Ryan, 10 years old, wondered if he would ever get the chance to play the game he loves so much after a rare neurological movement disorder call dystonia changed his young life. He went from being a rough and tumble multi-sport athlete to using a wheelchair to get from class to class in his elementary school.

Ryan became the 100^th patient at Cook Children’s to receive deep brain stimulation(DBS) surgery on Monday, Nov. 30, 2015. The surgery was performed by John Honeycutt, M.D., Cook Children’s medical director of Neurosurgery.

Nearly two year later, it’s hard to imagine this little boy once struggled to walk or hold a pencil in his right hand. The successful DBS surgery has brought him back to the normal the Conder family knew before DBS robbed him of his childhood for more than a year.

“I’m the happiest mom in the whole wide world,” Kayla, Ryan’s mom, said. “When he first got diagnosed we were shocked and it was really hard because we had to have help with almost everything. But now he doesn’t need help or want help. I’m just so excited and really amazed. I’m very thankful because he’s like he was before.”

Last year as Ryan his family wait out in the lobby for their appointment, Dr. Honeycutt happens to walk by on his way into the Jane and John Justin Neurosciences Center. After a couple of steps, he realizes who he has passed and stops in his tracks. He comes back to say hello and marvels at the success of Ryan’s surgery.

“It’s a modern medical miracle,” he tells Kayla.

Then it’s time for a visit with Warren Marks, M.D., a neurologist and medical director of the Movement Disorders and Rehabilitations Programs at Cook Children’s. He sees Ryan every three months.

Ryan spent significant time with Dr. Marks before and after surgery at the Cook Children’s Motion Lab, which is equipped with technology that enables a specialized team the ability to analyze the unique movement of each individual patient and plan a treatment plan for them.

Ryan goes through a routine checkup with Dr. Marks. Then they go out to toss a rubber ball to each other. Dr. Marks leaves the game for a bit and gets a reflex hammer that he uses as a make-shift bat. They are having fun and both are in a great mood.

“This is so rewarding. This is why you have a DBS program because you get kids like this," Dr. Marks said. They come back to being completely normal kids. They come back to doing everything they were doing before the surgery. Everything they want to do. It’s perfect.”

Ryan has been cleared to play baseball, basketball and at recess. He’s not allowed to play contact sports like football or soccer. But he does take his football to school to play catch.

His friends call him the robot because of the surgery that includes two battery-operated pulse generators, much like pacemakers, implanted near the collarbone. Ryan doesn’t mind the nickname at all. He kind of enjoys it.

He shows his friends a video Cook Children’s made as it followed Ryan toward his surgery last year. And what do they say?

“They are like, ‘Wow. It’s amazing what modern technology can do these days,” Ryan said.

Did we mention he’s a really funny kid? Even during his worst days, he maintained his sharp sense of humor. But now the smiles come much easier to everyone in the household and even the tears aren’t so bad lately.

“When he was looking at the video the other day, every time I watch it I cry because I get to see where he was and where he is now,” Kayla said. “It’s not crying because I’m sad. It’s crying because I’m happy. I tell him, ‘Ryan I love watching it but it makes me cry.’

Kayla noticed something was wrong with her little boy around September, 2014. She noticed Ryan running differently than normal during one of his baseball games.

When asked what was going on, Ryan said he couldn’t help it. Then after noticing that his toes on his right foot were curling in, Kayla took her son to the family doctor.

Kayla remembered she had cousins who had dystonia and called her aunt to talk to her about it. After the conversation, Kayla arranged a referral to see Dr. Marks.

Dr. Marks commented that Kayla reminded him of someone and then as they talked, he found out that one of her cousins was not only a dystonia patient, but the first one that Dr. Marks treated at Cook Children’s who had deep brain stimulation surgery performed on her. The surgery was done 15 years ago before Cook Children’s began its own DBS program.

After an initial diagnosis, Dr. Marks verified that Ryan had a genetic version of dystonia.

Dystonia is a disabling disease and sometimes painful condition that limits children in many ways, impacting motor, cognitive and social development. Because medications have a limited effect on most forms of dystonia, Cook Children’s began a Deep Brain Stimulation Program of its own in 2007.

Kayla admits to being scared and nervous at the unknown of surgery. But after talking to Dr. Marks and his team, she hoped this would be a fresh chance for Ryan to return to the little boy he was before dystonia began to take over his body.

Ryan walked with his right foot on his toes and his right arm is now curled in, making it difficult to use. She hoped for Ryan to be able to walk and run like before, but also to use his right hand to write. He had to dictate his work at his elementary school.

Kayla said the hardest part was watching the things Ryan could do and how active he was, playing sports and being a typical little boy, to where he needed help walking, taking a shower or cutting up his food.

