Therapeutic Recreation Handy Dandy Notebook: Spina Bifida

What is Spina Bifida?

Spina bifida, also known as myelomeningocele, is a birth defect. During the first 28 days of pregnancy, the brain and the spinal cord of an embryo (developing baby) form. For some reason that is not well understood, this development is interrupted in some babies and spina bifida occurs.

Spina bifida literally means split spine. The name comes from the fact that the bones or vertebrae of the spinal column which surround the developing spinal cord do not close as they normally would but, instead, remain open.

The figure below shows the process of when spina bifida occurs. The neural fold of a developing spinal cord starts to form at day 20 of a pregnancy. The neural tube starts closing at day 21, and by day 28 is should be completely closed. If there is incomplete closure of a Neural Tube at day 28, then a fluid cyst forms, therefore knowing the baby has spina bifida.

Types of Spina Bifida and Associated Symptoms

There are three different types of Spina Bifida: Spina Bifida Occulta, Meningocele, and Myelomeningocele.

Spina Bifida Occulta

Spina Bifida Occulta means “hidden split spine”. This is where only the bones of the spinal column will be incompletely developed and that the nervous tissue beneath will be normal. The skin of the back is also normal. It usually occurs at the lower end of the spine and rarely causes medical problems.

Spina Bifida Occulta is a common variation of normal spinal cord development and is found in 10-20 percent of the general population.

Symptoms. Forms of Occulta that causes problems:

Tethered spinal cord--the lower end of the spinal cord has an abnormal attachment to surrounding structures. The spinal cord gets stretched and damaged
Lipomyelomeningocele and lipomeningocele--A tethered spinal cord attached to a benign fatty tumor in the back
Thickened filum terminale--A thickening in the end section of the spinal cord
Fatty filum terminale--A fatty lump at the inside end of the spinal cord
Diastematomyelia (split spinal cord) and Diplomyelia--A spinal cord split in two, usually by a piece of bone or cartilage
Dermal sinus tract ( with involvement of the spinal cord)--A connection between the spinal canal and the skin of the back that appears as a band of tissue to the outside

Visual Signs of Spina Bifida Occulta:

Hairy patch
Fatty lump
Hemangioma--a reddish or purplish spot on the skin made up of a collection of blood vessels
Dark spots or biri the blue-black marks called Mongolian spots
Skin tract (tunnel) or sinus--this can look like a deep dimple, especially if it is too high (higher than the buttocks crease), or if its bottom cannot be seen
Hypopigmented spot--an area with decreased skin color

Meningocele

Meningocele is the rarest form of Spina Bifida. When this defect occurs, the meninges (membranes that surround and protects the spinal cord) extend through the unclosed spine to create a lump or sac on the dorsal side of an infant. The spinal cord remains intact and is often undamaged. Because the spinal cord itself is not damaged, neurological damage is not sustained. Therefore, surgery is typically successful to remove the cyst, allowing for normal development.

Myelomeningocele

Myelomeningocele is the most significant type of spina bifida. This is when a portion of the spinal cord is undeveloped, the overlying bones are not fully formed, and there is no skin covering the open bones or spinal cord. This can happen anywhere along the spine, but it is most common in the lower portions (from upper lumbar to the upper sacral areas). Most babies who are born with this type of spina bifida also have hydrocephalus (see pg. 9), an accumulation of fluid in and around the brain. Because of the damage to the spinal cord, a child with myelomeningocele typically has some paralysis. The degree of paralysis largely depends on where the opening occurs in the spine. The higher the opening is on the back, the more severe the paralysis tends to be.

Symptoms

According to E Medicine Health website, the symptoms for Myelomeningocele are as follows:

