Education

What is Spina Bifida?

Spina bifida is an abnormality of the nervous system that occurs in a baby’s development during the first month pregnancy. Most textbooks define spina bifida as a neural tube defect or a gap in the fusing of the neural tube that encloses the spinal cord. While a neural tube defect indicates a diagnosis of spina bifida, it is only part of what is a total nervous system disorder. The number of children born with spina bifida is approximately 1 in every 1500 births.There are three main types of Spina Bifida. The most common are:

  • Myelomeningocele
    A sac containing an abnormally formed spinal cord protrudes from an opening in a baby’s back. The nerves at and below the defect are damaged. The higher the defect occurs on the spine, the greater the number of nerves that are affected. Babies need surgery shortly after birth to repair the affected area of the back.
  • Meningocele
    A baby is born with a sac protruding from an opening in the back. Unlike with Myelomeningocele, the sac doesn’t contain the spinal cord. Most often, few nerves are affected. Babies need surgery shortly after birth to repair the affected area of the back.
  • Spina Bifida Occulta
    In Spina Bifida Occulta, the bones around a baby’s spinal cord fail to develop normally. The nerves of the spinal cord usually are normal, as is the skin on the back. Sometimes, however, there will be a dimple, hair patch or red discoloration on the skin at the point of the defect. Babies rarely need surgery shortly after birth to repair the defect.

 

Life Expectancy
Historically, a child born with a more serious form of spina bifida or more severe neural tube defect was expected to live a greatly abbreviated life. Today, however, increased education about spina bifida, emphasis on the importance of personal care, greater understanding of the accompanying health issues associated with the condition, as well as surgical advances have resulted in a much higher quality of life and greater life expectancy for persons with spina bifida. As many as 90 percent of persons born with spina bifida will survive into and possibly past their third decade of life. The current adult population living with spina bifida is reaching unprecedented levels of achievement.

 

Folic Acid and Spina Bifida
Folic acid, a B vitamin, has been found to play an important role in reducing the incidence of spina bifida. The U.S. Department of Health and Human Services’ Center for Disease Control recommends that all women of child bearing age consume 400 mcg of folic acid per day. It is important to note, however, that taking a folic acid supplement, or consuming the equivalent of 400 mcg of folic acid in the diet, is not a guarantee against a child being born with spina bifida. Folic acid cannot “cure” spina bifida; rather, it can help reduce the chances of a child developing the condition in utero.

 

Why me/my child?
Most parents, when learning of a diagnosis of spina bifida, wonder if they did something to cause their child to have this condition. Nothing a parent does or does not do results in a child being born with spina bifida. There is no known cause of spina bifida.

 

What happens now?
When a baby is diagnosed with spina bifida, the course of action a doctor may recommend depends entirely on the type of spina bifida and the location of the defect in relation to the baby’s spine.

In children born with Myelomeningocele, surgery is performed shortly after birth to repair the abnormality and prevent further damage to the spinal cord and nerves. During surgery, the opening in the baby’s back is sewn closed. The surgery does not bring back function, but serves to prevent further injury and infection. Because this opening is now closed, there is no way for cerebral spinal fluid (CSF) to drain and a condition called Hydrocephalus can occur. Hydrocephalus, commonly referred to as “water on the brain,” is an abnormal accumulation of CSF within cavities inside the brain. It occurs when there is an imbalance between the amount of CSF that is produced and the rate at which it is absorbed. As the CSF builds up, it causes the cavities, also called ventricles, to enlarge and the pressure inside the head to increase. Surgery is performed to put a shunt in place that will allow this fluid to drain and maintain an appropriate amount of intracranial pressure. A shunt is a flexible tube placed into the ventricular system that diverts the flow of CSF into another region of the body where it can be absorbed.

Babies born with Meningocele also require surgery to close the opening in the back. Since the sac does not contain the spinal cord, the rest of the central nervous system is generally not involved and prognosis for development is excellent.

Spina Bifida Occulta occurs in as many as 20-30% of the population and often is not diagnosed until adulthood. Oftentimes patients are seen for back pain, enuresis and scoliosis – to discover through x-rays of the spine that they have spina bifida occulta. This type of spina bifida results in the least frequency of impairment.

In recent years, surgeons have begun to perform prenatal, intrauterine surgical repairs on the fetus to correct an abnormality. Intrauterine surgical repair reduces the baby’s risk of contracting infection because the sac containing spinal cord tissue is no longer exposed to bacteria upon birth. This type of surgery is not without risks to both mother and baby, however, and the benefits must be carefully weighed against the risks.

While surgery will close the opening in the baby’s back and prevent infection, the damage caused as a result of the nerves being exposed during the baby’s development cannot be repaired.

