Carlie Jackson, Gretchen Simakov, Courtney Barnum
Table of Contents
Types of Spina Bifida 3
Spina Bifida Occulta 3
Spina Bifida Meningocele 4
Spina Bifida Myelomeningocele 4
TR Implications 22
Definition of Spina Bifida
Spina bifida is a neural tube defect that happens in the first month of pregnancy when the sides of the spine do not come together- leaving an open area where the spinal cord and its membranes, or meninges, may or may not push through and create a fluid filled sac on the exterior of the baby. This spinal defect occurs just a few weeks (21 to 28 days) after conception- usually before the woman knows that she is pregnant.
Spina bifida is the most common permanently disabling birth defect in the United States. Spina bifida is one of the most common severe birth defects, historically occurring in 1 live birth per 1,000 in the United States. The rates of Spina bifida are higher in Hispanics and whites of European descent.
Types of Spina Bifida
There are two classifications of Spina Bifida: SB Occulta and SB Manifesta. SB Manifesta is further divided into meningocele and myelomeningocele.
Spina Bifida Occulta
Spina bifida occulta is known as the “hidden spina bifida” because the defect is not easily seen and many who have this form of SB don't even know that they have this defect. Most of the time, SB occulta is diagnosed later in life accidentally when the patient has an X-Ray or MRI for some other reason. This type of spina bifida happens when the vertebrae are malformed but the spinal cord and meninge are undamaged. Spina bifida occulta is the mildest form of this disease and persons with this diagnosis may not have any symptoms at all, though if they do have symptoms, they may include:
- pain in the back or legs
- muscle weakness in the legs
- numbness or loss of feeling in the legs or back
- deformed feet, legs and back
- dysfunctional bowel and bladder systems
There are a few physical characteristics of occulta that include:
- small tuft of hair
- fat patch over the occulta
Spina Bifida Meningocele
Meningocele is a form of spina bifida in which the meninge layers push through the hole in the vertebrae. The meninge layers then form a fluid-filled sac on the baby's back that is usually covered in skin. This is not as severe as the last form of spina bifida because the nerves are not protruding through the hole in the vertebrae and the nerves usually remain unharmed. This condition must be corrected within a few days after birth so there is no future nerve damage. Though there are not major complications, there may be some mild disabilities to the patient including:
- fluid-filled sac visible on the baby's back
- muscle weakness
- can have loss of feeling in legs
Spina Bifida Myelomeningocele
Spina Bifida Myelomeningocele is the most severe and most common form of spina bifida. Myelomeningocele is when the meninges and spinal cord push through the hole in the baby's back creating a visible sac. This is serious because in this form of spina bifida, nerve damage can be severe. A baby with myelomeningocele requires surgery soon after birth to replace the nerves back into the spinal column. Today, most children with myelomeningocele survive and most are able to live productive lives with some independence. Some of the characteristics of myelomeningocele include:
- Partial of complete paralysis of the legs
- Complete injury
- Muscle weakness
- Leg, hip, and foot deformities
- Arnold Chiari Malformations
- Tethered spinal cord
- Latex allergy
- Bladder and bowel problems
- Urinary tract disorders
- Bone fractures
- Growth hormone deficiency
Symptoms of Spina Bifida
The following are the most common symptoms of spina bifida. However, each baby may experience symptoms differently. Symptoms may include:
- Abnormal appearance of the baby's back, varying from a small, hairy patch or a dimple or birthmark, to a sac-like protrusion that is found along the back bone area.
- Bowel and bladder problems (i.e., constipation, incontinence).
- Loss of feeling below the area of the lesion, especially in babies born with a meningocele or myelomeningocele.
- Inability to move the lower legs (paralysis).
The spinal cord lesion or the scarring due to surgery may result in a tethered spinal cord. In some individuals, this causes significant traction and stress on the spinal cord and can lead to a worsening of associated paralysis, scoliosis, back pain, and worsening bowel and/or bladder function.
68% of children with spina bifida have an allergy to latex, ranging from mild to life-threatening. The common use of latex in medical facilities makes this a particularly serious concern. The most common approach to avoid developing an allergy is to avoid contact with latex-containing products such as examination gloves, condoms, catheters, and many of the products used by dentists.
Many individuals with spina bifida have an associated abnormality of the cerebellum, called the Arnold Chiari II malformation. In affected individuals, the back portion of the brain is displaced from the back of the skull down into the upper neck. In about 90% of the people with myelomeningocele, hydrocephalus also occurs because the displaced cerebellum interferes with the normal flow of cerebro-spinal fluid, causing an excess of the fluid to accumulate. In fact, the cerebellum also tends to be smaller in individuals with spina bifida, especially for those with higher lesion levels.
The corpus callosum is abnormally developed in 70-90% of individuals with spina bifida myelomeningocele; this impacts the communication processes between the left and right brain hemispheres. Further, white matter tracts connecting posterior brain regions with anterior regions appear less organized. White matter tracts between frontal regions have also been found to be impaired.
Cortex abnormalities may also be present. For example, frontal regions of the brain tend to be thicker than expected, while posterior and parietal regions are thinner. Thinner sections of the brain are also associated with increased cortical folding. Neurons within the cortex may also be displaced.
