Spina Bifida and Jean Driscoll

Every hour, every day, somewhere in the US, a baby is born with a serious birth defect that is more common than muscular dystrophy, multiple sclerosis, polio, or cystic fibrosis combined... yet until recently most people had never heard of it (1). This disorder is Spina Bifida (SB), or Neural Tube Disorder (NTB), a neural tube defect caused by the failure of the fetus's spine to close properly during the first month of pregnancy (2). The neural tube is the embryonic structure that develops into the brain and spinal cord (3). Under normal circumstances, the neural tube forms in the developing baby early in the pregnancy and closes by the 26th day after conception (4). Then, later in the pregnancy, the top of this tube becomes the baby's brain and the remainder becomes the spinal cord (4).

However, in babies with Spina Bifida, a portion of the neural tube fails to develop or close properly, causing defects in the spinal cord and in the bones that make up the backbone (4). As of now, doctors are not sure as to what exactly causes the neural tube to not close properly. However, it appears to result from a combination of genetic and environmental risk factors, such as folic acid deficiency, a family history of neural tube defects, and medical conditions such as diabetes and obesity (4). Yet, ninety-five percent of babies with Spina bifida and other NTDs are born to parents with no family history of these disorders (5). Due to that fact, studies have shown that the best way to prevent Spina bifida is taking folic acid before pregnancy which can reduce the occurrence in babies by up to 75 percent (1). Therefore, the US Public Health Service recommends "that all women of childbearing age in the United States should consume 0.4 mg of folic acid daily to reduce the risk of having a pregnancy affected by a neural tube defect." (1).

Spina bifida, or open spine, results in the incomplete development of the brain, spinal cord, and/or their protective coverings (3). Additionally, although it can also occur in the middle or upper end of the spine, Spina bifida is most common in the lower spine (5). Spina bifida can be mild to severe depending on the type of defect, how big it is, where it is and if there are other problems (5). There are three forms of this neural tube defect that vary in severity: Spina bifida occulta, Meningocele, and Myelomeningocele.

First, Spina bifida occulta is the mildest form of the condition which is classified by a small separation or space in one or more of the vertebra of the spine (4). Also called closed neural tube defect, most children with this form of Spina bifida do not have symptoms and do not experience neurological problems (5). However, some signs to this type are an abnormal tuft of hair, a collection of fat, a small dimple or a birthmark on the newborn's skin above the spinal defect (4). Sometimes this is the only indication of the condition that can be visibly seen unless it is discovered during an x-ray for an unrelated reason.

The second form of Spina bifida is called Meningocele and is a rare form of this disorder. This type occurs when the protective membranes around the spinal cord, or meninges, are pushed out through the opening in the vertebrae (4). This sac-like formation includes cerebrospinal fluid but there is usually no nerve damage (6). Therefore, because the spinal cord develops normally, this sac, or membranes, can be removed by surgery with little to no damage to the nerve pathways (4). Although individuals with Meningocele may suffer only minor disabilities, new problems can develop later in life (6).

The third form., Myelomeningocele, or open Spina bifida, is the most severe form of the condition and is the form that people usually mean when they use the term "Spina bifida." In this form, the baby's spinal canal remains open along several vertebrae in the lower or middle back (4). Due to this opening, the meninges (the protective covering of the spinal cord) and spinal nerves come through the open part of the spine at birth, also forming a sac on the baby's back (6). In some cases skin covers the sac, but in most cases the tissues and nerves are exposed, making the baby more prone to life-threatening infections (4). This more severe type of Spina bifida usually results in nerve damage and partial or complete paralysis below the area of the sac (5).

