Disorder Zone Archives Picture Disorder Zone
Archives

 

Spina Bifida

Spina Bifida Poster Child
Armette Damon

Spina Bifida Poster Child
Lenzy Richards

Introduction

Spina bifida (SB) is a neural tube defect that is the most frequently occurring, permanently disabling birth defect. SB occurs from the failure of the fetus’ spine to close properly during the first month of pregnancy. Infants with SB often have an open lesion on their spine where significant permanent nerve and spinal cord damage has occurred. SB can range from a mild defect that causes no problems at all to a more serious defect which causes paralysis and loss of bowel/bladder control.

All women capable of becoming pregnant have a risk of having a child with SB, and 90-95% of all babies born with it have no family history of the defect. The occurrence rate of SB in the United States is 1 out of every 1,000 infants, which results in approximately 4,000 babies each year. However, if parents already have a child with SB, the recurrence rate increases to 1 out of 100. The cause of SB is unknown, however, recent studies show that one factor that increases the risk of having a child with SB is having a low folic acid level before conception and during the first couple weeks of pregnancy. It is now recommended that women of childbearing age should consume 0.4 mg of folic acid daily.

 

Features and Characteristics

There are three types of SB, ranging from mild to severe. Spina bifida occulta is an opening of one or more of the bones of the spinal column and since the spinal cord does not protrude, little or no damage is caused to the spinal cord. It can take the form of a dimple or a small hair growth on the back. It is the most mild form of SB and often times, this type of SB causes no disability at all.

Meningocele is a more serious type of SB in which the protective covering around the spinal cord pushes out through an opening in the spinal column, forming a sac or cyst. Early surgery can correct the problem with little or no damage to the spinal cord. This type of SB is the least common.

Myelomeningocele is the most severe type of SB. In this form, not only does the protective covering get pushed out through an opening of the spinal column, but the spinal cord itself also protrudes, again, forming a sac or cyst. Spinal fluid may leak out and result in a serious infection. This type of SB usually results in paralysis of the lower limbs and problems with bladder/bowel control, although the actual effect depends on many things including the size and location of the lesion, and the degree of damage to the spinal cord and nerves.

Following is a list of complications that you may find in a child with spina bifida:

Learning disabilities occur in approximately 80% of all children with spina bifida and are more likely to occur in children with hydrocephalus. The majority of children with learning difficulties will fall within the normal range of intelligence, however many will function at a low average level. Children with learning disabilities tend to have poor eye-hand coordination, attention deficits, hyperactivity, and problems with memory, sequencing, organizing, problem-solving, and decision-making.

 

Diagnosis

Prenatal testing for spina bifida (and other neural tube defects) is available by the use of AFP screenings. The AFP screening measures the amount of AFP in the mother’s blood. When a child has a severe form of spina bifida, often times the AFP is higher than usual (the screening detects 64-80% of babies born with SB). In addition to an AFP screening, a high resolution ultrasound or amniocentesis may be useful. For those cases not detected by prenatal testing, the defect is recognizable at birth.

 

Treatment

There is no treatment for SB because the nerve tissue cannot be repaired or replaced. When a child has one of the more severe forms of SB, surgery may be needed the first 24 hours after birth. In this case, doctors try to remove the cyst protruding from the spine, and cover the wound with muscle and skin. Surgery may not, however, prevent paralysis and other related complications. As the child gets older, surgery may be an option to strengthen the child’s spine, lengthen muscles, or control severe bladder problems.

Children with SB sometimes have an active physical therapy program to learn mobility skills such as using crutches, braces, or a wheelchair. In the early stages, physical therapists teach parents how to move their baby’s feet and legs in order to prepare for mobility. Occupational therapy and early intervention programs are also available to prepare the child for school.

In regards to a school setting, it is important that the child be placed in the least restrictive environment as early as possible. For children with learning disabilities, Dr. Donald J. Lollar recommends that the following be provided:

Most children can receive a mainstreamed education, and active participation in clubs, youth groups, and educational settings is recommended. As with any other child, acceptance by peers is also important for the child with SB.

 

What to Expect

With the latest medical advances, children born with SB will live into adulthood and can become independent, productive adults. Many adult individuals with spina bifida are college educated professionals in a variety of fields.

 

Personal Stories

My 3 year old daughter was born with spina bifida. She has the myelomeningocele type of spina bifida. Three years ago, my so-called perfect world came crashing down. My husband and I waited several years before we finally became pregnant. We learned very very late in the pregnancy that our daughter was going to be born with spina bifida. Neither one of us had ever heard of spina bifida and therefore did not know what it was. Over the next two weeks, I think that I cried more than most people do in a lifetime.