Several studies have demonstrated difficulties with executive functions in youth with spina bifida, with greater deficits observed in youth with shunted hydrocephalus. Unlike typically developing children, youths with spina bifida do not tend to improve in their executive functioning as they grow older. Specific areas of difficulty in some individuals include planning, organizing, initiating, and working memory. Problem-solving, abstraction, and visual planning may also be impaired. Further, children with spina bifida may have poor cognitive flexibility. Although executive functions are often attributed to the frontal lobes of the brain, individuals with spina bifida have intact frontal lobes; therefore, other areas of the brain may be implicated.
Individuals with spina bifida, especially those with shunted hydrocephalus, often have attention problems. Children with spina bifida and shunted hydrocephalus have higher rates of ADHD than typically developing children (31% vs. 17%).Deficits have been observed for selective attention and focused attention, although poor motor speed may contribute to poor scores on tests of attention. Attention deficits may be evident at a very early age, as infants with spina bifida lag behind their peers in orienting to faces.
Individuals with spina bifida may struggle academically, especially in the subjects of mathematics and reading. In one study, 60% of children with spina bifida were diagnosed with a learning disability. In addition to brain abnormalities directly related to various academic skills, achievement is likely affected by impaired attentional control and executive functioning. Children with spina bifida may perform well in elementary school, but begin to struggle as academic demands increase.
Children with spina bifida are more likely than their typically developing peers to have dyscalculia. Individuals with spina bifida have demonstrated stable difficulties with arithmetic accuracy and speed, mathematical problem-solving, and general use and understanding of numbers in everyday life. Mathematics difficulties may be directly related to the thinning of the parietal lobes(regions implicated in mathematical functioning) and indirectly associated with deformities of the cerebellum and midbrain that affect other functions involved in mathematical skills. Further, higher numbers of shunt revisions are associated with poorer mathematics abilities. Working memory and inhibitory control deficiencies have been implicated for math difficulties, although visual-spatial difficulties are not likely involved. Early intervention to address mathematics difficulties and associated executive functions is crucial.
Individuals with spina bifida tend to have better reading skills than mathematics skills. Children and adults with spina bifida have stronger abilities in reading accuracy than in reading comprehension. Comprehension may be especially impaired for text that requires an abstract synthesis of information rather than a more literal understanding. Individuals with spina bifida may have difficulty with writing due to deficits in fine motor control and working memory.
Compared to typically developing children, youths with spina bifida may have fewer friends and spend less time with peers. They may be more socially immature and more passive in social situations. These children have also reported feeling less close to their friends and feel they do not receive as much emotional support from their friendships. Many social difficulties tend to be stable, lasting into adulthood. Youth encountering the most social difficulties tend to have lower executive functioning and shunted hydrocephalus. However, not all studies have found social difficulties in these youth compared with their typically developing peers.
Causes of Spina Bifida
Spina bifida is a type of neural tube defect. Neural tube defects, including spina bifida (open spine) and anencephaly (open skull), are seen in one to two out of 1,000 live births.
During pregnancy, the human brain and spine begin as a flat plate of cells, which rolls into a tube, called the neural tube. If all or part of the neural tube fails to close, leaving an opening, this is known as an open neural tube defect (or ONTD). This opening may be left exposed (80 percent of the time), or covered with bone or skin (20 percent of the time).
Anencephaly and spina bifida are the most common types of ONTD, while encephalocele (in which there is a protrusion of the brain or its coverings through the skull) is much rarer. Anencephaly occurs when the neural tube fails to close at the base of the skull, while spina bifida occurs when the neural tube fails to close somewhere along the spine.
In over 90 percent of cases, an ONTD occurs without a prior family history of these defects. ONTDs result from a combination of genes inherited from both parents, coupled with environmental factors. For this reason, ONTDs are considered multifactorial traits, meaning "many factors," both genetic and environmental, contribute to their occurrence.
Some of the environmental factors that may contribute to ONTDs include uncontrolled diabetes in the mother, and certain prescription medications. According to the Centers for Disease Control and Prevention (CDC), the occurrence rate of ONTDs can vary from state to state and from country to country. The rate of ONTD occurrence in Arkansas is 7.8 out of 10,000 births and 30 out of 10,000 births in Washington. Ireland has the highest number of documented cases, where spina bifida occurs in approximately 4.2 out of 1,000 live births. The factors causing these differences are not well understood.
ONTDs are seen five times more often in females than males. Once a child with an ONTD has been born in the family, the chance for an ONTD to occur again is increased to 3 to 5 percent. It is important to understand that the type of neural tube defect can differ the second time. For example, one baby could be born with anencephaly, while a second baby could have spina bifida (not anencephaly).
Preventions of Spina Bifida
Because the neural tube closes 28 to 32 days after conception and before many women are aware they are pregnant, normal development of the brain and spinal cord may be affected during these first three to eight weeks of pregnancy by the following:
- Genetic problems.
- Exposure to hazardous chemicals/substances.
- Lack of proper vitamins and nutrients in the diet.