After one or more operations to correct the defects, some disability almost always remains. Various degrees of leg paralysis, spine curvature (scoliosis), hip, foot, and leg deformities, and problems with bowel and bladder control are the most common residual disabilities.
Abnormalities at the lower spine are always accompanied by upper spine abnormalities (Arnold-Chiari malformation), causing subtle coordination problems that usually can be improved by physical therapy.
Spine, hip, foot, and leg deformities are often due to imbalances in muscle strength and function resulting mostly from residual paralysis, but with a spasticity component.
The most common bladder and bowel problems are inability to voluntarily relax the muscles that hold urine in the bladder and stool in the rectum.
Hydrocephalus (accumulation of fluid in the brain) is another common residual problem, affecting about 90% of people with spina bifida. Having some fluid around the brain is normal and healthy, but in spina bifida the fluid often cannot drain naturally. Without treatment, this extra fluid can cause neurological problems or mental disabilities; however, these individuals are of intelligence if their hydrocephalus is treated aggressively. Hydrocephalus often recurs gradually after treatment.
Developing a tethered spinal cord (see pg.11)
Obesity due to inactivity.
Pathologic bone fractures. The bones break easily because of weakness or disease in the bones, not because of injury.
Growth hormone deficiency resulting in short stature.
Learning disorders.
Psychological, social, and sexual problems.
Allergy to latex. Thought to be a result of intense exposure to latex in the early years of life because of frequent surgeries and other medical procedures.

Causes

In the United States, there are around 60 million women of childbearing age and each one is potentially at risk of having a pregnancy affected by spina bifida. The cause of spina bifida is unknown to the medical world. Researchers have identified a few risk factors. Some of the risk factors are:

· Lack of folic acid

This vitamin is important to the healthy development of a fetus.

· Genetics

Some evidence suggests that a genetic factor plays a role in spina bifida, but in most cases there is no family connection. However, studies have shown that if a woman has a child with spina bifida, the woman’s daughters have a higher risk of having a child with spina bifida. Therefore, the daughter needs to take precaution when she is to get pregnant and double her intake of folic acid (to learn more about the importance of folic acid, go to pg. 13).

· Taking medication while pregnant

Women with epilepsy who have taken the drug valproic acid to control seizures may have an increased risk of having a baby with spina bifida.

· Diabetes

· High fever during pregnancy

· Mother’s age

Spina bifida is more common in children of teenage mothers

· History of miscarriage

· Birth order

First born children are at higher risk.

Effects

There are three primary areas of abnormal function: the central nervous system (brain and spinal cord), the urologic system (kidneys and bladder), and the musculoskeletal system (bones and muscles). These areas of functioning can be affected when the spinal cord is not fully formed. Messages passed up and down the spinal cord carry information between the brain and all areas of the body. The messages cannot be sent to or received from areas below the spina bifida. As a result, several body systems cannot function properly.

How does spina bifida affect the central nervous system?

Spinal cord. At the point of the spina bifida defect, the normal passage of electrical impulses, or messages, from the brain to the nerves serving the rest of the body and from the body back to the brain is interrupted. A person with spina bifida will not be able to have control of muscles served by the abnormal nerves. There is typically no feeling in the skin below the spina bifida. This point along the spinal cord where no functioning begins is referred to “level” of the spina bifida, but the level of abnormal function cannot be determined simply by where the opening is along the spinal cord of an infant. Therefore, special examination by a specialist is necessary to precisely determine spina bifida level. In some cases, individuals may have partial muscle functioning or parts of skin with feeling below the spina bifida. This is called “sparing” of function or “spared” function.

Before a baby is born, the fluids surrounding the baby protect the open spine and exposed abnormal nerves from damage and infection. After birth the opened area can become infected and damaged. In most cases, within 24 to 72 hours of being born a neurosurgeon will perform a surgery to return the spinal cord to as normal a position as possible and to protect any functioning nervous tissues from any injury and infection. Unfortunately there is not a surgery that will restore missing muscle control or skin sensitivity.

Brain. In individuals with spina bifida, the brain is developed further down in to the upper spinal column than it should be. This abnormal positioning of the brain is known as the Arnold Chiari malformation. This malformation blocks flow of the spinal fluid, which normally moves around and in the cavities or “ventricles” of the brain. This condition of overly filled ventricles is called hydrocephalus.

Chiari II malformation can cause other problems with the brain. It can lead to serious problems that include the follow:

· Inability to regulate breathing

· Loss of normal vocal cord function

· Difficulty swallowing

· Gagging with food or drink

· Poor strength or function in the arms and hands

Individuals with spina bifida that are experiencing any of these problems may require surgery to relieve pressure on the back of the brain.

