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Use of directed history and behavioral indicators in the assessment of the child with a developmental disability
ORIGINAL ARTICLE
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Use of Directed History and Behavioral Indicators in the Assessment of the Child With a Developmental Disability
ABSTRACT It can be very difficult to get a complete history and review of systems for children with developmental disabilities and poor communication skills. In addition, many children with developmental disabilities may engage in self-injurious or aggressive behavior. Although the causes of inappropriate behavior are frequently environmental, physiologic components may exist as well, particularly pain or discomfort. History taking must be focused and specific and may need to focus on the child’s behavioral patterns, because the child may not have sufficient communication skills to describe his or her problem and parents or guardians may not realize the relevance of certain behaviors. Gastrointestinal problems in particular may be a source of discomfort and should be reviewed with particular care. Referral to a psychologist who is able to perform a functional analysis of behavior may be necessary to treat problem behavior, especially if medical causes have been ruled out. J Pediatr Health Care. (2002). 16, 170-179.
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Jo n i Ja c o b s e n B o s ch , P h D, A R N P
A
ssessing a preverbal child for certain medical problems can be a diagnostic challenge because the child cannot describe symptoms, and the nurse practitioner (NP) must rely on the history provided by the child’s parents or guardians. The challenge is somewhat simplified by the comparatively limited differential diagnoses that apply to younger pediatric patients. The challenge grows considerably when the NP is faced with an older child, adolescent, or young adult whose communication skills are extremely limited by mental retardation. In addition to mental retardation, many persons in this group have comorbidities such as cerebral palsy, visual and hearing loss, and seizure disorders, and thus the differential diagnosis widens considerably. The NP must be prepared to ask detailed and probing questions to guide the evaluation of the older child, adolescent, or young adult who has no formal communication skills. Taking a history is a standard curricular component for NPs and other clinicians; therefore, this discussion will focus on strategies that may help in the assessment of persons with limited formal communication.
Joni Jacobsen Bosch, PhD, ARNP, is a Pediatric Nurse Practitioner and Family Nurse Practitioner at the Center for Disabilities and Development in Iowa City, Iowa. Reprint requests: Joni Jacobsen Bosch, PhD, ARNP, University of Iowa Center for Disabilities and Development, 100 Hawkins Dr, Iowa City, IA 52240; e-mail: [email protected]. Copyright © 2002 by the National Association of Pediatric Nurse Practitioners. 0891-5245/2002/$35.00 + 0
25/1/120393
doi:10.1067/mph.2002.120393
July/August 2002
PH ORIGINAL ARTICLE C HISTORY An accurate history is usually the single most important part of the assessment. Obtaining an accurate assessment may be complicated by the need to rely on persons who do not know the patient well, such as out-of-home care providers or family members who no longer live with the child or do not see him or her on a daily basis. It also may be complicated by care providers, including parents, who fail to volunteer essential information because they do not realize its value.
Behavior Self-injurious and aggressive behaviors are not uncommon among persons with mental retardation (Rincover, 1986). The incidence of these behaviors increases with decreasing cognitive and communicative abilities. Rates of self-injury and aggression are also higher among persons in out-of-home placements, such as group homes or intermediate care facilities/mental retardation. The causes for self-injurious and aggressive behavior are many and may be multifactorial as well. Assessment of self-injury and aggression is important because self-injury by definition causes tissue damage. Behavioral problems also may provide clues about the child’s physiologic status and assist the NP in ruling in or ruling out physiologic versus environmental functions of inappropriate behavior. Some self-injurious behavior has genetic causes, such as Lesch-Nyhan syndrome (Nyhan, 1976) and Rett syndrome (Perry, 1991). Hormonal changes and neurotransmitter imbalances have been implicated in behavioral abnormalities (Schroeder & Tessel, 1994; Taylor, Rush, Hetrick & Sandman, 1993; Winchel & Stanley, 1991). A strong environmental component may exist as well. Behavioral psychologists have identified environmental conditions, or functions, that may maintain inappropriate behavior (Iwata et al., 1994). When the function of the inappropriate behavior is identified, an individual behavior management plan can be implemented to address the inappropriate behavior. If self-injurious and aggressive behavior results from an attempt to communicate pain or discomfort, treating the underlying cause may reduce the inappropriate behavior to a considerable degree.