But that was then. Now Ryan is back to being the fun-loving, sports playing, ornery little boy he was before the surgery.

“The best part of all this … he’s right there,” Kayla said pointing to Ryan. “He’s walking, running and jumping. He’s able to take care of himself. No parent ever wants to see their child go through what Ryan went through. But hands down, we got more than we ever imagined.”

Learn more:

• What Is Deep Brain Stimulation?
• What Is Dystonia?
• The architect: Warren Marks, M.D.
• The surgeon: Helping kids like his own
• Cook Children's Motion Lab
• Cook Children's Movement Disorders Program

What is Deep Brain Stimulation Surgery?

Deep brain stimulation surgery involves two parts: implanting electrodes into the brain and a pacemaker under the skin of the chest. The two devices are connected by the surgeons and electrical impulses are sent from the pacemaker to the brain to correct the abnormal impulses of the movement disorder. The two surgeries take place about a week apart from each other. Following both procedures, the child usually goes home the next day.

The Path to Asleep DBS

When neurosurgeons at Cook Children’s first began performing DBS, patients had to be awake. Now, thanks to enhanced technology, patients can be under anesthesia and asleep. This change in the treatment’s technique came with the addition of an iMRI, which is essentially a giant magnet, at Cook Children’s. The iMRI allows neurosurgeons to have pinpoint accuracy while performing a delicate brain surgery.

“With all the technology we have, I know I am in the exact spot I want to be," said John Honeycutt, M.D., medical director of Neurosurgery at Cook Children’s “My accuracy for DBS is 0.5 millimeters."

Dr. Honeycutt is one of the world’s leaders when it comes to using DBS on patients with dystonia and will perform the hundredth surgery. It will be the fifteenth asleep surgery for Cook Children’s.

Building a DBS Program

Prior to 2003, DBS was done almost exclusively for Parkinson’s disease and tremors. Then, the Food and Drug Administration (FDA) allowed for DBS to be done on patients with dystonia, beginning at age 7. At that point, most hospitals weren’t doing the surgery on children.

Warren Marks, M.D., a neurologist and medical director of the Movement Disorders and Rehabilitations Programs at Cook Children’s, says Cook Children’s DBS program grew as part of an evolution of the movement program at the medical center.

Dr. Marks said it took two years of hard work to develop the DBS program at Cook Children’s. It involved assembling and organizing two entire teams – one to do the evaluations and postoperative management and another to do the surgery.

Cook Children’s performed its first DBS surgery in 2007.

“We have slowly and methodically grown the program so we try and do it in the best way we can and try to provide for the most kids we can,” Dr. Marks said. “The program is somewhat unique in that we are really focused on children. When we started our program there was no adult program to work off of. Virtually all DBS programs where surgeries are performed on children are in conjunction with adult programs. We didn’t have an adult program to work off of. We really did start from ground zero.”

The Road Ahead

Both Dr. Honeycutt and Dr. Marks take the hundredth procedure in stride. The milestone reinforces their success, but they say they never looked at this program as a race. They have been very careful in their approach to the surgeries and the patients they are treating.

What excites them is the fact that the last 15 cases were performed while the children were asleep.

Dr. Honeycutt says it can be a traumatic event for the kids to go through surgery awake, even after the efforts to keep them pain free while awake in previous surgeries. So with the advent of iMRI guided system, he saw the opportunity to perform the surgery on children who were asleep.

There will still be some surgeries where the patient has certain disorders that will require him or her to be awake. But for the most part, the surgeries will be done with the patients asleep.

*Illustrations/Graphics courtesy of Clearpoint.

Today, Dr. Marks looks back at that time as “an interesting diversion,” but it really helps explain who the man really is. After all, he’s built one of the nation’s most highly successful comprehensive clinical centers for pediatric movement disorders.

Dr. Marks, who is one of the first two Endowed Chairs at Cook Children’s, has molded a program where Cook Children’s deep brain stimulation serves as the centerpiece.

Most recently, Dr. Marks has seen the end of years of work with the addition of Cook Children’s Motion Lab, which sees children, teens and young adults who have a variety of complex movement disorders, including cerebral palsy, dystonia and traumatic brain and spine injuries.

He just keeps on adding to a legacy where he has brought together the most advanced technology with a kid-friendly atmosphere.

When he was a kid, Dr. Marks loved science and became fascinated with the brain and how it works. When he entered Texas Christian University, Dr. Marks considered a career in chemistry, earning a Bachelor of Science degree from TCU, and then he took that year to pursue architecture.

But once he entered Texas Tech University School of Medicine, Dr. Marks’ life’s work began to take focus. During his training he returned to studying the brain and found he had no choice but to make his career helping children.

