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Achieving continence in children with neurogenic bowel and bladder
ORIGINAL ARTICLE
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Achieving Continence in Children With Neurogenic Bowel and Bladder C a n d a c e F. Z i ck l e r, M S N , R N , C P N P, & Vi r g i n i a R i ch a r d s o n , D N S , R N , C P N P
I
ABSTRACT Bowel and bladder continence is a major developmental accomplishment for a child and his or her family. Children with neurogenic bowel and bladder dysfunction, which occurs with myelomeningocele and other neural tube defects, have a physical inability to attain continence. However, children with these limitations can attain continence when the appropriate modifications to the traditional routines are made. Enabling the child to attain continence improves parental relationships and self-concept. The nurse who is working with children with myelomeningocele in schools, clinics, private offices, and home health care settings can play a vital role in helping a child with a neural tube defect achieve continence. J Pediatr Health Care. (2004). 18, 276-283 .
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n children who undergo typical development, maturation has predictable patterns that are based on neurologic and physical growth (Stein, 2000). When children are born with medical conditions such as myelomeningocele (spina bifida), they have both sensory and motor impairments that affect their ability to achieve all of their developmental milestones. Toilet training is a significant developmental milestone in the life of a child. Complex learning such as mastery of bowel and bladder control often is challenging even for children who develop normally. Children are usually toilet trained by 21/2 to 4 years of age. Bowel control comes before bladder control, and daytime training is achieved before a child stays dry at night. Girls generally achieve dryness before boys, and for many children, complete continence is not achieved until the child reaches 5 to 6 years of age. When toddlers are ready to be continent they demonstrate readiness by coming to the parents, telling them that they are wet or dirty, and pulling off their diapers (Fox, 2002; Schmitt, 2004a). Continence is both a physiologically and psychologically based skill. Physical requirements for continence occur when the child can stand upright and the abdominal muscles are strong enough to demonstrate trunk control sufficient for walking, sitting, and standing. The child also must have the motor skills to be able to bend and sit and must be able to pull down his or her pants at the appropriate time. The psychological readiness occurs when the child has both the cognitive development and motivation to remain continent (Schmitt, 2004a). The child should be able to make
Candace F. Zickler is a Pediatric Nurse Practioner–Clinician, Team Clinics, Children’s Medical Center, Dayton, Ohio. Virginia Richardson is Assistant Dean for Student Affairs, Indiana University School of Nursing, School Based Clinic, Clarian Health Partners, Indianapolis, Ind. Reprint requests: Candace F. Zickler, MSN, RN, CPNP, 3372 Ridgeway Rd, Kettering, OH 45419-5014; email: [email protected]. 0891-5245/$30.00 Copyright © 2004 by the National Association of Pediatric Nurse Practitioners. doi:10.1016/j.pedhc.2004.05.005
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PH ORIGINAL ARTICLE C an association between the feelings elicited for voiding and bowel movements, ave a desire to learn the skill by demonstrating increasing periods of dryness during the daytime, be able to positively take the suggestion from the parent that they should sit on the toilet, and feel a sense of accomplishment when they are eliminating on the toilet (Stein, 2000). Frequently, mastery of continence is attained when the family is able to minimize other stressors and help the child focus on toileting. When a child continues to be incontinent in spite of the parents’ intense efforts at using their personal experiences, relatives’ suggestions, or professionals’ counseling, the parents become frustrated. The child’s continued incontinence results in stress between the parent and the child (Schmitt, 2004b; Stein, 2000). Children who do not achieve continence develop a poor self-image and have difficulties with peer and social interactions (Dixon, 2000). Just as normally developing children may resist toilet training, toddlers with spina bifida also may demonstrate resistance to mastery of this skill. Many nondisabled children have relapses after they have achieved dryness, especially at times of stress, but these relapses are only temporary (Schmitt, 2004b; Stein, 2000).
NEURAL TUBE ABNORMALITIES Neural tube defects (NTDs) are some of the most complex and disabling birth defects known (Kolaski, 2002). With the advances that have occurred in medicine and pediatrics and the multidisciplinary approach obtained by working with geneticists, neurosurgeons, and urology/reproductive and orthopedic surgeons, during the past 30 years the vast majority of children with spina bifida are living well into older adulthood. Although they should attend multidisciplinary clinics to receive subspecialty evaluations and care, children with spina bifida are seen for their routine well child and preventive health care needs in general pediatric and medical practices (Becske & Jallo, 2003; Nickel, 2000). Pediatric nurses are working with these children and helping them attain their age-appropriate developmental skills. At times the associated problems that accompany a diagnosis of spina bifida limit the child’s ability to master toilet
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training. In addition to the loss of bowel and bladder control and lower extremity function, children with spina bifida are at increased risk for hydrocephalus from an Arnold Chiari II malformation, fine motor and dexterity limitations, seizures, visual and perceptual problems, and cognitive deficits. Ten to twenty percent of children with spina bifida experience learning disabilities, especially with reasoning and remembering (Bonney, 2002; Nickel, 2000). NTDs have a multifactorial form of inheritance and account for the majority of congenital central nervous system disorders. The defects are the result of failure of the neural tube to close spontaneously at 3 to 4 weeks of gestation and result in partial or total paralysis below the level of the defect. The most common NTD compatible with life is myelomeningocele (spina bifida); its incidence is 1 in 1200 to 1400 live births. About 2500 babies are born with myelomeningocele each year (March of
A
t times the associated
problems that accompany
has also been implicated (Becske & Jallo, 2003). Parental exposure to Agent Orange, a herbicide used to defoliate the jungles during the Vietnam war, has been associated with NTDs. A fund has been established to help children born to parents who have been exposed to Agent Orange (Environmental Agents Service, 2003). In addition to the environmental and teratogenic relationships, there is ongoing research into the genetic links that may exist (Volcik et al., 2002). Folic acid, a B vitamin that acts as a co-factor for enzymatic processes involved with DNA and RNA synthesis, is recommended for all women of child-bearing age. Folate is important in the metabolism of homocysteine and is necessary for the synthesis of methionine. Methionine is needed in the metabolism of proteins, lipids, and myelin (Nickel, 2000). Furthermore, the inclusion of folic acid in a preconceptual and prenatal diet may be directly protective against neural tube defects in up to 70% of infants with the folate deficit. With insufficient quantities of folic acid, the genetic risks plus the environmental exposures are thought to result in approximately 50% of first-time neural tube defects and 70% of recurrent neural tube defects (March of Dimes, 2003).
a diagnosis of spina bifida
THE PHYSIOLOGIC PROCESS OF MICTURITION
limit the child’s ability to
The urinary system consists of the upper urinary tract and the lower urinary tract. The upper urinary tract includes the renal blood vessels, renal parenchyma, renal pelvises, the two ureters, and includes up to the uretero-vesical junction. The upper urinary tract system does not require innervations from the spinal cord to function normally. The lower urinary tract includes the uretero-vesical junction, the detursor muscle (bladder), the urethral control mechanism, the urethra, and the afferent and efferent nerves that are attached to the central nervous system (spinal cord). Normal lower urinary tract functioning consists of filling and storage of urine and emptying or voiding. The bladder neck and posterior urethra are controlled by the thoracic10 to lumbar-2 (T-10 to L-2) portions of the hypogastric nerve. In normally functioning urinary systems, somatic innervations of the external sphincter increase the tone at the pudendal nerve
master toilet training.
Dimes, 2003). Since the advent of prenatal diagnosis, elective abortions, and the increased prescribing of folic acid, the rate of babies born with NTD has decreased dramatically (Becske & Jallo, 2003; Kolaski, 2002). Although there is no definitive cause for myelomeningocele, a number of teratogens have been implicated as having a higher than normal incidence. Some of the teratogens include the season of conception, the geographic region, maternal diabetes, maternal age, maternal valprote use, maternal alcohol abuse, lower socioeconomic class, maternal zinc or folate deficiencies, and an episode of maternal hyperthermia such as a high fever or hot tub use. Maternal obesity is an independent factor that
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sacral-2 through sacral-4 (S-2 to S-4) and create a voluntary and efficient emptying of the bladder. The feeling of a need to urinate is triggered when the bladder fills to 300 to 500 mL (Churchill, Abramson, & Wahl, 2001). There are two types of neurogenic bladder dysfunction. Aspastic or hyperreflexive bladder is characterized by uncontrolled, frequent expulsion of urine and incomplete emptying. A spastic bladder is very rigid and stiff on voiding cystourethrogram. A flaccid or hypotonic bladder is weak and stretched out and can hold extremely large quantities of urine (as high as 2000 mL at a time) and can create a continuous dribbling from the urethra. Both types of dysfunction can result in reflux into the renal parenchyma, irreversible pyelonephritis with scarring of the renal tubules, and subsequent loss of renal function. The upper urinary tract is preserved in children and adults if urine is regularly and completely emptied from the bladder and the leak point pressures are kept below 40 cm H20 (Churchill et al., 2001). Management of neurogenic bladder should be of paramount concern to health care providers of children with spina bifida because renal failure continues to be the leading cause of death in patients after the first year of life (Kolaski, 2002).
below thoracic 12 (T-12) vertebrae. The T-12 lesions result in loss of sensation or urge and continuous oozing from the rectum. The children may have offensive odors and must wear diapers, which embarrasses them and makes them reluctant to socialize with peers. A child with reflexic bowel dysfunction or an upper motor neuron lesion has damage above thoracic 12 (T-12) vertebrae and the anal reflexes are intact, giving the child the ability to stimulate the rectum to prompt elimination (Feng & Churchill, 2001). However, because of the level of injury, the decreased mobility and poor fine motor ability, manual stimulation is generally limited. These children also will have to wear diapers because of incomplete emptying of the colon, flatus, and soiling.