- Prescription drug and alcohol consumption.
Although many factors are related to the development of spina bifida, research has found that folic acid (vitamin B-12), a nutrient found in some green, leafy vegetables, nuts, beans, citrus fruits, and fortified breakfast cereals, can help reduce the risk of neural tube defects. For this reason, the American College of Medical Genetics (ACMG) and the Centers for Disease Control and Prevention (CDC) recommend that all women of childbearing age take a multivitamin containing folic acid. If a couple has had a previous child with an ONTD, a larger amount of folic acid is recommended and can be prescribed by the woman's physician or healthcare provider. This allows the woman to take it for one to two months prior to conception, and throughout the first trimester of pregnancy, to reduce the risk of another child with ONTD.
Additional risk factors include:
- Maternal age (spina bifida is more commonly seen in teenage mothers).
- History of miscarriage.
- Birth order (first-born infants are at higher risk).
- Socioeconomic status (Children born into lower socioeconomic families are at higher risk for developing spina bifida. It is thought that a poor diet, lacking essential vitamins and minerals, may be a contributing factor).
Folic Acid and How it Relates to Spina Bifida
It's critical to have enough folic acid in your system by the early weeks of pregnancy to prevent spina bifida. Because many women don't discover that they're pregnant until this time, experts recommend that all women of childbearing age take a daily supplement of 400 micrograms (mcg) of folic acid. Several foods, including bread, pasta, rice and breakfast cereals, are fortified with 400 mcg of folic acid per serving. Folic acid may be listed on food packages as folate, which is the natural form of folic acid found in food.
What is folic acid?
Folic acid is a vitamin that the body needs to grow and be healthy. It is found in many foods, but the man-made or synthetic form in pills is actually better absorbed by our bodies
If you're actively trying to conceive, most pregnancy experts believe supplementation of at least 400 mcg of folic acid a day is the best approach for women planning pregnancy. Your body doesn't absorb folate as easily as it absorbs synthetic folic acid, and most people don't get the recommended amount of folate through diet alone, so vitamin supplements are necessary to prevent spina bifida. And, it's possible that folic acid will also help reduce the risk of other birth defects, including cleft lip, cleft palate and some congenital heart defects.
It's also a good idea to eat a healthy diet, including foods rich in folate or enriched with folic acid. This vitamin is present naturally in many foods, including:
- Citrus fruits and juices
- Egg yolks
- Dark green vegetables, such as broccoli and spinach
If you have spina bifida or if you've given birth to a child with spina bifida, you'll need extra folic acid before you become pregnant. If you're taking anti-seizure medications or you have diabetes, you may also benefit from a higher dose of this B vitamin. In these cases, the recommended dose of folic acid may be up to 4,000 mcg (4 mg) beginning one month prior to conception and during the first few months of pregnancy. However, check with your doctor before taking additional folic acid supplements.
Hydrocephalus and How it Relates to Spina Bifida
The term hydrocephalus is derived from the Greek words "hydro" meaning water and "cephalus" meaning head. As the name implies, it is a condition in which the primary characteristic is excessive accumulation of fluid in the brain. Although hydrocephalus was once known as "water on the brain," the "water" is actually cerebrospinal fluid (CSF) — a clear fluid that surrounds the brain and spinal cord. The excessive accumulation of CSF results in an abnormal widening of spaces in the brain called ventricles. This widening creates potentially harmful pressure on the tissues of the brain.
The ventricular system is made up of four ventricles connected by narrow passages.. Normally, CSF flows through the ventricles, exits into cisterns (closed spaces that serve as reservoirs) at the base of the brain, bathes the surfaces of the brain and spinal cord, and then reabsorbs into the bloodstream.
CSF has three important life-sustaining functions: 1) to keep the brain tissue buoyant, acting as a cushion or "shock absorber"; 2) to act as the vehicle for delivering nutrients to the brain and removing waste; and 3) to flow between the cranium and spine and compensate for changes in intracranial blood volume (the amount of blood within the brain).
The balance between production and absorption of CSF is critically important. Because CSF is made continuously, medical conditions that block its normal flow or absorption will result in an over-accumulation of CSF. The resulting pressure of the fluid against brain tissue is what causes hydrocephalus.
What are the different types of hydrocephalus?
Hydrocephalus may be congenital or acquired. Congenital hydrocephalus is present at birth and may be caused by either events or influences that occur during fetal development, or genetic abnormalities. Acquired hydrocephalus develops at the time of birth or at some point afterward. This type of hydrocephalus can affect individuals of all ages and may be caused by injury or disease.
Hydrocephalus may also be communicating or non-communicating. Communicating hydrocephalus occurs when the flow of CSF is blocked after it exits the ventricles. This form is called communicating because the CSF can still flow between the ventricles, which remain open. Non-communicating hydrocephalus - also called "obstructive" hydrocephalus - occurs when the flow of CSF is blocked along one or more of the narrow passages connecting the ventricles. One of the most common causes of hydrocephalus is "aqueductal stenosis." In this case, hydrocephalus results from a narrowing of the aqueduct of Sylvius, a small passage between the third and fourth ventricles in the middle of the brain.
