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Living with Spina BifidaA Guide for Families and Professionals$

Adrian Sandler, M.D.

Print publication date: 2004

Print ISBN-13: 9780807855478

Published to North Carolina Scholarship Online: July 2014

DOI: 10.5149/9780807867860_sandler

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The Adolescent and Young Adult

The Adolescent and Young Adult

Chapter:
(p.187) Chapter 9 The Adolescent and Young Adult
Source:
Living with Spina Bifida
Author(s):

Adrian Sandler

Publisher:
University of North Carolina Press
DOI:10.5149/9780807867860_sandler.14

Abstract and Keywords

This chapter argues that there are important medical issues in this age group, and that there also exist unique and critical developmental and psychological issues specific to adolescents and young adults. What services are available to meet these complex needs? Sadly, this age group has historically been neglected when it comes to provision of services. A recent survey from the National Center for Youth with Disabilities (NCYD), an information and resource center in Minneapolis, showed the continued existence of major service gaps for youth with disabilities such as spina bifida. A large proportion lack access to adequate primary health care. Huge numbers of disabled youngsters are uninsured or lack health care financing. Preventive and health promotion services are variable.

Keywords:   important medical issues, psychological issues, adolescents, young adults, NCYD, Youth with Disabilities, NCYD

A dolescents with chronic conditions are living longer. Almost a third of the individuals with spina bifida in our clinic are thirteen years of age or older. Not only are there important medical issues in this age group, but there also exist unique and critical developmental and psychological issues specific to adolescents and young adults. What services are available to meet these complex needs? Sadly, this age group has historically been neglected when it comes to provision of services. A recent survey from the National Center for Youth with Disabilities (NCYD), an information and resource center in Minneapolis (NCYD, Box 721, University of Minnesota Health Center, 420 Delaware Street, Minneapolis, Minnesota 55455; telephone number [800] 333–6293), showed the continued existence of major service gaps for youth with disabilities such as spina bifida. A large proportion lack access to adequate primary health care. Huge numbers of disabled youngsters are uninsured or lack health care financing. Preventive and health promotion services are variable. Too often there are no services to deal adequately with the complex psychosocial needs of disabled youth. The schools, which are seriously overloaded and underfunded, are left to try to provide needed services. The lack of adequate transportation is a major obstacle to service provision. From an organizational standpoint, the various agencies that are involved, including public health, vocational rehabilitation, social services, and the schools, are often poorly coordinated; as a result, individuals find it difficult or impossible to access available services. Whereas we have committed a lot of our resources to younger children with disabilities, we have left the older ones out in the cold. The tragic consequence is a generation of wasted potential, as we fail to provide the assistance needed for many youngsters to take the next steps toward independence, maturity, and adult fulfillment.

(p.188) Developmental Issues

Progressions and Expectations

The popular stereotype of adolescence as a sudden explosion of hormones, stress, and family conflict has been greatly exaggerated in recent times. Although hormone surges certainly do occur, adolescence is not all “storm and stress”! Parents, take heart! For most families, these years are challenging yet especially rewarding. This is not the place for advice about parenting a teenager: there are many excellent books on that subject. Just remember that the goal is to teach responsibility and independence. In the early adolescent years, this means clear and consistent limit setting and the establishment of rules and responsibilities. In the later adolescent years, the emphasis shifts to encouraging and supporting young adults in their fledgling efforts to spread their wings. And always, keep the lines of communication open.

Adolescence is really a stepwise maturational process. We are talking about the maturation and unfolding of an adult person, not an adult body. Puberty is a related but different process, one that is not always in phase with adolescent development. It is possible for a young person to be in midadolescence yet be lacking in pubertal development. In spina bifida, the reverse is frequently true: a thirteen-year-old girl may have a fully mature body, yet she is only beginning to move into early adolescence.

The tasks of adolescence are shown in Figure 9.1. First and foremost, adolescence is about the development of identity, the sense of unique individuality. This task requires some experimentation on the part of the young adolescent, who tries out new identities with different clothes, different ways of talking, different friends. The young person sees how these identities sit with others and modifies them, discards them, and thereby matures.

The process of identity development also requires a separate identity from one's parents. To know who I really am, I have to know who I am not, and that means “I'm not like you, Mom!” Separating from parents, the second task of adolescence, is thus a necessary step in the development of identity. This stage of development hearkens back to the toddler years. The struggle for independence is an ongoing quest about which the adolescent may be quite ambivalent, at times perhaps refusing to join the family on outings or not wanting parents around, and at other times feeling insecure and needing parents' support and help.

