Samantha Staab: Living with dystonia
Four years ago, Tyler Staab underwent surgery to treat his dystonia.Now his 8-year-old sister, living with the same disorder begins her own journey
Published: Monday, January 25, 2010 at 6:01 a.m.
Last Modified: Friday, February 26, 2010 at 5:05 p.m.
Rick and Michelle Staab hoped it wouldn't happen again.
After Tyler Staab's dystonia diagnosis, his parents created Tyler's Hope for a Dystonia Cure. The non-profit organization is committed to funding neurology research to prevent and cure dystonia, as well as creating awareness about the disorder. Their top fundraisers include an annual golf tournament and poker tournament.
Each year, they also host a major summit on dystonia at the University of Florida. It brings top doctors and researchers from around the world together to share research and develop new ideas. The foundation has raised about $1.5 million with the promise of large renewable pledges, Rick Staab said.
Last year, all the Waffle House locations in Atlanta and the surrounding areas collected $22,000 for Tyler's Hope.
Its biggest accomplishment was funding the Tyler's Hope Center for Comprehensive Dystonia Care at UF's Movement Disorder Center. It opened in 2008 to offer dystonia patients care that goes beyond the examination room and includes speech and physical therapists, psychologists and other interdisciplinary specialists. Research is another big focus and within the next three months, the center will have three dystonia studies up and running, said program coordinator Jennifer Reichwage.
“We brought all these groups together so that if the patients need it, it's here,” she said. “All these people know what it is and know how to treat it and we can send them to really get comprehensive care in one place, which is very rare.”
Tyler's Hope and the UFMDC are presenting a seven-day Caribbean cruise July 31-Aug. 7 for dystonia awareness. Cost is $1,047.81 per person. For reservations, contact 1-800-959-SHIP or email@example.com.
To donate or get more information about dystonia, visit www.TylersHope.org or http://mdc.mbi.ufl.edu or call 273-5575.
A year and a half ago, their 7-year-old daughter Samantha started to limp and hop on her left foot. Then on a family walk through the neighborhood, she began walking backwards. She told them it was easier for her.
They'd seen unusual movements like this before with their son, Tyler.
At age 7, he was diagnosed with the rare neurological movement disorder dystonia. With the condition, muscles experience involuntary, and sometimes painful, contractions that cause twisting, repetitive movements or abnormal postures or positions. It can affect just one muscle, a group of muscles or the entire body. Tyler's dystonia, a genetic form, affected his ability to talk, walk and use his hands. He struggled to form a sentence, hold a glass or take a step because of the progressive disorder.
Today, Tyler is 12. Samantha is 8.
Tyler doesn't use a wheelchair anymore. Samantha can only get around in one.
Tyler is writing again. Samantha can only write briefly before fatigue sets in.
Tyler can put on his own pants. Samantha needs help getting dressed.
The Staabs, who also have a 5-year-old son, Luke, hoped it wouldn't happen again. But they knew there was a chance.
Samantha was diagnosed with dystonia in June 2008 — just five months after her older brother had the final procedure to help him live a better, more functional life. The procedure, deep brain stimulation, is a two-phase surgical therapy that aims to interrupt the faulty signals between the body's muscles and the part of the brain that controls movement — think rewiring a circuit or untangling crossed telephone lines.
Tyler has undergone DBS multiple times to treat his dystonia. Now it's Samantha's turn. On Wednesday, she spent more than six hours in surgery at Shands at the University of Florida for the first part of the procedure.
With Tyler, the Staabs have seen great improvements; for Samantha, they are hopeful for the same outcome.
“The biggest thing for us and for her is her inability to walk,” Rick Staab says. “Samantha is uncomfortable a lot of the time with sitting and sleeping because she is always leaning over and twisted, and we're hoping the DBS will allow her to do a lot that she can't do now.”Soundslide Update on Samantha Staab
“The biggest thing for us and for her is her inability to walk,” Rick Staab says. “Samantha is uncomfortable a lot of the time with sitting and sleeping because she is always leaning over and twisted, and we're hoping the DBS will allow her to do a lot that she can't do now.”
It's math time inside Pam Latsko's third-grade gifted class at Williams Elementary School. Desks are pushed together, forming mini-groups throughout the classroom. The lesson today is concurrent and similar shapes. Red, yellow and blue three-dimensional shapes are piled at each group's desks.
Kristan Smallwood sits next to Samantha. Smallwood is a paraprofessional with the public school system. She aides Samantha in the classroom and helps her get around the school in her black and turquoise wheelchair.
Dystonia first hit Samantha in her left leg. Then it went to the right arm, right leg and left arm. Her neck and back are the latest targets. Unlike Tyler, Samantha's dystonia has not affected her mouth and swallowing muscles.