THE PHYSIOLOGIC PROCESS OF BOWEL EVACUATION
defects are the distal
The major areas of the gastrointestinal tract that are affected by neural tube defects are the distal portions of the rectum, anus, and anal sphincter. The proximal gastrointestinal system is not directly affected by neural tube defects. The two primary involuntary reflexes that initiate defecation are the intrinsic and parasympathetic defecation reflexes, located at the sacral 2 to 4 level (S2 to S-4) of the spinal cord. The pudendal nerve is responsible for voluntary defecation and controls the opening and closing of the external anal spinchter. Because the sensation of a need to defecate often is absent in children with spina bifida, the fecal material is infrequently eliminated and constipation with subsequent fecal impaction results (Feng & Churchill, 2001). There are two types of neurogenic bowel dysfunction, areflexic and reflexic. If a child has an areflexic or lower motor neuron dysfunction, the lesion is
portions of the rectum,
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EXAMINATION OF THE CHILD History
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he major areas of the
gastrointestinal tract that are affected by neural tube
anus, and anal sphincter.
The child’s ability to attain continence begins with an understanding of the families’ cultural expectations, especially the timing and “words” that the family uses to describe the process to the child. It is helpful to determine how the child will be treated if an “accident” occurs, so the parent appreciates the role that readiness and the child’s desire to please influences the outcome. Ask the parent if there is any family history of relatives or siblings with bowel or bladder dysfunction. If so, an assessment of signs and symptoms that may indicate an inherited condition and further data should be pursued. Eliciting the frequency of bowel movements and the timing that the child usually has
bowel movements helps establish a pattern from which to begin the bowel program teaching. Asking the parent about the size of the bowel movement and the degree of difficulty the child has with elimination can help with identifying constipation or diarrhea. (If the child has a long-standing history of constipation or diarrhea, it is advantageous to remedy this problem before moving on with the toilet training.) In normally developing young toddlers, voiding every 1 1/2 to 3 hours is common (Murphy, 2000). The frequency and volume of urine can be estimated from the child’s history of wet diapers. If the parent mentions that the child’s urine is cloudy or has a strong odor, the urine should be examined for bacteria. Any history of kidney or flank pain, dysuria, ureteral colic, or hesitancy should be thoroughly evaluated with an abdominal examination, a culture and urinalysis, and possibly a flat plate of the abdomen, looking for calcifications or stones. It is important to determine if the child is taking medications, such as anticholinergics or laxatives, that may influence elimination. Ask if there is any history of nausea, vomiting, anorexia, or hematemasis. Determine if the child has been taking the medications for a brief or extended period of time, and review the dosages and instructions that accompany the medication administration (Taketomo, Hodding, & Kraus, 2002). Reviewing the child’s meal times and the types of solids and fluids consumed at each meal is helpful in determining if sufficient fiber and fluid is present to facilitate normal bowel and bladder elimination. Many young children are picky eaters, and a diet history can help with counseling to optimize the quantity and quality of intake. Briefly review the child’s ability to eat normally by assessing such concerns as the child’s tolerance for thin and thick fluids, ability to masticate food properly, and ability to swallow without choking. If a history of frequent aspiration or pneumonia is present, a feeding study and a barium swallow are indicated (Bonney, 2002).
Physical Examination Spina bifida is a complex neuromotor and anatomic malformation that necessitates a thorough, systematic approach to the physical examination. Children
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PH ORIGINAL ARTICLE C should be examined while wearing gowns with all of their underwear removed. Using age-specific and developmentally appropriate examination skills facilitates comfort and cooperation from the child. The weight in kilograms, length and height in centimeters, the basal metabolic index, and head circumference are measured and plotted on the standard National Center for Health Statistics graph to ensure growth rates appropriate for age. Serial measurements plotted on the same growth grid assist in identifying abnormal trends of increasing or decreasing weight and increasing head circumference. Increasing head circumference can indicate hydrocephalus or shunt malfunction. Because some children with spina bifida are non-weight bearing, upper arm span measurements should be used to approximate the height (Kleinman, 2004). Routine monitoring of blood pressure and temperature can detect early renal and bladder dysfunction and need to be graphed and evaluated on standardized grids at each visit. Children who are developing typically will probably be 2 to 5 years of age when toilet training is initiated. Children who happen to have spina bifida also will be ready for toilet training at that age and will act and behave in a fashion similar to that of their age-related peers who are not disabled. Observation of the child’s interaction with the parent and active listening to the conversation facilitates a determination of the developmental level of the child. The use of a Denver Developmenatal Screening Test can assist with determining the gross motor, fine motor, personal, social, and communication skills of the child. If formal developmental evaluations have been performed in an early intervention program, review the reports for language age, comprehension, and problem-solving level to validate your assessment of the child’s functional level. The child’s head and neck should be examined and palpated for a cerebral spinal fluid shunt and tubing, which is present in 90% of children with myelomeningocele (Nickel, 2000). Do not pump the shunt, because doing so can cause headache and discomfort for the child. Vision and hearing should be evaluated during the visit, but formal testing of sight and hearing identifies
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deficits that may interfere with learning. The cranial nerves are generally intact but the examiner may note a child with a torticollis, facial weakness, or nystagmus. Any abnormalities should be more thoroughly evaluated and appropriate referrals made (Bickley & Szilagyi, 2003). Assessment of the abdomen should include evaluation of the skin for scars, rashes, or lesions, examination of the shape, contour, and symmetry of the surface, and examination of the umbilicus for location and any signs of hernia or inflammation. There may be a small scar where the cerebral spinal fluid shunt was routed through the abdomen. Auscultation, percussion, and deep and light palpation are performed over the abdominal surface to detect the presence of a full bladder or a fecal mass in the lower quadrants. Palpation of the lateral flanks for the size and lo-
D
etermining if an
umbilical reflex is present or absent can assist in locating the level of the neurologic loss.
cation of the kidneys bilaterally may detect pain or tenderness. Determining if an umbilical reflex is present or absent can assist in locating the level of the neurologic loss. Because sensation often is decreased or absent below the level of the spina bifida repair, examining, smelling, and palpating the entire skin surface is essential. If dampness or excoriation is noted, aggressive therapy to relieve the moisture and the pressure from the area is indicated (Bickley & Szilagyi, 2003). Following the abdominal examination, help the child roll onto his or her side and explain that you are going to examine their rectum. The back and buttocks should be examined for any dimples, scarring, or fistulas. If the child is cooperative and is able to bear down, ask him or her to do so as you in-
sert a gloved, lubricated finger into the anus. If the examiner can feel the anal spinchter tighten around the finger, increased or normal tone is present. If the child has not had a bowel movement recently, fecal matter also may be palpated. In children without neurologic loss of the anal reflex, the “anal wink” can be elicited by using a dull object and stroking the edges of the anus (Bickley & Szilagyi, 2003). The presence of the anal reflex indicates that nerves are intact that will aid with bowel elimination. Above the level of the repaired lesion, the child may have few neurologic abnormalities. Many children with spina bifida have good strength and tone in their shoulders, arms, and hands. Lower motor neuron lesions result in loss of muscle tone, absent or decreased deep tendon reflexes, hypotonia, and thin wasted extremities (Bickley & Szilagyi, 2003). The lower extremities will have varying degrees of muscle wasting (hypotonia). Performing passive, gentle range of motion and assessments of both deep and light sensation of the lower extremities locates areas where the child may have some sensation and/or motor control. Upper motor neuron lesions anywhere along the spinal cord result in spastic muscle tone, with passive resistance on range of motion movements. If the child is able to ambulate, the gait and ability to stand independently should be evaluated. The presence of scars on the hips, thighs, and feet may indicate previous orthopedic surgeries. Children who ambulate should be evaluated by an orthopedist to optimize their ability to stand, walk, run, and play. Some children with spina bifida may have dislocated hips and contractures. The motor level is a good predictor of functional status (Nickel, 2000). Popliteal and pedal pulses should be assessed and located in both legs. In assessing the genitourinary and reproductive systems, inspect the skin and note any excoriation. Determine if the urethral orifice is located appropriately for the sex of the child. Palpate for the presence of femoral pulses. In male patients, it is important to inspect the penis for any inflammation and the presence of a foreskin, for the location of the urethral meatus, the descent and size of the testicles in the scrotum, and the presence of any inguinal hernias. In
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PH ORIGINAL ARTICLE C female patients, a careful examination of the external genitalia may identify a prolapsed urethra, cystocele, or a varicocele. Associated anomalies frequently can be present.
Diagnostic Tests for Assessing Kidney and Bladder Function The level of the spinal lesion correlates poorly with urodynamic and radiologic studies, necessitating regular systematic evaluation of bladder function. Normal residual urine volume, after voiding, is less than 5 mL at all ages. Normal bladder storage pressure should remain at less than 20 cm H20 throughout life. These standard results can be utilized in evaluating both children and adults with bladder dysfunction (Churchill et al., 2001). A number of diagnostic tests should be done twice yearly to assess the integrity of the urinary tract system. Routine urinalysis with culture and sensitivities are indicated for a person of any age with a neurogenic bladder. Serum blood urea nitrogen and the creatinine level help determine renal clearance and kidney function (Churchill et al., 2001; Nickel, 2000). Radiologic examinations that evaluate bladder dysfunction and incontinence are performed annually or more frequently if indicated. Abdominal films of the abdomen can be used to examine the kidneys, ureters, and bladder and assess for the presence of calculi. A voiding cystourethrogram is done to visualize the integrity of the entire urinary collecting system. A cystoscopy also is recommended for any person with frequent urinary tract infections (Churchill et al., 2001). An ultrasound of the kidneys is used to evaluate the upper urinary tract and determine if obstruction, scarring, masses, or calculi are present. Periodically urodynamics will be done to evaluate filling, storage, bladder capacity, bladder pressures, detursor muscle function, leak points, bladder compliance, and the ability to voluntarily void. The urodynamics are used as a gauge of stable/deteriorating bladder or renal status (National Institutes of Health, 2002).
TECHNIQUES IN PRESERVING KIDNEY FUNCTION Children with neurogenic bladders are at life-long risk for kidney and bladder infections, incomplete bladder empty-
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ing, vesicoureteral reflux, poor kidney growth, renal cortex damage from reflux, and urinary incontinence (Churchill et al., 2001; Kolaski, 2002). Without interventions and regular evaluations, more than 90% of infants and children with a myelomeningocele defect will demonstrate symptoms and urodynamic changes of renal deterioration and/or bladder dysfunction (Churchill et al., 2001). When diagnostic tests and the child’s symptoms indicate a deteriorating condition, clean intermittent catheterization (CIC) will be started. Sometimes CIC will be initiated in the newborn nursery. The infant with urinary reflux at birth benefits from early initiation of CIC. If the newborn does not demonstrate reflux, delaying the initiation of CIC until there is noticeable change in
T
he level of the spinal
lesion correlates poorly with urodynamic and radiologic studies, necessitating regular systematic evaluation of bladder function.
the renal function is not harmful and in fact is easier for the parents to accept, given the overwhelming stress encountered during the newborn period. Depending on the level of the spina bifida and the degree of involvement to the spinal cord, some children will never need to be catheterized (Churchill et al., 2001). Many children can and do learn to do their own CIC, with supervision, by the time they are 6 to 8 years of age (Spinal Cord Injury Information Network, 2001). When parents are told that they will need to learn bladder catheterization, they fear hurting the child or introducing infection. With proper teaching and practice, good hand washing, and cleansing of the genitals, the risks are
minimized (Clean Intermittent Catheterization, 2002). Teaching videos are available through catheter vendors such as Astra (http://www.astratechusa. com) and Mentor Corporation (http:// www.mentorcorp.com/bladdercontrol/index. htm). The videos enable parents to view the procedure before they are taught to perform it on their child. The parents need to be instructed how to perform a sterile catheterization on their child because the procedure is initially frightening and stressful for them. Prior to being sent home to do CIC, the parent is given prescriptions for catheters and lubricant, written instructions, and a contact phone number. Follow-up after the instruction is often done by phone, but occasionally the parent may need to return for more hands-on practice (Association for Continence Advice, 2003). Persons who do CIC or perform it for the infant or child should be told that over time bacterial “accumulation” occurs in the urine. These “resident” organisms do not cause renal damage unless there is significant reflux and are considered normal flora for the child (Morton et al., 2002). The most frequently isolated organisms found in the urine of individuals performing CIC are Escherichia coli, Klebsiella, and Proteus. If on routine culture and sensitivity analysis of the urine the rates of bacteria exceed 100,000 organisms per field or the organisms are considered pathologic, such as Enterobacter, Pseudomonas, Serratia or Candida, then a urinary tract infection exists and must be treated. To evaluate the urine of a patient who uses CIC, it is recommended that a sterile catheterized specimen be obtained. All catheterizations should be carried out with a latex free, lubricated catheter to ensure that latex sensitivity does not occur. Antibiotic prophylaxis has been shown to significantly reduce bactiuria in acutely injured spinal cord patients, and there is some reduction in bactiuria in nonacute, chronic spinal cord patients. However, antibiotic prophylaxis results in a two-fold increase in resistant organisms and should be avoided as a treatment modality, especially in persons who are at increased risk of urinary tract infections (Morton et al., 2002).
MEDICATIONS THAT HELP WITH NEUROGENIC BLADDER In children who have stiff, noncompli-
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PH ORIGINAL ARTICLE C ant bladders, antispasmodic medications such as oxybutynin (Ditropan, Ditropan XL) or propantheline (ProBanthine, Propanthel) work directly on the bladder to relax the muscle and inhibit the action of acetylcholine. These medications increase the capacity of the bladder, thus decreasing potential reflux. Both medications come in tablet form. The oxybutynin comes in both a long-acting and a syrup form and is approved by the Food and Drug Administration for use in children 1 year of age and older (Taketomo et al., 2002).
SURGICAL PROCEDURES FOR IMPROVING CONTINENCE There are surgical procedures that can be performed to preserve renal function by decreasing vesicoureteral reflux. The traditional method of ureteral implantation moves the ureters higher up on the bladder wall, thus decreasing the potential for reflux. Recently, an endoscopic technique of injecting a polysaccharide compound into the mucous membrane of the ureter has been utilized. The “bulge” that is created in the ureter acts as a one-way opening for the urine to exit the kidney and prevents urinary reflux. The endoscopic procedure is approved by the Food and Drug Administration for vesicoureteral reflux grades II to IV. There have been no long-term safety or efficacy concerns for the procedure, and 70% to 80% of such procedures have a high long-term success rate (Capozza & Caione, 2002; Kirsch, Perez-Brayfield, & Scherz, 2003). Vesicostomies and bladder augmentations can be performed if the bladder wall is so noncompliant that little urine is able to be stored within it. The vesicostomy is a surgical opening on the abdomen that drains urine continuously. This necessitates that the child wear diapers to prevent soiling of clothes. Bladder augmentation utilizes tissue from the stomach, the intestine, or the ureter to “enlarge or expand” the size of the bladder. When the bladder augmentation is performed, it tends to lower the pressure by increasing the capacity of the bladder (Association for Continence Advice, 2003; Churchill et al., 2001). Auretheral sling or bladder neck suspension procedure can be done to decrease urinary dribbling. The surgically created “sling” lifts up the bladder, thus exerting pressure on the urethra to in-
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crease urine retention. In rare instances, a cuff is surgically placed around the urethra or bladder neck to help prevent urinary leakage. The cuffs enable the urethra to retain urine until voiding is desired. The cuff is part of a balloon reservoir that has been placed in the perineum and is squeezed by the child at the time of desired voiding (Churchill et al., 2001). When children become old enough to manage their own catheterizations and desire independence, if they have poor manual dexterity or if their caretakers have difficulty getting the child in a position to catheterize, an appendicovesicostomy or Mitrofanoff procedure may be performed. The Mitrofanoff procedure uses the appendix to create a stoma for catheterizing the bladder from the abdominal wall much as a vesicostomy, but without the leaking and odor that accompany it. The stoma location enables the parent and the child to access the bladder with less effort than reaching between the child’s legs. A 20-year follow-up study of 23 continent Mitrofanoff procedures/cystostomies showed that the procedure is safe and long-lasting (Liard, Seguier-Lipszyc, Mathiot, & Mitrofanoff, 2001). The Mitrofanoff procedure is well accepted by the children and their families, improves continence rates, rarely presents difficulties with the catheterizations, and simplifies the process of catheterizing the bladder (Churchill et al., 2001). If school-aged children or adolescents continue to have bowel incontinence in spite of compliance with medical recommendations or if there is a strong desire on the part of the child for increased independence, the surgeon may recommend a Malone procedure. The Malone Antegrade Colonic Enema procedure is a surgical opening that is created from a segment of colon and is externalized to the abdomen. This enables the child or caretaker to irrigate the colon in an antegrade fashion to completely remove fecal debris from the upper and lower colon segments. If the school-aged or young adolescent is able to perform problem-solving skills at or near the level of their peers, the Malone procedure enables the child to develop the skills to do their own Malone irrigations. After the procedure was performed on 20 children, the global self-esteem scores for the children on the Self-Perception Profile for
Adolescents were significantly improved (Aksnes et al., 2002). Some adolescents will have both a Mitrofanoff procedure and a Malone procedure done to optimize self-care and personal hygiene. These procedures do not eliminate the need for healthy eating, regular exercise, and scheduled bowel and bladder routines. The Malone and Mitrofanoff procedures merely facilitate easier access to the orifices. Children who have these procedures done should be comfortable with self-catheterizing and enemas before the procedures are performed because these will be life-long techniques required to maintain good kidney and bowel function.
