sidnee Stormes is in the second grade at the Joe Serna Elementary School. This charter school has a concurrent Spanish bilingual program, where she is learning Spanish. It's a program that Jennie Stormes would like to see her son in one day.

Tim Stormes says that he and his wife were both naive about their son's condition when they made the first trip to the hospital, almost four years ago.

He thought doctors would tell him, "This is the problem, here's how you fix it, let's go." But, he says, over the years he has learned that doctors aren't gods, they're just people who have gone to school longer than most of the rest of us.

Every father wants their boy to grow up and play football, Tim Stormes said, but what he really wants for his son is for him to stop having seizures and grow up healthy. It's that wish that lets him keep trying what the doctors tell him to do.

"He wakes up smiling. He tells everybody 'Hi,' but he drops out sometimes (because of the epilepsy)," Tim Stormes said about his son. "Otherwise, he's just like a normal kid."

But when Jackson Stormes was born, Jennie Stormes seemed to sense a large change looming in front of her. Unlike the storm that's been her son's disease, it was a winter fog, a mysterious intuition. So soon after his birth, she quit her job at the doctor's office and started her own business as a notary public.

"I didn't know what was coming, I just knew I had to be with him," Jennie Stormes said.

Two weeks after she quit her job, he had his first seizure.

Jennie Stormes' experience working at a doctor's office was invaluable, she says. It taught her how to deal with the medical bureaucracy and insurance companies. Fortunately, Tim Stormes' insurance has paid for the medi-flights the emergency room trips, doctors and medications.

Now the couple hope to create a special educational program for Jackson here in Lodi. It's called the Son-Rise program, and was created for a severely autistic child on the East Coast. The program has been profiled in a book called "Son-Rise: The Miracle Continues."

Jennie Stormes read that book, a Christmas gift from her mother-in-law. She hopes the program might be right for Jackson Stormes. Jennie and Tim Stormes are even taking a trip to Sheffield, Mass., in February to learn more about it.

As for the seizures, the Stormes, and Jackson's doctors still hope to find something that will control the tonic-clonics. Those are the ones that keep getting in the way at school, at the supermarket, at home and everywhere else.

"Every time we try a new drug, we hope that's the one," Tim Stormes said.

Contact reporter Alejandro Lazo at alexl@lodinews.com.



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