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First, the NP needs detailed information about the behavior. What type of behavior is exhibited? Is it aggression? Is it directed toward children, adults, or something else? Is there self-injury? What is the topography or type of the self-injury—for example, face slapping, head banging, hand biting, or body slamming? What is the setting in which the inappropriate behavior occurs? This information is important, but it must be remembered that any given inappropriate behavior may have many functions. Children may tug on an ear because they have otitis or because they enjoy tugging on their ear occasionally. Likewise, a child may bang his head because of a headache or to escape an unpleasant task demand. The fact of head banging tells us little about the cause of the behavior. More important are data concerning the antecedents, behavior, and consequences (also known as the ABCs) of the behavior. Observers may or may not be aware of the ABCs of a given behavior. They may report that nothing triggers the behavior. However, a careful observer may have noted that
I
f self-injurious and
aggressive behavior results from an attempt to communicate pain or discomfort, treating the underlying cause may reduce the inappropriate behavior to a considerable degree.
the child was not receiving attention before the behavior occurred and did receive attention after the behavior occurred. Attention itself can be a very powerful behavioral reinforcer for inappropriate behavior (Iwata et al., 1994).
BOX Resources • Local University Centers for Excellence in Developmental Disabilities Education, Research, and Service/University-affiliated Programs: http://www.aauap.org/data/ directory/directory.cfm • Links to various disability related sites: http://www.ninds.nih.gov/health_ and_medical/disorder_index.htm http://www.disability.gov/CSS/ • Parker, S., & Zuckerman, B. (1995). Behavioral and developmental pediatrics: A handbook for primary care. Boston: Little, Brown and Company. • The membership page of the Association for Behavioral Analysis may be searched by name or state: http://www.wmich.edu/cgi-bin/ aba/db.cgi?
The ABCs can give you some clues as to differential diagnoses as well. If inappropriate behavior occurs during high allergen times of the year, environmental allergies may need to be considered as a cause. Cyclical behavior problems in young women occurring on a monthly basis may represent menstrual cycle discomfort (Taylor et al., 1993). Inappropriate behavior just prior to meals may be an indicator of hunger. Likewise, dysphagia, esophagitis, gastritis, or enteric ulcers may lead to behavior problems associated with mealtimes (Bosch, VanDyke, Smith, & Poulton, 1997). Behavior that worsens when lying down, after meals, or at night may be indicative of gastroesophageal reflux associated with painful esophagitis (Sondheimer, 1988). Dysuria resulting from a urinary tract infection (UTI) as well as constipation may cause behavior problems associated with toileting. A good history regarding the ABCs of behavior may help focus a differential diagnosis toward a body system or toward a behavioral diagnosis. Correct identification of the cause is essential for the development and implementation of an appropriate treatment plan. Some questions that may help include the following:
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FIGURE Algorithm for assessment. • Is the behavior worse if the child is being ignored or during busy/noisy times of the day? • Is the behavior worse when the child is asked to complete a task? • Does worsened behavior lead to attention? (What’s wrong? Don’t do that!) • Does worsened behavior lead to an escape from a demand situation (time out)? • Does worsened behavior lead to access to preferred objects or activities?
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Gastrointestinal One study (Bosch et al., 1997) found that 7 of 25 patients being treated for behavioral problems had previously unidentified but significant medical problems that could be expected to cause discomfort or pain. Six of those seven patients had new diagnoses related to the gastrointestinal tract; therefore, the gastrointestinal tract is an important place to initiate a more focused medical history. Neuromotor problems, such as cerebral palsy, can affect the
gastrointestinal tract as well as other body systems. For example, neuromotor problems may interfere with the ability to chew, form a bolus, or correctly and safely swallow, leading to a diagnosis of dysphagia (Eicher, 1997; Tuchman 1989). Likewise, the incidence of gastroesophageal reflux can be as high as 75% in children with central nervous system disease (Tuchman, 1989). Questions related to swallowing difficulties should be detailed and include the following areas (Eicher, 1997): • Neuromotor problems • Recurrent pneumonia/bronchitis/sinusitis • Coughing/choking/gagging with meals • Preference for certain textures, especially soft or puree • Refusal of food • