Potential Winter 2013

potential A publication of Kennedy Krieger Institute

To the Brink

and Back:

Severe Behavior & Self-Injury

Rehabilitation:

Three-time Cancer Survivor Finds Her Strength

Overcoming Chronic Pain Is it Picky Eating or Something More?

We are all born with great

potential

shouldn’t we all have the chance to achieve it?

A publication of Kennedy Krieger Institute Volume 13, No. 2 • Winter 2013

Inspiring Potential Autographs Special education teacher Katie Bates is inspired by a student who comes into his own at Kennedy Krieger High School.

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FEATURES

Letter from our

President It’s often the simplest moments in life that we take for granted—a hug from a child, sitting down with our families for a meal, or sharing laughter with family and friends.

Yet for many families of children with severe behavioral or feeding disorders, chronic pain, and other disorders and injuries, these moments are difficult to come by. For these families, Kennedy Krieger is often the place they turn to when hope is all but lost.

In this issue, you’ll read about families who came to Kennedy Krieger for the care they couldn’t find anywhere else. When Luke McNair was in the third grade, his behavioral problems grew so severe that he was breaking windows with his head and hitting his three-year-old brother.

His parents became so desperate that they considered making him a ward of the state, until a doctor recommended Kennedy Krieger’s neurobehavioral unit as a last resort.

They flew across the country to get him the treatment he needed, and Luke emerged a “ten-times-better version of himself,” according to his mother.

When 13-year-old Perry Zimmerman needed intense physical therapy after surgery for osteosarcoma left her without the ability to walk, her family traveled to Kennedy Krieger because it was the only rehabilitation facility that offered what she needed.

And after Katie Bickford suffered debilitating chronic pain that left her unable to walk or attend school, an expert recommended the Pediatric Pain Rehabilitation Program at Kennedy Krieger; today, Katie is a regular teenager again.

All of these families turned to us for the expertise to help their children heal, and we gave them life-changing treatment. We are proud to play a role in helping these and so many other families get their lives back.

Sincerely, Gary W. Goldstein, MD

Stronger After cancer and chemotherapy left her so fragile doctors said she might never walk again, Perry found her strength with the help of Kennedy Krieger’s Specialized Transition Program.

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Beating the Pain After multiple doctors could not help relieve her suffering from pain so severe she could no longer walk, Katie Bickford sought help at Kennedy Krieger’s Pediatric Pain Rehabilitation Program.

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The Comeback Kid The Neurobehavioral Unit at Kennedy Krieger helps turn around a young boy with self-injurious and aggressive behavior.

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Program Spotlight Is it Picky Eating or Something More?

Kennedy Krieger’s Pediatric Feeding Disorders Program helps children overcome feeding problems.

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RESEARCH Frontiers Identifying the Cause, Pursuing the Cure A medical breakthrough by Kennedy Krieger researchers offers hope for new treatments for Sturge-Weber syndrome.

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IN MY Own Words Marshall Garber, Colorado Dreaming: When a spinal cord mass left him paralyzed, Marshall’s life changed forever. It changed again during an adaptive ski trip arranged by Kennedy Krieger.

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News briefs & Events Kennedy Krieger in the news and upcoming Institute events. 13

potential Editor

Art Director

Contributing Writers

Photography

Kristina Rolfes Katie Bates, Martie Callaghan, and Abigail Green

Proofreader

Nina K. Pettis

Creative Services manager

Sarah Mooney

Amy Mallik

Steve Parke and Bill Schilling AVP of Marketing and PR

Bryan Stark

Media Inquiries

Elise Babbitt-Welker Publication Inquiries

443-923-7330 or TTY 443-923-2645

For appointment and patient referrals, please visit kennedykrieger.org or call 888-554-2080.

Potential is published by the Marketing and Public Relations Department of the Kennedy Krieger Institute, 707 North Broadway, Baltimore, Maryland 21205. Kennedy Krieger Institute recognizes and respects the rights of patients and their families and treats them with courtesy and dignity. Care is provided in a manner that preserves cultural, psychosocial, spiritual and personal values, beliefs, and preferences. We encourage patients and families to become active partners in their care by asking questions, requesting resources, and advocating for the services and support they need.

If you do not want to receive future communications from Kennedy Krieger Institute, you may notify us by emailing unsubscribe@kennedykrieger.org or visiting kennedykrieger.org/unsubscribe. © 2013, Kennedy Krieger Institute

On the cover: Thirteen-year-old Luke McNair, former patient at the Neurobehavioral Unit at Kennedy Krieger Institute.

To view Potential online, visit potentialmag.kennedykrieger.org.

Inspiring potential

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potentialmag.kennedykrieger.org

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After cancer and chemotherapy left her so fragile doctors said she might never walk again, Perry found her strength with the help of Kennedy Krieger’s Specialized Transition Program.