The third task of adolescence is the establishment of love and intimacy outside the family. In the next section I will discuss the development of (p.189)

The Adolescent and Young Adult

Figure 9.1. The main developmental tasks of adolescence

sexuality that underlies this task and the special challenges that it poses for adolescents with spina bifida. The fourth major task of adolescence is the development of a vocation, the desire and motivation to study, to earn a living, and to become a self-reliant and productive citizen. Of course, these four broad tasks are interrelated, and their accomplishment to a greater or lesser extent is what being an adult is all about.

Achievement of these developmental tasks can be facilitated by a number of personal attributes and environmental supports (Figure 9.2). In the same way, the presence of a physical disability can alter and interfere with the developmental trajectory of adolescence. It is important to appreciate that it is not so much the disability itself that does this but rather the way in which others respond to the disabled individual and limit his or her ability to make it in the world as an adult.

For example, an overprotected adolescent will perpetually receive reminders, supervision, and help from parents. There will be lower expectations for responsibility and self-reliance. For some parents who are stuck in this pattern of parenting, there may be some deep emotional investment in leaving things the way they are, in providing for their teenager's needs just as they did in earlier years. These are “codependent parents,” (p.190)

The Adolescent and Young Adult

Figure 9.2. Steps toward independence: an adolescent's progress can be helped (or hindered) by many important personal and environmental factors.

who unwittingly help overprotected children become immature and dependent adults.

Another constraint to adolescent development is the social isolation experienced by many adolescents with disabilities. With fewer occasions for socialization with peers, it is harder to make those bonds of identification with a peer group that allow a youngster to pull away from the parents. If such a teenager is not going out to parties and on dates, there will be fewer opportunities for kissing, falling in love, and the other familiar and fumbling steps toward intimacy with another young person.

As we have discussed, identity formation depends on the freedom to experiment with appearances, behaviors, and roles. To hang out with the other kids, an adolescent needs to have some freedom to ride the bus downtown, hang out at the beach, go on an overnight campout, or cruise around the neighborhood. Access to these places for a youngster in a wheelchair may be limited, leading to a lag behind peers in identity development.

The development of identity in adolescence is also inextricably linked with body image, and many youngsters with physical disabilities have some difficulty reconciling their own body images with the perfect, athletic physique that is glorified by the media. There are few, if any, disabled role models for many young adolescents, which hinders their efforts to develop a fuller sense of self.

Finally, finding a vocation and a means to economic independence can be frustrating and hard for a young person who is physically or mentally (p.191) challenged. Although there has been limited progress in recent years, prejudicial attitudes, limited opportunities, and inadequate supports and services continue to make life difficult for a disabled young adult. Fewer than 20 percent of employment-age people with disabilities are actually employed, and 60–80 percent of such young adults still live with their parents. This situation has to change. One powerful force driving this change is the Americans with Disabilities Act (ADA) of 1991. The potential impact of the ADA will be discussed in Chapter 11.

Sexuality

As young adolescents become increasingly preoccupied with themselves and the changes that are occurring in their bodies, their interest in sex comes to the fore. They may seem quite obsessed with sex and begin the process of dating and early sexual exploration. Although there is great variation in the ages at which different teens go through this, the stages of sexual relationships are pretty similar. Going out on a date, kissing, and petting are typical staging posts in the progression to sexual intimacy.

I have spoken with many adolescents with spina bifida, and it is absolutely clear that all of the above holds true for them. These youngsters are just as interested in sex and just as hungry for knowledge about sexual matters as their peers without spina bifida. Sex education and open communication at home about sex is especially important for these youngsters, because the social isolation to which many of them are subject serves to isolate them from another very important source of information—namely, peers. In a survey we conducted of sexual knowledge among adolescents with spina bifida, it was clear that these teenagers knew less about sex and contraception than other teenagers, and they were less likely to learn about sex from their friends (Sandler et al. 1994). Although the accuracy of talk among peers is certainly open to question, the peer group is a powerful teacher of prevailing attitudes and norms, and this is arguably just as important to a young adolescent in search of sexual identity. It is therefore especially important for educators and parents to teach young adolescents with spina bifida about sex.

Issues of sexuality are also of enormous importance in clinical encounters: it is essential that adolescents understand that it is OK to talk privately with their clinic doctors about their sexual concerns. Of course, sex is not just about bodily sexual functions. Therapeutic efforts need to focus broadly on attitudes, communication, and sexual behavior. I like to ask adolescents in clinic about whether they have been on dates, about crushes, and about steady boyfriends and girlfriends. The problems that (p.192) they describe are the usual ones that other teenagers have, and the approaches to counseling and anticipatory guidance are not significantly different.

