Spina Bifida

DISABILITY CATEGORY: Other Health Impairments—Spina Bifida, neural tube defects

TYPES: Occulta (mild), Meningocele (moderate), and Myelomeningocele (severe)

DEFINITION: 

Our nation’s special education law, the Individuals with Disabilities Education Act (IDEA) defines diabetes under “other health impairment,” which means…

            “…having limited strength, vitality, or alertness, including a heightened alertness to environmental stimuli, that results in limited alertness with respect to the educational environment, that–

• is due to chronic or acute health problems such as asthma, attention deficit disorder or attention deficit hyperactivity disorder, diabetes, epilepsy, a heart condition, hemophilia, lead poisoning, leukemia, nephritis, rheumatic fever, and sickle cell anemia; and

• adversely affects a child’s educational performance.”

[34 code of Federal Regulations §300.7(c)(12)]

Other Definitions: “Neural Tube Defects (NTDs) are a group of malformations of the spinal cord, brain, and vertebrae.  The resulting disorders vary in severity according to their location, the extent of bony opening, and the exposure of spinal cord or brain.”  The most common NTD is spina bifida, which is a split of a section of the vertebral arches (the bony arches projecting from the body of the vertebra) (Source:  Batsahw, M.L., 2002, Children with Disabilities, 5th ed. Baltimore, MD: Brookes Publishing Co.). 

Spina bifida (SB) is a neural tube defect (a disorder involving incomplete development of the brain, spinal cord, and/or their protective coverings) caused by the failure of the fetus's spine to close properly during the first month of pregnancy. Infants born with SB sometimes 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. Even when there is no lesion present there may be improperly formed or missing vertebrae and accompanying nerve damage. In addition to physical and mobility difficulties, most individuals have some form of learning disability. The three most common types of SB are: myelomeningocele, the severest form, in which the spinal cord and its protective covering (the meninges) protrude from an opening in the spine; meningocele in which the spinal cord develops normally but the meninges protrude from a spinal opening; and occulta, the mildest form, in which one or more vertebrae are malformed and covered by a layer of skin. SB may also cause bowel and bladder complications, and many children with SB have hydrocephalus (excessive accumulation of cerebrospinal fluid in the brain) (Source: National Institute of Neurological Diseases and Strokes (NINDS)). 

FACTS AND STATS: Spina Bifida occurs in 7 out of every 10,000 live births in the United States and 40% of Americans may have spina bifida occulta but they experience no symptoms and few even know they have it.  The other two forms, meningocele and myelomeningocele, occur one in every thousands of births.  Of these births, 4% have meningocele and 96% have myelomeningocele form.  Spina Bifida occurs most frequently amongst Hispanics and whites of European extraction.  It is less common among Ashkenazi Jews, most Asian ethnic groups and African-Americans (Source: SBAA, March of Dimes, National Dissemination Center for Children with Disabilities (NICHCY)). 

CAUSES: Scientists believe environment and genetics are the causes of spina bifida and other NTD’S.  However, 95% of the patients with NTD’s are born to families who do not demonstrate any history of having these birth defects.  If a child is born with spina bifida, the chances of reoccurring in the next pregnancy is one and 40.  Spina bifida can also occur as a syndrome with other birth defects.  Women who have chronic health issues including diabetes or seizures have a higher risk of giving birth to a child with spina bifida (1/100) (Source:  March of Dimes). 

PREVENTION: Studies show that 70% of NTD’s risks can be reduced or prevented if women took 4 milligrams of B Vitamin folic acid every day before pregnancy and during the early days.  B vitamin folic acid can be found in multivitamins and the following foods:  orange juice, other citrus fruits and juices, leafy green vegetables, beans and whole-grain products.  Multivitamins, fortified breakfastcereals, and enriched grain products contain a synthetic form of folic acid that is more easily absorbed by the body than the natural form.  Doctors recommend women who could become pregnant to take 400 micrograms but no more than 1,000 micrograms (1 milligram) a day.  Women who have had a baby with NTD or Spina Bifida or have chronic health issues should see their doctor to reduce the risk of reoccurrence (Source: March of Dimes).   

CHARACTERISTICS:

Physical Symptoms

Occulta (mild):  Symptomless form where the patient has small defect or gap in one or more of the vertebrate. 

Meningocele (moderate):  Protective covering around the spinal cord has pushed through the opening of the vertebrae. However, the spinal cord remains intact. 

Myelomeningocele (severe):  Portions of the spinal cord protrude through the back and nerves and tissues can be exposed.  Most serious form of Spina Bifida may include muscle weakness or paralysis below the spine, loss of sensation below the cleft, loss control of bowel and bladder control.  In addition, fluid may be built up in the brain (this is called hydrocephalus).  Other complications that occur with Spina Bifida are obesity, gut and urinary tract disorders, psychological and sexual issues, and learning disabilities (Source:  NICHCY). 