ROLE OF THE PEDIATRIC NURSE PRACTITIONER Initiating the toileting process really begins with the inclusion of a balanced, varied diet. By following the recommended American Dietetic Association food pyramid and using a variety of tastes, textures, and manageable portions, children with spina bifida should be offered a high-fiber, high fluid, wellbalanced intake. Children are encouraged to drink milk as their major beverage, up to 24 ounces per day, with minimal amounts of juice, Kool-Aid, and soda. Children with a neurogenic bowel and bladder should be encouraged to drink beverages of at least 40 to 50 mL/kg of their body weight daily (Kleinman, 2004). To promote bowel elimination prior to toilet training age, young infants can be placed in their pumpkin seat with a blanket or cushion under their feet. This technique works well for infants with foot movement and kicking capabilities. Digital stimulation of the anus to initiate elimination may be necessary for many infants with low-level lesions. When the child attains the age of 2 or 3 years, a potty chair with arms can be purchased. The arms on the chair are necessary to assist the child in positioning and in bearing down. The potty chair can be placed next to the standard toilet and used when the child shows interest. The child’s feet should be flat on the floor, because this also enables bearing down. Sitting children on adult toilet seats may be frightening and uncomfortable because the seats are usually inappropriately sized and high off of the ground. Establishing a routine
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PH ORIGINAL ARTICLE C time for bowel elimination enhances bowel emptying. When children become constipated or impacted, they complain of stomachache and bloating, have a poor appetite, or have flatus. To relieve the initial fecal blockage, a series of cleansing enemas should be used for 3 to 4 days prior to initiating the use of any laxative. Three main types of laxatives can be used: the bulk-forming/hyperosmotics, the stimulants, and the stool softeners/lubricants. Dosages vary with weight and age and usually need to be titrated by the parent for optimal results (Taketomo, 2002). Any child with a neurogenic bowel will benefit from a bowel management program. Regular, daily rituals of eating three well-balanced meals, taking laxatives, and sitting on the toilet and performing their toileting at the same time of day facilitate the process of elimination. A good bowel program also is crucial to avoiding urinary tract infections (Churchill et al., 2001). Regular exercise, even if the exercise is shuffling their weight in their wheelchair, is advantageous in strengthening muscle tone and thus assisting with elimination (Dunn, 2000). Parents should be reminded that not all children are ready to toilet train at 2 years of age; however, role modeling and using positive reinforcement when interest is shown are great incentives for success. Children with learning disabilities, such as those that occur with spina bifida, learn best with demonstration and repetition. Toileting routines take time to establish, and every child is unique. Many children will not need to have an enema each day but may require one every second or third day, and most will need to take a laxative daily. Digital stimulation also may aid in beginning the bowel movement. Regular evacuation of the colon is necessary if optimal bowel continence is to be attained.
CONCLUSION NTDs are among the most common of the complex birth defects and account for 6000 to 11,000 births in the United States each year (Becske & Jallo, 2003). When children are born with an NTD such as myelomeningocele, the physiologic readiness for toilet training may never come. Approximately 95% of children with myelomeningocele have
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BOX Resources for children with neurogenic bowel and bladder http://www.afud.org American Foundation of Urologic Disease http://www.marchofdimes.com March of Dimes Birth Defects Foundation http://www.nafc.org National Association of Continence http://www.nichcy.org National Information Center for Children and Youth with Disabilities http://www.kidney.org National Kidney Foundation, Inc. http://www.simonfoundation.org The Simon Foundation for Continence [email protected] National Kidney and Urologic Disease Information Clearing House http://www.suna.org Society of Urologic Nurses and Associates http://www.sbaa.org Spina Bifida Association of America located in Washington, DC
W
ith the advent of
CIC, regular bowel routines, and new and innovative surgeries, the typical child with neurogenic bowel and bladder is able to become continent.
a neurogenic bladder and bowel. In the past, children born with myelomeningocele have struggled to achieve a satisfactory level of continence. With the advent of CIC, regular bowel routines, and new and innovative surgeries, the typical child with neurogenic bowel and bladder is able to become continent. In children with spina bifida, continence can be achieved for both bladder and bowel if adaptations are made to the routines. For nurses working with children who have spina bifida, an understanding of the physical limitations and the vast array of options available facili-
tates the child’s ability to become more typical and more acceptable to typical children. The goals of the health care team are to have a child with (a) bladder function that is free of kidney damage (b) bowel function that promotes regular, controlled elimination, and (c) the ability to wear underwear instead of diapers or Pull-ups. The Box provides resources for those seeking further information on achieving continence in children with neurogenic bowel and bladder dysfunction.
REFERENCES Aksnes, G., Diseth, T., Helseth, A., Edwin, B. Stange, M., Aafos, G., et al. (2002). Appendicostomy for antegrade enema: Effects on somatic and psychosocial functioning in children with myelomeningocele. Pediatrics, 109, 484-489. Association for Continence Advice. (2003). Clean intermittent catheterization. Nursing Times, 99, 44-47. Becske, T., & Jallo, G. (2003, July 17). Neural tube defects. Retrieved from http://www.emedicine. com/neuro/topic244.htm Bickley, L. S., & Szilagyi, P. G. (2003). Bates’ guide to physical examination (8th ed., pp. 144, 317-345, 373-376, 392-400, 506-517, 567-592, 594, 614, 628-630). Philadelphia: Lippincott, Williams & Wilkins. Bonney, S. (2002). Developmental difficulties. In J. A. Fox (Ed.), Primary health care of children and adolescents (pp. 869-876). St. Louis: Mosby. Capozza, N., & Caione, P. (2002). Dextranomer/ hyaluronic acid copolymer implantation for vesico-ureteral reflux: A randomized comparison with antibiotic prophylaxis. Journal of Pediatrics, 140, 230-234. Churchill, B. M., Abramson, R. P., & Wahl, E. F. (2001). Dysfunction of the lower urinary and distal gastrointestinal tracts in pediatric pa-
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PH ORIGINAL ARTICLE C tients with known spinal cord problems. Pediatric Clinics of North America, 48, 1587-1630. Clean Intermittent Catheterization. (2002, October 28). Retrieved from http://health.allrefer. com/health/clean-intermittent-self-catheterization_info.html Dixon, S. (2000). Four years: Clearer sense of self. In S. Dixon & M. Stein (Eds.), Encounters with children: Pediatric behavior and development (3rd ed., pp. 346-353). St. Louis: Mosby. Dunn, A. M. (2000). Elimination patterns. In C. E. Burns, M. A. Brady, A. M. Dunn, & N. BarberStarr (Eds.) Pediatric primary care: A handbook for nurse practitioners (2nd ed., pp. 327-31). Philadelphia: WB Saunders Co. Environmental Agents Service. (2003, September 11). Agent Orange. Retrieved from http:// www1.va.gov/agentorange/ Feng, W. C., & Churchill, B. M. (2001). Dysfunctional elimination syndrome without obvious spinal cord diseases. Pediatric Clinics of North America, 48, 1489-1504. Fox, J. A. (2002). Common parenting concerns. In J. A. Fox (Ed.), Primary health care of children and adolescents (pp. 266-267). St. Louis: Mosby. Kirsch, A. J., Perez-Brayfield, M. R., & Scherz, H. C. (2003). Minimally invasive treatment of vesicoureteral reflux with endoscopic injection of dextranomer/hyaluronic acid copolymer: The Children’s Hospitals of Atlanta experience.
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Journal of Urology, 170, 211-215. Kleinman, R. E. (Ed.). (2004). Pediatric nutrition handbook: American Academy of Pediatrics. (5th ed., pp. 629-642). Ill: AAP Press. Kolaski, K. (2002, May 30). Myelomeningocele. Retrieved from http://www.emedicine.com/ PMR/topic83.htm Liard, A., Seguier-Lipszyc, E., Mathiot, A., & Mitrofanoff, P. (2001). The Mitrofanoff procedure: 20 years later. The Journal of Urology, 165, 2394-2398. March of Dimes. (2003). Folic acid: Quick reference and fact sheets. Retrieved from http://www. marchofdimes.com/folicacid Morton, S. C., Shekelle, P. G., Adams, J. L., Bennett, C., Dobkin, B. H., Montgomeri, J., et al. (2002). Antimicrobial prophylaxis for urinary tract infections in persons with spinal cord dysfunction. Archives of Physical Medicine and Rehabilitation, 83, 129-138. Murphy, M. A. (2000). Developmental management of toddlers and preschoolers. In C. E. Burns, M. A. Brady, A. M. Dunn, & N. BarberStarr (Eds.). Pediatric primary care: A handbook for nurse practitioners (2nd ed., pp. 128-130). Philadelphia: WB Saunders Co. National Institutes of Health. (2002, June). Urodynamic testing. NIH publication No. 02-5106. Retrieved from http://kidney.niddk.nih.gov/ kudiseases/pubs/urodynamic
Nickel, R. E. (2000). Myelomeningocele and related neural tube defects. In R. E. Nickel & W. Desch (Eds.). The physician’s guide to caring for children with disabilities and chronic conditions (pp. 426-458). Baltimore: Paul H. Brookes Publishing. Schmitt, B. (2004a). Toilet training: Getting it right the first time. Contemporary Pediatrics, 21, 105108, 111-112, 115-116. Schmitt, B. (2004b). Toilet training problems: Underachievers, refusers, and stool holders. Conhtemporary Pediatrics, 21, 71-80. Spinal Cord Injury Information Network. (2001, April). Bladder care and management. Retrieved from http://www.spinalcord.uab.edu/ Stein, M. (2000). 15 to 18 months: Asserting independence and pushing the limits. In S. D. Dixon & M. T. Stein (Eds.). Encounters with children: Pediatric behavior and development (3rd ed., pp. 281-284). St. Louis: Mosby. Taketomo, C. K., Hodding, J. H., & Kraus, D. M. (2002). Pediatric dosage handbook (9th ed., pp. 211-212, 386-387, 524, 627, 673, 738, 811-812, 863, 878-879, 909-910, 923, 975, 992). Hudson, OH: Lexi-Comp, Inc. Volcik, K. A., Blanton, S. H., Kruzel, M. C., Townsend, I. T., Tyerman, G. H., Mier, R. J., et al. (2002). Testing for genetic associations with the PAX gene family in a spina bifida population. American Journal of Medical Genetics, 110,
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Achieving Continence in Children With Neurogenic Bowel and Bladder C a n d a c e F. Z i ck l e r, M S N , R N , C P N P, & Vi r g i n i a R i ch a r d s o n , D N S , R N , C P N P
I
ABSTRACT Bowel and bladder continence is a major developmental accomplishment for a child and his or her family. Children with neurogenic bowel and bladder dysfunction, which occurs with myelomeningocele and other neural tube defects, have a physical inability to attain continence. However, children with these limitations can attain continence when the appropriate modifications to the traditional routines are made. Enabling the child to attain continence improves parental relationships and self-concept. The nurse who is working with children with myelomeningocele in schools, clinics, private offices, and home health care settings can play a vital role in helping a child with a neural tube defect achieve continence. J Pediatr Health Care. (2004). 18, 276-283 .