“I always liked it that if you are going to do pediatrics, you know it up front,” Dr. Marks said. “If you look at the personality inventory of pediatricians, they don’t look like the rest of the physicians, they look like social workers. I talk to medical students when I have them over at the office and the ones that are going to go into pediatrics have no doubt that’s what they are doing. The ones who have hesitation about it are usually not destined to go into pediatrics.”

Dr. Marks joined Cook Children’s in 1988 and today serves as the medical director for the Movement Disorder and Neurorehabilitation Program.

Dr. Marks said he loves the multidisciplinary approach he finds at Cook Children’s, often working directly with rehabilitation therapists, orthotists, neurosurgeons, orthopedists and others. He has developed several multidisciplinary rehabilitation teams, including the transitional care unit, and specialized multidisciplinary clinics that have been developed for children with spasticity, movement disorders, and neuromuscular disorders.

“You have different people coming from different backgrounds,” Dr. Marks said. “Everybody’s perspective is different and we are all bouncing ideas off one another. It’s one of the great things about Cook Children’s.”

Dr. Marks said that team approach creates better care for patients. He said the goal of the neurology team at Cook Children’s is not just to treat children or find a quick fix, but to make their overall quality of life better. He calls this an exciting time for the Neuroscience Program at Cook Children’s, exploring new and innovative approaches to complex patient issues such as movement disorders and epilepsy.

“We are doing as much as anybody and more than most in the country when it comes to improving children’s lives,” Dr. Marks said. “We continue to expand our offerings. We continue to push the limits of treatment. In the future we will have the ability to treat more children and more complex neurological diseases and make them even better. I’m really excited about our ability to bring these new and innovative approaches to solving some very complex issues.”

During his tenure, Dr. Marks remembers fondly certain patients and how to see their lives dramatically impacted. He recalls sisters he treated who went from being bed ridden to being in wheel chairs to dancing at their senior prom and eventually getting married, leading normal and productive lives.

“Those are the stories you look back and say, ‘Man was I lucky.’ You found the magic key for those kids.”

How do we break down silos for innovative design?

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Innovative ideas often require a team approach. It’s difficult to make changes that truly move the needle when you consider a problem from just one perspective. Broadening the team allows physicians to consider other ideas. These new ideas might just open up possibilities that they may have never thought of.

Dr. Marks is a pediatric neurologist with Cook Children’s in Fort Worth, Texas. He has a particular interest in movement disorders and has been involved in Cook Children’s effort to advance the use of deep brain stimulation in the pediatric population. His latest project has been the design and implementation of an innovative pediatric motion lab, which uses a multidisciplinary approach, and advanced technology to assess and develop plans for children, teens and young adults with movement disorders.

Questions to work through on today's topic:

• How are things going with the new lab?
• Going back to when it started. Walk us through some of the different professionals who had input on the design of the lab. How did each member of the team contribute to the big picture?
• What were some of the challenges associated with having a big/diverse team?
• What’s next on the horizon for the lab?
• What part of your work are you most proud of?
• What is the most important quality for a leader in pediatrics?

Quotes:

In order to develop a lab like this, it takes a lot of people who have a great deal of understanding about all the components.

The most important people are the physical therapists who run the lab every day.

The technology itself is just to tool but it does allow for better procedures and better outcomes.

I enjoy team building. I have done that throughout my career…This team was unique because there are constant moving pieces.

There is a huge need to study how childhood obesity affects movement…it should allow us to intervene and better understand what we are doing.

All the things that I have done have required teams.

Neurologists, orthopedists and neurosurgeons desperately need each other.

At the end of the day, we are all in this to help the patient and to make their life better.

I think it is important to surround yourself with good people…and allow them to be creative.

Re: Good ideas-This is not about ownership, it’s about outcome.

Before we see a kid from far away, we do a lot of pre-screening via video and questionnaires.

Links:

• Cook Children’s Motion Lab
• Cook Children’s Neurosciences
• Dr. Warren Marks
• Referring a patient

Warren Marks, M.D., a neurologist and medical director of the Movement Disorders and Rehabilitation Programs at Cook Children’s, says he sees a dozen or more cases often in clusters.

“Influenza can be a trigger, so flu season often triggers some cases,” Dr. Marks said. “There have been some cases associated with mostly older flu vaccines, however in general, immunizations are safe and should be given as recommended. If you are concerned that your child is having progressive weakness, they should be seen by a physician.”

GBS is a condition that can follow many different infections. The process starts after the infection activates the immune system. Sometimes part of the bacteria or virus can look like our own nerves. If this happens, our immune systems can mistakenly attack those nerves which can lead to weakness or paralysis.

At this time, Dr. Marks is not ready to say there’s a relationship to GBS and the ZIka virus. Although it’s not out of the possibility – “there are literally thousands of potential triggers for Guillian Bare’. It is a nonspecific response to an infection.”