Because of this neurological impairment, bowel and bladder problems, and seizures are common complications of this disorder (4). The first step in the treatment process for a child with a Myelomeningocele is surgery to close the defect in the back. This operation is not to restore any neurological problems, but is to prevent further trauma to the exposed spinal cord (5). In addition, if the child has associated hydrocephalus, an abnormal accumulation of cerebrospinal fluid (CSF) within cavities called ventricles inside the brain, treatment to eliminate this is also necessary (5 & 7). Hydrocephalus is common with this form, and occurs when there is an imbalance between the amount of CSF that is produced and the rate at which it is absorbed (7). As the CSF builds up, it causes the ventricles to enlarge and the pressure inside the head to increase (7). The most common method to treat hydrocephalus is to have a shunt surgically implanted into the child’s head to divert the flow of CSF to another region of the body where it can be absorbed (7).

Each case of Spina bifida is different from any other, and the degree of each form will dictate whether that child will be have to be in a wheelchair or whether they will ever be able to walk. In babies who have Myelomeningocele, irreparable nerve damage has already occurred and they will most likely have to be in a wheelchair due to paralysis. However, there is still a chance for walking depending on the degree of paralysis, and certainly more of a chance with the first two forms of Spina bifida. To increase the child's chances of walking, parents are taught by a physical therapist, as soon after surgery as possible, how to exercise their baby's legs and feet to prepare for walking with leg braces and crutches (5). Also, ongoing therapy, medical care, and/or surgical treatments may be necessary to prevent and manage complications throughout the individual's life (2).

People with disabilities, like anyone else, need to work, socialize, recreate, and receive adequate health care. With the enactment of the Americans with Disabilities Act in 1990, individuals with disabilities gained more control of their life with the legal right to participate in all aspects of life (8). This created a whole new world for people with disabilities, making it easier for them to live a more normal, independent life. This need and desire for recreation is a natural part of life and individuals with disabilities have the same need and desire for recreation as any other child or adult and can experience the same physical, mental, social, and emotional benefits (8). Ten years ago, an individual with Spina bifida did not expect to go snow skiing with his/ her peers; today this person can expect to join in the fun of recreation and friendships with more accessible facilities and the aid of adaptive sports equipment (8).

One big problem among those with Spina bifida is obesity. Very young children who have Spina bifida usually grow at about the same rate as their non-disabled peers and are quite physically active, so they usually do not become obese (6). However, beyond age six at least 50 percent of children who have Spina bifida are overweight, and in adolescence and adulthood over 50 percent are obese (6). One reason for this is that obesity further limits mobility, leading to an escalating problem of decreased energy use and weight gain, thus making it harder to keep up with peers in social, work, and daily living situations (6). In addition, social rejection, which may already be a problem due to lack of sensitivity and understanding from others, may become worse (6).

In addition to healthy eating to reduce chances of obesity later in life, another important strategy is to teach children who have Spina bifida to enjoy exercising and engage in recreation activities with others. Physical activity and recreation offers many benefits to those with Spina bifida; it burns calories, can decrease hunger by resetting the body's "thermostat," improves physical functioning and health, expands social skills and networks, and increases abilities of independent living skills (6 & 8). Physical activity also helps to minimize pressure sores and increase balance and endurance (8).

Most children with Spina bifida can walk for at least short distances, usually with the assistance of braces, canes or crutches, although they may require wheelchairs for longer distances (4). Using these devices is very beneficial in helping a child regain mobility that was lost as a result of the condition, or to gain more independence (4). Still, other children are unable to walk by any means, but can play sports adapted to individuals in wheelchairs to help them stay in healthy.

In addition, children with Spina bifida (with or without) hydrocephalus can have visual-motor problems (6). This means that the child may have trouble finding their way around, completing activities requiring eye-hand coordination, and are generally less coordinated (6). These weaknesses typically interfere with their daily activities but with practice and exercises focusing on these problem areas, they stand to greatly improve. One way to help children with these visual-motor problems is to help them learn basic relational ideas (up/down, in/out, left/right, and top/bottom) in early childhood (6). Another good tip is to spend time on games and activities that encourage eyes and hands working together such as threading objects on a string, building blocks, and throwing and catching balls according to age (6).