Hydrocephalus ex-vacuo occurs when stroke or traumatic injury cause damage to the brain. In these cases, brain tissue may actually shrink. Normal pressure hydrocephalus can happen to people at any age, but it is most common among the elderly. It may result from a subarachnoid hemorrhage, head trauma, infection, tumor, or complications of surgery. However, many people develop normal pressure hydrocephalus even when none of these factors are present for reasons that are unknown.
Who gets this disorder?
The number of people who develop hydrocephalus or who are currently living with it is difficult to establish since there is no national registry or database of people with the condition. However, experts estimate that hydrocephalus affects approximately 1 in every 500 children.
What causes hydrocephalus?
The causes of hydrocephalus are still not well understood. Hydrocephalus may result from inherited genetic abnormalities (such as the genetic defect that causes aqueductal stenosis) or developmental disorders (such as those associated with neural tube defects including spina bifida and encephalocele). Other possible causes include complications of premature birth such as intraventricular hemorrhage, diseases such as meningitis, tumors, traumatic head injury, or subarachnoid hemorrhage, which block the exit of CSF from the ventricles to the cisterns or eliminate the passageway for CSF into the cisterns.
What are the symptoms?
Symptoms of hydrocephalus vary with age, disease progression, and individual differences in tolerance to the condition. For example, an infant's ability to compensate for increased CSF pressure and enlargement of the ventricles differs from an adult's. The infant skull can expand to accommodate the buildup of CSF because the sutures (the fibrous joints that connect the bones of the skull) have not yet closed.
In infancy, the most obvious indication of hydrocephalus is often a rapid increase in head circumference or an unusually large head size. Other symptoms may include vomiting, sleepiness, irritability, downward deviation of the eyes (also called "sunsetting"), and seizures.
Older children and adults may experience different symptoms because their skulls cannot expand to accommodate the buildup of CSF. Symptoms may include headache followed by vomiting, nausea, papilledema (swelling of the optic disk which is part of the optic nerve), blurred or double vision, sunsetting of the eyes, problems with balance, poor coordination, gait disturbance, urinary incontinence, slowing or loss of developmental progress, lethargy, drowsiness, irritability, or other changes in personality or cognition including memory loss.
Symptoms of normal pressure hydrocephalus include, problems with walking, impaired bladder control leading to urinary frequency and/or incontinence, and progressive mental impairment and dementia. An individual with this type of hydrocephalus may have a general slowing of movements or may complain that his or her feet feel "stuck." Because some of these symptoms may also be experienced in other disorders such as Alzheimer's disease, Parkinson's disease, and Creutzfeldt-Jakob disease, normal pressure hydrocephalus is often incorrectly diagnosed and never properly treated. Doctors may use a variety of tests, including brain scans (CT and/or MRI), a spinal tap or lumbar catheter, intracranial pressure monitoring, and neuropsychological tests, to help them accurately diagnose normal pressure hydrocephalus and rule out any other conditions.
Arnold Chiari and How it Relates to Spina Bifida
Chiari malformations (Cs) are structural defects in the cerebellum, the part of the brain that controls balance. Normally the cerebellum and parts of the brain stem sit in an indented space at the lower rear of the skull, above the foramen magnum (a funnel-like opening to the spinal canal). When part of the cerebellum is located below the foramen magnum, it is called a Chiari malformation.
CMs may develop when the bony space is smaller than normal, causing the cerebellum and brain stem to be pushed downward into the foramen magnum and into the upper spinal canal. The resulting pressure on the cerebellum and brain stem may affect functions controlled by these areas and block the flow of cerebrospinal fluid (CSF)— the clear liquid that surrounds and cushions the brain and spinal cord—to and from the brain.
What causes these malformations?
CM has several different causes. It can be caused by structural defects in the brain and spinal cord that occur during fetal development, whether caused by genetic mutations or lack of proper vitamins or nutrients in the maternal diet. This is called primary or congenital CM. It can also be caused later in life if spinal fluid is drained excessively from the lumbar or thoracic areas of the spine either due to injury, exposure to harmful substances, or infection. This is called acquired or secondary CM. Primary CM is much more common than secondary CM.
How are they classified?
CMs are classified by the severity of the disorder and the parts of the brain that protrude into the spinal canal.
Type I involves the extension of the cerebellar tonsils (the lower part of the cerebellum) into the foramen magnum, without involving the brain stem. Normally, only the spinal cord passes through this opening. Type I—which may not cause symptoms—is the most common form of CM and is usually first noticed in adolescence or adulthood, often by accident during an examination for another condition. Type I is the only type of CM that can be acquired.
Type II, also called classic CM, involves the extension of both cerebellar and brain stem tissue into the foramen magnum. Also, the cerebellar vermis (the nerve tissue that connects the two halves of the cerebellum) may be only partially complete or absent. Type II is usually accompanied by a myelomeningocele—a form of spina bifida that occurs when the spinal canal and backbone do not close before birth, causing the spinal cord and its protective membrane to protrude through a sac-like opening in the back. A myelomeningocele usually results in partial or complete paralysis of the area below the spinal opening. The term Arnold-Chiari malformation (named after two pioneering researchers) is specific to Type II malformations.