Sitting is hard for her. She arches her back a lot and frequently repositions her body, searching for a slice of comfort. The dystonia has turned Samantha's feet slightly inward. Her head constantly tilts to one side.
“I think she hit that point to where she was uncomfortable, and she couldn't really focus on learning,” says Michelle Staab, who carries the gene for dystonia but doesn't have any symptoms. “It started to get in the way of school, and that's when we kind of knew DBS was the solution. She ultimately made the decision. ... She's a straight-A student, and she takes pride in that.”
When Samantha knows which shapes she needs, Smallwood picks it up and holds it down with one finger against Samantha's notebook. Samantha, holding her pencil like a chopstick, drags the pencil around the shape. As she works, Samantha sways back and forth in the swivel office chair that she uses when she needs a break from the wheelchair. It has arm rests, a feature she needs when sitting. When she tires of the office chair, she has another option — a large, red corduroy bean bag chair that sits in the corner.
“Sometimes she'll just stand up out of her chair or sit on the floor, whatever she can do to just make herself a little bit more comfortable,” Smallwood says.
Samantha slowly stands up when it's time to move to the next class. She leans on the desk as Smallwood rolls in her wheelchair from the hallway. Samantha grabs the sides and pulls herself into the chair. Smallwood puts Samantha's legs in the foot rests. As the kids wait to change classrooms, the girls crowd around Samantha. They sweep her hair from her face or make her laugh.
Latsko says Samantha's dystonia is deceiving. She moves, thinks and talks like all the other kids. The differences are subtle.
“Even the kids forget how much pain she is in because she's sitting on her own and moving her hands, but when she has to get up or catch a ball, you see it,” she says.
Tyler, Samantha and DBS
Neurosurgeon Dr. Kelly Foote and neurologist Dr. Michael Okun started UF's Movement Disorder Center in 2002. Four years later, the pair performed DBS on Tyler to help improve his severe dystonia symptoms. At the time, he was one of the youngest dystonia patients in the country to undergo the surgery.
During the procedure, doctors drill holes in the skull and insert electrodes that find the part of the brain that needs to be targeted for reprogramming. The patient is awake for some of the surgery so the doctors can know if they're in the right site. Once the area is pinpointed, the leads are put in place, and during a second surgery weeks later, pulse generators are implanted in the chest to power the leads. DBS is a standard treatment for essential tremor and Parkinson's disease. It is not standard for dystonia, but Okun says it's on its way.
“You have different brain regions, which are like different islands,” Okun says. “They're all speaking different languages to each other, and the signals between them get scrambled up. We put these devices in, eavesdrop on their conversation, and we change the way they communicate with each other. If we can, in some way, either normalize or change those connections, we have the potential to improve the symptoms of the disease.”
In 2007, Tyler's leads were repositioned because he experienced some minor skull growth. A year later, doctors added another lead to his brain's right side. After all the operations, his parents say he's more productive and can function on his own. It's still hard to understand him when he speaks. His hip is twisted, and his contorted leg pulls up and turns inward with each step. But this past summer, Tyler stopped using his wheelchair. He can run, climb stairs and use his hands again. He just started Bishop Middle School this year and continues to make straight As.
“These are really remarkable changes that happen, but they take time, and the families have to be really committed and very patient and understand the process,” Okun says of DBS. “This is not a light switch. ... This is a process, and it's potentially a very powerful process.”
The Movement Disorders Center has grown into one of the busiest medical centers for DBS surgery in the nation. More than 100 new DBS operations are performed there each year, Okun says.
For Samantha's DBS surgery, the main concern is her ankle. Dystonia can get so bad the muscles get stuck, and the bones and tendons change. If this happens, DBS might help with the pain but not relax the muscle. One of Samantha's ankles is moving toward that lockdown process, and Okun says they are “racing a bit on the clock.”
“If we can get the stimulator in now, maybe we can save some function of that ankle so that she can walk,” Okun says. “Our overall goal with Samantha is we want to get her out of the wheelchair. We want to get her walking again.”
Samantha wants that the most, too. She also misses writing, jumping on the trampoline and playing tennis and soccer.
Sitting on the floor in her house, Samantha whispers, “I can't wait until the 20th.”
The parking lot at Gainesville Country Club's clubhouse is packed on a recent brisk Sunday afternoon.
Samantha and her dad are heading there to eat for a father-daughter date. But instead of brunch, Samantha is going to get gifts and eat cake.
Inside the two front rooms, a surprise party with friends and family is waiting for her. Samantha plans to shave her head for the surgery and donate the hair to Locks of Love. So for the party, everybody was asked to bring hats for her.