Hydrocephalus means “water on the brain”. It is found on prenatal ultrasounds in most infants with myelomeningocele. The fluid will need to be drained from the brain, so neurosurgeons implant a device called a “shunt” to allow drainage of the spinal fluid and prevent severe hydrocephalus. The shunt device can become clogged, may break, or be outgrown. When the shunt fails it will need to be replaced. The table on the right shows signs of shunt failure at particularly young-aged children.

Signs of hydrocephalus in infants may include:

· Rapid head growth

· Full or tense soft spot

· Unusual irritability

· Repeated vomiting

· Inability to look up, Crossed eyes

· Periods in which the baby stops breathing

· Difficulty swallowing

· Horse or weak cry

· Difficulty in keeping the infant awake

· Worsening brain function

How does spina bifida affect the urologic system?

Bladder and kidneys. Normal bladder control is usually not possible in individuals with spina bifida because nerves that control the bladder are almost always abnormal. This abnormality causes individuals to not feel when their bladder is full. If the bladder is not emptied properly and on a regular basis it can cause the bladder to force the urine backward into the kidneys. This process is called reflux. Because a bladder can change as an individual ages, regular checkups are encouraged so that kidney and bladder problems can be detected and prevented.

Most children with spina bifida cannot be toilet trained because they do not have a normal control of urination. Therefore, most children learn to empty their bladders with a catheter. A catheter is a small flexible straw-like device that helps empty the bladder. In both males and females, the catheter is inserted through the urethra and into the bladder. When urine flow stops and the bladder is empty the catheter is removed. The procedure is repeated at intervals prescribed by a urologist, and each individual is going to have a different case.

How does spina bifida affect the musculoskeletal system?

Muscles and bones. Since nerve development and function does not occur below the level of spina bifida, the higher up the spinal cord it is, the greater the paralysis. Each individually must always be evaluated according to their own signs. The table to the right shows the typical amount of function found with different levels of spina bifida. There are a variety of tests that can be used to determine the level of paralysis.

Most people with spina bifida will need to wear leg braces to walk along with crutches or canes. Those with levels at or above lumbar 3 will often use braces for short distances and wheelchair for more physically demanding activities such as shopping. Children with good control of their legs will start using braces so that their legs can be supported and protected from weak muscles and vulnerable joints.

Tethered Spinal Cord. Tethering of spinal cord is a condition where the spinal cord becomes attached to the spinal column and surrounding structures. Whereas a normal spinal cord hangs loose allowing movement freely up and down with growth, bending and stretching, a tethered spinal cord does not move. Rather it is pulled tightly at the end of it, causing blood flow to slow down and the spinal nerves do not get enough blood to them. Therefore a tethered spinal cord causes damage to the spinal cord from both the stretching and decreased blood supply.

Tethering can happen either before or after birth. It can take place in children and adults. It most often occurs in the lower level of the spin (lumbar). Tethering can happen in all types of spina bifida, but it seldom occurs with spina bifida occulta. In children, the spinal cord is stretched with growth because it is stuck down or tethered at the lower end (as seen in the figure below); in adults, the spinal cord will be stretched during the course of normal activity like bending and stretching. If surgery is not performed to treat the tethered spinal cord, it can lead to progressive, permanent spinal cord damage.

Symptoms of a tethered cord can occur when the shunt used to control hydrocephalus fails to work properly. Shunt function may need to be investigated if symptoms of a tethered cord develop.

Treatment

There is no cure for Spina Bifida because the nerve tissues that are damaged cannot be repaired nor function restored. Treatment for the different effects of spina bifida may include surgery, medication, and physiotherapy. Many of those with Spina Bifida will need assistive devices that might include braces, crutches, or wheelchairs. Beginning special exercises for the legs and feet when children are young helps to better prepare the child for walking with corrective devices as they get older. Ongoing therapy, medical attention, or surgery may be required to prevent or manage different complication throughout an individual’s life.

Occulta: Children with the mildest form, spina bifida occulta, may need no treatment, though surgery may become necessary as they get older.

Meningocele: For those diagnosed with meningocele, an operation during infancy is most often required. In this surgery, doctors push the meninges back and close the hole in the vertebrae. Most should not face any other healthy problems later in life.

Hydrocephalus: A baby born with hydrocephalus will need to undergo surgery to have a shunt placed on the brain to relieve pressure on the brain and drain or divert any extra spinal fluid.