At the tender age of 13, Perry Zimmerman has already battled a lifetime’s worth of illnesses. Born with retinoblastoma, a type of eye cancer, Perry developed a related brain tumor at age two that doctors did not expect her to survive. For the Zimmerman family, watching their two-year-old daughter go through chemotherapy was heartwrenching, but when it was over, Perry emerged cancer-free.

For several years, cancer remained a distant, long-ago memory, until one morning two years ago, Perry complained about a lump on her left thigh, and showed her mother. “The second I saw it, I knew what it was,” her mother, Anne Zimmerman, recalls.

An MRI showed a large tumor. For the third time, Perry had cancer—this time, osteosarcoma.

Limb-sparing surgery removed the tumor, but because it was so large, surgeons had to reconstruct her leg. She wore a cast from waist to toe for three months, and doctors said she might never walk again. The surgery and nine months of chemotherapy took their toll, leaving her body ravaged and her bones weak.

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potentialmag.kennedykrieger.org

Everything She Needed All in One Place

Keeping Up With School

For patients like Perry who have gone through surgery and chemotherapy, their struggle does not end when the cancer is gone.

They still face fatigue, weakness, pain, immobility, and low bone density, and need months of rehabilitation to recover.

In between therapy, Kennedy Krieger teachers continued her schooling, so she wouldn’t fall behind. Kids recovering from all types of injuries or conditions are grouped together in a classroom, but each has an individualized school curriculum and therapy regimen.

Despite the multitude of rehabilitation facilities near the family’s home in Manhattan, none of them could offer the intensive five-day-a-week rehabilitation therapy Perry needed. “She just wasn’t getting what she needed in New York, and I knew it,” says Zimmerman. So they temporarily relocated to Maryland, so Perry could receive treatment at Kennedy Krieger’s Specialized Transition Program (STP).

“The educational piece here in an outpatient setting is unique,” says Perry’s teacher at STP, Ali Adler. “Since teachers are employed by Kennedy Krieger and not a school system, we have the flexibility to implement individualized curriculums. For Perry, I was able to implement the curriculum for the school she would be attending upon discharge…We try to simulate as much of a normal school day as possible to help ensure a smooth reentry into school.”

Assistant teacher Brandt Dunn, who most days can be found wielding a guitar and singing songs to the kids, brought a little bit of whimsy to the classroom.

“He made the whole experience fun for Perry,” says Zimmerman. And Perry also made an impression on Dunn. In honor of Perry, he cut and donated his long hair to make real-hair wigs for others with cancer.

“STP really is a unique day program,” says Katlyn Recchia, Perry’s physical therapist.

“It’s set up as a school day, but kids also receive three to four hours of intensive physical, occupational, and speech therapy, along with neuropsychology. To have that in such an intensive format allows kids like Perry, who have such great challenges, the opportunity to progress at their own speed.”

When she arrived at Kennedy Krieger, Perry was fragile, weighing only 45 pounds, and in a wheelchair. She couldn’t move or bear any weight on her leg, and had lost nearly all muscle tone, recalls Recchia.

Intensive daily physical therapy helped Perry transition from a wheelchair to a walker, and rebuild muscle that was lost.

Weight-bearing exercises helped strengthen her muscles and bones, and electrical stimulation helped re-teach her muscles how to work again. A series of casts and braces helped give Perry the ability to extend her leg.

Dunn says he is inspired almost every day by the kids who come to STP. He sees kids recovering from all types of accidents, brain injuries, surgeries, and cancers in his classroom. “I tell the kids all the time, ‘You guys are way tougher than I am,’” he says. As for Perry, he says, “She’s always been tough, but now she’s stronger, more confident, and vocal…I’ve seen so much growth in Perry.”

Taking it All in Stride

learn about “It was great to on er’s rehabilitati Kennedy Krieg was no similar program. There York where hospital in New y hysical therap you could get p t day and patien several times a rn a e same time, le th t a d n a , re ca .” hool curriculum and follow a sc y’s mother man, Perr –Anne Zimmer

Through it all, Perry kept working hard. When setbacks occurred— Perry broke both an ankle and a wrist—therapists adjusted her regimen and kept her moving forward. “She had fallen so many times at home that she had a fear about movement,” says Recchia.

“She learned to trust me and the other therapists when we said, ‘You are going to be okay if you take this step.’ She worked incredibly hard and was very brave.” (above) Intensive physical therapy and educational instruction helped Perry recover without falling behind in school. Assistant teacher Brandt Dunn made the classroom fun.

Slowly, Perry progressed from not being able to put any weight on her leg to walking with a walker, to crutches, and finally, to taking her first steps unassisted. For Perry and her family, walking was a monumental feat. “You have to realize how hard it is to get someone to walk again,” says Perry’s mom.