GBS can affect people of virtually any age, although it is rare in infants and toddlers. GBS symptoms usually begin with achiness of the muscles or a burning or tingling of the feet and legs.

“Weakness follows, beginning in the feet and progressing up the body, usually over the course of a few days,” Dr. Marks said. “It can occasionally happen more quickly. GBS can involve the diaphragm and breathing muscles which can be life threatening.”

In the mildest cases, monitoring of progress in the hospital is sufficient. If there is loss of ability to walk 100 feet or the weakness is progressing and breathing is endangered, the treatment is usually a five day course of IVIG (intravenous immunoglobulin). Steroids and plasma exchange are other options.

Related articles:

• Zika virus confirmed in Tarrant County
• 11 facts about the Zika virus
• Zika: From nuisance to genuine threat to public health

About the source

Warren Marks, M.D., is one of the the first two Endowed Chairs at Cook Children’s. Dr. Marks joined Cook Children’s in 1988 and today serves as the medical director for the Movement Disorder and Neurorehabilitation Program. Learn more about Dr. Marks and the contributions he's made to Cook Children's here.

In the United States, nearly 1 in 6 children, or about 15 percent, of children from 3 to 17 years have one or more developmental disabilities, according to the Centers for Disease Control and Prevention.

Today, people and organizations worldwide are taking time to acknowledge the challenges those with disabilities face by observing International Day of Persons with Disabilities.

The annual observance was proclaimed in 1992 by the United Nations.

The goal:

• To promote an understanding of disability issues.
• To mobilize support for dignity, rights and well-being of persons with disabilities.
• To create awareness of gains to be derived from the integration of people with disabilities.

At Cook Children's, our physicians work with children with disabilities every day.

So what is considered to be a disability?

"A disability is when an impairment causes a limitation in a life skill," says Warren Marks, M.D., a neurologist and medical director of the Movement Disorders and Rehabilitations Programs at Cook Children’s. "From a medical standpoint, that could be a motor impairment, cognitive impairment or even a social impairment, especially when related to a chronic medical condition."

Dr. Marks treats patients with cerebral palsy, muscular dystrophy and dystonia, all of which are disabilities that can create lifelong challenges.

"Physical disabilities impair the way children interact with their families and peers, and the way their peers interact with them. This can result in secondary social disabilities," says Dr. Marks. "Also, children with motor disabilities have more difficulty participating in many activities which can cause problems throughout their life – a higher incidence of obesity and diabetes and all the complications related to those disorders."

Dr. Marks and his team of physical therapists at Cook Children's have spent countless hours researching complex movement disorders. Currently, they are using the newly developed motion analysis laboratory at Cook Children's to work with experts in endocrinology, orthopedics and nutrition in hopes of understanding the impact of obesity on all aspects of mobility. Their work is not only significant for the medical community, it also offers to patients that their quality of life can be greatly improved.

"We're working hard, even investigating rehabilitative approaches including interactive robotics and assistive technology," says Dr. Marks. "But we have to remember that these investigations take time, equipment and money."

About the source:

 Warren Marks, M.D., a neurologist and medical director of the Movement Disorders and Rehabilitations Programs at Cook Children’sCook Children's Movement Disorders Clinic cares for kids who have challenges with body movement due to illness, injury or congenital ailments. Our team provides your child with a thorough evaluation including a physical examination, complete medical history and testing in order to diagnose the specific type of movement disorder your child has. Based on the diagnosis, the team creates a plan of care that meets your child's unique needs.

The second patient operated on was a dystonia patient and since then the majority of patients have had some form of this disabling and sometime painful condition.

“Dystonia can limit children in so many ways, impacting motor, cognitive and social development,” said Warren Marks, M.D., a neurologist and medical director of the Movement Disorders and Rehabilitations Programs at Cook Children’s. “The reduced independence experienced by these children also affects their entire family. Because medications have a limited effect on most forms of dystonia, we use DBS surgery as another tool to improve the lives of children affected by dystonia. Even small gains in motor function or reduced pain can be life changing for these children.”

Dystonia is a syndrome of intermittent or continuous sustained muscle contractions, frequently causing twisting and repetitive movements or abnormal postures of virtually any part of the body.

Here are some quick facts about dystonia:

• Dystonia patients are sometimes misdiagnosed with other muscular diseases or with claims that it’s purely psychological.

• It’s a symptom or sign of an inherited or acquired brain disease that usually involves the basil ganglia – a series of structures located deep in the brain responsible for motor movements.

• The goal of DBS surgery is to make significant improvements to the quality of life for patients. Patients may not see improvement for three to six months.

• Dystonia can affect multiple parts of the body or just one such as legs, arms, neck, face, eyes and vocal cords.

• A goal of DBS surgery is to prevent the progression of dystonia from spreading throughout other areas of the body.