Spina Bifida: Making Mobility Happen

"He will probably never walk, never talk, and will be completely mentally disabled."

Kevin Felton, CO, LO, FAAOP, wraps fiberglass casting tape around the right leg of Texas Scottish Rite Hospital for Children patient, Grace Plue, 5, to make the negative mold for an AFO. Photograph courtesy of TSRHC. Doctors said these shocking words to Johnna Throckmorton shortly after her first child, Chase, was born.

She did not know her son would have spina bifida (SB). Prenatal tests had not revealed any problems.

After Chase was born, he was wrapped up and whisked away. "The next time I saw him, he was in an incubator with all kinds of wires hanging from him," Throckmorton says. "My world was devastated."

Physicians told her that her son had been born with a neural-tube defect, with part of his spine outside his back. Within an hour, the tiny child was on his way to the Medical College of Virginia Hospital in Richmond.

When Lynsey Stevenson was born, prenatal tests also had not revealed any abnormalities. "We had four ultrasounds, and no one...spotted anything," says her mother, Tammy. "So we thought everything was fine, until the doctor said, 'She has an open wound on her back.'"

After Lynsey's first operation, the physician told Stevenson and her husband Matt that there was a chance that Lynsey would never walk, talk, breathe on her own, or swallow. "Basically, we walked out of there and thought our kid was a vegetable," Stevenson says.

Today, Chase is 16, on-target academically, active in sports, articulate, outgoing, and popular with peers and adults alike. (Editor's note: Read more about Chase in " Two Teenagers' Perspectives on Spina Bifida " in the sidebar at the end of this article.)

Lynsey is now three and a half, bubbly, happy, and enjoying her world. In a story posted on the website of American Prosthetics & Orthotics, Iowa, which provides Lynsey's orthotic care, Stevenson describes Lynsey's dance-recital performance. "She went on the stage and she was waving, dancing up a storm, and blowing kisses. As soon as she got off the stage, she jumped into my arms and said, 'Do it again, Mom.'"

Children with SB-and their parents as they provide care-often face a broad and daunting array of challenges: mobility difficulties, bowel and bladder complications, learning difficulties, psychosocial issues, and possible multiple surgeries.

SB is a neural-tube defect (NTD), a disorder involving incomplete development of the brain, spinal cord, and/or their protective coverings caused by the failure of the fetus' spine to close properly during the first month of pregnancy.

Infants born with SB often have an open lesion on their spine where significant damage to the nerves and spinal cord has occurred. Although the spinal opening can be surgically repaired shortly after birth, the nerve damage is permanent, resulting in varying degrees of paralysis of the lower limbs, notes the National Institute of Neurological Disorders and Stroke (NINDS) of the National Institutes of Health (NIH). Even when there is no lesion present, there may be improperly formed or missing vertebrae and accompanying nerve damage.

Although there are several types of SB, the three most common are: (1) myelomeningocele, the most severe form, in which the spinal cord and its protective covering, the meninges, protrude from an opening in the spine; (2) meningocele, in which the spinal cord develops normally but the meninges protrude from a spinal opening; and (3) occulta, the mildest form, in which one or more vertebrae are malformed and covered by a layer of skin.

Neufeld Along with heart defects, spina bifida is one of the most common birth defects in the United States. About one in every 2,500 babies born in the United States will have spina bifida, according to the Centers for Disease Control and Prevention (CDC).

To meet the wide range of issues that children with SB face, a multidisciplinary team of healthcare specialists must be marshaled. Children's Hospital & Research Center, Oakland, California, for example, emphasizes a team approach to care. The team includes a physical medicine and rehabilitation (PM&R) physician, orthopedic physician, urologist, social worker, nurse, and other specialists, according to Jacob Neufeld, MD, MSPH, chief of the Division of Pediatric Rehabilitation at the hospital. When a child is ready for potential ambulation, "I like to have the orthotist and physical therapist with me at the clinic so we can evaluate the child together," Neufeld says. "I don't always have that, but that's the ideal." He stresses good communication between the ordering physician and the orthotist. "If the orthotist has any concerns or questions, he should call the ordering physician. If he can't reach him, keep trying. Most of the rehabilitation doctors that I know would be very happy to have a conversation with the orthotist." Likewise, if the orthotist and the physical therapist see that an orthosis is not working as expected, or if other problems are developing, they also should talk with the physician, he says.