“They got her walking and that was her goal.”

“To her, it is just so freeing to be able to walk around carrying something instead of crutches taking up her hands,” she adds. “She’s proud and happy.”

In August, the family returned to New York with a home program to follow. And despite her medical problems over the past two years, Perry has not fallen behind on her schoolwork and will return to her same school with all of her friends, making it as seamless a transition as possible.

“Coming to Kennedy Krieger was worth the travel and relocation,” says Zimmerman.

“It’s so amazing to see how far she’s come.” n Kristina Rolfes

(opposite) Perry in Annapolis after rehabilitation.

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Beating the Pain After multiple doctors could not help relieve her suffering from pain so severe she could no longer walk, Katie Bickford sought help at Kennedy Krieger’s Pediatric Pain Rehabilitation Program and today, she has her life back.

k

atie Bickford remembers the pain starting in the 7th grade—it began in her spine and radiated out all over her body. The pain got so bad she couldn’t walk. To make matters worse, she fractured her heel, and then her arm, so it hurt to use crutches. She used a wheelchair to get around, but in her house where the wheelchair wouldn’t fit, she resorted to crawling, developing thick calluses on her hands and knees. She could no longer function and stopped going to school.

Her bone fractures were a result of weak bone density, likely caused by the chemotherapy she underwent at age 4 for acute lymphoblastic leukemia, now in remission. But doctors could find no direct cause for the full body pain she was experiencing. She saw multiple specialists, and despite trying medication and outpatient therapy, nothing seemed to help. Over the years, the pain only worsened. Eventually, doctors recommended that she see a psychiatrist.

Katie felt like the doctors didn’t believe her and thought she was making up the pain. “I used to stay up at night and cry,” says Katie. “I felt like I was defective.”

“It was terrible,” recalls Mary “ML” Bickford, Katie’s mom. “We were scared.

We believed in her and we knew the pain wasn’t in her head, so we kept fighting for her.”

More than 30 percent of children and adolescents experience chronic pain significant enough to cause suffering, school avoidance, limited participation in physical and social activities, and disruption of sleep and appetite.

A psychiatrist told them about the Pediatric Pain Rehabilitation Program at Kennedy Krieger, which specializes in treating chronic pain resulting in disability. At 14, she was diagnosed with complex regional pain syndrome, a chronic pain condition that is believed to be the result of dysfunction in the central or peripheral nervous systems. Sometimes, the pain may begin with an illness, injury, viral infection, or developmental or psychological challenge. In some cases, like Katie’s, chronic pain can continue even when the original illness or injury has healed, or when no source of the pain can be identified.

When Katie arrived at Kennedy Krieger, she was cautious about expecting too much. She had been through therapies before, but nothing had helped. This is not unusual, according to Cynthia Ward, PsyD, pain services coordinator. “When patients come to us, they have tried everything—numerous medications, multiple specialists, outpatient services—without results,” she says.

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(Pictured top to bottom, left to right)

Katie, 18, after her recovery from debilitating pain: enjoying some tunes; with her new pickup truck, Dixie; working as a baker at Café on 26 in Ocean View, DE; with her parents, ML and Gary Bickford.

Understanding Chronic Pain What’s different about Kennedy Krieger, Dr. Ward says, is its integrated approach to treating pain. Patients are evaluated and treated by an interdisciplinary team, including specialists in rehabilitation medicine, education, neuropsychology, therapeutic recreation, psychiatry, and occupational, physical, and behavioral therapy. The team not only addresses the underlying biological basis of the pain, but also teaches patients and their caregivers the tools they need to cope through behavior therapy.

“Behavior therapy is ideal for helping patients like Katie cope with their pain and their anxiety about pain, so they can do the challenging work of rehabilitation,” explains Keith Slifer, PhD, director of the Pediatric Psychology Consultation Program. By setting achievable short-term goals for patients, therapists help patients gain confidence as they develop better tolerance and regain physical conditioning. This approach reinforces small successes, instills a sense of hopefulness, and challenges the individual to work hard to return to their typical activities. “We help patients reset the focus from what they can’t do to what they can do,” says Dr. Slifer.

One of the techniques therapists use is biofeedback—using instruments to measure physiological responses, such as brainwaves, heart function, breathing, muscle activity, and skin temperature. When patients implement cognitive and behavioral coping strategies, they can see themselves calming down on the computer screen, reestablishing a sense of control over their physiology.

For Katie, guided imagery helped her cope with the pain. “I imagine I’m with my horse Piper or doing other things, and it helps me be okay with the pain,” she says. “When it gets really bad I’ll stop and listen to a CD and focus on the music, and it resets where I am. My whole brain takes a break for a second.”