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Living with Spina Bifida

Individuals with spina bifida can have vastly varying degrees of impairment, from virtually no impairment to severe sensory, motor, autonomic and cognitive impairment. Myelomeningocele is the most severe form of spina bifida where many systems can be impacted in direct relation to how severe the abnormality is and where it occurs. Generally speaking, the higher the abnormality is on the spinal cord, the more systems that are involved. Some common problems/conditions that individuals born with spina bifida experience include:

  • Allergic reactions caused by prolonged exposure to latex
  • Bladder, bowel and kidney problems
  • Eye problems
  • Fractures
  • Hydrocephalus (excess fluid on the brain)
  • Learning disorders
  • Seizures
  • Skin problems
  • Tethered spinal cord
  • Social skills deficits
  • Weight gain/obesity
  • Depression and other psychological / psychiatric issues

It is likely that a child born with spina bifida will require numerous surgeries over the course of his/her life. Frequent hospitalizations require a great commitment of time, finances and energy on the part of the family and/or caregiver and can have quite an emotional impact on the family. There are local and national support groups available to parents and caregivers and the Spina Bifida Association of Western PA can offer suggestions for additional support.

Just as surgeries and hospitalizations impact families, they also take a physical and emotional toll on the individual with spina bifida. A loving and supportive family is only one critical factor in the emotional development and well being of a child born with spina bifida. Interaction and socialization with peers apart from a family setting is equally vital to his/her development. Camps and retreats specifically created for individuals with spina bifida, such as those offered year-round by the Spina Bifida Association of Western PA, provide an encouraging, supportive environment where children can make friends and learn how to care for themselves. Sometimes attending a camp or retreat is the first exposure a child has to others with the same challenges as him/her.

Common Physical Characteristics
Because of surgery performed to repair the spinal cord, individuals with spina bifida are often short in stature. Limited mobility and lower metabolic rate due to the condition often result in a greater likelihood of obesity.

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What can I do?

Understanding and Acceptance
Understanding and acceptance are key to living with spina bifida. All parents dream of having a “perfect” child, and a diagnosis of spina bifida can be daunting, causing fear and uncertainty. The fear of the unknown sometimes causes greater anxiety than the reality of a situation. Competent and caring medical professionals can help ease fears, as can reading through the information provided in this web site. Treatment methods may vary, depending on the practice philosophies of different medical professionals, but there is a core of best practices that can be followed to ensure that a child with spina bifida has the best possible chance to thrive in life.

Experienced Medical Care
It may seem obvious, but it is important to seek qualified, experienced medical professionals. Not all physicians and care practitioners are experienced in treating patients with spina bifida. Ask your physician for a referral to a specialist. Contact a local spina bifida clinic. If your health plan has a patient care hotline, call and ask for referral information.

Promote Independence/Interdependence
Encourage independence and also interdependence. Promoting independence is a vital aspect of any parent’s or caregivers’ role. Independence is necessary to achieve self confidence, but not at the expense of one’s well-being. It is important for a child to grow up to be as independent as possible; yet, it is equally as important that caregivers encourage the child to ask for and be willing to receive help when it is needed. We all rely on various supports and other people to be successful in life. Reinforce the fact that no one is completely independent and it is OK to need and accept assistance from others.

Emphasize Personal Care
The importance of personal care increases as a child grows older and parents are less inclined to closely monitor their child’s personal hygiene routine. Well-established, independent grooming habits that have been taught and reinforced from a very young age will help a child achieve and maintain better health as he/she matures into adulthood.

SBAWP Programs and Services
The Spina Bifida Association of Western PA has many resources for individuals with spina bifida as well as their families and caregivers. We’re here to help. Our programs and services provide a continuum of service, education and advocacy for all stages of life.

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The Stages of Life

Individuals with spina bifida can lead very active lives, work, socialize, get married, have children and find fulfillment in a broad range of areas. While each individual is different, children with spina bifida can be expected to go through all of the same developmental stages that children without spina bifida do. However, they may experience additional challenges directly related to the type of spina bifida that they have and the severity of their impairment.

Spina bifida is not a progressive condition and does not worsen as an individual ages. However, the health issues faced by a person with spina bifida tend to vary with age. What is common in a young child may not be prevalent in an adult with spina bifida and the impact of these health issues is not the same throughout the lifespan.

The stages of development for a child with spina bifida can be broken down into age groups beginning with birth and continuing into adulthood. Just as all children learn to walk and talk, go through puberty and experience a growing need for independence at different ages, so do children with spina bifida. Thus, some physical and emotional developmental areas overlap and blend and there is no measuring stick for developmental success.