Felton

"Here at TSRHC [Texas Scottish Rite Hospital for Children, Dallas, Texas], we have a multidisciplinary team of clinicians," says Kevin Felton, CO, LO, FAAOP, education coordinator for the Orthotics Department. "When a child is being evaluated for possible orthotic management, we evaluate the child as a team and work as a team. A developmental pediatrician heads up the team, which includes a physical therapist, occupational therapist, therapeutic-recreation therapist, and orthotist." The child may be referred to neurosurgery, radiology, or other specialties as needed, he added. "We look at the...child in a holistic way," Felton says. The child also may be referred to Early Childhood Intervention (ECI), a joint federal and state program for children with developmental delays and disabilities, which is available nationwide to children between birth and three years of age.

On the positive side, research is revealing more about SB, and improvements in surgical techniques and overall care, plus new design and materials developments in orthoses and other mobility aids, are improving the outlook for a higher quality of life for children with SB.

Orthotic Management Although, as Felton notes, orthotic management is only a small part in overall SB treatment, it is a vital one for youngsters with ambulatory potential.

At TSRHC, a child with possible ambulatory function is evaluated for walking potential and device use at around one year of age. Since the child is too young to follow the directions for movement used in standard muscle-strength testing, the orthotist and physical therapist observe the child's movements and ask the parents what the child can do.

"The bottom line is, for a child to be a candidate for walking, he needs to have knee-extension strength," Felton says. "If he doesn't have that strength [because the nerves innervating the muscles never formed], his chances for being able to stand and walk are very small."

The level of spinal cord injury is the main determining factor in whether the child will be able to ambulate, Neufeld explains. If the injury is at the thoracic level, the child will be paraplegic, but if it begins at lumbar levels L2 and L3, the child has potential for ambulation.

However, Neufeld and other experts point out that each child is different. Sometimes a child will exceed the expected functional level, and sometimes he or she will not achieve it. "It's not an exact science," Neufeld says.

Felton concurs. "Children don't always go by the book," he says. Many times, one side of the lesion may be lower than the other, and that also can affect functional capacity.

"After we evaluate the knee extension," Felton continues, "we next look at the child's overall tone, head and trunk stability-whether the child is able to sit well, stabilize his head and upper limbs. We look at the child's motivation and cognitive level-where he is developmentally."

Often, the child may be developmentally delayed, Felton adds, and not yet showing any interest in pulling to a stand. "So trying to start intervention to encourage walking at this point could seem frustrating," he says. "We might ask the parents to encourage the child to explore, for instance, placing toys a little farther away, encouraging the child to go places. Most of these children are also being seen by ECI."

If the child has adequate knee-extension strength and is motivated to move, orthotic intervention may begin. "We look at how much stability the child needs. Some children don't need any orthotic intervention, but when a child is pulling to stand and his lower limbs are not supporting him well, then that's when we consider orthotic management."

The child is fit with AFOs to stabilize the feet and ankles. In follow-up, if the child needs hip support, the orthotist provides an HKAFO, generally with knee locks but with hips having free flexion and extension. "We work with the child to ambulate with a walker or push toy, which places the lower limbs well under the child's body and allows the child to ambulate more effectively. Knee locks will hopefully be unlocked as soon as the child can control his knees on his own," Felton adds. A child generally comes into the hospital and works with a physical therapist for a couple of days when the HKAFO is fitted.

In the last decade, TSRHC has changed how children with higher level lesions but some ambulatory capacity are managed, Felton says. "In the past, we might have provided an RGO, a swivel walker, or an HGO. But now we have almost entirely discontinued using those devices in favor of a wheeled stander, the Rifton Dynamic Stander , which is like a standup wheelchair."

The purpose of any of these devices is to provide upright mobility so a child can be at the same level as his or her peers. "These other devices require a great deal of energy expenditure, but wheeled standers require very little extra energy, and kids can use both hands for activities like T-ball."

When the children are small, they can use the smaller-sized standers, but when they start to reach the teenage years, they require standers that are so large that they are hard to put in a van or store in a house, Felton explains. At that point, they generally switch to wheelchair use.

SB patients tend to lose ambulatory capacity as they get older, Felton and Neufeld say. One reason is that smaller, shorter children don't need as much energy to stand up and ambulate. Weight gain and other factors may also play a role. However, even if they use a wheelchair most of the time, many still use AFOs for transfers or to walk short distances in situations that are not wheelchair-accessible. The AFOS help stabilize knees and ankles. "The reason the knee collapses is because the ankle dorsiflexes," Felton explains. "We tend to think of the quads as being the main knee extensors, but the gastroc-soleus plantorflexors are as well. They keep the ankle from dorsiflexing and thus prevent the knee from collapsing. If you can lock the ankle up, that will help keep the knee from buckling, so you can stand with enough knee stability to pivot and sit down on another surface for an easier transfer."

The orthotists use polyurethane gel to support areas susceptible to skin breakdown. "We also use the low-friction interface, ShearBan®," Felton says.