In addition to discussing sexuality for the adolescent age group, we need also to consider the young adult with spina bifida who may be sexually active or about to become sexually active. There are a few myths and misconceptions that may need to be dispelled, but it is my experience that these myths are more often held by medical students and doctors than by the young adults themselves or their parents. For example, it is commonly held that people with spina bifida cannot be sexually active. Interviews with adults with spina bifida provide clear evidence that many, if not most, are indeed sexually active. There are many different sex acts and many ways to achieve sexual gratification and give sexual pleasure to others. The disability may interfere with some of these, but certainly not with all. Nor does it eliminate desire.

Another misconception is that people with spina bifida cannot have babies. Every spina bifida clinic in the country knows of several adult women who have successfully delivered healthy babies and men who have fathered them. It is important to stress the importance of contraception in the clinic setting and to make contraceptive services available when needed. Likewise, the importance of safe sex must be stressed to young adults who are sexually active or likely to become so. One important warning concerns condom use. Latex condoms have the potential to cause severe allergic symptoms in sensitive individuals, and thus the use of polyurethane, tachylon, or “natural” (sheep gut) condoms may be advisable (two of the natural condoms are supposed to be worn to prevent transmission of the virus that causes AIDS).

Until recently, the medical profession tended to steer clear of inquiries about sexuality. Many doctors feel uncomfortable about this area of medicine. Moreover, very limited information exists in the medical literature about sexual function in individuals with spina bifida, which makes it difficult to give sound advice or definite answers to our patients' questions. There has been more research in the area of spinal cord injury, and although some differences are likely, this research helps inform us about sexual function in spina bifida. In our own work with young men with spina bifida, we have found that the majority report that they are able to stimulate erections by masturbation and other techniques of touch (reflex erections). In our research, this was especially likely in those with low-lumbar or sacral lesions. By contrast, few have psychogenic erections (that is, erections from imagining or seeing something “sexy”) or spontaneous nocturnal erections. We (p.193) have actually used a reliable technique of recording penile tumescence and rigidity overnight in a small group of fifteen young men, finding that only two had “normal” nocturnal erections, while seven others had partial erections (Sandler et al. 1996). By inference, it would appear that the individuals with spontaneous erections have “incomplete lesions” of the spinal cord, whereas the majority have “complete lesions.” The medical and surgical management options for erection dysfunction have grown in recent years; thus it is possible to obtain erections using papaverine injections (PIPE, or papaverine-induced penile erections), external sheaths and rings, suction devices, or surgical implants (Sloan 1994).

Erections are mediated mainly by the pelvic parasympathetic nerves, whereas ejaculation is mainly a function of the sympathetic nerves. Ejaculation is uncommon in men with spina bifida, although some of the men we interviewed reported ejaculation with orgasm. For others, there may be retrograde ejaculation into the bladder. The use of electrical stimulation may be very effective in producing emission of semen for the purposes of conception. Both men and women with spina bifida in candid interviews have reported pleasurable and climactic sensations with sex, although these may be appreciated in areas of the body other than the genital region.

I believe that others with physical disabilities may be better able to advise young adults about how to prevent or cope with problems that arise with sex. To this end, we have begun to run same-sex groups about sex at a retreat for young adults with spina bifida, where we have an older and more experienced disabled adult as the group facilitator. It is often helpful to remember that sex for people without disabilities is frequently difficult, frustrating, or embarrassing and not the way it appears in the movies! We are all human and we all have something we're embarrassed about. A sense of humor, a simple explanation, an understanding partner, and somebody to ask for advice can all be very helpful.

Habilitation Issues

In this section, we will address issues such as mobility, access, and transportation. Also, the importance of developing independent living skills is stressed.

Mobility

A number of factors make it hard for many adolescents with spina bifida to continue walking as much as they did when they were younger. Increasing obesity may make walking exceedingly tiring. Spinal deformities and (p.194) contractures of the knees or hips may pose a severe mechanical disadvantage. For some, the occurrence of a fracture or the need for major surgery may cause a prolonged hospitalization, from which they never recover full ambulatory function. About 50 percent of those who are walking with braces at age twelve will have abandoned walking by the age of twenty. This is especially likely for those with lesions at L3 or above. Giving up on walking can be a painful loss for the young adolescent and her or his parents, who all may have invested so much emotionally over the years in the trials and tribulations of walking. In the end, however, one has to be pragmatic, and those who stop walking do so because of the energy expenditure advantages of the wheelchair.

Accessibility

I was talking with a young adult in clinic recently. She was tired of her secretarial job and was in the process of applying to graduate school at a major university. She needed financial aid and had arranged an appointment with the program officer, only to find that the office was on the second floor of an old building that had no wheelchair access and no elevator. How ironic that a program designed to break down barriers actually imposes new ones! Clinicians often take it for granted that patients have fully accessible homes, schools, and workplaces, which often is not the case. Inadequate ramps, temporary classrooms, and insufficient space to maneuver a wheelchair are common problems at school. Even at home, narrow entrances, tight corners, small bathrooms, and other barriers make mobility and transfers difficult or impossible.