 

Infants and Toddlers (Birth to Age 3)

Potential Topics:

  • Expectations of Developmental Progression
    • Developmental milestones that parents can anticipate
    • Potential developmental delays
  • (The Value of) Early Intervention Services
    • How & Where to get these services
  • Habilitation Issues
    • Mobility
    • Toileting
    • Nutrition
  • Medical Issues
    • Unstable Hip
    • Shunt Function & Malfunction
    • Arnold-Chiari Malformation – a congenital anomaly of the brain that occurs in almost all children born with both spina bifida and hydrocephalus, In infants, the most common symptoms are stridor and swallowing difficulties. In older children, upper limb weakness and breathing difficulties may occur. Patients may experience no symptoms or remain asymptomatic until early adulthood, at which point they will often experience severe headaches and neck pain. Fatigue, dizziness, vertigo, neuropathic pain, visual disturbances, difficulty swallowing, ringing in the ears, impaired fine motor skills, muscle weakness, and palpitations are other common symptoms. Some patients may go an entire lifetime without having noticeable symptoms. Or, symptoms can be minimal, then turn severe suddenly due to head trauma which alters the condition of the spine, brain, or cerebellar tonsils and begins to cause more difficulties. Once these “onset of symptoms” occurs, the most frequent treatment is decompression surgery, in which a neurosurgeon seeks to open the base of the skull and through various methods unrestrict CSF flow to the spine.
    • Failure to Thrive
    • Strabismus – also known as “heterotropia”, “squint”, “crossed eye”, “cockeyed’, “wandering eye”, or “wall eyed”, is a condition in which the eyes are not properly aligned with each other.

 

Preschool Years (Age 3 to 5/6)

Potential Topics:

  • Expectations of Developmental Progression
  • A Developing Sense of Self
  • Introduction to the Social World
  • The Emergency of Sexual Identity
  • Habilitation Issues (dressing skills & other ADLS )
  • Wheelchairs & Transfers
  • Promoting Ambulation
  • Fine-Motor Function
  • Expectations of Developmental Progression
  • Visual Perceptual Function
  • Medical Issues:
    • Knees & Ankles
    • Foot Problems

 

Middle Childhood (Age 6-12)

Potential Topics:

  • Expectations of Developmental Progression
  • Learning Abilities & Disabilities
  • Common Learning Characteristics
  • Attention Deficit Disorder (ADD)
  • Habilitation Issues
    • Gait Analyses
      • PT
      • Adaptive PE
      • CIC
    • Clean Intermittent Catheterization
    • Pharmacological Management of Incontinence (Managing incontinence with the aid of prescription drugs)
    • Management of the Neurogenic Bowel
  • Medical Issues
    • Growth & Growth Problems
    • Precocious Puberty – Puberty is the period in life when the body changes rapidly and develops reproductive capability. Precocious puberty is the development of body characteristics that normally occur during puberty before the age of 8 (girls) or 9 (boys). Children (of both sexes) with early sexual development are more likely to have psycho-social problems. Children and adolescents generally want to be the same as their peers, and early sexual development can make them appear “different”. This can result in self-esteem problems, depression, acting out at school and home, and alcohol and illegal substance abuse. (National Library of Medicine)
    • Nutrition
      • Obesity Prevention
    • Urinary Tract Infections
    • Tethered Cord – Tethered spinal cord syndrome is a neurological disorder caused by tissue attachments that limit the movement of the spinal cord within the spinal column. These attachments cause an abnormal stretching of the spinal cord. The course of the disorder is progressive. In children, symptoms may include lesions, hairy patches, dimples, or fatty tumors on the lower back; foot and spinal deformities; weakness in the legs; low back pain; scoliosis; and incontinence. Tethered spinal cord syndrome may go undiagnosed until adulthood, when sensory and motor problems and loss of bowel and bladder control emerge. This delayed presentation of symptoms is related to the degree of strain placed on the spinal cord over time. Tethered spinal cord syndrome appears to be the result of improper growth of the neural tube during fetal development, and is closely linked to spina bifida. Tethering may also develop after spinal cord injury and scar tissue can block the flow of fluids around the spinal cord. Fluid pressure may cause cysts to form in the spinal cord, a condition called syringomyelia. This can lead to additional loss of movement, feeling or the onset of pain or autonomic symptoms. (National Library of Medicine)
    • Detethering & Retethering
  • Scoliosis Management
  • Seizures
  • Emotional Issues / Self-Esteem

 

Adolescence (Age 13 – High School)

Potential Topics:

  • Expectations of Developmental Progression
  • Developing one’s identity
  • Separating from parents
  • Learning about love, intimacy & sexuality
  • Preparing for the world of work
  • Habilitation Issues
  • Mobility
  • Accessibility
  • Wheelchair Maintenance
  • Driving & Transportation
  • Medical Issues
  • Transition to Adult Health Care services
  • Weight Control & Exercise
  • Osteoporosis Prevention
  • Shunt Problems & Chiari Symptoms
  • Spinal Cord Problems
  • Urinary Track Infections
  • Bladder & Kidney Stones
  • Hypertension
  • Renal Insufficiency
  • Skin Injury & Pressure Sores
  • Non-compliance
  • Adjustment Problems – Confronting Disability
  • Depression
  • Learned Helplessness

 

Transition Time (Grade 10 – High School)

Transition Services
Generally begin in 10th grade. The child may or may not be the same age as peers. High School education can continue until age 21 years

 

Adulthood (Age 18 or Post-High School)

The more education you have, the more choices you have for work and career. Many people find that a high school education is not enough. You may want to consider an educational program after high school to prepare you for work and career:

  • Vocational education (hands-on training in job skills such as word processing, hair dressing, car repair)
  • Junior or community college
  • Technical education (training programs lasting several months to several years leading to specialized skills such as drafting, dental assisting, or commercial photography)
  • College or university

Students with disabilities are eligible for accommodations at most colleges, universities, and training programs. This is because Section 504 prohibits schools that receive any federal funding from discriminating against people with disabilities. Helpful accommodations might include a special parking permit, a tape recorder for class notes, or extra time for taking tests and completing assignments. You can find out about accessibility by:

  • Calling the school or program you are considering
  • Asking your guidance counselor
  • Looking at resources in your public library, such as Colleges with Programs for Students with Learning Disabilities (published by Peterson’s Guides) or Survival Guide for college Students with ADD or LD by Kathleen G. Nadeau.

If you decide to continue your education after high school, you’ll have to decide whether to live at home or on campus. The advantages and disadvantages of each choice are basically the same for you as for anyone making this decision. For example, if you live at home, costs will be less. On the other hand, living on campus gives you the experience of being on your own.

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Aging with Spina Bifida

Protecting the Mobility of the Aging Person with Cerebral Palsy or Spina Bifida
Joan S. Bergman

While the population in general is aging, so also are those who have cerebral palsy or spina bifida (Machemer & Overeynder, 1993). Unfortunately, members of these two groups are reporting with loud voices alarming messages: they are experiencing pain, loss of function, and loss of independence (Murphy, 1993; Overeynder, Turk, Dalton, & Janicki, 1992; Segalman, 1993). These problems are often thought of as being characteristic of people who are aging, but in these two groups their onset is being reported as beginning in the mid-twenties. Dr. L. G. Pawlson (1993) points out, “An injury such as a rotator cuff tear, which might be merely an inconvenience for a young person, may precipitate nursing home admission in an older woman who has preceding functional loss in dexterity and mobility from osteoarthritis and osteoporosis.” The compounding effects of trauma and aging on disability from cerebral palsy or spina bifida can be monumental.

Careful studies of what is known about aging in general, what is known about characteristics of cerebral palsy and spina bifida, and what happens when the former is overlaid on the latter are long overdue. Are people who have cerebral palsy or spina bifida receiving the best advice on how to maximize their potential and protect their future well-being?

Mobility and Aging in the General Population

The general population is aging at such a rate that architects, city planners, health providers, and merchants are among those taking notice. Despite the growing interest in meeting the needs of older people (variously referred to as aging, aged, geriatric, or senior citizens), there is not even agreement on the age that defines the younger boundary of this group (Machemer & Overeynder, 1993). For example, 65 years of age has often been considered the line marking off the workers from the retired, the capable from those who are becoming incapable of working and even of caring for themselves.

The widespread use of the magical age of 65 as a specific cut-off seems to have taken root in the rule that age 65 is when one is eligible to retire and draw full benefits from Social Security. It is interesting that so many assign significance to age 65 since it was apparently an arbitrarily chosen age at the time that Social Security was established. The age of eligibility for many senior citizen programs is younger, 60 years of age, while the American Association of Retired Persons, the largest organization and a very effective lobbying body for older people, requires one to have attained only 50 years of age to join (AARP, 1990). As the lower boundary moves to younger levels, some people interested in the aging process have found it necessary to distinguish the old from the very old or old-old (frail), terms that refer to people 85 years and older (Machemer & Overeynder, 1993).

For the purposes of this paper, I look at aging not as a point reached on the life-span continuum but as a process by which function begins to show a decline. I begin with a brief discussion of some of the more commonly reported areas of concern relating to mobility of all aging persons.

 

Loss of Bone Density (Osteoporosis)
Osteoporosis, an absolute decrease in bone density to the point at which fractures begin to occur, is the most common age-related pathologic condition of bone (Ben-Yishay & Zuckerman, 1993). In addition to osteoporosis that results from an underlying disease process (e.g., hypothyroidism), there are two types of osteoporosis, both considered typical complications of aging. The first affects primarily vertebrae and distal radii; the second type affects primarily the hips (Ben-Yishay & Zuckerman, 1993; Smith, Gilligan, & Kwiatkowski, 1993).