New materials and components also enable orthotists to fabricate lighter-weight, thinner orthoses, which is especially helpful as children get older and heavier-a time, which, in the past would have required bulkier, heavier orthoses, Felton adds.

Creative Thinking Lynsey Stevenson, almost 2 and walking like a pro. Photographs courtesy of Matt and Tammy Stevenson. John Kamp, CPO, who first saw Lynsey Stevenson when she was about 15 months old, decided she needed something better than the AFOs she was using.

"Lynsey could not stand without hanging on to a chair; her feet were basically rotated about 180 degrees. If she was standing, her foot could be completely pointed to the back. I thought we could come up with a better design that would at least stabilize her and enable her to stand and use her hands. So I asked the physician if I could try another design or add-on."

Kamp designed some AFOs that were much more supportive and corrective and attached them to anti-torsion, or twister, cables. "I was able to keep her foot in normal alignment because her hips didn't have any stability whatsoever," Kamp says.

Soon, Lynsey could stand without support and walk well in her orthoses with the help of a walker. "Then she was able to walk without the walker, and things just got better and better and better. She's a very precocious, cute little kid with a great personality-and she just loved her braces! If I took them off, she would get so upset, and then when I brought them back, she would clap her hands, she was so happy! She knew they were her ticket to movement-and she could really go!"

Lynsey Stevenson walks with AFOs only

As Lynsey grew, Kamp replaced the AFOs. "I used solid-ankle AFOs for knee stability and put clamshells on the front. Her hips and knees just started to either correct themselves or just tightened up, so she was able to get out of the twister cables and walk, even almost trot, with just her AFOs. She's very mobile."

Lynsey, who is now about three-and-a-half, is currently undergoing serial casting, which began last November, says her mother. "She wears a cast about five days a week, then has about a day and a half off. We're trying to straighten out her feet and gain some muscle, so her feet will turn out more, not be so pigeon-toed. She's also wearing TheraTogs™, which we got from American Prosthetics. It's basically a little blue suit she wears under her clothes, with elastic straps that pull her ankles, legs, and knees into the correct position. When she wears her TheraTogs suit, she walks so wonderfully!"

Felton also makes adaptations to suit some of his older pediatric patients. He is making AFOs to fit patients who like to wear boots or high-heeled shoes. Although AFOs for pediatrics are designed for tennis shoes, teenagers are more fashion-conscious and don't want to wear tennis shoes when very few of their friends are, so Felton picks a reasonable heel height, such as three-fourths inch to an inch, and fabricates an AFO to fit, since changing the heel height changes the angle of the ankle, which affects the whole alignment of the body. These AFOs also can be worn with tennis shoes simply by adding a wedge inside the shoe.

Case Study 2 - ESR AFO (energy storage and return AFO): (1) copolymer (colyene) base material; (2) posterior reinforcement (¼ inch polypropylene) molded inside colyene; (3) plantar reinforcement molded outside colyene; (4) overlap of colyene and polypropylene; (5) anterior cuff (11 6 polyethylene thermofoam); (6) anterior pull back straps; (7) slots (cuts) in plastic to extend energy storage and return section. Diagram courtesy of Don Weber, CO(c), BSc, FCBC, Hamilton Health Sciences Hospital, Ontario, Canada.

Another exciting innovation, a design free for use by orthotists, is the Energy Storage and Return Ankle Foot Orthosis (ESR AFO), developed by Don Weber, CO(c), BSc, FCBC, Hamilton Health Sciences Hospital, Hamilton, Ontario, Canada; and Gordon Ruder, CO (c), MSc, BSc, George Brown College P&O Programs, Clinical Orthotic Consultants, Toronto, Ontario, Canada, from an idea conceived by Ruder. Originally called the Reinforced Ankle Foot Orthosis (RAFO), the ESR AFO was presented at the International Society for Prosthetics and Orthotics (ISPO) World Conference 2007 and at the 2008 National Assembly of the American Orthotic and Prosthetic Association (AOPA).

The ESR AFO design embeds polypropylene reinforcements up to one-half-inch thickness in the tibial and plantar surfaces of a posterior-leaf AFO (colyene base material) to produce a rigid foot-plate section and a dynamic posterior section capable of clinically significant energy-storage-and-return characteristics. "The plastic architecture of the posterior tibial section can be altered to create a broad range of motion resistance, from minimal control, such as for a child pulling to stand, to maximal resistance for sport applications such as soccer and basketball," Weber noted in a paper presented during the ACPOC 2008 Annual Conference. The device can be clinically applied to a variety of activity levels and pathologies, including cerebral palsy, SB, Down syndrome, spinal muscular atrophy, and idiopathic toe walking.