Myelomeningocele: When babies are diagnosed with myelomeningocele, action must be taken to prevent any sort of infection from developing in the exposed nerves and tissue through the spina defect. A child born with spina bifida will often have surgery within the first few days of birth to close the defect and protect from infection or trauma.

Prenatal surgery:

Another option for babies diagnosed with myelomeningocele is prenatal surgery. Specific medical centers perform fetal surgery, which is performed in utero (within the uterus) on the fetus before birth. This surgery involves opening the mother’s abdomen and uterus and then sewing up the opening over the baby’s spinal cord. Many doctors feel that the earlier the defects of this disease are corrected, the better the child’s outcome. This surgery still cannot restore any lost neurological function, but it can help to prevent future loss.

Prevention

Spina bifida is a birth defect that can happen to anyone. Though we don’t know the exact causes of the disease, research has shown that the best way to prevent spina bifida is for the mother to have sufficient folic acid levels before and during the first few months of pregnancy. Studies have showsn that as many as 70% of cases of sever spina bifida could have been prevented by adequate folic acide intake by the mother.

Folic Acid is a B-vitamin needed in DNA synthesis. Without the proper levels of folic acide, both the DNA and the cells of the fetus aren’t able to form properly, which formation begins immediately after conception. Folate (natural folic acid) is a nutrient that can be found in: leafy green vegetables, egg yolks, beans and whole-grain products, orange juice, and other citric fruits. Many foods, including breakfast cereals, enriched breads, flours, pastas, rice, and other grain-products, now have folic acid added to them.

Eating a folic acid rich diet is good for you; however, most people do no get enough through food alone. Because of this, it is recommended that all women of childbearing age take a multivitamin containing folic acid. The suggested dose for most women is 400 mcg (0.4 mg) of folic acid daily. Women who plan to become pregnant who have spina bifida themselves, have had a previous child with spina bifida, or have had a previous pregnancy affected by spina bifida or any other neural tube defect are told to take 10x this dose (4,000 mcg, or 4 mg) for a few months before becoming pregnant.

Due to greater awareness regarding the importance of high folic acid levels and advancements in technology that allow for prenatal diagnoses, the number of children who are born with spina bifida are steadily decreasing.

Current research also suggests that a cesarean delivery can reduce the chances and severity of paralysis in babies with spina bifida. Parents who have received a prenatal diagnosis of spina bifida for their baby may wish to choose a planned cesarean delivery.

Rising Above a Disability

Often, the world places limits on people with disabilities and the things they can accomplish. It is the same for people with spina bifida. People assume that they are not able to do everything we can do because of the limitations of their disabilitiy. Luckily for us, there are people who have every kind of disability who rise above and show the world what they’re really made of. Here are a few people with spina bifida who did just that:

Jean Driscoll

Jean is a world-renowned athlete who is now inspiring the world as a professional speaker, motivating corporations, non-profit enterprises, and the U.S. Government.

Jean is an American wheelchair racer. She has won the women’s wheelchair division of the Boston Marathon eight times, which is more than any other female athlete in any division. These wins include seven consecituve first places finishes in the years 1990-1996. In her 1991 race, she set a world record with a time of 1:42:42 and then broke her own world record in 1994. She has also participated in the Summer Paralympic Games and has won five gold medals, three silver, and four bronze medals in events ranging from the 200 meters to the marathon. She is an Olympian and Paralympian and advocates for people with disabilities all around the world.

Though born with spina bifida, Jean never let this stop her from competing and being active just like anyone without this disease. She began using a wheelchair in high school and immediately became involved in many different wheelch air sports. She was recruited by the University of Illinois to play wheelchair basketball for them and ended up joining their wheelchair track and field team as well.

Jean was honored as the Women’s Sports Foundation’s Sportwoman of the year in 1991. In 2000, she was voted #25 of the top 100 female athletes of the 20^th century by Sports Illustrated for Women. In the year 2006, Jean was awarded the Wilma Rudolph Courage Award from The Women’s Sports Foundation. She is an amazing example of a person who refused to let a disability limit what could be accomplished.

Born Carys Davina, “Tanni” Grey-Thompson is a Welsh former wheelchair racer and is now a parliamentarian and television presenter. She is considered to be one of the msot successful disabled athletes in the UK.