Fortunately, the Americans with Disabilities Act is ushering in an era of increasing public accessibility. Lifts are readily available for vans, and are now required on public transportation in the United States. New bus and train stations must be accessible. Wheelchairs on buses need to be secured or fastened to the body of the vehicle, facing forward.

Wheelchair Maintenance

When it comes to choosing among the enormous array of available wheel-chairs, young people with spina bifida need to be knowledgeable and sophisticated consumers. Will the chair be used mainly indoors or outdoors? Will it be used during transport in a van or car? Must it fold or dismantle easily for traveling by car? Choices have to be made regarding the frame, the seating, the wheel types, and even the color! Youngsters put great time and effort into the choice of an individualized wheelchair; (p.195) moreover, a new chair is likely to cost between two thousand and twenty-eight hundred dollars!

Naturally, it is important to maintain the chair well to get good value from the investment. When we presented a wheelchair maintenance class at a recent young adult retreat, I was very impressed with the expertise of the group in this area. Your local wheelchair distributor and the manufacturer of your chair should provide you with guidelines regarding optimal maintenance.

Driving and Transportation

There is no more powerful and exciting symbol of independence to an American teenager than getting one's own car! For the adolescent with spina bifida, this may pose a few special challenges. The physical needs are really quite easily met with certain modifications, such as hand controls, raised tops, drop floors, and so on. The mental barriers, on the other hand, are more difficult. Poor depth perception, lack of judgment, and other cognitive limitations may truly make driving unsafe. Many parents are good judges as to their teenager's driving potential. If the prospect of driving strikes fear into the hearts of the teenager's parents, their feelings are often justified. It is important to explore this issue and examine the reasons for their fears. If uncertainties remain and expert opinion is needed, driver assessment centers can evaluate the prerequisite skills and provide very helpful information about driving instruction and adaptations. General Motors operates a Mobility Program that can both identify local assessment centers and equipment specialists and offer certain financial breaks for adaptations (call [800] 323–9935). The National Mobility Equipment Dealers Association (NMEDA; call [800] 833–0427) operates a hotline to provide information about the availability of dealers in van conversions, lifts, ramps, and other custom driving equipment.

Enhancing Self-Awareness and Self-Reliance

Studies have repeatedly shown that an individual's employability and independence are not determined by his or her IQ score or the level of spina bifida. Among the most powerful influences on these long-term outcomes is the individual's sense of self. Self-awareness is critically important for the development of self-confidence. When we know our strengths and weaknesses, we develop a realistic and positive outlook. We learn to use our assets to face challenges and gain new skills. Self-awareness leads to self-confidence, and self-confidence leads to self-reliance. Far too many (p.196) adolescents with spina bifida are lacking in critical skills of self-reliance. About one-third need help with washing, grooming, and hygiene. Most don't know how to plan meals, shop wisely for food, or cook safely. Housekeeping and laundry skills are usually inadequate. Few have the skills necessary to manage a bank account or pay the bills. Of course, in their defense, they have often had little exposure to many of these essential independent living skills because of their disabilities. But even in the area of accessing health care, seeking advice, reporting their problems to their doctors, and getting prescriptions filled, many lack the self-reliance that they will need to make it on their own. In one study, only one-third of those young adults with spina bifida and average intelligence could explain anything about what myelomeningocele is. In the clinic, I conduct my clinical interview with the adolescent alone, and I frequently ask these young people what they would say if a friend asks them to explain spina bifida. Few get beyond “It's a hole in the back”! It all starts with self-awareness.

Independent Living Skills Programs

In the United States, a complex maze of community-based services and programs supports individuals with disabilities. There are residential independent living skills programs, day programs, school-based vocational rehabilitation services, group homes, personal care attendants, and so forth. Unfortunately, these services are highly variable and are not organized “under one roof.” It can be exceedingly difficult for people to negotiate the maze, through all kinds of institutional roadblocks and barriers. Other countries have developed more integrated approaches to providing a necessary range of services. For example, Sweden has community-based “handicapped adult teams” that are responsible for ongoing service coordination in health care, habilitation, job training, housing, and welfare.