The day came that our beautiful little girl was born. She had a closed sac on her back about the size of a plum. She was delivered vaginally and the sac did not rupture. She was taken immediately after birth to the ICU. She was then transferred the next morning to the children's hospital for her back closure. I did not get to hold my daughter until she was 3 days old. I was so scared, and I had emotions running through my body that I had never experienced before. The doctors told us that she had nothing - no movement, no feeling from her knees on down. My world once again went dark. Of course, we could not help ourselves in asking the obvious: Would she ever walk? The one thing that I have found out about SB doctors is when they are asked that question, most of the time the answer is very vague - if you can even get an answer.

My daughter also has hydrocephalus and does have a shunt. She has had one shunt revision when she was 13 months old. She stayed in the hospital for the first 10 days of her life. She is level L4. When she was 18 months old, she had surgery on her right foot. She was having problems with the foot turning in, so the orthopedic surgeon performed a right posteromedial release with fractional tibialis posterior lengthening (basically, the tendon running along the inside of her foot was lengthened).

She started to crawl when she was about 10 months old and since then, has continued to do very well. She has no feeling in her feet, and only has a small amount of movement in her feet. She walks with AFO's and a twister cable on her right leg. She started walking independently when she was 28 months old.

In the past 3 years, I have learned so much about spina bifida and met so many wonderful new friends. I want to know everything there is to know to help our daughter be the best she can be. My daughter has taught me so much about life. She has shown me how to love unconditionally, and to see her for who she is, not what she has. She also has a wonderful grandma and grandpa who love her very much.

Today, she is just my daughter and I love her dearly. - Debbie Duckworth

Our daughter, Jennifer, is 3 years old and was born with myelomeningocele at a very low level. Jennifer is adopted. She was a planned adoption for the birth mom and the SB wasn't discovered until delivery. To make a long story short, we brought Jen home from the hospital at 2 weeks of age. She had her back closed and a VP shunt placed. Some of our relatives thought we were crazy to "take on" such a responsibility; others said that we were "saintly." We were just happy to be a family!! We just think we are normal. Now that she is older and everyone has watched her grow, they think she is just like any kid. Sure she has her issues - she is catheterized for urine every 4 hours, and has bowel issues (she is chronically constipated, so we work on managing that and getting her to have a BM on a regular basis).

Jennifer has shunted hydrocephalus and an Arnold-Chiari malformation, all of which are related to SB. She had one shunt revision at 1 month of age, she had ureter reimplantation surgery at 14 months of age, and at the same time, had her strabismus surgery (crossed eye). She began walking at about 18 months of age with AFO's. Now she has shoe inserts only and does great! She is jumping and trying to run, and is doing great learning how to do stairs. She is receiving speech and physical therapy through her preschool program, which she attends 5 half days a week, and she loves every minute of it.

She is delightful! She picks on her younger brother, and makes messes to beat the band. She is funny and loving and, oh so sweet!!! She can also make you want to tear your hair out! All in all, she is a three year old kid. - Mary Beth Cox

Jimmy will be 15 years old on May 2, 1999. He is an honor roll student at Mount View High School where he is a freshman class representative to the Student Government Association, a member of several clubs, and a manager of the girl's softball team. He enjoys computers, Nintendo 64, and music. He's a very typical teenager except he is living his life with spina bifida and hydrocephalus, and is in a wheelchair.

Jimmy, a.k.a., James Carter Crouse, was born in 1984 with spina bifida, a neural tube birth defect. At birth, he had a sac (it almost looked like an inflated balloon) on his lower back. His spinal cord and nerves were exposed and damaged at this sight in the lumbar region of his spine. Because of the nerve involvement, it was diagnosed as a myelomeningocele, the worst type of spina bifida. After only one week in the hospital, he came home to join his older brother, Joe, and to complete our family.

Hydrocephalus, an accumulation of fluid in the head due to improper drainage, developed by the age of three weeks, and his first shunt was placed in the right ventricle of his brain to drain the fluid into his peritoneal cavity.

During his first four years, his life, medically, was unspectacular. By the age of eighteen months, it was determined that his shunt was not functioning, but local doctors shrugged their shoulders and said he apparently didn't need it because he was not showing symptoms of shunt failure.

Jimmy amazed the doctors, when at the age of two and a half years he began to walk unaided. At first, when he tried to stop, he would fall, so the doctors at Children's Hospital in Virginia prescribed a quad cane for him to use. We knew his accomplishments were just beginning when he would walk across the yard with that cane slung over his shoulder like a soldier's rifle, learning to stop on his own in just a few short months.