Tanni was born with spina bifida and refused to let her disability limit her even at a young age. Her paralympic career began at the Junior National Games for Wales in 1984 swhere she competed in the 100m. At her first international competition in Seoul, she competed in the 400m race and took third place, winning her first bronze medal. Tanni participated in five different Paralympic games, with her last games being in Athens in 2004, where she won two gold medals in wheelchair racing for the 100m and the 400m. In total, her Paralympic career won her 16 medals: 11 gold, 4 silver, and a bronze. She also won 13 World Championship medals: 6 gold, 5 silver, and 2 bronze. She announced her retirement in 2007 , having held over 30 different world records and won the London Marathon six times.

She has sat on the board of the National Disability Council, The Sports Council for Wales, and UK Sport and currrently sits on the board of the London Marathon, the London Legacy Development Corporation, the Board of Transport for London, and is Chair of the Women’s Sports and Fitness Foundation Commission on the Future of Women’s Sport. She has received many different honors in her like and was conferred the title of a Baroness by the House of Lords in March of 2010. She now holds a place in parliament, serving both England and Wales as a crossbencher.

Tanni is another example of a person who wouldn’t let the limits that society places on her determine what she could accomplish. Instead of letting her spina bifida be a hinderance, she has used her disease to push her forward, earn international recognition, and show the world that a disability is not what defines a person. Life’s really about what you can accomplish.

Other Famous People Born with Spina Bifida

· Roald Dahl, Children’s book author

· Rene Kirby, U.S. actor, popular for the movie “Shallow Hal”

· Hank Williams Sr., Country Music Singer and Songwriter

· Mighty Mike McGee, American 2006 World Slam Poetry Champion

· James Connelly, U.S. Paralympian, 2006 Bronze Medal Winner

· Robert M. Hensel, American Poet and world record holder

· John Mellencamp, singer and song writer

· Blaine Harrison, of the British band “Mystery Jets”

· Bruce Payne, American Actor

· Frida Kahlo, Mexican painter

· Aaron Fotheringham, U.S. Wheelchair skateboarder

Special Needs

Because of damage to the spinal cord, paralysis of varying severity can occur as previously discussed. Different equipment is available to people with spina bifida that can be used in each case according to a person’s individual need.

· Ankle-foot braces: when the lesions are located on the lower regions of the spine, sometimes paralysis only affects a person’s ankles. In this case, foot braces can be used to help with mobility, to correct disfigurement and enable the person to get around.

· Walker: a walker is often used when the patient is first learning to walk. These walkers give children with spina bifida the ability to move around, requiring less coordination than crutches or a wheelchair. Many children start by using a walker and then advance to crutches after their legs have developed more strength and the child has gained confidence in their ability to walk more independently.

· Crutches: as with the walkers, crutches are normally used by children whose lesions are found in the lower regions of the spinal cord. It’s possible that they have lost some of the feeling in their legs but do not experience complete paralysis. Because of this, they are able to use what feeling they do have in their legs to use crutches to get around. The crutches used by children with spina bifida are not the same as we would see for someone who had broken a leg and has under-the-shoulder crutches. These crutches have a band that attaches around a child’s arm at the elbow and then has supportive handles just below to hold on to. Crutches are used long term and allow the child to stabilize themselves and move more independently.

· Wheelchairs: for those patients who have experienced more extreme paralysis, meaning the lesions are in a higher location along the spine, wheelchairs are often the best solution to create mobility. A person is not required to have complete paralysis to use a wheelchair, but other means of assistance are more difficult because of the high level of paralysis. Wheelchairs are also used when the patient is going to be out and about for extended periods of time, like a trip to the store or a school field trip. If the lesions are located high enough on the spine that the person experiences extreme paralysis in all four limbs, an electric wheelchair may be needed in order to allow the person to create their own mobility.

· Other needs for children with spina bifida might include physical therapy in order to prevent osteoporosis, urinary catheter, or emotional therapy if the child is teased, rejected, or isolated, which sometimes occurs because of struggles with bladder and bowel drainage and control.

Will a Person with Spina Bifida Walk?

Whether or not a person with spina bifida will be able to walk or not depends on the locations of the lesions. The higher the lesion takes place on the spinal cord, the more paralysis will be present. The diagram below shows the likelihood of a child walking based on the level or location of their lesions. The shaded portions show the parts of the body that is affected by paralysis and loss of feeling.