With her physical therapist, Katie gradually began rebuilding the muscle she had lost while in a wheelchair. She used desensitization techniques to learn to put weight on her foot, and soon moved on to walking with crutches, and then with a cane.

Getting Her Life Back When her parents visited her, they were thrilled with her progress. “When she saw us coming down the hallway, she got up out of her wheelchair and was walking,” recalls Gary Bickford, Katie’s father. “By the second week, she said, ‘I’m done with the wheelchair. I’m done with the crutches. I don’t need these anymore.’” The bright light was back in her eyes and the smile back on her face.

Katie’s parents met with the team to learn how they could help Katie learn to not focus on the pain. “It’s hard because when my only baby says ‘I hurt,’ I want to hold her and make sure she’s okay, says ML. “But what I really need to do is help her focus on something else—not deny that she’s in pain, but not focus on it.” With the help of her therapists, Katie learned how to better communicate what she was feeling, and her parents learned how to respond to her.

When she had progressed enough to be discharged, Katie was armed with the coping techniques she had learned to help get back to the life she knew before the pain. When summer came, she was at the beach with her friends every day like any other teenager. And in the fall, Katie was able to go back to school, participate in activities like JROTC, and even ride her horse again.

The highlight of her year was going to the prom with her new boyfriend.

Although she still feels pain sometimes, Katie is able to cope with it, and it no longer rules her life. Now she has time to focus on more important things, like being a teenager.

“I definitely feel Kennedy Krieger gave me my life back. I couldn’t ask for anything more.” n Kristina Rolfes

“

The quality of staff at Kennedy Krieger was amazing. They’re like angels. There is no way I can put into words how wonderful a relationship they made with Katie, and how well they were able to motivate her and turn her around.

–ML Bickford, Katie’s mother

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potentialmag.kennedykrieger.org

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The

Comeback Kid

The Neurobehavioral Unit at Kennedy Krieger helps turn around a young boy with self-injurious and aggressive behavior.

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Chrissy McNair describes her son Luke, 13, as “one of the happiest kids I’ve ever met.” He usually wakes up in a good mood and likes cracking jokes with his two brothers. He loves Top-40 music and animals, and has been riding horses since he was 3 years old. “The best word for him is passionate,” says his mother. “He passionately loves the things he loves and he passionately hates the things he hates.” But when Luke would get upset, that passion began to manifest in disturbing and even dangerous ways.

Diagnosed with autism at age 2, Luke had always struggled with getting frustrated easily when he didn’t get what he wanted or had to wait for something. But his outbursts weren’t out of the ordinary compared to other Luke McNair with horse, Izzy. kids his age, and he could be easily redirected. By the time he was 8 years old, however, his behavior had gotten worse. Luke would bang his head on hard surfaces when he got upset. He’d occasionally hit his teachers or other adults who worked with him. But when he started getting aggressive towards his 3-year-old little brother, his parents knew they had to do something.

The McNairs sought treatment for Luke at an outpatient autism program near their home. Luke went there every day during the summer of 2008 and improved a lot. But several months later, his behavior began to take a turn for the worse again. By spring break of his third-grade year, Luke was breaking windows with his head, says Chrissy. “Every little thing set him off.”

“Things were so desperate we were considering making him a ward of the state, because our home was not safe anymore and we didn’t know what to do,” she says. A psychologist at the University

of Nebraska recommended the Neurobehavioral Unit (NBU) at Kennedy Krieger.

Treatment of Last Resort Established in the 1980s, the NBU’s unique, 16-bed inpatient program is known around the country and even abroad for providing intensive behavioral assessment and treatment for children and young adults with developmental and intellectual disabilities who have severe behavioral problems.

“We’re the only very intensive inpatient program like this in the country, if not the world,” says Lee Wachtel, MD, medical director of the program. The NBU is often the last resort for families who have spent years trying different therapies and medications without success.

“Historically, it’s been a very successful program, and it’s built an excellent reputation within the field of applied behavior analysis,” says behavior analyst Sung Woo Kahng, PhD. During the last five years, 88 percent of patients discharged had reduced problem behaviors by at least 80 percent, and 84 percent had maintained those gains months later. >> potentialmag.kennedykrieger.org

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The NBU takes an interdisciplinary approach to treatment, relying on input from behavior analysts, psychiatrists, pediatricians, neurologists, nurses, social workers, and speech and language pathologists. “Our interdisciplinary team is outstanding because we work so well with one another, and the range of services we’re able to provide patients and their families is very comprehensive as a result,” says Louis Hagopian, PhD, program director of the NBU.

“The staff was phenomenal.

They made us feel at ease…

They didn’t downplay how hard it was for us and they didn’t make pie-in-the-sky promises, but they were clearly very confident in what they do and very professional.”