Minimally, osteoporosis can result in compression of vertebrae, which in turn results in a loss of height and range of motion of the spine. Particularly in slender, Caucasian women, the loss of bone density of the spine results in spontaneous compression fractures of the thoracic and lumbar parts of the spine. The consequences may include pain, disturbance of nerve function of the lower extremities, and loss of bladder and bowel control.

A second primary target of osteoporosis is the hip, where 95% of the fractures are either of the femoral neck or intertrochanteric (Smith, Gilligan, & Kwiatkowski, 1993). Fractures of the hip are often seen as heralding the beginning of the end for older people.

 

Reduction of Ability to Maintain Balance
A reduced ability to maintain balance is manifested in a person’s efforts to broaden his or her base of support while standing and walking. Women, for example, move from stylish pumps with heels to oxfords with broader, lower heels. Older people move more slowly and take special care with foot placement on stairs and on uneven terrain. How much of this increased caution is due to a reduction of righting and protective reflexes (Lewis & Knortz, 1993), loss of visual ability, or other factors is currently the subject of much research (Horak, Mirka, & Shupert, 1989; Winter, 1993; Woollacott, 1989).

 

Reduction of Muscle Strength and Endurance
For people in general, aging muscles remain relatively stable biochemically, and older sedentary men show only a small decrease in blood supply in com-parison with younger sedentary men. However, other typical changes of aging significantly affect muscle strength and endurance.

Aging is accompanied by a loss in the total number of muscle fibers and atrophy of those remaining. There is a reduction in the number of muscles in each motor unit and possibly in the number of motor units. As a result, a smaller amount of contractile tissue can be mobilized when a given motor unit is being recruited. Additionally, aging muscle is not able to contract as rapidly as younger muscle. Collagen fiber content increases in aging muscles, making them less compliant and, therefore, more susceptible to injury. Skeletal muscle changes in those who are aging are summarized by Menard (1993) as “a loss of endurance, strength, mass, contraction speed, and compliance.”

 

Reduction of Recuperative Abilities after Trauma
Connective tissues are highly dynamic structures that form the framework of many of the body’s structures, including those of ligaments and tendons. Diversity of composition allows for variations in the important properties of compliance and tensile strength. The composition varies according to function and use of structures, as well as with age. Changes associated with the aging process include increasing brittleness and vulnerability to fracture of elastic fibers, reduced shock-absorbing capacity, and reduced tissue compliance. Severely injured aging ligaments do not regain their original length, and their stress-strain properties are permanently disrupted (Menard, 1993).

 

Wearing Out of Joint Structures (Osteoarthritis)
As people age, their joints are typically negatively affected by several factors, including the thinning of articular cartilage, reduction of flexibility of tendons and ligaments, and reduction of vascular support (Menard, 1993). The result of years of wear on joint structures are commonly manifested in older people by pain and swelling of the knees, hips, fingers, and spine.

Compensatory measures include attempts to reduce further wear by limiting activities, strengthening muscles surrounding bothersome joints, and surgical joint replacements.

 

Pain in Response to Repetitive Movements
Repetitive microtrauma can result in multiple small tears in cartilage, tendons, and ligaments, and may lead to bursitis. Clinically, the person experiences debilitating pain and loss of function.

 

Resistance to the Use of Assistive Technology
The use of canes, crutches, walkers, scooters, and wheelchairs has a negative connotation and is too often considered by a person only when it becomes absolutely necessary, frequently after a fall instead of as a preventive measure.

 

Flattening of the Normal Curves of the Spine
To accommodate for the flattening of the spinal curve, the elderly person unconsciously flexes hips and knees when standing and walking (Nasca, 1993). Consequences of these changes in relationships of body structures may include pain, decrease in balance, and reduction in strength and endurance.

 

Misperceptions of the Conditions
Perhaps the most detrimental thing that can be done to someone who has cerebral palsy or spina bifida is done when that person is still an infant and the parent(s) are told that the condition is nonprogressive. A review of medical texts and articles on these two conditions affirms that the lesions causing the conditions are indeed nonprogressive (NIH, in press). Unfortunately, cerebral palsy and spina bifida are lifelong conditions, and although the contributing lesions may not be progressive, the conditions are now recognized as having progressive qualities. There is much variance within each condition depending upon the particular location and timing of the insult causing the abnormality.