Nerve Loss Chart:

· T2-T12: lack of feeling at chest and below

· L1: lack of feeling at waist and below

· L2: lack of feeling at upper and outer thighs and below

· L3: lack of feeling at inner thighs, knees, and below

· L4: lack of feeling at inner calves and below

· L5: lack of feeling at outer calves and below

· S1: lack of feeling at heel

· S2: lack of feeling in genitals and buttocks. Wil have almost normal movement and strength in legs, feet, and toes. May affect bowel, bladder, and sed sexual function.

TR Implications

Children who are born with spina bifida are children first, with a disability second. The greatest things we can do as recreational therapists is to teach people struggling with the effects of spina bifida that they are not defined by their disability and are capable of great things. We can help them to build confidence, develop talents, and accomplish their goals. Because of the spectrum of severity with this disability, it is important to make a plan unique to each individual client that addresses their needs and plays to their strengths.

Social development for children with spina bifida can be affected and interrupted by frequent visits to the hospital, limits of mobility, possible learning disabilities, and the lack of bladder control that accompanies a neural tube dysfunction. It is important that we create every opportunity we can for these children to allow them to succeed and experience autonomy.

It is important to encourage people with spina bifida to become involved in physical games and sports as best they can. Sports and other activities can be adapted to the ability of each individual person, making it possible for them to complete the task while pushing them to improve. More than anything, we want to make sure that children with spina bifida feel included and get to experience those things that any “normal” child gets to experience. Here are some possible activities to try:

Horseback riding

Horse therapy is becoming one of the most popular and effective methods of therapy for children dealing with any kind of disability, including spina bifida. The goal of these programs is not only to teach children to ride a horse, but for them to learn how to move with the horse. The movements of a horse’s hips is used to help build up musculoskeletal and motor responses in people with disabilities and has been proven to have physical and mental benefits. As the horse walks, the rider sways and is thrown off balance, requiring them not only to be flexible in their movements, but also to contract and relax different muscles to help them regain their balance.

During therapeutic horseback riding, an certified instructor teaches the children to ride the horse and properly care for the animal. Many of the individuals who participate in these programs develop a relationship with the horse they are caring for, helping to teach them patience, trust, and confidence. For children who struggle socially, this developed relationship is especially important and therapeutic as the child finds someone they can love and trust.

The movement from horseback riding, the development of a relationship with the horse, and the confidence that comes from riding are the incredible benefits of participating in horse therapy.

Adaptive Cycling

As we have learned from earlier discussion regarding Paralympic athletes, cycling is a great option for people with disabilities to gain strength and confidence and compete at any level they desire. This is a sport that can be accomplished either by using upper body or lower body strength, making it very adaptable to the needs of any client, and is something that can be done indoors or outdoors.

The tricycle can be built to be elongated (as seen in the picture above), which uses lower body strength to pedal and has handlebars to steer. The other option is a tricycle built similarly to a wheelchair with an extra wheel just in front and requiring the rider to tuck their legs back. This kind of tricycle focuses on using upper-body strength to propel oneself and steer. Adaptive cycling is a great option for people with spina bifida because of its versatility, the many opportunities it provides for competition and growth, and the muscles that are required and developed to be successful in this sport.

Wheelchair Basketball

Wheelchair basketball is one of the most popular methods of adaptive recreation and has competitions on a local, national, and international level. In wheelchair basketball, the wheelchair is considered part of the player and general rules of contact apply. There are specifications and rules for the chairs to ensure that no player has an unfair advantage. Players cannot take more than two strokes while holding the ball and can wheel and dribble if they would like.

This sport is physically demanding and very competitive. It is a great option because it is easy to mix people who have disabilities with people who do not, which we call inclusion. Wheelchair basketball is great for helping people to build up muscle strength, gain confidence and boost self-esteem, allow them to create relationships with teammates and opposing teams, and provides a serious level of competition so that people with disabilities aren’t missing out on the intensity of sports like basketball and others.

Other popular and effective recreational activities:

· Water skiing

· Snow skiing

· Swimming

· Bowling

· Archery

· Fishing

· Rock climbing

· Scuba Diving

· Ice skating

· Any kind of sport: tennis, soccer, golf, football, etc.

· Wheelchair hockey