Type III is the most serious form of CM. The cerebellum and brain stem protrude, or herniate, through the foramen magnum and into the spinal cord. Part of the brain’s fourth ventricle, a cavity that connects with the upper parts of the brain and circulates CSF, may also protrude through the hole and into the spinal cord. In rare instances, the herniated cerebellar tissue can enter an occipital encephalocele, a pouch-like structure that protrudes out of the back of the head or the neck and contains brain matter. The covering of the brain or spinal cord can also protrude through an abnormal opening in the back or skull. Type III causes severe neurological defects.
Type IV involves an incomplete or underdeveloped cerebellum—a condition known as cerebellar hypoplasia. In this rare form of CM, the cerebellar tonsils are located in a normal position but parts of the cerebellum are missing, and portions of the skull and spinal cord may be visible.
Another form of the disorder, under debate by some scientists, is Type 0, in which there is no protrusion of the cerebellum through the foramen magnum but headache and other symptoms of CM are present.
What are the symptoms of a Chiari malformation?
Individuals with CM may complain of neck pain, balance problems, muscle weakness, numbness or other abnormal feelings in the arms or legs, dizziness, vision problems, difficulty swallowing, ringing or buzzing in the ears, hearing loss, vomiting, insomnia, depression, or headache made worse by coughing or straining. Hand coordination and fine motor skills may be affected. Symptoms may change for some individuals, depending on the buildup of CSF and resulting pressure on the tissues and nerves. Persons with a Type I CM may not have symptoms. Adolescents and adults who have CM but no symptoms initially may, later in life, develop signs of the disorder. Infants may have symptoms from any type of CM and may have difficulty swallowing, irritability when being fed, excessive drooling, a weak cry, gagging or vomiting, arm weakness, a stiff neck, breathing problems, developmental delays, and an inability to gain weight.
Are other conditions associated with Chiari malformations?
Individuals who have a CM often have these related conditions:
Hydrocephalus is an excessive buildup of CSF in the brain. A CM can block the normal flow of this fluid, resulting in pressure within the head that can cause mental defects and/or an enlarged or misshapen skull. Severe hydrocephalus, if left untreated, can be fatal. The disorder can occur with any type of CM, but is most commonly associated with Type II.
Spina bifida is the incomplete development of the spinal cord and/or its protective covering. The bones around the spinal cord don’t form properly, leaving part of the cord exposed and resulting in partial or complete paralysis. Individuals with Type II CM usually have a myelomeningocele, a form of spina bifida in which the bones in the back and lower spine don’t form properly and extend out of the back in a sac-like opening.
Syringomyelia, or hydromyelia, is a disorder in which a CSF-filled tubular cyst, or syrinx, forms within the spinal cord’s central canal. The growing syrinx destroys the center of the spinal cord, resulting in pain, weakness, and stiffness in the back, shoulders, arms, or legs. Other symptoms may include headaches and a loss of the ability to feel extremes of hot or cold, especially in the hands. Some individuals also have severe arm and neck pain.
Tethered cord syndrome occurs when the spinal cord attaches itself to the bony spine. This progressive disorder causes abnormal stretching of the spinal cord and can result in permanent damage to the muscles and nerves in the lower body and legs. Children who have a myelomeningocele have an increased risk of developing a tethered cord later in life.
Spinal curvature is common among individuals with syringomyelia or CM Type I. Two types of spinal curvature can occur in conjunction with CMs: scoliosis, a bending of the spine to the left or right; and kyphosis, a forward bending of the spine. Spinal curvature is seen most often in children with CM, whose skeleton has not fully matured.
CMs may also be associated with certain hereditary syndromes that affect neurological and skeletal abnormalities, other disorders that affect bone formation and growth, fusion of segments of the bones in the neck, and extra folds in the brain.
Diagnosing Spina Bifida
There are many tests and exams that are optional but can be done before birth to identify a fetus with spina bifida. There are non-invasive methods such as blood tests and ultrasounds as well as invasive methods such as an amniocentesis.
- Blood Test- testing the mother's blood for the presence of AFP, a plasma protein that is normally produced by the fetus, in the mother's blood. Checking for other protein's will also help give an idea as to whether or not to take the more invasive tests.
- Ultrasound- this test allows doctors to see an image of the baby still in the womb. When the fetus has the most severe form of spina bifida, myelomeningocele. This is a more sure way of finding out if the fetus has spina bifida because you can visually see the deformity on the back of the baby.
- Amniocentesis- this is the best way to diagnose spina bifida during pregnancy but it also has the most risks involved. An Amniocentesis test is inserting a needle to withdraw amniotic fluid from within the amniotic sac which surrounds the baby and holds some of the baby's DNA. Chromosomal testing is done on the fluids to determine any defects.
Testing can also be done after birth to diagnose spina bifida. Sometimes there is a hairy patch of skin or dimple on the baby's back that may initiate a scan or x-ray to get a clearer view of the spine. Sometimes spina bifida is not diagnosed until after the baby is born because the mother did not receive prenatal care or it didn't show on the ultrasound or other tests.