Many resources have been expended to successfully reduce the incidence of both cerebral palsy and spina bifida. However, once the lesion has occurred, it cannot be erased. Once it is identified, the energies of professionals, caretakers, and patients are channeled into habilitation, the reduction of the effects of the lesion. Great harm can be done to a person and family by a failure to warn them that although the lesion may be nonprogressive, the condition may be progressive. Additionally, people can be seduced by the zeal of professionals into believing that if they carry out a prescribed program, all effects of the condition will disappear.

Another problem is that cerebral palsy is commonly considered to be a condition affecting only children. Recently a major lawsuit was brought to court because services were withheld from a young man of 16 years because it was thought that “people who have cerebral palsy do not need treatment after the age of 16.”

Finally, secondary disabilities have been all too easily overlooked. A secondary disability is a negative condition that develops because of the presence of a primary disability. Much attention has been given to the prevention of primary disabilities of such conditions as cerebral palsy and spina bifida, but professionals have been slow to put concerted efforts into the prevention of secondary disabilities (Pope & Tarlow, 1991).

 

Physical Malleability
The human body is malleable. It can be molded and shaped over time, as evidenced by people from other cultures who have necks that are a foot or more long, ear lobes that reach to their knees, or lips that protrude out from their faces like large platters. These unusual features are the result of molding from early childhood, not of genetic endowment.

People who have abnormal neuromuscular systems are prime candidates for finding that their bodies are being molded into nonfunctional shapes or positions. Persistent pulling of spastic muscles, for example, can cause the development of dislocated joints and scoliosis. Hypotonia, low muscle tone, puts one at the mercy of that strong force-gravity. Lack of or inappropriate support promotes joint dislocations, scoliosis, decrease in cardiopulmonary function, and digestive disorders. Care must be taken that the body is supported to grow in a way that promotes optimal function and comfort.

Overstretching Body Parts. The lack of sensation experienced by people who have spina bifida promotes hyperextension of joints, particularly of the lumbar spine and hips. This can lead to further loss of function, change in balance, subluxation or dislocation of the hips, and entrapment of nerves and blood vessels.

Body parts may be subjected to undue stretching when stereotypical movement patterns are allowed to be repeated over and over. Additionally, overstretching is often an unintentional part of a treatment program or functional behavior. For example, it is not unusual for a child, as a part of the treatment regimen, to be positioned in a way that promotes a certain desired motor behavior but that ignores the excessive excursion of a body part. A typical scenario might go as follows: A child is positioned prone over a wedge to promote head control. In this position the child is instructed, “Hold your head up.”

Books, pictures, or other enticements may be positioned to make holding the head up (cervical spine in extension) rewarding. Mercury switches may be used to control radios or tape players to provide immediate positive feedback. Unfortunately, when the child relaxes or muscles fatigue, the head may fall far forward. The neck is now in a position of extreme flexion, which causes overstretching of posterior neck muscles. If this, or other contraindicated movements, are allowed repeatedly over a period of time, the structures will become irrevocably overstretched. A secondary disability has now been created unnecessarily.

In the same fashion, in the name of promoting function, switch controls are sometimes placed so that a person accesses them by use of an abnormal reflex pattern. This strengthens an abnormal movement pattern while allowing body parts to move through a range so large that structures are frequently inappropriately stressed.

The Overlay of Normal Aging. People who have cerebral palsy or spina bifida traditionally spend many years perfecting motor skills that allow them mobility. Slight changes in their environment-for example, stairs with other than standard risers-can cause major problems in adjustment. It should be expected, then, that an overlay of the insidious changes brought about by the normal aging process can play havoc with a person’s ability to function. For example, there is the person who has cerebral palsy who, after years of practice, has perfected balance and ambulation. But carefully perfected balance in the body is lost when, as a natural part of the aging process, spinal curves flatten, tendons and ligaments lose flexibility, muscles lose strength, and righting and protective reflexes slow. There may be just enough change to cause falls that result in injuries such as fractures.

The effect of repetitive microtrauma that results from walking on hips where there are abnormal biomechanics must be considered. Complaints of bursitis and osteoarthritis are frequent among relatively young people with cerebral palsy.

It is not uncommon for people who have spina bifida or cerebral palsy with low muscle tone to be overweight. The extra weight adds to the stress of ambulating with crutches, so that people in their mid-twenties frequently find themselves with severely traumatized shoulder structures.

By ignoring such potential damage to body structures, are we setting people up for pain, cardiopulmonary compromise, and tissue breakdown as early as their twenties? We are seeing people who have made extraordinary efforts throughout childhood, adolescence, and early adulthood now facing unemployment, loss of their homes, and, ultimately, the loss of their independence.