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n children who undergo typical development, maturation has predictable patterns that are based on neurologic and physical growth (Stein, 2000). When children are born with medical conditions such as myelomeningocele (spina bifida), they have both sensory and motor impairments that affect their ability to achieve all of their developmental milestones. Toilet training is a significant developmental milestone in the life of a child. Complex learning such as mastery of bowel and bladder control often is challenging even for children who develop normally. Children are usually toilet trained by 21/2 to 4 years of age. Bowel control comes before bladder control, and daytime training is achieved before a child stays dry at night. Girls generally achieve dryness before boys, and for many children, complete continence is not achieved until the child reaches 5 to 6 years of age. When toddlers are ready to be continent they demonstrate readiness by coming to the parents, telling them that they are wet or dirty, and pulling off their diapers (Fox, 2002; Schmitt, 2004a). Continence is both a physiologically and psychologically based skill. Physical requirements for continence occur when the child can stand upright and the abdominal muscles are strong enough to demonstrate trunk control sufficient for walking, sitting, and standing. The child also must have the motor skills to be able to bend and sit and must be able to pull down his or her pants at the appropriate time. The psychological readiness occurs when the child has both the cognitive development and motivation to remain continent (Schmitt, 2004a). The child should be able to make
Candace F. Zickler is a Pediatric Nurse Practioner–Clinician, Team Clinics, Children’s Medical Center, Dayton, Ohio. Virginia Richardson is Assistant Dean for Student Affairs, Indiana University School of Nursing, School Based Clinic, Clarian Health Partners, Indianapolis, Ind. Reprint requests: Candace F. Zickler, MSN, RN, CPNP, 3372 Ridgeway Rd, Kettering, OH 45419-5014; email: [email protected]. 0891-5245/$30.00 Copyright © 2004 by the National Association of Pediatric Nurse Practitioners. doi:10.1016/j.pedhc.2004.05.005
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PH ORIGINAL ARTICLE C an association between the feelings elicited for voiding and bowel movements, ave a desire to learn the skill by demonstrating increasing periods of dryness during the daytime, be able to positively take the suggestion from the parent that they should sit on the toilet, and feel a sense of accomplishment when they are eliminating on the toilet (Stein, 2000). Frequently, mastery of continence is attained when the family is able to minimize other stressors and help the child focus on toileting. When a child continues to be incontinent in spite of the parents’ intense efforts at using their personal experiences, relatives’ suggestions, or professionals’ counseling, the parents become frustrated. The child’s continued incontinence results in stress between the parent and the child (Schmitt, 2004b; Stein, 2000). Children who do not achieve continence develop a poor self-image and have difficulties with peer and social interactions (Dixon, 2000). Just as normally developing children may resist toilet training, toddlers with spina bifida also may demonstrate resistance to mastery of this skill. Many nondisabled children have relapses after they have achieved dryness, especially at times of stress, but these relapses are only temporary (Schmitt, 2004b; Stein, 2000).
NEURAL TUBE ABNORMALITIES Neural tube defects (NTDs) are some of the most complex and disabling birth defects known (Kolaski, 2002). With the advances that have occurred in medicine and pediatrics and the multidisciplinary approach obtained by working with geneticists, neurosurgeons, and urology/reproductive and orthopedic surgeons, during the past 30 years the vast majority of children with spina bifida are living well into older adulthood. Although they should attend multidisciplinary clinics to receive subspecialty evaluations and care, children with spina bifida are seen for their routine well child and preventive health care needs in general pediatric and medical practices (Becske & Jallo, 2003; Nickel, 2000). Pediatric nurses are working with these children and helping them attain their age-appropriate developmental skills. At times the associated problems that accompany a diagnosis of spina bifida limit the child’s ability to master toilet
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training. In addition to the loss of bowel and bladder control and lower extremity function, children with spina bifida are at increased risk for hydrocephalus from an Arnold Chiari II malformation, fine motor and dexterity limitations, seizures, visual and perceptual problems, and cognitive deficits. Ten to twenty percent of children with spina bifida experience learning disabilities, especially with reasoning and remembering (Bonney, 2002; Nickel, 2000). NTDs have a multifactorial form of inheritance and account for the majority of congenital central nervous system disorders. The defects are the result of failure of the neural tube to close spontaneously at 3 to 4 weeks of gestation and result in partial or total paralysis below the level of the defect. The most common NTD compatible with life is myelomeningocele (spina bifida); its incidence is 1 in 1200 to 1400 live births. About 2500 babies are born with myelomeningocele each year (March of
A
t times the associated
problems that accompany
has also been implicated (Becske & Jallo, 2003). Parental exposure to Agent Orange, a herbicide used to defoliate the jungles during the Vietnam war, has been associated with NTDs. A fund has been established to help children born to parents who have been exposed to Agent Orange (Environmental Agents Service, 2003). In addition to the environmental and teratogenic relationships, there is ongoing research into the genetic links that may exist (Volcik et al., 2002). Folic acid, a B vitamin that acts as a co-factor for enzymatic processes involved with DNA and RNA synthesis, is recommended for all women of child-bearing age. Folate is important in the metabolism of homocysteine and is necessary for the synthesis of methionine. Methionine is needed in the metabolism of proteins, lipids, and myelin (Nickel, 2000). Furthermore, the inclusion of folic acid in a preconceptual and prenatal diet may be directly protective against neural tube defects in up to 70% of infants with the folate deficit. With insufficient quantities of folic acid, the genetic risks plus the environmental exposures are thought to result in approximately 50% of first-time neural tube defects and 70% of recurrent neural tube defects (March of Dimes, 2003).
a diagnosis of spina bifida
THE PHYSIOLOGIC PROCESS OF MICTURITION
limit the child’s ability to
The urinary system consists of the upper urinary tract and the lower urinary tract. The upper urinary tract includes the renal blood vessels, renal parenchyma, renal pelvises, the two ureters, and includes up to the uretero-vesical junction. The upper urinary tract system does not require innervations from the spinal cord to function normally. The lower urinary tract includes the uretero-vesical junction, the detursor muscle (bladder), the urethral control mechanism, the urethra, and the afferent and efferent nerves that are attached to the central nervous system (spinal cord). Normal lower urinary tract functioning consists of filling and storage of urine and emptying or voiding. The bladder neck and posterior urethra are controlled by the thoracic10 to lumbar-2 (T-10 to L-2) portions of the hypogastric nerve. In normally functioning urinary systems, somatic innervations of the external sphincter increase the tone at the pudendal nerve
master toilet training.
Dimes, 2003). Since the advent of prenatal diagnosis, elective abortions, and the increased prescribing of folic acid, the rate of babies born with NTD has decreased dramatically (Becske & Jallo, 2003; Kolaski, 2002). Although there is no definitive cause for myelomeningocele, a number of teratogens have been implicated as having a higher than normal incidence. Some of the teratogens include the season of conception, the geographic region, maternal diabetes, maternal age, maternal valprote use, maternal alcohol abuse, lower socioeconomic class, maternal zinc or folate deficiencies, and an episode of maternal hyperthermia such as a high fever or hot tub use. Maternal obesity is an independent factor that
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sacral-2 through sacral-4 (S-2 to S-4) and create a voluntary and efficient emptying of the bladder. The feeling of a need to urinate is triggered when the bladder fills to 300 to 500 mL (Churchill, Abramson, & Wahl, 2001). There are two types of neurogenic bladder dysfunction. Aspastic or hyperreflexive bladder is characterized by uncontrolled, frequent expulsion of urine and incomplete emptying. A spastic bladder is very rigid and stiff on voiding cystourethrogram. A flaccid or hypotonic bladder is weak and stretched out and can hold extremely large quantities of urine (as high as 2000 mL at a time) and can create a continuous dribbling from the urethra. Both types of dysfunction can result in reflux into the renal parenchyma, irreversible pyelonephritis with scarring of the renal tubules, and subsequent loss of renal function. The upper urinary tract is preserved in children and adults if urine is regularly and completely emptied from the bladder and the leak point pressures are kept below 40 cm H20 (Churchill et al., 2001). Management of neurogenic bladder should be of paramount concern to health care providers of children with spina bifida because renal failure continues to be the leading cause of death in patients after the first year of life (Kolaski, 2002).
below thoracic 12 (T-12) vertebrae. The T-12 lesions result in loss of sensation or urge and continuous oozing from the rectum. The children may have offensive odors and must wear diapers, which embarrasses them and makes them reluctant to socialize with peers. A child with reflexic bowel dysfunction or an upper motor neuron lesion has damage above thoracic 12 (T-12) vertebrae and the anal reflexes are intact, giving the child the ability to stimulate the rectum to prompt elimination (Feng & Churchill, 2001). However, because of the level of injury, the decreased mobility and poor fine motor ability, manual stimulation is generally limited. These children also will have to wear diapers because of incomplete emptying of the colon, flatus, and soiling.