There are many exciting and innovative programs in this country that focus on the development of independent living skills for older adolescents and young adults. Information about these programs can be obtained from the SBAA. Among the programs that have come to my attention are the Gateway Program in Pittsburgh, the Transition to Adult Living Project at Yale (in New Haven, Connecticut), and the Shepherd's Program about Real Experiences (SPARX) in Atlanta. Successful programs of this type may focus primarily on teaching participants independent living skills while providing important group support as the young people take critical steps toward independence. For example, the adolescents may be enrolled in a kind of “camp” experience, through which they have (p.197) community outings, access community resources, and engage in problem-solving activities. They may be active learners in a curriculum involving health education, fitness, nutrition, and sexuality. There may be an emphasis on vocational planning and counseling and supervised opportunities to visit and participate in different workplaces. To assist the adolescent in making the transition from the parents' home to independent living, transitional living arrangements may be available, such as supervised, accessible apartments and group homes. Here, the adolescent may receive guidance in shopping, cooking, housekeeping, and financial management. Continuing group support is an important ingredient, as young adults face new, threatening situations. Successful programs also recognize that parents need help and support in managing this transition.

Medical Issues

Transition to Adult Health Care Services

Around 90 percent of babies with spina bifida survive into adulthood, which presents a special challenge to health care providers oriented toward pediatrics. Approximately 25 percent of our multidisciplinary clinic population is over sixteen years of age, and other clinics around the country are in a similar situation. It just doesn't make sense for me, a pediatrician, to be examining a twenty-eight-year-old married woman in a pediatric clinic with pink elephants and balloons painted on the walls!

Of course, many of the medical issues of childhood continue to apply in this age group. More important, however, many unique issues exist, and these need to be adequately addressed through adult-oriented health care services. The majority of young adults attending multidisciplinary clinics in this country look to the clinic to provide primary care. If the clinic intends to assume a primary health care role, this must include preventive services such as tetanus boosters and other immunizations. Family planning services and Pap smears should also be readily available. Shunt problems and spinal cord-related symptoms may account for deteriorating health in the young adult, and such conditions may be preventable if spotted early in their course. There are also important health education, vocational, and psychological issues that are specific to this age group.

It is clear that we have the responsibility to plan for an appropriate transition to adult-oriented health care services for tomorrow's adults with spina bifida. Many child-oriented clinics try to group their patients by age, such that on a given day, the clinic team is seeing older individuals. This is a good start and affords the opportunity to use appropriate models (p.198)

The Adolescent and Young Adult

Figure 9.3. The comprehensive health care team for young adults, with key aspects of health care to be addressed in a comprehensive annual review

of care and to provide appropriate health education. The health care team may be broadened to include, for example, an internist, gynecologist, and vocational counselor who are available for consultations. Some clinics have been developed specifically for young adults, providing comprehensive care with indepth annual review (Figure 9.3). The goal is to assist the young adult in his or her efforts to be healthy, independent, employed, (p.199) and satisfied and to prevent secondary disabling conditions. These goals are achievable to some extent for all young adults!

Weight Control and Aerobic Exercise

Obesity is a common problem and one that poses a major threat to health. Adults with spina bifida are usually short (the average male is five feet tall and the average female is four feet, six inches) and therefore tend to show excess weight more obviously than most other people. Moreover, the harmful effects of carrying around too much weight are compounded by spinal deformities and small chests that often accompany thoracic spina bifida. In adolescents with limited respiratory reserve, it doesn't take much to tip them into severe respiratory disease. Being substantially overweight and out of condition greatly increases the risk of these life-threatening problems. I recently saw a young teenager who had gained far too much weight during the past few years. He has thoracic spina bifida and a very small chest, and his lung capacity is therefore restricted. He developed bronchitis and pneumonia and had acute respiratory failure, which required intensive care in hospital and dependence on oxygen at home following this hospitalization. After beginning a program of supervised weight control and aerobic exercise, he rapidly lost weight. As his cardiovascular fitness improved, he no longer needed the oxygen. In addition, I am sure that he will be far more likely to withstand the next respiratory infection that comes his way.

Obesity is associated with other health problems as well. Some of these, like hypertension, can be dangerous, whereas others, such as skin infections (intertrigo), are chronic annoyances. Obesity also has the potential to interfere with self-care, personal hygiene, mobility, and participation in some recreational activities.

Adolescents with spina bifida often have low self-concept about their physical appearance. Obesity adds to this burden, and they certainly don't need another reason to feel ashamed. For this reason alone I stress the importance of diet and exercise. It is important for families to understand that caloric needs of youngsters with spina bifida are usually lower than those of people of similar age but without spina bifida. This knowledge alone is not likely to be enough, however. Efforts to lose weight are frequently unsuccessful because the same high-calorie foods are coming home from the grocery store, and there is not sufficient support and reinforcement for losing weight. A weight loss program is greatly enhanced by the use of “the buddy system,” in which a peer or a family member is (p.200) also trying to lose weight. The two offer each other encouragement, advice, and mutual positive feedback as they shed the excess pounds!