As talked about before, exercise and social recreation is very important for those with disabilities such as Spina bifida, plus most physical activities that other children enjoy can be adapted for those who have mobility impairments. Children with Spina bifida have varying degrees of paralysis of the lower extremities, which determines how much adaptation is necessary for the child to participate in the sport (5). Some examples of activities great for those with Spina bifida are horseback riding, tennis, swimming, and wheelchair sports (6).

Swimming is very beneficial to those with Spina bifida because it is a non-weight-bearing activity and they do not necessarily have to be able to walk to exercise in the water. Also, when engaging in activities in a pool, one's body weight is less than that on dry land allowing the individual to move more freely and gain more range of motion (11). Some examples of wheelchair activities that can be engaged in are basketball, archery, track, fencing, softball, and racing (8). Other recreational activities are fishing, table tennis, bowling, adapted kayaking, and beach activities all of which are life-long activates that children, peers and families can enjoy together (6). Many opportunities can be found in community organizations such as the YMCA or around the nation in unique camps for the disabled. Remember, even with the worse type of Spina bifida, Myelomeningocele, these individuals can move all parts of their bodies above the sac or spinal defect and need to adequately exercise their bodies.

One activity in particular, horseback riding, has been found to be particularly helpful to individuals with Spina bifida. Therapeutic riding has been used since the early 1950s in Europe as a tool for improving the lives of individuals with physical disabilities (10). In the physical realm, the benefits of modern therapeutic horseback riding include improved balance, circulation, respiration, body metabolism and muscle strength and flexibility (9). All of which are very important for individuals with Spina bifida in keeping a healthy body and their independence.

In addition, some psychosocial benefits of riding are improved self-image, self-esteem, and interpersonal skills (9). Again, all of which are important factors to those with this disorder who battle with society and their peers to be accepted and liked. It should be noted that riders must use protective helmets and allow trained assistants to aid them in performing the advised exercises during this activity (9). Because horseback riding gently and rhythmically moves the rider's body in a manner similar to a human gait, riders with physical disabilities often show improvement in flexibility, balance and muscle strength (10). It may seem less like therapy and more like play, but "equine-assisted therapy" really can help handicapped patients (9). In addition to being helpful to those with Spina bifida, therapeutic horseback riding also benefits those with cerebral palsy, Down syndrome, and muscular dystrophy, mental retardation, amputations, deafness, and many more (9).

When involving individuals with Spina bifida in activities, sports, or general exercise there are a few guidelines you should be aware of. First, many people with Spina bifida are allergic to latex so always check exercise equipment to make sure it is latex-free before any exercise commences (11). Second, they should be breathing normally and not holding their breath anytime during an activity (11). Try to carry on a conversation with them, making it almost impossible for them to hold their breath. Third, make sure the individual retains good posture by sitting up tall during relevant exercises and support their back, by a rolled up towel if needed (11). Last, make sure they adequately rest their muscles and do not have any delayed onset of muscle soreness (DOMS) (11). If the individual does have DOMS then the activity needs to be less strenuous and sometimes more spread out.

In general, individuals with Spina bifida report that their quality of life is high and contrary to popular belief, the level of spinal lesion, the need for assistive devices such as a wheelchair, or a limitation in learning are not significant predictors of quality of life for these individuals (8). Instead, 'quality of life' ratings were based on many different factors and influences of daily living and were associated with a variety of challenges, experiences, and expectations (8).

One individual in particular, who overcame her Spina bifida, is Jean Driscoll. She is an Olympian, Paralympian, author, and advocate for persons with disabilities around the world (12). Driscoll was born in 1966 in Milwaukee, Wisconsin with Spina bifida, an improper development of her spine, which left the nerves in her lower body unconnected and her in a wheelchair (13). However, despite her disorder, she went on to become a world-renown athlete with many great achievements. First, she went on to become the only athlete, in any division, to ever win the Boston Marathon eight times (12). In addition, during her career as an elite wheelchair racer, she won two silver medals in the 1992 and 1996 Summer Olympic Games, 12 Paralympic medals [Tables 1 & 2] and many prestigious awards including the Women's Sports Foundation's Sportswoman of the Year in 1991 (12).