THE PHYSIOLOGIC PROCESS OF BOWEL EVACUATION
defects are the distal
The major areas of the gastrointestinal tract that are affected by neural tube defects are the distal portions of the rectum, anus, and anal sphincter. The proximal gastrointestinal system is not directly affected by neural tube defects. The two primary involuntary reflexes that initiate defecation are the intrinsic and parasympathetic defecation reflexes, located at the sacral 2 to 4 level (S2 to S-4) of the spinal cord. The pudendal nerve is responsible for voluntary defecation and controls the opening and closing of the external anal spinchter. Because the sensation of a need to defecate often is absent in children with spina bifida, the fecal material is infrequently eliminated and constipation with subsequent fecal impaction results (Feng & Churchill, 2001). There are two types of neurogenic bowel dysfunction, areflexic and reflexic. If a child has an areflexic or lower motor neuron dysfunction, the lesion is
portions of the rectum,
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EXAMINATION OF THE CHILD History
T
he major areas of the
gastrointestinal tract that are affected by neural tube
anus, and anal sphincter.
The child’s ability to attain continence begins with an understanding of the families’ cultural expectations, especially the timing and “words” that the family uses to describe the process to the child. It is helpful to determine how the child will be treated if an “accident” occurs, so the parent appreciates the role that readiness and the child’s desire to please influences the outcome. Ask the parent if there is any family history of relatives or siblings with bowel or bladder dysfunction. If so, an assessment of signs and symptoms that may indicate an inherited condition and further data should be pursued. Eliciting the frequency of bowel movements and the timing that the child usually has
bowel movements helps establish a pattern from which to begin the bowel program teaching. Asking the parent about the size of the bowel movement and the degree of difficulty the child has with elimination can help with identifying constipation or diarrhea. (If the child has a long-standing history of constipation or diarrhea, it is advantageous to remedy this problem before moving on with the toilet training.) In normally developing young toddlers, voiding every 1 1/2 to 3 hours is common (Murphy, 2000). The frequency and volume of urine can be estimated from the child’s history of wet diapers. If the parent mentions that the child’s urine is cloudy or has a strong odor, the urine should be examined for bacteria. Any history of kidney or flank pain, dysuria, ureteral colic, or hesitancy should be thoroughly evaluated with an abdominal examination, a culture and urinalysis, and possibly a flat plate of the abdomen, looking for calcifications or stones. It is important to determine if the child is taking medications, such as anticholinergics or laxatives, that may influence elimination. Ask if there is any history of nausea, vomiting, anorexia, or hematemasis. Determine if the child has been taking the medications for a brief or extended period of time, and review the dosages and instructions that accompany the medication administration (Taketomo, Hodding, & Kraus, 2002). Reviewing the child’s meal times and the types of solids and fluids consumed at each meal is helpful in determining if sufficient fiber and fluid is present to facilitate normal bowel and bladder elimination. Many young children are picky eaters, and a diet history can help with counseling to optimize the quantity and quality of intake. Briefly review the child’s ability to eat normally by assessing such concerns as the child’s tolerance for thin and thick fluids, ability to masticate food properly, and ability to swallow without choking. If a history of frequent aspiration or pneumonia is present, a feeding study and a barium swallow are indicated (Bonney, 2002).
Physical Examination Spina bifida is a complex neuromotor and anatomic malformation that necessitates a thorough, systematic approach to the physical examination. Children
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PH ORIGINAL ARTICLE C should be examined while wearing gowns with all of their underwear removed. Using age-specific and developmentally appropriate examination skills facilitates comfort and cooperation from the child. The weight in kilograms, length and height in centimeters, the basal metabolic index, and head circumference are measured and plotted on the standard National Center for Health Statistics graph to ensure growth rates appropriate for age. Serial measurements plotted on the same growth grid assist in identifying abnormal trends of increasing or decreasing weight and increasing head circumference. Increasing head circumference can indicate hydrocephalus or shunt malfunction. Because some children with spina bifida are non-weight bearing, upper arm span measurements should be used to approximate the height (Kleinman, 2004). Routine monitoring of blood pressure and temperature can detect early renal and bladder dysfunction and need to be graphed and evaluated on standardized grids at each visit. Children who are developing typically will probably be 2 to 5 years of age when toilet training is initiated. Children who happen to have spina bifida also will be ready for toilet training at that age and will act and behave in a fashion similar to that of their age-related peers who are not disabled. Observation of the child’s interaction with the parent and active listening to the conversation facilitates a determination of the developmental level of the child. The use of a Denver Developmenatal Screening Test can assist with determining the gross motor, fine motor, personal, social, and communication skills of the child. If formal developmental evaluations have been performed in an early intervention program, review the reports for language age, comprehension, and problem-solving level to validate your assessment of the child’s functional level. The child’s head and neck should be examined and palpated for a cerebral spinal fluid shunt and tubing, which is present in 90% of children with myelomeningocele (Nickel, 2000). Do not pump the shunt, because doing so can cause headache and discomfort for the child. Vision and hearing should be evaluated during the visit, but formal testing of sight and hearing identifies
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deficits that may interfere with learning. The cranial nerves are generally intact but the examiner may note a child with a torticollis, facial weakness, or nystagmus. Any abnormalities should be more thoroughly evaluated and appropriate referrals made (Bickley & Szilagyi, 2003). Assessment of the abdomen should include evaluation of the skin for scars, rashes, or lesions, examination of the shape, contour, and symmetry of the surface, and examination of the umbilicus for location and any signs of hernia or inflammation. There may be a small scar where the cerebral spinal fluid shunt was routed through the abdomen. Auscultation, percussion, and deep and light palpation are performed over the abdominal surface to detect the presence of a full bladder or a fecal mass in the lower quadrants. Palpation of the lateral flanks for the size and lo-
D
etermining if an
umbilical reflex is present or absent can assist in locating the level of the neurologic loss.
cation of the kidneys bilaterally may detect pain or tenderness. Determining if an umbilical reflex is present or absent can assist in locating the level of the neurologic loss. Because sensation often is decreased or absent below the level of the spina bifida repair, examining, smelling, and palpating the entire skin surface is essential. If dampness or excoriation is noted, aggressive therapy to relieve the moisture and the pressure from the area is indicated (Bickley & Szilagyi, 2003). Following the abdominal examination, help the child roll onto his or her side and explain that you are going to examine their rectum. The back and buttocks should be examined for any dimples, scarring, or fistulas. If the child is cooperative and is able to bear down, ask him or her to do so as you in-
sert a gloved, lubricated finger into the anus. If the examiner can feel the anal spinchter tighten around the finger, increased or normal tone is present. If the child has not had a bowel movement recently, fecal matter also may be palpated. In children without neurologic loss of the anal reflex, the “anal wink” can be elicited by using a dull object and stroking the edges of the anus (Bickley & Szilagyi, 2003). The presence of the anal reflex indicates that nerves are intact that will aid with bowel elimination. Above the level of the repaired lesion, the child may have few neurologic abnormalities. Many children with spina bifida have good strength and tone in their shoulders, arms, and hands. Lower motor neuron lesions result in loss of muscle tone, absent or decreased deep tendon reflexes, hypotonia, and thin wasted extremities (Bickley & Szilagyi, 2003). The lower extremities will have varying degrees of muscle wasting (hypotonia). Performing passive, gentle range of motion and assessments of both deep and light sensation of the lower extremities locates areas where the child may have some sensation and/or motor control. Upper motor neuron lesions anywhere along the spinal cord result in spastic muscle tone, with passive resistance on range of motion movements. If the child is able to ambulate, the gait and ability to stand independently should be evaluated. The presence of scars on the hips, thighs, and feet may indicate previous orthopedic surgeries. Children who ambulate should be evaluated by an orthopedist to optimize their ability to stand, walk, run, and play. Some children with spina bifida may have dislocated hips and contractures. The motor level is a good predictor of functional status (Nickel, 2000). Popliteal and pedal pulses should be assessed and located in both legs. In assessing the genitourinary and reproductive systems, inspect the skin and note any excoriation. Determine if the urethral orifice is located appropriately for the sex of the child. Palpate for the presence of femoral pulses. In male patients, it is important to inspect the penis for any inflammation and the presence of a foreskin, for the location of the urethral meatus, the descent and size of the testicles in the scrotum, and the presence of any inguinal hernias. In
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PH ORIGINAL ARTICLE C female patients, a careful examination of the external genitalia may identify a prolapsed urethra, cystocele, or a varicocele. Associated anomalies frequently can be present.