The decision to send their 9-year-old son more than 1,000 miles away from home for five months was not an easy one for the McNairs. “I’d never been away from Luke before,” says Chrissy.

“But we didn’t see that we had any other options. It was either that or medicate him to the point he wasn’t himself anymore.”

In April 2009, Chrissy, her husband, Matt, and Luke arrived at Kennedy Krieger after a horrible trip from Nebraska to Maryland, during which –Chrissy McNair Luke screamed and hit his parents the entire flight. “If there was any doubt that we were doing the right thing, the flight out confirmed everything,” says Chrissy. When they arrived at Kennedy Krieger, the staff greeted them and took Luke off for evaluation while his parents met with various therapists. “That first day I cried the whole day,” says Chrissy. “The staff was phenomenal.

They made us feel at ease…They didn’t downplay how hard it was for us and they didn’t make pie-in-the-sky promises, but they were clearly very confident in what they do and very professional. By the time I left a few days later, I felt like he was in really good hands.”

The McNairs flew out to visit Luke almost every week, making 20 trips to Baltimore in five months, thanks to frequent-flier miles and grant money from some autism groups in Nebraska. And every night, Luke called home at bedtime to say goodnight.

The Power of Rewards During the first several weeks of his stay, Luke’s behavior was assessed under safe conditions to identify the triggers for his problem behavior. When he arrived at the NBU, Luke was engaging in self-injurious or aggressive behaviors more than 400 times a day. The following weeks were spent eliminating those triggers and teaching him new skills, including how to more appropriately get what he wants. “We used a token economy with Luke to help him learn to control his behavior,” explains Dr. Kahng. If he did not engage in aggressive behaviors, he would earn tokens that he could trade in for access to preferred activities or outings, like being able to play a favorite video game or go out to a fast-food restaurant.

Luke’s NBU treatment team also implemented a levels system for him, explains his mother. When he was not having aggression, he was on Level 3, and was rewarded with attention and access to Luke enjoying lunch and his things he wanted. Any sign favorite video game. of aggression sent Luke to Level 2, where he lost access to his preferred things and did not receive a lot of positive reinforcement. If his behavior worsened to the extent that there was risk for injury, he would reach Level 1, and his parents or caregivers would have to use physical means to keep him safe. Another key to the levels system was earning rewards at several very specific times each day—for example, 10 a.m., not “after breakfast”—which appealed to Luke’s need for order and routine. This behavioral treatment, in combination with medication, greatly improved his behavior and mood.

Four years later, Chrissy says the systems implemented at Kennedy Krieger still work. “The principles of what we learned there, we still do.” Over time, the family has been able to relax the behavioral plan somewhat. They no longer need to train babysitters and everyone who works with Luke on the three-step prompting system, and Luke no longer needs to receive his rewards immediately or at very specific times. While pleased with her son’s progress, Chrissy is careful with her word choice: “He’s not cured—he’s always going to struggle with this stuff—but he’s changed.” >> continued on page 10

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After spending five months in intensive inpatient neurobehavioral treatment, Luke is now back to horseback riding and enjoying life again.

Luke with his teacher now, Mr. George Rieger.

Program Spotlight

Is it Picky Eating or Something More?

Kennedy Krieger’s Pediatric Feeding Disorders Program helps children overcome feeding problems.

Catering to a child who is a picky eater is like being a short-order cook: chaotic. Dinnertime becomes a war zone, leading to hopeless battles fought over vegetables and macaroni and cheese.

Picky eating is as normal as potty-training, a rite of passage in childhood development. Taste buds evolve and food preferences expand in these early years. Even the best of parents can have a difficult time getting their child to eat. In fact, picky eating is one of the most common occurrences in children, often outgrown as the child reaches adolescence.

But if eating behavior inhibits normal developmental and physical growth processes, it could be something much more severe—a pediatric feeding disorder.

“The difference between a fussy eater and a child with a feeding disorder is the impact the eating behavior has on a child’s physical and mental health,” says Peter Girolami, PhD, clinical director of the Pediatric Feeding Disorders Program at Kennedy Krieger—a leading program that was one of the first of its kind in the United States and the largest in the world to treat pediatric feeding disorders.

Pediatric feeding disorders are more common than most think, afflicting up to 10 percent of all

infants and children, according to published literature.