Table 1: 1996 Paralympic Summer Games in Atlanta, GA

Table 2:

Another accomplishment was being voted #25 of the Top 100 Female Athletes of the 20th Century in 2000 by Sports Illustrated for Women (12). Driscoll is also a world record holder in the 10,000 meter wheelchair track event as well as the 10K and wheelchair marathon road racing distances (12). Driscoll is the co-founder of a non-profit organization called Determined to win and in 2002 she was inducted into the Wheelchair Sports, USA, Hall of Fame (12). She has become a motivational speaker and author, and is also very active as a global advocate and hands-on coach with Wheels for the World, a program that restores wheelchairs and gives them to individuals in third world countries (12). She travels annually to Ghana, West Africa to teach potential athletes and coaches about the sport of wheelchair track (12). Driscoll is not only living proof that a great life lies ahead of those who strive to achieve but that you can accomplish almost anything, even with Spina bifida.

"You've got to be willing to take risks. Dream big, set big goals, and then work hard to make your dreams happen." ~ Jean Driscoll

REFERENCES

1. Spina Bifida Association of North Carolina http://sbanc.home.mindspring.com/index.html
             Accessed June 9, 2006.

2. National Institute of Neurological Disorders and Stroke – Disorders – Spina Bifida
          ;http://www.ninds.nih.gov, Last updated January 25, 2006.

3. March of Dimes – Spina Bifida, http://www.marchofdimes.com/pnhec/4439_1224.asp
        Accessed June 10, 2006.

4. CNN.com Health Library – Spina Bifida
http://www.cnn.com/HEALTH/library/DS/00417.html
October 04, 2005

5. Mayo Clinic – Spina Bifida, http://www.mayoclinic.org/peds-spinabifida-rst/index.html, Accessed June 10, 2006.

6. Spina Bifida Association, http://www.sbaa.org/, Accessed June 8, 2006.

7. Hydrocephalus Association, http://www.hydroassoc.org/, © 2002; Accessed June 11, 2006.

8. Growing Up With Spina Bifida. (Online version of book).
NCODH North Carolina Office on Disability and Health. http://www.fpg.unc.edu/~ncodh/pdfs/SpinaBifida.pdf, Accessed June 10, 2006.

9. Bliss, Beth. "Therapeutic horseback riding?." RN Journal 60.n10 (Oct 1997): 69(2).
Expanded Academic ASAP. Thomson Gale. University of Florida.
10 June 2006
http://find.galegroup.com.lp.hscl.ufl.edu/itx/infomark.do?&contentSet=IAC-Documents&type=retrieve&tabID=T002&prodId=EAIM&docId=A20042770&source=gale&srcprod=EAIM&userGroupName=gain40375&version=1.0

10. North American Riding for the Handicapped Association (NARHA), http://www.narha.org/, June 6, 2006.

11. NCPAD - Exercise/Fitness: Exercises for Individuals with Spina Bifida, http://www.ncpad.org/, January 6, 2006.

12. Jean Driscoll: Olympian ~ Paralympian ~ Motivational Speaker ~Corporate Spokesperson ~ Author ~ Professional Athlete,           http://www.jeandriscoll.com/index.htm, Accessed June 12, 2006.

13. Daily Celebrations – Jean Driscoll, http://www.dailycelebrations.com/112601.htm, November 26, 2005.

Table 1: 1996 Paralympic Summer Games in Atlanta, GA - 10,000 Meters, Resource: International Paralympic Committee (IPC) – Paralympic Games Results http://www.paralympic.org/, Accessed June 13, 2006.

Table 2: Olympic & Paralympic Games Medal breakdown; Resource #12.

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