Though there are significant challenges involved when caring for children with SB, advances in orthotic management, coupled when a healthy dose of creativity, are going a long way toward improving mobility and quality of life for these children.

Miki Fairley is a contributing editor for The O&P EDGE and a freelance writer based in southwest Colorado. She can be contacted via e-mail at linkEmail('miki.fairley','gmail.com'); .

Editor's note: To view videos and abstracts about orthotic application, fabrication techniques, measuring stiffness, and other information about the ESR AFO, visit web.me.com/gordon.ruder/Site_2/ESR-AFO.html . For more information about the challenges of spina bifida care, read our online exclusive article .

The O&P EDGE conducted interviews with several parents of children with spina bifida. To read the interviews and learn what these parents do to help their children succeed, go to the article titled Families Help Spina Bifida Kids Succeed: Interviews with Parents .

Two Teenagers' Perspectives on Spina Bifida Julia Grace Collins

Julia Grace Collins is 13 and in the eighth grade. Her spina bifida level is L4, 5; her functional level is considered L3. Her sister Jessica, 18, is a senior in high school, and her brother Christopher, 16, is a junior in high school. Photo at right courtesy of Susan Collins.

Q: Your mother mentioned that you enjoy reading and handcycling. What other activities and sports do you enjoy?

A: I like basketball, and I also do after-school chorus. I like to do things around the house, and I am not big on shopping like other teenage girls. I love my dog Jaan (pronounced John), and I love to hang out with my friends.

Q: What are your favorite subjects in school?

A: English and science. I also like social studies, but I don't always do well in it.

Q: What would you like to do after you graduate from high school?

A: I would like to go to a four-year university and maybe get a job in counseling or maybe just a job that deals with children.

Q: What have been the biggest challenges you've had to meet in your life so far?

A: I guess some of the challenges I have faced have been not always being able to transfer to places that I need or want to go. I always try to do what I can, but sometimes it's just not possible.

Q: What advice would you give other kids with spina bifida to get the most out of life?

A: I would tell kids not to let having a disability stop you from doing what you want with your life. You may have to adjust things, but you can usually do what you want. I think that it is important to know what your limitations are, but don't let everything be [a limitation]. All you can do and all anyone expects out of you is to try your best-that's all that is important.

Q: What advice would you give to parents to best help their children to truly enjoy life and realize their potential?

A: I would tell parents to always encourage their kids to do everything they possibly can. I would also tell them that they shouldn't baby their kids but always push them to do their best.

Q: Are there any other comments you would like to make?

A: Never feel sorry for yourself, because there is always someone out there who is worse off than you are. You should try to enjoy life and never let anything stop you...but don't do anything that will hurt you in the long run.

Chase Throckmorton

Chase Throckmorton is a 16-year-old high school freshman; his spina bifida level is T10. He has a younger sister, Karis, 12, who is in the seventh grade. Photo at right courtesy of Johnna Throckmorton.

Q: What are your favorite sports and/or recreational activities?

A: My favorite sports are basketball, baseball, football, NASCAR, bowling, etc.; I pretty much like all sports.

Q: What are your favorite subjects in school?

A: My favorite subject is world history. I'm really into presidents. My mom says I'm going to be president one day because I know so much about them.

Q: What do you like to do best when you are with friends?

A: I like to play video games, listen to music, play sports, and hang out.

Q: What would you like to do when you graduate from high school?

A: I would like to get involved in law enforcement.

Q: What have been the biggest challenges you've had to meet in your life so far?

A: The strongest challenges I've had in life would have to be being in the hospital so much and missing so much school because of all the surgeries I have had.

Q: What advice would you give to other young people with spina bifida to get the most out of life and accomplish their goals?

A: Do what you want to do, and don't let anything stop you. Just because you're in a wheelchair doesn't mean you can't do something everyone else can do; it just means you have to do it differently.

Q: What advice would you give to parents to best help their children to truly enjoy life and realize their potential?

A: I would tell them not to treat their children any differently, to encourage them to do their best, and not let anything stop them. My mom doesn't let me say the word "can't" because she says it's not in our vocabulary, and it's a bad word. So I would tell them to teach their kids not to use that word. My parents have always treated me and my sister the same, and everybody else's parents should too. When I get in trouble, I get punished just like my sister does when she gets in trouble.

Q: Are there any other comments you would like to make?

A: I love my life and, although I wish I could walk, I have a lot of friends, and they don't treat me any different because I am in a wheelchair. When I get tired, at least I can be pushed, and I don't have to sit for a while to catch my breath like they do!

Spina Bifida: Making Mobility Happen

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