Diagnostic Tests for Assessing Kidney and Bladder Function The level of the spinal lesion correlates poorly with urodynamic and radiologic studies, necessitating regular systematic evaluation of bladder function. Normal residual urine volume, after voiding, is less than 5 mL at all ages. Normal bladder storage pressure should remain at less than 20 cm H20 throughout life. These standard results can be utilized in evaluating both children and adults with bladder dysfunction (Churchill et al., 2001). A number of diagnostic tests should be done twice yearly to assess the integrity of the urinary tract system. Routine urinalysis with culture and sensitivities are indicated for a person of any age with a neurogenic bladder. Serum blood urea nitrogen and the creatinine level help determine renal clearance and kidney function (Churchill et al., 2001; Nickel, 2000). Radiologic examinations that evaluate bladder dysfunction and incontinence are performed annually or more frequently if indicated. Abdominal films of the abdomen can be used to examine the kidneys, ureters, and bladder and assess for the presence of calculi. A voiding cystourethrogram is done to visualize the integrity of the entire urinary collecting system. A cystoscopy also is recommended for any person with frequent urinary tract infections (Churchill et al., 2001). An ultrasound of the kidneys is used to evaluate the upper urinary tract and determine if obstruction, scarring, masses, or calculi are present. Periodically urodynamics will be done to evaluate filling, storage, bladder capacity, bladder pressures, detursor muscle function, leak points, bladder compliance, and the ability to voluntarily void. The urodynamics are used as a gauge of stable/deteriorating bladder or renal status (National Institutes of Health, 2002).
TECHNIQUES IN PRESERVING KIDNEY FUNCTION Children with neurogenic bladders are at life-long risk for kidney and bladder infections, incomplete bladder empty-
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ing, vesicoureteral reflux, poor kidney growth, renal cortex damage from reflux, and urinary incontinence (Churchill et al., 2001; Kolaski, 2002). Without interventions and regular evaluations, more than 90% of infants and children with a myelomeningocele defect will demonstrate symptoms and urodynamic changes of renal deterioration and/or bladder dysfunction (Churchill et al., 2001). When diagnostic tests and the child’s symptoms indicate a deteriorating condition, clean intermittent catheterization (CIC) will be started. Sometimes CIC will be initiated in the newborn nursery. The infant with urinary reflux at birth benefits from early initiation of CIC. If the newborn does not demonstrate reflux, delaying the initiation of CIC until there is noticeable change in
T
he level of the spinal
lesion correlates poorly with urodynamic and radiologic studies, necessitating regular systematic evaluation of bladder function.
the renal function is not harmful and in fact is easier for the parents to accept, given the overwhelming stress encountered during the newborn period. Depending on the level of the spina bifida and the degree of involvement to the spinal cord, some children will never need to be catheterized (Churchill et al., 2001). Many children can and do learn to do their own CIC, with supervision, by the time they are 6 to 8 years of age (Spinal Cord Injury Information Network, 2001). When parents are told that they will need to learn bladder catheterization, they fear hurting the child or introducing infection. With proper teaching and practice, good hand washing, and cleansing of the genitals, the risks are
minimized (Clean Intermittent Catheterization, 2002). Teaching videos are available through catheter vendors such as Astra (http://www.astratechusa. com) and Mentor Corporation (http:// www.mentorcorp.com/bladdercontrol/index. htm). The videos enable parents to view the procedure before they are taught to perform it on their child. The parents need to be instructed how to perform a sterile catheterization on their child because the procedure is initially frightening and stressful for them. Prior to being sent home to do CIC, the parent is given prescriptions for catheters and lubricant, written instructions, and a contact phone number. Follow-up after the instruction is often done by phone, but occasionally the parent may need to return for more hands-on practice (Association for Continence Advice, 2003). Persons who do CIC or perform it for the infant or child should be told that over time bacterial “accumulation” occurs in the urine. These “resident” organisms do not cause renal damage unless there is significant reflux and are considered normal flora for the child (Morton et al., 2002). The most frequently isolated organisms found in the urine of individuals performing CIC are Escherichia coli, Klebsiella, and Proteus. If on routine culture and sensitivity analysis of the urine the rates of bacteria exceed 100,000 organisms per field or the organisms are considered pathologic, such as Enterobacter, Pseudomonas, Serratia or Candida, then a urinary tract infection exists and must be treated. To evaluate the urine of a patient who uses CIC, it is recommended that a sterile catheterized specimen be obtained. All catheterizations should be carried out with a latex free, lubricated catheter to ensure that latex sensitivity does not occur. Antibiotic prophylaxis has been shown to significantly reduce bactiuria in acutely injured spinal cord patients, and there is some reduction in bactiuria in nonacute, chronic spinal cord patients. However, antibiotic prophylaxis results in a two-fold increase in resistant organisms and should be avoided as a treatment modality, especially in persons who are at increased risk of urinary tract infections (Morton et al., 2002).
MEDICATIONS THAT HELP WITH NEUROGENIC BLADDER In children who have stiff, noncompli-
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PH ORIGINAL ARTICLE C ant bladders, antispasmodic medications such as oxybutynin (Ditropan, Ditropan XL) or propantheline (ProBanthine, Propanthel) work directly on the bladder to relax the muscle and inhibit the action of acetylcholine. These medications increase the capacity of the bladder, thus decreasing potential reflux. Both medications come in tablet form. The oxybutynin comes in both a long-acting and a syrup form and is approved by the Food and Drug Administration for use in children 1 year of age and older (Taketomo et al., 2002).
SURGICAL PROCEDURES FOR IMPROVING CONTINENCE There are surgical procedures that can be performed to preserve renal function by decreasing vesicoureteral reflux. The traditional method of ureteral implantation moves the ureters higher up on the bladder wall, thus decreasing the potential for reflux. Recently, an endoscopic technique of injecting a polysaccharide compound into the mucous membrane of the ureter has been utilized. The “bulge” that is created in the ureter acts as a one-way opening for the urine to exit the kidney and prevents urinary reflux. The endoscopic procedure is approved by the Food and Drug Administration for vesicoureteral reflux grades II to IV. There have been no long-term safety or efficacy concerns for the procedure, and 70% to 80% of such procedures have a high long-term success rate (Capozza & Caione, 2002; Kirsch, Perez-Brayfield, & Scherz, 2003). Vesicostomies and bladder augmentations can be performed if the bladder wall is so noncompliant that little urine is able to be stored within it. The vesicostomy is a surgical opening on the abdomen that drains urine continuously. This necessitates that the child wear diapers to prevent soiling of clothes. Bladder augmentation utilizes tissue from the stomach, the intestine, or the ureter to “enlarge or expand” the size of the bladder. When the bladder augmentation is performed, it tends to lower the pressure by increasing the capacity of the bladder (Association for Continence Advice, 2003; Churchill et al., 2001). Auretheral sling or bladder neck suspension procedure can be done to decrease urinary dribbling. The surgically created “sling” lifts up the bladder, thus exerting pressure on the urethra to in-
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crease urine retention. In rare instances, a cuff is surgically placed around the urethra or bladder neck to help prevent urinary leakage. The cuffs enable the urethra to retain urine until voiding is desired. The cuff is part of a balloon reservoir that has been placed in the perineum and is squeezed by the child at the time of desired voiding (Churchill et al., 2001). When children become old enough to manage their own catheterizations and desire independence, if they have poor manual dexterity or if their caretakers have difficulty getting the child in a position to catheterize, an appendicovesicostomy or Mitrofanoff procedure may be performed. The Mitrofanoff procedure uses the appendix to create a stoma for catheterizing the bladder from the abdominal wall much as a vesicostomy, but without the leaking and odor that accompany it. The stoma location enables the parent and the child to access the bladder with less effort than reaching between the child’s legs. A 20-year follow-up study of 23 continent Mitrofanoff procedures/cystostomies showed that the procedure is safe and long-lasting (Liard, Seguier-Lipszyc, Mathiot, & Mitrofanoff, 2001). The Mitrofanoff procedure is well accepted by the children and their families, improves continence rates, rarely presents difficulties with the catheterizations, and simplifies the process of catheterizing the bladder (Churchill et al., 2001). If school-aged children or adolescents continue to have bowel incontinence in spite of compliance with medical recommendations or if there is a strong desire on the part of the child for increased independence, the surgeon may recommend a Malone procedure. The Malone Antegrade Colonic Enema procedure is a surgical opening that is created from a segment of colon and is externalized to the abdomen. This enables the child or caretaker to irrigate the colon in an antegrade fashion to completely remove fecal debris from the upper and lower colon segments. If the school-aged or young adolescent is able to perform problem-solving skills at or near the level of their peers, the Malone procedure enables the child to develop the skills to do their own Malone irrigations. After the procedure was performed on 20 children, the global self-esteem scores for the children on the Self-Perception Profile for
Adolescents were significantly improved (Aksnes et al., 2002). Some adolescents will have both a Mitrofanoff procedure and a Malone procedure done to optimize self-care and personal hygiene. These procedures do not eliminate the need for healthy eating, regular exercise, and scheduled bowel and bladder routines. The Malone and Mitrofanoff procedures merely facilitate easier access to the orifices. Children who have these procedures done should be comfortable with self-catheterizing and enemas before the procedures are performed because these will be life-long techniques required to maintain good kidney and bowel function.