Treating Spina Bifida
Treatment depends on the type of spina bifida you are diagnosed with. Spina bifida occulta does not usually need treatment of any kind but those diagnosed with either spina bifida manifesta will need treatment and correction depending on the severity. Surgery is used to correct meningocele to place the exposed layers of meninge back into the vertebrae and close the opening in the back. For those with myelomeningocele will need the most immediate treatment, either performed in utero or within the first 48 hours after birth. The surgery consists of doctors pushing the spine and its meninges back into the vertebrae and closing the hole to protect the spine. These surgeries have an 80 percent success rate. Surgery before birth is only done when the mother and baby are seriously endangered.
Physical therapy early on helps, counseling to improve self-image. Intense surgery can be done on infants to increase their survival rate. Many children will need braces or crutches to help walk, and physical therapy early can prepare them for that.
Those born with myelomeningocele suffer from hydrocephalus. In order to stop the rush of fluid to the brain, doctors can insert a shunt, or thin tube, that will relieve the pressure on the brain by diverting the fluid to other parts of the body. Further surgeries will need to be done to correct legs, hips, and feet in someone with spina bifida.
People with Spina Bifida must learn mobility skills and, with the use of crutches, braces, or wheelchairs, can achieve more independence. Also, with new techniques, children can become independent in managing their bowel and bladder problems. Physical disabilities like Spina Bifida can have profound effects on a child's emotional and social development. It is important that health care professionals, teachers and parents understand the child's physical capabilities and limitations. To promote personal growth, they should encourage children (within the limits of safety and health) to be independent, to participate in activities with their non-disabled peers and to assume responsibility for their own care.
With help, children with Spina Bifida can lead full lives. Most do well in school, and many play in sports. Because of today’s medicine, about 90 percent of babies born with Spina Bifida now live to be adults, about 80 percent have normal intelligence and about 75 percent play sports and do other fun activities.
Accommodation ideas for individuals with Spina Bifida:
- Bowel and/or Bladder Impairments: An individual with spina bifida may benefit from a flexible work schedule, frequent restroom breaks throughout the work day, moving the workstation closer to the restroom, or working at home.
- Lifting: Accommodations for lifting limitations vary depending on the situation. Accommodations may include assistance with lifting, lifting devices, or a transfer to another job that does not require lifting.
- Impairment of the Use of the Arms and Legs: A wheelchair or scooter may accommodate individuals who have difficulty moving around a workplace. Adjustable workstations for office and industrial settings also help individuals convert standing areas to sitting workstations or vice versa. For building access, accessible parking, ramps, stair and wheelchair lifts, and elevators are helpful.
- Low Vision: An individual with a spina bifida may have low vision. For information on accommodations for individuals with vision impairments, visit SOAR's Accommodations for Vision Impairments or JAN's general publication on Work-site Accommodation Ideas for Individuals with Vision Impairments.
- Use of a Wheelchair: Some individuals with spina bifida use wheelchairs and the limitations and accommodations vary. For accommodations for individuals who use wheelchairs, visit SOAR's Accommodations for Wheelchair Use or JAN's general publication on Work-site Accommodation Ideas for Office Workers who Use Wheelchairs.
- Brain: Those with hydrocephalus (a common condition for those with spina bifida) can be associated with impairments in cognitive function, learning problems, and behavior challenges.
Perceptual Motor Problems: Children with shunted hydrocephalus often have problems with eye-hand (visual-motor) activities. Visual perception problems mean the child may have problems "seeing" things in their head, finding their way around, and generally being less coordinated. Fine-motor skills are often rather poor among spina bifida children. Together these weaknesses typically interfere with the ability to move around, use materials or tools and perform academics such as reading, arithmetic and writing.
- It is very important that children have all senses stimulated - seeing, hearing, smelling, touching, tasting and moving.
- Youngsters with spina bifida require physical exercise and help developing awareness in space. This includes rolling, turning, somersaulting, etc. These are seen as basic skills needed by children with spina bifida.
- Beginning in the early years, help children learn basic relational ideas (up/down, in/out, right/left, top/bottom, etc.).
- Spend time and energy on games and activities which encourage eyes and hands working together, including threading objects on a string, building blocks, legos, throwing and catching balls according to age, etc. Even games like Nintendo can be helpful in this development, if used sparingly and not as the total program.
- In addition, encourage children to color and paint, work with clay, cut (blunt scissors) and paste.
- Children often can enjoy eye-hand activities which are also imaginative like "take-apart" toys, or old broken clocks, radios, etc.
- Because of difficulty in copying from board to paper, when possible, provide a printed copy of board material or allow a responsible peer to share his/ her notes.
Comprehension: Children with spina bifida sometimes have a hard time understanding things even though they seem to understand. This is particularly true of some kids who speak well, but when they have to explain what they said, or respond to questions, they seem disorganized, and talk about irrelevant things. They may change the subject in the n-dddle of what they are saying.
This problem usually goes away by the time the child is about 10 years old but comprehension problems often go beyond this age. When it goes beyond this time, working to make it better is difficult. Several strategies can be used to help children who are having trouble understanding.