Pressure from Social and Lifestyle Values. Our society values youth, physical prowess, extraordinary feats of physical ability, and normal appearance and behavior. Only when someone is particularly successful in some aspect of life does society readily accept that person’s deviation from the norm. It is the norm for people to attempt to retain or regain their youth by using creams and even surgery to reduce wrinkles, dying hair to hide dullness or gray, dieting and exercising to attain younger-looking bodies, and wearing stylishly youthful clothes.

There is a fairly narrow band of gait that is accepted as being within the norm; beyond this, we speak of abnormal gait patterns. Parents of young children who have cerebral palsy or spina bifida are encouraged by professionals (e.g., physical therapists, occupational therapists, physicians) to strive to have their children achieve a pattern of walking that is as close to normal as possible, to practice for hours on end (could this be considered developing a lifetime pattern of overuse?), and to use this ability to walk as much as possible. How can we then justify promoting the idea that someone with a spastic gait, obviously ambulating with biomechanics that are not optimal, complete a marathon run?

The same question might be asked with regard to someone with spina bifida, where the means of mobility include undue wear and tear on shoulder, elbow, and wrist joints and, frequently, repeated movements of extreme lordosis of the lumbosacral spine. Is it appropriate for distance walking to be a part of that person’s individualized education program (IEP)? And should a teenager be allowed to arrive five minutes late for each class and leave ten minutes early because of the length of time required to ambulate or operate a manual wheelchair from room to room? We must return to thinking about the reason for ambulation-to accomplish getting from one place to another.

People who have disabilities of mobility should be taught to be discriminating about their choice of mode of mobility under various circumstances. Internalizing the concepts of age-appropriate and condition-appropriate use of assistive technology should start early and be reinforced throughout life.

The Lack of Knowledge of Professionals. Although there may not be enough of them, there are people in the medical and health care professions who have specialized information and who can provide good care to persons who have cerebral palsy or spina bifida while they are young. Unfortunately, as they move out of childhood, they will find very few people who are prepared to provide them quality care (Murphy, 1993; Segalman, 1993). To compound the problem, even those people who are aging without primary disabilities find it difficult to receive quality medical care (Lewis & Knortz, 1993). The lack of knowledge about the special problems of aging leaves people to struggle on their own to find answers and guidance, a situation that certainly leads to further problems.

Interdisciplinary teams dealing with developmental disabilities must be broadened to include people who are efficiency and ergonomics experts. It should be standard practice that videotapes are taken of people in their “regular” lives. A broad array of people, including the people who have cerebral palsy or spina bifida, should analyze these, looking not only at the present physical behaviors but also considering the likely future so that the questions like “If this behavior continues for a period of years, what will be the long-term effect on the body?” and “Can the person afford that?” can be answered. This careful scrutiny can help us find alternative ways of approaching tasks and at least avoiding the effects of:

  • repetitive movements when there are abnormal biomechanics;
  • repeated overextension of joints from: reflex movements, spastic or athetoid (unceasing, involuntary) movements without graded co-contraction, inappropriate positions such as extreme lordotic posture, and entrapment of nerves, blood vessels, or both;
  • joint dislocations (e.g., hip, temporal mandibular, shoulder);
  • adopting abnormal postures for stabilization (e.g., wrapping arms around push handles and legs around uprights of wheelchairs) with consequent tearing up of joints, overstretching of structures, nerve entrapments, pain, and loss of function.

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Preventing or Delaying Secondary Disability

In Infancy and Early Childhood. Early intervention programs are prevalent and, although the criteria differ somewhat state by state, services should be available to any infant or toddler who has a developmental disability or who is at risk, and to that child’s family. This is the time to establish a philosophy of care and lifestyle pattern. The establishment of a healthy lifestyle requires a strong interdisciplinary approach in which all team members come together and identify the characteristics specific to the individual and the behaviors that would therefore be therapeutic.

These behaviors can then be incorporated in a normal fashion into the person’s daily life. For example, if an infant has very low muscle tone, professionals should not teach the parent to undress the infant twice a day for facilitory brisk rubbing but instead should teach them methods of bathing, dressing, and diapering that incorporate the treatment techniques into the child’s regular routine.

Attention during early childhood should be focused on assisting the youngster to do what peers are doing. Assistive technology should be provided as age appropriate and condition appropriate. This is the time to imbue the child with self-confidence, not with the need to be a superperson

During Transition Periods. The times when people are moving from one stage of life to another provide optimal opportunities for carefully examining all aspects of life and reassessing the best approaches to problems. Transition times are good times to convene the interdisciplinary team appropriate for that particular stage of life to help the person develop a revised plan for accomplishing his or her goals. These times are excellent for determining needed supports (e.g., medical, therapeutic, family, financial, assistive technology) and the best means to obtain them.