Opportunities for sustained aerobic exercise are not always easy to find for young adults with disabilities. Wheeling on exercise tracks and the use of other adaptive exercise equipment may be helpful. Videotapes for wheelchair aerobics are commercially available, such as Richard Simmons's Reach for Fitness (Reach Foundation).

Prevention of Osteoporosis

Inactivity leads to osteoporosis, or brittle bones. Bones are a living organ, and there occurs a constant process of bone formation and remodeling. Calcium and minerals are laid down in an organized matrix that makes bones strong. This process is affected by the food we eat (calcium and vitamin D), by our hormones (estrogen), by activity and weight bearing, and by genetic factors. If we look at bone density studies in individuals with spina bifida, the bones are clearly deficient in calcium and minerals. Bones that are lacking in such minerals are prone to fractures. Sometimes we see severe leg fractures in patients who have no recollection of falling or sustaining any trauma. For adolescents and young adults with spina bifida, osteoporosis is especially likely to occur if they are immobilized for long periods because of surgery or if they decide to give up walking.

Fortunately, there are a few things that can be done to help prevent osteoporosis (Figure 9.4). Eating a diet with plenty of vitamin D and calcium is important (skim milk, low-fat yogurt, and calcium-enriched orange juice are good sources). Exposure to sunlight helps provide us with the active form of vitamin D. Therapeutic standing in braces for at least fifteen minutes each day is probably helpful for those who are using wheel-chairs exclusively for mobility. For some women, there may also be benefits in taking hormonal supplements in the form of the oral contraception pill. Much research is currently being conducted on osteoporosis prevention. Hopefully, in the years to come, we will know more precisely how to prevent this problem in susceptible people.

Shunt Problems and Chiari Symptoms

Shunt failure is not rare in adolescents and adults. The valve systems may deteriorate over time, failing to regulate CSF pressures consistently. More common, the growth that occurs with puberty may in effect pull the shunt tubing up and out of the abdominal cavity, so that the shunt does not drain normally or becomes obstructed. When shunt problems occur, they may take many forms and become apparent in different ways. The (p.201)

The Adolescent and Young Adult

Figure 9.4. Inactivity leads to brittle bones that fracture easily. Also shown are some factors that are important in preventing osteoporosis.

onset of hydrocephalus may be acute, with severe headache, vomiting, lethargy, and change of consciousness, but it may be far more insidious, characterized by difficulty concentrating, intermittent headaches, sleepiness, or mood changes.

If these subtle changes have occurred, it is important for doctors to investigate the shunt function. A shunt series is a series of plain X-rays which show the shunt and its tubing over its entire length and which enable the doctors to check that the shunt is intact, in the right position, and sufficiently long. The shunt series may show disconnection of the shunt system, or it may show that a young person has “outgrown” the shunt—that is, the shunt tubing is too short. An unenhanced CT scan is the usual technique to establish whether there has been an increase in hydrocephalus. If the ventricles are obviously larger than they were previously, this would be consistent with shunt failure and would require shunt revision. If there are no previous studies with which to compare the CT or if some uncertainty exists about whether the shunt is functioning as it should, the neurosurgeon may elect to tap the shunt. This involves inserting a needle under sterile conditions into the shunt system to examine pressure and flow of CSF. If these investigations suggest shunt malfunction, the neurosurgeon may recommend shunt revision.

One of the problems that commonly arises is the asymptomatic shunt disconnection. A substantial proportion of adolescents with shunts are found on routine examination or shunt series to have a disconnected (p.202) shunt—that is, a gap or disconnection exists in the shunt tubing (most often in the scalp or neck). The adolescent is unaware of this and exhibits no symptoms to suggest a problem. A CT scan shows no change in ventricle size. We usually assume that the shunt is still draining across the disconnected space or that the youngster is no longer shunt dependent. Some neurosurgeons decide to leave well enough alone and to watch for any signs of deterioration or increasing hydrocephalus. Others feel that there is a substantial risk of sudden, increasing hydrocephalus and that the shunt has to be revised.

Chiari malformation may occasionally cause symptoms in this age group, and the doctor has to be watchful for increasing cranial nerve dysfunction (for example, swallowing difficulties, gagging), dizziness, unsteady gait, neck pain, or weakness in the arms. Chiari symptoms such as these usually indicate pressure on the brainstem associated with shunt malfunction, and they should be investigated as just discussed. It is usually the case that these symptoms resolve themselves after successful shunt revision or posterior cervical decompression.

Spinal Cord Problems

CSF passes from the ventricles into the narrow central canal of the spinal cord. With raised intracranial pressure, the central canal can become dilated, in effect developing a fluid-filled cyst in the spinal cord. This swelling is called hydromyelia (water in the spine) and is fairly common in young adults with spina bifida (Figure 9.5).