Children classified as having a feeding disorder do not consume enough calories and nutrients to promote healthy growth and development. It is important to differentiate between a picky eater, who may consume a restricted but still nourishing diet, from a child who consumes, for example, only three to four types of foods, eliminating entire food groups “The difference and compromising healthy growth.

between a fussy eater Feeding disorders are not all created equal. Some children can have and a child with a aversions to food groups or certain feeding disorder is textures and colors, while others lack the skills to self-feed or refuse to eat the impact the eating any food at all. The assortment of behaviors that constitute a feeding behavior has on a problem vary from child to child and child’s physical and include food refusal (e.g., crying, head turning, spitting out food, throwing mental health.” utensils, packing or holding food in mouth, aggression, and getting –Peter Girolami, PhD Clinical Director of the Pediatric Feeding out of the seat), coughing/gagging, Disorders Program at Kennedy Krieger and vomiting. Many children with feeding disorders also suffer from a serious medical, behavioral, or psychological condition that can contribute to the onset of the feeding disorder—30 percent of children with developmental delays have difficulty feeding at some point during their childhood.

Other common medical conditions that are associated with feeding disorders include gastroesophageal reflux disease (GERD), food allergies, cancer, and complications from premature birth. Children who undergo surgeries unrelated to food may also develop feeding problems, as feeding tubes can throw them off track. >> potentialmag.kennedykrieger.org

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Early diagnosis and treatment are extremely important when it comes to a feeding disorder. The longer it is left untreated, the more complex it can become—in extreme cases, children may need a feeding tube. Feeding disorders can also lead to serious complications from nutritional deficiencies, such Common symptoms of a as anemia. Undernourished pediatric feeding disorder: children are also at risk for developmental delays, • An abrupt change in eating habits including the inability to lasting longer than 30 days crawl, walk, and talk, which • Delayed development of the skill set could lead to oral, motor, and necessary to self-feed or consume sensory problems. continued from page 9

higher textures

Since there is no common cause or symptom that is characteristic of all pediatric • Choking/coughing during meals feeding disorders, each case is unique with varying • Unexplained fatigue, loss of energy challenges to effective • Disruptive behavior during mealtime treatment. Both biological and social interactions need to be individually addressed for each child during diagnosis and when developing an appropriate treatment program. Kennedy Krieger’s Pediatric Feeding Disorders Program takes an interdisciplinary approach. A team of professionals with • Weight loss or failure to gain appropriate weight

expertise in many specialties—from leaders in pediatrics to behavioral psychology and speech pathology—works together to help each child modify behavior, build motor skills, and treat associated medical conditions.

“It’s not a one-size-fits-all model,” explains Dr. Girolami.

“Our team of leading professionals addresses the many causes of feeding disorders and their associated complications so we can give the child and family the best care available.”

Part of the child’s care involves training for the family by every member of the child’s treatment team. “The goal is that when the family leaves, they’re self-sufficient to do the meals and introduce new foods, and to trouble-shoot any problems that may come up,” says the medical director of the program, Eric Levey, MD. “I’d say 80 to 90 percent of the time, families continue to make progress without any additional help on a regular basis.”

For the many families whose children have emerged from the program no longer saddled by feeding disorders, the program has made a profound difference in their daily lives. “Every parent wants their child to be able to sit at a table and eat a meal together as a family,” says Dr. Levey.

And thanks to the feeding disorders program, many more families will be able to do just that this Thanksgiving.

To learn more about feeding disorders and treatment options, visit feedingdisorders.kennedykrieger.org.

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A Complete 180 Dr. Kahng says the McNairs are an outstanding example of the importance of training caregivers to continue the behavioral plan once a child is sent home from the NBU. “We can change the behavior on our unit, but if it doesn’t transfer to home, that’s basically a failure,” he says. “Our goal is to teach parents to be the expert in their child’s behavioral treatment. That’s a crucial role in their success.”

Luke, whose aggressive behaviors reduced from 400+ a day to less than nine, is happy 90 percent of the time now, says his mother.

The McNair family (pictured left to right): Luke; his father, Matt; brother Marcus; mother, Chrissy; and brother Jackson.

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Chrissy says that while the decision to send Luke to Kennedy Krieger was difficult, she would do it again 100 times over given the “complete 180” she saw in Luke’s behavior.

“It used to be, 90 percent of the time he was upset and 10 percent of the time he was happy—his normal self,” she says. Now, it’s reversed. A couple weeks after he left the NBU and went back home, Luke was down from 400+ aggressive behaviors a day to less than nine. These days, he has even fewer. And when he does get upset now, the episodes are much less intense than they used to be, says his mother. “He’s just a 10-times-better version of himself than he was before.” n Abigail Green To learn more about the Neurobehavioral Unit and treatment options, visit kennedykrieger.org/ neurobehavioral-continuum.

research frontiers

Sturge-Weber Syndrome and Port-Wine Stain Birthmarks: Identifying the Cause, Pursuing the Cure

Anne Comi, MD and Jonathan Pevsner, PhD

Researchers at Kennedy Krieger recently announced the groundbreaking discovery of the genetic mutation that causes Sturge-Weber syndrome and port-wine stain birthmarks.