Before Samantha arrives, her brothers write well wishes on slips of neon paper and drop them in a bucket. A sign says the “words of hope” will be read to Samantha after surgery. The rooms are decorated with balloons and Hershey's Kisses candy. On the gift table sit two foam wig heads. “Dystonia Sucks!” baseball cats, made by Tyler, cover the long, dark wigs. Behind the table is a sheet cake decorated with “We Love You Sam” and pink, purple and yellows hats.
In the corner is the biggest decoration: a giant hat. It's covered in pink and white tulle, boas, glitter and pictures of Sam with friends and family.
Soon the room is full of people. Then, Michelle Staab gets the call from her husband. “Shhh!” The room quiets; a minute later, the door turns, and Rick Staab pushes Samantha inside the crowded room.
“Surprise!” everyone yells. Her brothers and their friend burst out of the top of the giant hat, showering the room with colorful confetti.
Samantha giggles, her dimples appearing through a shy smile. Her face turns a rosy pink. Tyler and Luke run over to her. Tyler lovingly rubs her shoulder. Luke hands his sister a bouquet of red lollipops.
Samantha is shocked but happy to see everyone.
“Tyler had so much attention around him when he was going through this stuff that we wanted to do something special for Samantha and encourage her,” Rick Staab says. “We thought it would be fun.”
She leaves the restaurant with a car full of stylish hats and a big smile.
Reality sets in
A week after the hat party, on a brighter, warmer day, Samantha arrives at the Regis salon in the Oaks Mall.
Stylist Joy Kubotsuka greets her with a happy smile and grabs a smock to put around her neck.
Michelle Staab, holding a wig, helps her daughter get out of her wheelchair. Kubotsuka compliments Samantha on her beautiful hair. Then she gathers it into mini pigtails with rubber bands. Samantha squirms a little with each ponytail she finishes. Slowly, her calm attitude shifts to nervousness. She bites her bottom lip and steals glances of herself in the mirror. The stylist finishes all the ponytails and then takes her scissors to snip them off. She starts from the back. She makes her way towards the front, and Samantha drops her head into chest.
She begins to sob. Hard. Michelle Staab rushes to her side. She holds her little girl tight. Their faces are pressed together, and she talks to her softly.
It's a moment that makes everything real — the dystonia, the surgery, the pain. Tyler and his friend stand outside. He sends his mother a text message that he wants to shave his head to make his sister feel better. In fact, the whole family and some of the children's friends' moms also had offered. Samantha told them not to.
Moments pass, and Michelle Staab, with tears on her face, moves back into her seat in front of Samantha. Kubotsuka explains it's time for the clippers. She says comforting words to Samantha as she buzzes off her short hair.
Samantha still cries and objects. Michelle Staab seems like she just wants it to end. Kubotsuka finishes and pulls the wig on Samantha's head. She is still sad, quiet.
“It's a lot easier with a boy,” says Michelle Staab, wiping tears from her face.
The Staabs wake Samantha at 5:30 a.m. to get ready to head to Shands. She slept with them the night before. They wanted to make sure she got enough rest.
She says goodbye to her brothers. Tyler seems more nervous than she is. They get to the hospital by 6 a.m. and head upstairs to the second floor. When Samantha leaves for pre-op, she's holding a gift from Tyler — a stuffed bear wearing scrubs. When Tyler had his DBS, he had one, too.
Samantha is calm. No tears. No fear. Rick Staab says she's prepared and wants it to happen. They're a bit anxious though.
He thought it would be easier. They've done this several times before with Tyler. But the feeling of helplessness, concern and worry are still there. They got Samantha a metal Jesus pin for the surgery. A nurse pins it on Foote's scrubs before surgery.
At almost 9 a.m., the surgery starts. For four hours, the Staabs don't hear anything. Then the nurse calls. Things are moving slowly, but going well. An hour later, Okun calls with an update and lets them speak to Samantha. Still lying on the operating table, she tells her father she's doing fine. Shortly after 3:30 p.m., the surgery is over. Okun comes to brief the Staabs about the surgery. He takes out his iPhone and shows them a video he shot during surgery. It's Samantha telling Tyler that her brain is bigger than his. The Staabs crack up with laughter.
Samantha finally makes it to her room. She's still groggy and a bit nauseous. They order a CT scan just to see if everything is as it should be. The test comes back clean. Later, they flip the TV to “American Idol.” Samantha loves watching the auditions. When it ends, Rick Staab heads home, and his wife stays the night at the hospital.
The next day, Samantha is home before noon. She's sitting with her father on the couch, resting. She'll spend most of the day sleeping. Her parents will ice her head throughout the day to help with the swelling and itching. It will take several weeks to months to see if DBS was a success, but everyone seems optimistic.
“She's doing very well,” Rick Staab says. “They were very pleased with the placement of the leads. Based on her reaction during the surgery, they think she'll see some good results. It gives us a lot of hope.”
Lashonda Stinson Curry can be reached at firstname.lastname@example.org or 374-5038.
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