Usually, hydromyelia is an incidental finding and does not cause symptoms (McEnery et al. 1992). But hydromyelia may be found in association with rapidly progressive scoliosis, weakness, and increasing tightness in the legs. The neurosurgeon must determine whether the shunt is working normally, for hydromyelia may be caused by inadequately treated hydrocephalus. If hydromyelia symptoms persist despite a functioning shunt, the surgeon may need to place a special shunt to drain fluid from the spinal cord.

Bladder or Kidney Stones

One of the reasons for continuing to monitor the urinary tract with periodic urine checks and ultrasounds is the detection of stones. Stones can form in the bladder or in the kidneys and can develop in the setting of chronic urinary tract infection. They are especially likely to occur in people with augmented bladders. Stones in people with spina bifida are usually painless. One of the tip-offs that there may be a stone is the presence (p.203)

The Adolescent and Young Adult

Figure 9.5. Cysts of fluid in the spinal cord (hydromyelia) are fairly common in young adults and can cause rapidly progressing scoliosis and neurologic changes in the legs.

of blood in the urine. Another is the recurrence of bacteriuria after treatment in a person who does not typically have urinary tract infections. Stones are usually evident on ultrasound examination. Once a stone is diagnosed, it is important to determine if it is doing any harm, such as causing troublesome infections or obstructing the kidneys. The urologist may recommend lithotripsy, which is the use of underwater shock waves to shatter the stones and make them easier to pass.

Hypertension and Renal Insufficiency

Most adults with spina bifida can look forward to a long life with functioning kidneys. Like most organs of the body, the kidney has much functional reserve; it is therefore possible to get by quite adequately on only a small percentage of normal kidney tissue. In other words, although many children have significant reflux and hydronephrosis, they generally have more than enough good remaining kidney and do not develop signs of renal insufficiency. Unfortunately, some young adults who have repeated, severe kidney insults may not have much functional reserve. Thus it is important to monitor renal function to pick up early signs of renal insufficiency. Among the signs to watch is elevated blood pressure, or hypertension. Although there are many causes of hypertension, the presence of hypertension in a person with spina bifida raises a suspicion that kidney function is deteriorating. It is also helpful to check serum creatinine periodically. (p.204) The first line of therapy for people with hypertension is to lose weight, to exercise, and to restrict salt intake. If these measures are not sufficient, your internist or family physician may recommend antihypertensive medication.

Skin Injury and Pressure Sores

The most underrated organ in our bodies is the skin! Young people with spina bifida have to be constantly vigilant for skin injury. Even as I write, one young man I know is in hospital following a complicated operation to close an enormous pressure sore. Previously, he had developed a sore so large and so deep that it actually eroded into his rectum and necessitated placement of a colostomy. A young woman who had very recently moved away from home into her own apartment developed a pressure sore while standing at a new job and felt compelled temporarily to shelve her independent living plans as a result.

The most common cause of skin injury is pressure from prolonged sitting in one position. This kind of pressure sore usually begins as an area of redness just below the buttock, where the “sit bone,” or ischial tuberosity, can be easily felt. If the injury affects the tissues under the skin, there may be a “knot” of hard, swollen, or inflamed tissue under the skin. If the injury progresses, a blister may appear on the skin surface as a result of the damaged or dead skin, along with a discharge of blood and pus, which may be evident on clothing. At this stage, the pressure sore may appear as a clean, punched-out hole that can be superficial or deep, depending on the extent of the damage to underlying skin. It is not uncommon for apparently small areas of redness to reveal much more extensive damage to the underlying tissues.

Emotional Issues

Compliance: Whose Problem Is It Anyway?

Anyone who has ever been told to take some new medicine three or four times a day knows that it is not always easy for a responsible adult to do the right thing! A teenager who doesn't exactly share those adult attributes of responsibility and planfulness is likely to find it harder still. One of the problems that I frequently encounter in the clinic involves teenagers being “noncompliant” with their medical treatments. Usually they have stopped taking their Ditropan or other medications, and they have stopped cathing. Parents express their deep frustrations to me, as their efforts to bribe, beg, coerce, and cajole their youngsters back onto the (p.205) straight and narrow get nowhere! “He just doesn't seem to care anymore!” they tell me.

Most often, this noncompliance is a result of an adolescent identity crisis, with a touch of adolescent rebellion. Young teenagers with disabilities are having a hard time confronting the fact of their disability, and some may try to deny its existence altogether. Having to take pills at school and having to take scheduled bathroom breaks are irritating reminders of their condition. What better way of denying it than giving up on the treatments? Another powerful factor that comes into play concerns the “ownership” of the disability. As younger children, these kids are used to having their parents give them their medicines, administer their treatments, and perform their catheterizations. It almost feels as if the spina bifida belongs to their parents and the children are just lending their bodies! Then along comes adolescence, a time for asserting one's own identity and separating from one's parents, a time to refuse to do those things that parents bug their teenagers about—cleaning one's room, making the bed, and following the bowel program and doing caths!