ROLE OF THE PEDIATRIC NURSE PRACTITIONER Initiating the toileting process really begins with the inclusion of a balanced, varied diet. By following the recommended American Dietetic Association food pyramid and using a variety of tastes, textures, and manageable portions, children with spina bifida should be offered a high-fiber, high fluid, wellbalanced intake. Children are encouraged to drink milk as their major beverage, up to 24 ounces per day, with minimal amounts of juice, Kool-Aid, and soda. Children with a neurogenic bowel and bladder should be encouraged to drink beverages of at least 40 to 50 mL/kg of their body weight daily (Kleinman, 2004). To promote bowel elimination prior to toilet training age, young infants can be placed in their pumpkin seat with a blanket or cushion under their feet. This technique works well for infants with foot movement and kicking capabilities. Digital stimulation of the anus to initiate elimination may be necessary for many infants with low-level lesions. When the child attains the age of 2 or 3 years, a potty chair with arms can be purchased. The arms on the chair are necessary to assist the child in positioning and in bearing down. The potty chair can be placed next to the standard toilet and used when the child shows interest. The child’s feet should be flat on the floor, because this also enables bearing down. Sitting children on adult toilet seats may be frightening and uncomfortable because the seats are usually inappropriately sized and high off of the ground. Establishing a routine
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PH ORIGINAL ARTICLE C time for bowel elimination enhances bowel emptying. When children become constipated or impacted, they complain of stomachache and bloating, have a poor appetite, or have flatus. To relieve the initial fecal blockage, a series of cleansing enemas should be used for 3 to 4 days prior to initiating the use of any laxative. Three main types of laxatives can be used: the bulk-forming/hyperosmotics, the stimulants, and the stool softeners/lubricants. Dosages vary with weight and age and usually need to be titrated by the parent for optimal results (Taketomo, 2002). Any child with a neurogenic bowel will benefit from a bowel management program. Regular, daily rituals of eating three well-balanced meals, taking laxatives, and sitting on the toilet and performing their toileting at the same time of day facilitate the process of elimination. A good bowel program also is crucial to avoiding urinary tract infections (Churchill et al., 2001). Regular exercise, even if the exercise is shuffling their weight in their wheelchair, is advantageous in strengthening muscle tone and thus assisting with elimination (Dunn, 2000). Parents should be reminded that not all children are ready to toilet train at 2 years of age; however, role modeling and using positive reinforcement when interest is shown are great incentives for success. Children with learning disabilities, such as those that occur with spina bifida, learn best with demonstration and repetition. Toileting routines take time to establish, and every child is unique. Many children will not need to have an enema each day but may require one every second or third day, and most will need to take a laxative daily. Digital stimulation also may aid in beginning the bowel movement. Regular evacuation of the colon is necessary if optimal bowel continence is to be attained.
CONCLUSION NTDs are among the most common of the complex birth defects and account for 6000 to 11,000 births in the United States each year (Becske & Jallo, 2003). When children are born with an NTD such as myelomeningocele, the physiologic readiness for toilet training may never come. Approximately 95% of children with myelomeningocele have
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BOX Resources for children with neurogenic bowel and bladder http://www.afud.org American Foundation of Urologic Disease http://www.marchofdimes.com March of Dimes Birth Defects Foundation http://www.nafc.org National Association of Continence http://www.nichcy.org National Information Center for Children and Youth with Disabilities http://www.kidney.org National Kidney Foundation, Inc. http://www.simonfoundation.org The Simon Foundation for Continence [email protected] National Kidney and Urologic Disease Information Clearing House http://www.suna.org Society of Urologic Nurses and Associates http://www.sbaa.org Spina Bifida Association of America located in Washington, DC
W
ith the advent of
CIC, regular bowel routines, and new and innovative surgeries, the typical child with neurogenic bowel and bladder is able to become continent.
a neurogenic bladder and bowel. In the past, children born with myelomeningocele have struggled to achieve a satisfactory level of continence. With the advent of CIC, regular bowel routines, and new and innovative surgeries, the typical child with neurogenic bowel and bladder is able to become continent. In children with spina bifida, continence can be achieved for both bladder and bowel if adaptations are made to the routines. For nurses working with children who have spina bifida, an understanding of the physical limitations and the vast array of options available facili-
tates the child’s ability to become more typical and more acceptable to typical children. The goals of the health care team are to have a child with (a) bladder function that is free of kidney damage (b) bowel function that promotes regular, controlled elimination, and (c) the ability to wear underwear instead of diapers or Pull-ups. The Box provides resources for those seeking further information on achieving continence in children with neurogenic bowel and bladder dysfunction.
REFERENCES Aksnes, G., Diseth, T., Helseth, A., Edwin, B. Stange, M., Aafos, G., et al. (2002). Appendicostomy for antegrade enema: Effects on somatic and psychosocial functioning in children with myelomeningocele. Pediatrics, 109, 484-489. Association for Continence Advice. (2003). Clean intermittent catheterization. Nursing Times, 99, 44-47. Becske, T., & Jallo, G. (2003, July 17). Neural tube defects. Retrieved from http://www.emedicine. com/neuro/topic244.htm Bickley, L. S., & Szilagyi, P. G. (2003). Bates’ guide to physical examination (8th ed., pp. 144, 317-345, 373-376, 392-400, 506-517, 567-592, 594, 614, 628-630). Philadelphia: Lippincott, Williams & Wilkins. Bonney, S. (2002). Developmental difficulties. In J. A. Fox (Ed.), Primary health care of children and adolescents (pp. 869-876). St. Louis: Mosby. Capozza, N., & Caione, P. (2002). Dextranomer/ hyaluronic acid copolymer implantation for vesico-ureteral reflux: A randomized comparison with antibiotic prophylaxis. Journal of Pediatrics, 140, 230-234. Churchill, B. M., Abramson, R. P., & Wahl, E. F. (2001). Dysfunction of the lower urinary and distal gastrointestinal tracts in pediatric pa-
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PH ORIGINAL ARTICLE C tients with known spinal cord problems. Pediatric Clinics of North America, 48, 1587-1630. Clean Intermittent Catheterization. (2002, October 28). Retrieved from http://health.allrefer. com/health/clean-intermittent-self-catheterization_info.html Dixon, S. (2000). Four years: Clearer sense of self. In S. Dixon & M. Stein (Eds.), Encounters with children: Pediatric behavior and development (3rd ed., pp. 346-353). St. Louis: Mosby. Dunn, A. M. (2000). Elimination patterns. In C. E. Burns, M. A. Brady, A. M. Dunn, & N. BarberStarr (Eds.) Pediatric primary care: A handbook for nurse practitioners (2nd ed., pp. 327-31). Philadelphia: WB Saunders Co. Environmental Agents Service. (2003, September 11). Agent Orange. Retrieved from http:// www1.va.gov/agentorange/ Feng, W. C., & Churchill, B. M. (2001). Dysfunctional elimination syndrome without obvious spinal cord diseases. Pediatric Clinics of North America, 48, 1489-1504. Fox, J. A. (2002). Common parenting concerns. In J. A. Fox (Ed.), Primary health care of children and adolescents (pp. 266-267). St. Louis: Mosby. Kirsch, A. J., Perez-Brayfield, M. R., & Scherz, H. C. (2003). Minimally invasive treatment of vesicoureteral reflux with endoscopic injection of dextranomer/hyaluronic acid copolymer: The Children’s Hospitals of Atlanta experience.
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Journal of Urology, 170, 211-215. Kleinman, R. E. (Ed.). (2004). Pediatric nutrition handbook: American Academy of Pediatrics. (5th ed., pp. 629-642). Ill: AAP Press. Kolaski, K. (2002, May 30). Myelomeningocele. Retrieved from http://www.emedicine.com/ PMR/topic83.htm Liard, A., Seguier-Lipszyc, E., Mathiot, A., & Mitrofanoff, P. (2001). The Mitrofanoff procedure: 20 years later. The Journal of Urology, 165, 2394-2398. March of Dimes. (2003). Folic acid: Quick reference and fact sheets. Retrieved from http://www. marchofdimes.com/folicacid Morton, S. C., Shekelle, P. G., Adams, J. L., Bennett, C., Dobkin, B. H., Montgomeri, J., et al. (2002). Antimicrobial prophylaxis for urinary tract infections in persons with spinal cord dysfunction. Archives of Physical Medicine and Rehabilitation, 83, 129-138. Murphy, M. A. (2000). Developmental management of toddlers and preschoolers. In C. E. Burns, M. A. Brady, A. M. Dunn, & N. BarberStarr (Eds.). Pediatric primary care: A handbook for nurse practitioners (2nd ed., pp. 128-130). Philadelphia: WB Saunders Co. National Institutes of Health. (2002, June). Urodynamic testing. NIH publication No. 02-5106. Retrieved from http://kidney.niddk.nih.gov/ kudiseases/pubs/urodynamic
Nickel, R. E. (2000). Myelomeningocele and related neural tube defects. In R. E. Nickel & W. Desch (Eds.). The physician’s guide to caring for children with disabilities and chronic conditions (pp. 426-458). Baltimore: Paul H. Brookes Publishing. Schmitt, B. (2004a). Toilet training: Getting it right the first time. Contemporary Pediatrics, 21, 105108, 111-112, 115-116. Schmitt, B. (2004b). Toilet training problems: Underachievers, refusers, and stool holders. Conhtemporary Pediatrics, 21, 71-80. Spinal Cord Injury Information Network. (2001, April). Bladder care and management. Retrieved from http://www.spinalcord.uab.edu/ Stein, M. (2000). 15 to 18 months: Asserting independence and pushing the limits. In S. D. Dixon & M. T. Stein (Eds.). Encounters with children: Pediatric behavior and development (3rd ed., pp. 281-284). St. Louis: Mosby. Taketomo, C. K., Hodding, J. H., & Kraus, D. M. (2002). Pediatric dosage handbook (9th ed., pp. 211-212, 386-387, 524, 627, 673, 738, 811-812, 863, 878-879, 909-910, 923, 975, 992). Hudson, OH: Lexi-Comp, Inc. Volcik, K. A., Blanton, S. H., Kruzel, M. C., Townsend, I. T., Tyerman, G. H., Mier, R. J., et al. (2002). Testing for genetic associations with the PAX gene family in a spina bifida population. American Journal of Medical Genetics, 110,
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