- Use visual maps, demonstrations and simplified verbal explaining to help the youngster "get a picture" of what is being said (or read).
- Encourage the child to assume roles and act out characters in picture story books. In this way, the child actually experiences what is happening and can understand better.
- Begin early to help the child explore topics at their own developmental level. Ask questions that help the child stay on the topic, and let you know the child understands what they're talking about.
- As the child begins to read, talk with him/her about what they are reading to be sure they know what they're reading.
- Help the child capture "the main idea" of a story, movie or conversation.
- Encourage the child to read the questions following a chapter prior to the reading of the Chapter. This can help with organization and comprehension of the material by emphasizing its most important points.
Attention: It is quite common for children with spina bifida to have trouble paying attention to parents, teachers, friends, tasks, etc. This at times gets mixed up with a child being emotionally self-centered and not being attentive to other people's needs. Both may be true. Inattention, however, is particularly a problem in school. Children may miss assignments, miscopy assignments or work, generally be slow in completing work (beyond visual-motor speed problems), or miss social cues from others. Children with spina bifida generally are better able to pay attention when listening than when seeing.
- Beginning in the early years encourage your child to pay attention to their room; home, yard, and neighborhood. Take walks and emphasize attention to sights and sounds.
- Let the child know that you (parent/teacher/ professional) realize she/he may have trouble paying attention. Make an agreement that you will give them a "secret sign" just between the two of you to let them know they are not paying attention. Embarrassment and conflicts are reduced and better relationships are formed, plus attention is better.
- Be sure you have the child's attention before telling or showing them something. This can be insured by waiting for eye contact before you begin.
- As much as possible, give the youngster a quiet place to work with few distractions. At school, a seat on the front row or close to the teacher may reduce distractions.
- Give short assignments or chores that can be done successfully; this increases concentration.
- Work on tasks in several short periods of time with breaks between. Let the breaks include helpful chores. Whether at school or at home, do the tasks frequently to encourage complete learning. You may try to increase the length of work periods as time goes on.
- A system of reinforcements for work completed on time may be needed. This might require help from a social worker, nurse, or psychologist to set up a program.
- Finally, if inattention continues to be a problem evaluation by a psychologist and pediatrician or neurologist for an attention deficit should be completed. A therapeutic trial of medication under the supervision of a physician may be considered. Medication often can allow children to pay attention better, thereby learning more and relating better.
Hyperactivity/ Impulsivity : Children with spina bifida often exhibit restlessness and are fidgety. It is often surprising to teachers or even parents who think since an orthopedic disability slows the child down, they can't be hyperactive. But this is not surprising when one is aware of visual-motor problems. While the physical impairment may mask the restlessness, it is often present. Often associated with inattention and hyperactivity is impulsivity Children who are impulsive act before they think. This lack of stopping and thinking often gets them into trouble because they end up doing things quickly and carelessly, which gets them into trouble with adults and friends, and can be unsafe.
- Set up an exercise routine to reduce physical tension.
- The youngster should be encouraged to stop and think about what is to be done and how it is to be done. The old adage "count to 10 before you act" may be helpful.
- Give positive reinforcement for taking a longer time on a task already successfully completed.
- Do not require long periods of independent work.
- Notice situations which seem to increase restlessness of impulsivity, and discuss these with the child.
- If the behaviors continue as time goes on, as with inattention, an evaluation by a psychologist and pediatrician or neurologist should he completed. Medication, again, may be helpful when given under the supervision of a physician.
- Provide an opportunity for "light" physical exercise or movement between periods of study or work.
Memory: Children with spina bifida often have difficulty in remembering things they see or hear. Even if they understand it, they may not remember it later. So it's like they have to learn it over and over again. This can happen when people are telling them things to do or when they are copying assignments from the board. It seems hard to remember one thing, while they're trying to do another.
- There are different kinds of memory - seeing (visual) memory, hearing (auditory) memory, short-term and long-term -memory to name some. Most children have some parts of memory better than the others. Help the child or adolescent identify which kind of memory they're better at.
- Help children pair (associate) things together which need to be remembered.
- Don't hesitate to use reminders, writing events or appointments on a central calendar at home, or carrying a personal calendar. Computers and calculators also can help.
- Use watches with regular alarms for remembering IIL's for example.
- Often one can remember better if you will say something over and over to yourself (or even out loud), like when you're learning multiplication tables or spelling words (rehearsal). Or you can help the child or adolescent think of mental pictures which they can see each time they want to remember a certain event.
- Teach and encourage the use of note-taking, outlining and summary skills.
- The use of a tape recorder can be helpful in enhancing memory.
Organization: Children with spina bifida may have trouble keeping things organized. This is clearly seen when school materials, papers, etc. need to be in order. Things tend to get lost or misplaced, creating frustration, anxiety, and anger among parents, teachers and even the child, at times.
- An old saying goes "Everything has a place and a place for everything." Very early in a family's life, it is particularly important to help the youngster learn this organizational principle.
- As much as possible, parents and teachers should model organization. Keep your own things organized as best you can.