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Recommendations

  • Revise the definitions of cerebral palsy and spina bifida to include consideration of secondary disabilities.
    Compare the characteristics of young adults with cerebral palsy or spina bifida who received intensive early intervention with those who received the more traditional kinds of care provided before the mid-1970s.
  • Alert those who have mobility concerns and their care providers (physicians, physical therapists, and occupational therapists) to the potential dangers inherent in activities that use abnormal movement patterns.
  • Develop curricula for preservice, inservice, and specialization programs and promote their incorporation into the general curricula. To avoid the unending battle to get one more new concept into academic and training programs, promote the incorporation of special curricula at national accrediting levels.
  • Disseminate information regarding concerns through professional journals, conferences, and meetings.
  • Bring together people from traditional and nontraditional disciplines to attack the problems. Include at least pediatricians, neurologists, orthopedists, phys-iatrists, gerontologists, physical therapists, occupational therapists, nutritionists, dentists, ergonomists, architects, recreation professionals, and rehabilitation technologists. Make certain that people who have cerebral palsy or spina bifida are well represented.
  • Develop and implement a program for communicating concerns and recommendations regarding secondary disability and aging to service providers.
  • Until more is definitively known, promote the use of assistive technology to save body parts wherever possible.
    Gather data-both cross-sectional and longitudinal-and analyze the facts carefully from an interdisciplinary perspective. Develop informational and training programs based upon the data.
  • Begin teaching in early childhood that people are responsible for monitoring and promoting their own health.
    Because of the dangers of inactivity, develop and promote alternate forms of exercise and activity for people with mobility disabilities.

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References

AARP. (1990). A profile of older Americans. Washington, D.C.: American Association of Retired Persons (1909 K St., NW, 20049).

Ben-Yishay, A., & Zuckerman, J. D. (1993). Hip injuries: An orthopedic perspective. In C. B. Lewis & K. A. Knortz (Eds.), Orthopedic assessment and treatment of the geriatric patient. St. Louis, Mo.: C. V. Mosby.

Horak, F. B., Mirka, A., & Shupert, C. L. (1989). The role of peripheral vestibular disorders in postural dyscontrol in the elderly. In M. H. Woollacott & A. Shumway-Cook (Eds.), Development of posture and gait across the life span. Columbia: University of South Carolina Press.

Lewis, C. B., & Knortz, K. A. (Eds.). (1993). Orthopedic assessment and treatment of the geriatric patient. St. Louis, Mo.: C. V. Mosby.

Machemer, R. H., & Overeynder, J. C. (Eds.). (1993). Aging and developmental disabilities-An in-service curriculum. Rochester, N.Y.: University of Rochester.

Menard, D. (1993). Neuromuscular considerations. In C. B. Lewis & K. A. Knortz (Eds.), Orthopedic assessment and treatment of the geriatric patient. St. Louis, Mo.: C. V. Mosby.
Murphy, K. P. (1993). Medical and social issues in adults with cerebral palsy-The California study. Unpublished report.

Nasca, R. J. (1993). Low back injuries: An orthopedic perspective. In C. B. Lewis & K. A. Knortz (Eds.), Orthopedic assessment and treatment of the geriatric patient. St. Louis: C. V. Mosby.

National Institutes of Health, National Institute of Neurological Disorders and Stroke. (in press). Cerebral palsy. Hope through research.

Overeynder, J. C., Turk, M., Dalton, A. J., & Janicki, M. P. (1992). “I’m worried about the future . . .”: The aging of adults with cerebral palsy. Albany, N.Y.: New York State Developmental Disabilities Planning Council.

Pawlson, L. G. (1993). Foreword. In C. B. Lewis & K. A. Knortz (Eds.), Ortho-pedic assessment and treatment of the geriatric patient (p. vii). St. Louis, Mo.: C. V. Mosby.

Pope, A. M., & Tarlow, A. R. (Eds.). (1991). Summary & recommendations: Disability in America-Toward a national agenda for prevention. Washington, D.C.: Institute of Medicine, National Academy Press.

Segalman, R. Z. (1993). A personal experience with cerebral palsy with total body involvement: Solving medical problems in midlife. Oakland, Calif: United Cerebral Palsy (1970 Broadway, #605, 94612).

Smith, E. L. Gilligan, C., & Kwiatkowski, B. S. (1993). Osteoporosis: Background and management. In C. B. Lewis & K. A. Knortz (Eds.), Orthopedic assessment and treatment of the geriatric patient. St. Louis, Mo.: C. V. Mosby.

Winter, K. (1993). PT at University of Oregon studies balance training for the elderly. PT Bulletin, 8(5), 12-22.

Woollacott, M. H. (1989). Aging, posture control, and movement preparation. In M. H. Woollacott & A. Shumway-Cook (Eds.), Development of posture and gait across the life span. Columbia: University of South Carolina Press.

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