Allergies

Due to extensive latex exposure during surgery and other medical procedures, 18-73 percent of children with spina bifida tend to be allergic to latex. Symptoms include rash, wheezing, watery eyes, and even life-threatening anaphylactic reactions (Source: SBAA).

Learning Disabilities

70 % of children with Spina Bifida have normal intelligence but some children have learning disabilities. 

Precocious puberty

Children who have spina bifida with hydrocephalus will more likely have precocious puberty. Precocious puberty is when the body sexually matures earlier. Symptoms of precocious puberty are early breast development for girls, before the age of seven, and, for boys, an increase in the size of their testicles before age nine. Precocious puberty also starts bone growth to become more adult-like and become incapable of further growth. Most children who start puberty too early will be shorter than had they begun puberty at a normal age (Source: SBAA).   

MEDICAL TREATMENT: 

Occulta:  Usually no treatment

Meningocele:  A child with this form usually has surgery and usually has no signs of paralysis.  However, a child should still be examined for hydrocephalus and bladder and be treated promptly if such symptoms occur. 

Myelomeningocele:  Surgery is performed within 24-48 hours of birth to tuck the exposed spinal cord and nerves back into the spinal canal and cover it up with muscle and skin.  However, already damaged nerves cannot be reversed. 

A large percentage of children who have myelomeningocele have hydrocephalus which is fluid in the brain.  When the cerebrospinal, which is fluid that protects the spine and the brain does not drain normally, it causes the head to enlarge and possibly neurological damage.  Surgery is needed to insert a shunt which runs from the chest into the abdomen or chest to drain the fluid and passes around in the child’s body. 

Since those with myelomeingocele usually have precocious puberty, leuprolide (lupron) can be injected with the guidance of a physician into the muscles to delay puberty.  This may also cause individuals to be taller than those without the medication (Source:  March of Dimes and NICHCY). 

PROGNOSIS: Soon after surgery, a physical therapist teaches parents how to help their child’s legs and feet get ready to walk with braces and crutches. 70% of these children show they can walk with or without these devices, although many require a wheelchair. 

Most children with Spina Bifida have a tethered spinal cord that does not slide up or down with movement. Although most children do not suffer symptoms, some children have scoliosis, or progressively lose the function of their legs. If a child’s spinal cord is untethered, the child can usually function normally (Source:  March of Dimes). 

EDUCATIONAL IMPLICATIONS:

Each child with spina bifida and hydrocephalus may have difficulties which affect their performance in the classroom.  An IEP (Individualized Education Plan) formed with the help of school professionals and the child’s parents will best meet the child’s needs.

To protect children from hydrocephalus, surgery is performed 48 hours after the child is born, and they will have surgery throughout their childhood.  Schools will need to be flexible and accommodate children with these needs.  (Source:  NICHCY).

To participate in school activities, courts have found it necessary for students with Spina Bifida to have an intermittent catheterization.  In addition to traditional academic curriculum, schools may provide a successful bladder management program to help children learn to catheterize and control their bowel and bladder functions (Source:  NICHCY). 

Children with spinal bifida may need additional supports to assist their organizing and sequencing of materials and ideas.  You may observe the child experiencing problems in attention, memory, decision making, and recall of visually presented materials. Children with shunt hydrocephalus may also a range of perceptual problems and encounter problems moving around, using materials, or writing.  More time may be necessary for children to respond although many will be more successful in experiences that require aural skills rather than visual skills.  Clear choices should be presented to help in decision making and prompts that can assist all children in memory tasks will be particularly useful for children with spina bifida.  Accommodations may be necessary in the placement of furniture and materials in the classroom to allow the child to move easily and as independently as possible.

Having spina bifida, especially those who also have a precocious puberty, can negatively affect a child’s emotional development, social development, and self esteem.  It is important for teachers and parents to understand and be sensitive to the limitations of child with spina bifida.  To encourage social development, parents and teachers should consider activities involving typical children and make proper adaptations accordingly (Source: NICHCY). 

ORGANIZATIONS:

Spina Bifida Association of America
4590 MacArthur Boulevard, NW. , Suite 250
Washington, DC 20007-4226
Telephone: (202) 944-3285 or (800) 621-3141
http://www.sbaa.org/site/PageServer?pagename=index

Since 1973, SBAA is dedicated to enhancing the lives of those with spina bifida and those whose lives they touch. Through its network of 57 chapters, SBA has a presence in more than 125 communities nationwide and serves thousands of people each year.

SBA’s programs give hope and strength to people confronted with the challenges of spina bifida. 

RESOURCES:

Websites for Parents:

http://www.waisman.wisc.edu/~rowley/sb-kids/wbwsb.html

This website compiled by Linda Rowley gives an overview about Spina Bifida and has a variety of resources and support groups for parents to connect with in their respective area. 

Websites for Children:

http://www.sparktop.org/intro.html

A free interactive website created by Charles Schwab for kids who have dyslexia.  Children with dyslexia have similar learning difficulties as those with spina bifida (organizing their thoughts, problem solving, etc…).  Thus, children are able to strengthen their skills at home to help them succeed in school and life. 

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