The most serious medical consequences of such noncompliance are usually incontinence and constipation, although there can be more significant problems in those youngsters with high-risk urinary tracts. It is important for teenagers to hear and understand that the spina bifida is their problem, not the parents' problem, and that ultimately they themselves must take responsibility. Organizational cues, incentives, and negative consequences can all be employed systematically. Sometimes it is necessary to refer a noncompliant youngster for counseling regarding the underlying troubling questions of identity and self-concept.

Adjustment Problems: Confronting Disability

Adolescence is a difficult period of transition, and many young people have trouble adjusting to the changing demands and circumstances of these years. Additional stresses associated with having a disability—or other family and environmental stresses—may lead to temporary changes in mood and behavior. Some teens may become unusually irritable, angry, or defiant. There may be reports of acting out at school or withdrawal from social activities. Problems of this sort are very common and often pass spontaneously or with love and support. Keeping lines of communication open during early adolescence is essential. As a general rule, if problems persist and you, as parents, are worried about them, it is a good idea to consult your doctor, a psychologist, the school guidance counselor, or a mental health professional.

(p.206) Learned Helplessness

Children who have had things done for them, rather than doing for themselves, are likely to be substantially delayed in the quest for independence that characterizes adolescence. I see many young teenagers with spina bifida who request assistance for everything, including self-help skills, schoolwork, and commonsense, daily problem solving. It is very frustrating for their parents and teachers, who know that they have the skills but are just not in the habit of using these skills independently. Such children seem to lack some problem-solving strategies that come naturally to others. This “learned helplessness” and its accompanying “fear of failure” can prove to be significant barriers to greater independence. One of the most effective interventions that I know is camp or some other similar experience in which the young person leaves home and her or his watchful, attentive parents for a few days and has fun with others of a similar age. An opportunity to see what others do for themselves or a brief heart-to-heart with a camp counselor can turn the young person on to the fun of trying something new by oneself.

Depression

Around 5 to 10 percent of all adolescents get significantly depressed at some time during these years. We are not talking here about feeling sad once in a while but about having a persistent mood change, often accompanied by other symptoms of depression, such as sleep disturbance, loss of concentration, and withdrawal from people and activities. The percentage is higher among young people with disabilities. Depression has a large familial incidence, so that the risk is likely to be much higher if there is a strong family history of depression. It is important for parents, school personnel, and clinicians to be mindful of depression as they evaluate young people with spina bifida. The treatment of depression has come a long way in recent years, both in terms of the availability of therapists experienced in short-term supportive psychotherapy and in terms of the newer medications that are proving to be relatively effective and safe.

Parent-to-Parent

“One of the most difficult aspects of having a child with spina bifida was that I never had heard of it and there were no support groups. Of course, this was nearly twenty years ago. Benjamin's prognosis was not good. The doctors said he would never live, and if he did, he would be a blob in a bed.

(p.207) Benjamin is now almost twenty years old and will be entering his second year of college in August. He has achieved more than most “normal” kids. With God's help, he will continue to be the best he can be each day.

As a family we never dwelled on what Benjamin couldn't do but on what he could do. He is a child any parent would be proud to have. He has never used his disability as a cop-out but instead to achieve his goals. What a kid!”

“Mike and I have the normal brother-sister relationship. We pick on each other, encourage one another, fight with each other, and love each other. He is a very special person, and I am proud to be his sister. He has achieved much in his life, and I rarely think of him as a ‘handicapped’ person. Never would I park in a handicapped parking space. Mike can walk like everyone else, and he could use the exercise! My family has never let Mike use spina bifida as a crutch to lean on. He can think for himself and take care of himself. I look forward to the achievements that are yet to come in Mike's life.

Basically, Mike is as ‘normal’ as the next guy. He is physically challenged, and I must say he deals with the challenge very well. We are all disabled in one way or another … in Mike's case you just happen to be able to see his disability.”

“Our home is like most homes with a thirteen-year-old teenager, with me constantly saying, ‘Turn that music down!’ and ‘Have you finished your homework?’ and ‘Go in your room and pick up your clothes!’ I smile at myself when I say these things because it was not so long ago that I thought I would never have your typical, everyday things go on in my home. Little did I know how much love and happiness Jonathan could bring into our lives. He continues to teach us the true meaning of love and how to enjoy life to its fullest.” (p.208)