After almost fifteen years of study, Anne Comi, MD, director of the Institute’s Hunter Nelson Sturge-Weber Center, and Jonathan Pevsner, PhD, director of Bioinformatics, confirmed their original hypothesis: the syndrome and the birthmark are caused by the same somatic mutation (an alteration in DNA that occurs after conception) now known to be in the GNAQ gene.

The discovery is important news for individuals and families affected by the syndrome, a neurological and skin disorder associated with the port-wine birthmark and with glaucoma, seizures, intellectual impairment, and weakness on one or both sides of the body. For the first time, parents can be assured that their child’s Sturge-Weber syndrome (SWS) was not caused by an injury sustained during pregnancy. “There is nothing the mother or the family did or did not do to cause SWS,” Dr. Comi says.

By establishing this genetic mutation as the cause of SWS, researchers also have proven that SWS is not an inherited condition.

“We don’t know exactly why SWS happens in some individuals and not others,” Dr. Comi explains. “Somatic mutations occur at random throughout the lifetime of an individual in a localized area of the body. We think this particular alteration occurs in the first trimester of fetal development.”

Dr. Pevsner’s laboratory employed whole genome sequencing, a technology that allows examination of the billions of nucleotide pairs that form DNA, to identify the

mutation. Affected tissue and unaffected tissue and blood samples from individuals with SWS were analyzed.

Matt Shirley, then a graduate student in the Pevsner lab, was able to identify one somatic mutation common to affected samples.

The study, published in May 2013 in the New England Journal of Medicine, represents a turning point, Dr. Pevsner says. “We suspected for decades that a somatic mutation was the cause of Sturge-Weber syndrome and port-wine birthmarks, but the technology to test that theory did not exist—until now. The advancements associated with whole genome sequencing and the development of nextgeneration sequencing tools finally allowed us to test and prove the hypothesis.”

Sturge-Weber syndrome is rare, affecting approximately one in 20,000 births, while port-wine stain birthmarks are more common, affecting approximately one million individuals in the United States. Current treatment options for children with SWS are limited, but include medications to reduce the likelihood of seizures and strokelike episodes, eye drops or surgery to manage glaucoma, and physical rehabilitation.

With this new finding, much optimism surrounds future clinical trials. “We have real hope that in the next five to ten years—perhaps sooner, perhaps a little longer—there will be new treatments developed to inhibit the overactivation of those pathways,” Dr. Comi said. “We hope to move quickly toward targeted therapies, offering families the promise of new treatments for the first time.” n Martie Callaghan

For patients with Sturge-Weber syndrome, like Madisynn Rodriguez (left), the discovery offers hope for new targeted therapies.

To learn more about current research initiatives at the Institute, visit kennedykrieger.org/research.

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In My own words

In My Own Words: Marshall Garber

“I went from having no idea I could stand to realizing that my aspirations and my hopes and dreams could be fulfilled.”

Marshall Garber is a patient at Kennedy Krieger’s International Center for Spinal Cord Injury.

A

few years ago, I developed a mass on my spinal cord that left me paralyzed. My family sought out the best place to help me, which was Kennedy Krieger. Going into treatment, I didn’t have any intention of forming a family there, but in a way, I did. Multiple people at Kennedy Krieger had an impact on me, but Dr. Becker has been one of the most influential people in my recovery.

so beautiful. The speed of going down the mountain and the adrenaline from knowing I was skiing... it was one of the best experiences of my life. My core muscles were pushed in ways that they hadn’t been before. It felt so good to be able to conquer that mountain. I was just so overwhelmed with how awesome that place was—it’s hard to put into words. I really fell in love with Colorado.

During an adaptive ski trip to Colorado that Dr. Becker arranged for patients, I told him that I had come to terms with the knowledge that I would never walk again. The next day, I had a test done as part of a study Dr. Becker was working on, and he told me that my muscles were strong enough to stand. I didn’t believe him, but he said, “Let’s try it.”

He helped me up and then let go, and I stood free. It blew my mind away. I started crying like a baby. I was so overwhelmed, because I had no idea any of that was going to happen. I’ll never forget it. I went from having no idea I could stand to realizing that my aspirations and my hopes and dreams could be fulfilled. Everyone has been saying from the beginning, “You gotta get there.” He showed me I’m there.

Since the ski trip, I’ve been able to work at a level much greater. I can feel and move my legs, and just recently, I’ve gained the ability to do some walking. If I hadn’t gone on the trip, I don’t think I would know right now that I could stand or walk.