- Reduce verbal explanations that tend to be confusing. (Don't explain everything.)
- Keep the number of items as few as possible that have to be used at any one time - whether at school or home - e.g. regularly take toys or materials, etc. not being used out of the child's room, school locker, or desk.
- Encourage children to think about what they will need for an activity (e.g. social, school, or self-care) beforehand and get those things together (social might include what we take on a picnic, school might include what materials and books are to be taken, and self-care might include what is needed for doing IC's).
Sequencing: Children and adolescents with spina bifida often have trouble keeping ideas or doing activities in their proper order. This problem may be related to not paying attention, not remembering or not being organized. The result is the same - the child, parent and/or teacher begin to feel confused and frustrated because the steps are there - just mixed-up. It may also seem that the child or adolescent doesn't understand or comprehend the situation or question. These sequencing (ordering) problems can be seen in the school subjects of math and written language. It is also seen in not being able to tell time and count change. The young person can verbally tell a good story or report what they've seen in an orderly way until they have to write it down. They cannot organize (sequence) the ideas in their head.
- Early in life, help the youngster work on getting their eyes and hands working together. This is a classic early step toward sequencing.
- Encourage dot-to-dot and other similar activities in which order and sequence are important.
- Work consistently on time concepts and learning to do things in specific steps, one at a time.
- Start with 2-step instructions and try to help the child develop so that they can follow 3-4 step activities. This will be particularly important when the child needs to learn to do their own bladder and bowel programs.
- Develop "games" where the parent or teacher tries to change the order of certain everyday activities such as a) putting food down without utensils or glasses or b) instructing the child to start work on an assignment without giving the page number. Let the child, then, provide the proper sequence of events.
- With older children and adolescents, allow them to begin scheduling daily activities, perhaps initially by writing out a schedule each day or week.
- Provide exercises which emphasize the concept of sequence such as sequencing pictures cut from a comic strip can provide logical ordering of events. This is a prerequisite for reading comprehension.
Decision Making / Problem Solving: As you can imagine, if any person has trouble paying attention, remembering information, organizing things and keeping ideas in order, they will probably have difficulty making decisions and solving problems. Making a decision is a different process from solving a problem. One makes a decision when he/she has two or three choices and has to choose one of them, for example, what to eat or what to wear. Solving a problem usually means that you have a situation that requires you to use what you've learned in the past to solve a new problem now.
- Beginning early in life, give children a choice from two or three options, e.g. water, milk, or juice, or the white dress or the blue dress or playing indoors or outdoors.
- The next step is to help children realize that certain consequences go with their decisions. If you choose this, then this will happen. Actions become paired with consequences.
- Overall problem solving is usually best learned informally over many years in many situations little-by-little. As with decision making, start early and be a model for your child. Talk to them about how you decide things and solve problems. If there comes a time when the child seems not to be continuing to improve their problem solving or decision making, contact professionals.
- Be aware of the general rules of problem solving.
- Figure out the problem
- Come up with different ways to solve the problem.
- Decide on one way by exploring the advantages and disadvantage of the different choices.
- Try it and then see how it works. If it doesn't work, then try again. As you try different approaches, the child and adolescents can learn to be a better problem solver and can make decisions more effectively and timely.
Children with myelomeningocele often experience restricted play and recreational opportunities because of limited mobility and physical limitations. This inactivity decreases the potential for normal development in all spheres and can exert a negative impact on self-esteem.
For the infant and toddler with myelomeningocele, recreational therapy enhances opportunities for environmental exploration and interaction with other children. For the school-aged child, recreational therapy provides opportunities for participation in adapted sports and exercise programs, which can result in long-term interest in personal fitness and health.
Recreational and physical fitness goals include socialization, weight control, and improved fitness (eg, flexibility, strength, aerobic capacity, cardiovascular fitness, coordination). Recreational therapy is helpful for promoting independence with adult living skills and often is used to assist the patient with shopping for and purchasing personal items, use of public transportation, and development of appropriate leisure activities.
Sports and other recreational activities are an important part of a healthy lifestyle, and a good way to build self-esteem. Children with spina bifida can participate in many sports and leisure activities. Participation will not only provide excellent social opportunities, but will also help them to control their weight more easily.
NOTE: Individuals with shunted hydrocephalus should avoid contact sports (ie football or boxing), prolonged pressure on the head (ie head stands), and excessive stretching of the shunted tube (ie somersaults).
Children should be encouraged to become involved in physical games and sports to the extent of their ability. Find ways to adapt activities so that they can participate. Some activities to try:
- horseback riding
- adapted baseball
- canoeing / rowing
There are also many wheelchair sports available that individuals with decreased may want to participate in:
- racing and/or athletics
- quad rugby
There is an association for spina bifida in almost every state or region and their websites can be found online. Example: Spina Bifida association of Arkansas or Colorado Spina Bifida Association.
There are also a summer camps available to those who have spina bifida, a list of them can be found at spinabifidaassociation.org
- Spina Bifida(March of Dimes Birth Defects Foundation)
- Spina Bifida(National Institute of Neurological Disorders and Stroke) -Short Summary
- Spina Bifida(Nemours Foundation)