When Jimmy was four, he attended a private nursery school in our neighborhood. The following year, he began kindergarten in the public schools in our southern West Virginia hometown of Welch. It was also during these early years that we began to notice a pronounced curvature of his spine (scoliosis) developing. Our local pediatrician made an orthopedic referral to Duke University's Myelo Clinic, and at age seven, Jimmy's medical complications from spina bifida escalated.

During Jimmy's first visit to Duke, the neurosurgeon was appalled that he did not have a working shunt. After the appropriate x-rays, which showed enlarged ventricles, a second shunt was the first order of business. Then, following CT scans, MRI's, and myelograms, it was determined that he had a tethered cord (his spinal cord was caught in scar tissue from closure surgery), thus prompting surgery to release his cord. During this six week recuperation period, a homebound teacher was provided for his education. The following summer after his eighth birthday, he had his first spinal fusion, a growth fusion, where a rod was placed to correct what had become a sixty-eight degree curvature of the spine. For eight months following this surgery - almost his entire third grade year of school - he could not even sit up without a molded turtle shell brace (it extended from armpits to hips and the front and back of the brace hooked together with velcro).

The rod was removed the following summer and within one year, the curvature was back to sixty degrees, which resulted in his second, permanent fusion (he has one rod called an Isola instrumentation) of ten thoracic vertebra.

But, despite the medical "life," Jimmy persevered. When he was six, he played on a T-ball team. Because of the wonderful small town we live in and the caring people who live here, every time Jimmy was at bat he was always safe on first, and that one memorable evening, he traveled completely around the bases and scored! There probably wasn't a dry eye at the Little League field that night and every person there gave him a standing ovation.

He remained an honor roll student throughout elementary school, participated in the marching band as a drummer (using a wheelchair for distances, like parades), and at the end of his sixth grade year, he was chosen the boy Good Citizen of Welch Elementary by our local American Legion.

Middle school years were more difficult. The school was on a hill and the buildings were too old for an elevator, so Jimmy was confined to only one floor and programs were brought to him. During these two years, he also needed two new shunts placed, and the neurosurgeon, during the last surgery in April, 1998, did an endoscopic third ventriculostomy, which was placing a hole in the third ventricle to allow for additional drainage. At this time, the procedure was not being done at many hospitals, so Jimmy likes to think of himself as being on the cutting edge of medical technology. During these two years, he was a member of the Junior Beta Club (a middle school honor society) and represented his school at the county spelling bee. He also had a major role in several plays written by a local author and performed in churches and schools in the county.

When I think back to that day in 1984 when Jimmy entered our lives, I can remember that the doctors told us he would never walk, but he did; I remember they told us he would never be continent (control of bowel and bladder), but he is; I remember they told us he might be mentally impaired (because of hydrocephalus), but he is far from it; I remember they told us if we chose surgery to close his back, it needed to be done within twenty-four hours or that we could choose no surgery. I thank God everyday that we chose the aggressive, surgical closure of his back. Since that time, he's probably had fifteen or more surgeries. Following his last surgery on his leg and ankle, walking has become more difficult and he now uses his wheelchair most of the time.

Jimmy faces life with a smile. He's an easy-going, compassionate, and friendly human being. He touches the lives of almost everyone he knows because of his brave determination to meet the challenges of everyday life. His strong faith and reliance on God helps him, not only during his times of crisis, but in his everyday life. He realized early in life that things may be difficult, but that life is good and worth the fight; that he controls his quality of life. He has set many goals for himself, many of which he has already achieved. I know he will reach them all! - Cathy Crouse

 

Resources

If you are interested in meeting other parents and individuals who are involved in raising a child with spina bifida, the following listserv is available:

SB-Parents List - Send an e-mail to listserv@waisman.wisc.edu, leave the subject field blank, and in the body of the e-mail type the words "Subscribe SB-Parents" (without the quotes), followed by your first and last name. You will then receive a confirmation.

For more information on spina bifida, visit these helpful sites:

 

The Disorder Zone has been created for educational purposes only and is not intended to serve as medical advice. The information provided in The Zone should not be used for diagnosing or treating a health problem or disease. It is not a substitute for professional care. If your child has any health concerns, please consult your health care provider.

Welcome | Editor's Note | Success Stories | Horror Stories | Family Issues | Legal Files | Information Avenue | Disorder Zone | Archives | Diagnosis Search | Tips | Bulletin Board | Marketplace | Parent-Matching Program | Suggestion Box | Guestbook | Sponsors | Donations | Featured Special Child | Home

Copyright © 1997-2000, The Resource Foundation for Children with Challenges. All rights reserved.
By using Special Child and related services, you agree to abide by the terms and conditions.