That ski trip had such an amazing impact on me. It took a couple of days to figure out how to ski, but once I had it down, it was like riding a bike. It was the fastest and farthest I’ve gone without wheels in as long as I can remember. The freedom was just incredible, and the scenery was 12

I’m not sure what the future will hold, because there are so many options. For the time being, I want to focus on therapy, so I can get back on my feet. I want to study photography in college, hopefully in Colorado. I don’t know where I’m going to end up, but I know I’m going to be moving forward.

news briefs & events

Festival of Trees

NOVEMBER 29 — DECEMBER 1, 2013 MARYLAND STATE FAIRGROUNDS

Celebrating its 24th year, Kennedy Krieger Institute’s Festival of Trees is an exciting three-day, holiday-themed extravaganza that raises funds for research, treatment, education, and community programs at Kennedy Krieger Institute. Event features include: • More than 600 spectacular holiday trees, wreaths, and gingerbread houses • Live holiday entertainment including kids’ band Milkshake • Holiday readings by local celebrities • SantaLand—the ultimate fun zone for kids, featuring a carousel, “reindeer” pony rides, carnival games, a remote control racetrack, and visits with Santa • Holiday shopping from 100 seasonal merchants • Silent auction featuring exciting getaway packages, autographed sports memorabilia, and more

Us e th is CO UP ON fo r $1.00 of f on e Ad

2013 NOV EMB ER 29 — DEC EMB ER 1,9PM FRID

AY & SATURDAY 10AM – SUNDAY 10AM – 6PM

mi ss ion (PTN)

For more event information, to purchase discounted advance tickets, or to learn how to earn FREE TICKETS, call 443-923-7300 or visit festivaloftrees.kennedykrieger.org.

MAR YLA ND STAT E FAIR GRO UND S

Tim oniu m, Mar ylan d iors $7 Adu lts $13 • Kid s & Sen

on.

Must present original coup

SAVE THE DATE: Art of Giving Fashion Show Wednesday, February 5, 2014

Have a great event idea?

Are you interested in hosting an event to benefit Kennedy Krieger Institute? From bake sales and dinner parties to golf tournaments and 5K runs, fundraising events come in all shapes and sizes. We at Kennedy Krieger are fortunate to have families, businesses, and organizations throughout the country actively supporting the Institute by coordinating their own fundraising events. These events are initiated, managed, and executed entirely by volunteers, and benefit the 20,000 children and families who come through our doors each year.

To learn more about organizing a fundraiser, visit events.kennedykrieger.org, or call 443-923-7300.

Grand Prix Zooms Through Baltimore and Stops at Kennedy Krieger’s Fairmount Campus Last May, organizers of the Grand Prix announced a fundraising campaign to support Kennedy Krieger’s ongoing research to develop new and innovative treatments for children recovering from brain injuries.

J.P. Grant, the managing partner of Grand Prix of Baltimore promoter Race On, LLC, presented Kennedy Krieger Institute with a check for $50,000.

The initial contribution seeded a fund that race fans could donate to when purchasing their tickets.

Also, Racing for Kids Foundation, the official charity of the Baltimore Grand Prix, arranged a special visit from drivers of the Andretti Autosport racing team to Kennedy Krieger’s Fairmount Campus. Our patients Student Danny Vasquez “test drives” a race car. and students had a chance to take photos with the Andretti Autosports showcar and get autographs from the many drivers who visited. The children also received goody bags, which included hats, coloring books, photos, and other race goodies. It was a special day for our kids, and everyone enjoyed wishing the athletes luck in their upcoming races.

To learn more, get involved, and stay connected, visit kennedykrieger.org/connect.

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NON-PROFIT U.S. POSTAGE

PAID

PERMIT #7157

U N L O C K I NG P OT E N T I A L

BALTIMORE MD

707 North Broadway Baltimore, Maryland 21205

Give the Gift of Potential By donating to Kennedy Krieger, you can make a difference in the lives of children with developmental disorders and injuries. Your donation enables us to pursue groundbreaking research, provide leading-edge treatment and therapies, and offer high-quality education and community programs for children with special needs to help them achieve their potential. Your gift truly transforms lives.

No matter what your age or financial resources, you can support Kennedy Krieger. There are so many ways to give!

Give monthly as a Partner in Potential Donate in honor or memory of someone n Set up a charitable gift annuity or other life income gift n Include Kennedy Krieger in your will as a charitable bequest n Give through your retirement plan or life insurance policy n Attend one of our special events, or host one of your own n n

To find out more, contact the Kennedy Krieger Office of Development at 1-800-HELP-KIDS or visit helpkids.kennedykrieger.org.

Debra Jeter-Thomas and Kokayi Thomas

Your support is the key to unlocking a child’s potential.

“When I think about what I want to leave behind at the end of my life, I think first of the needs of children. Inspired by my nephew Tommy, I have chosen to support research in developmental disorders in hopes that those children with the greatest needs may enjoy happy and productive lives.”

–Kennedy Krieger Legacy Donor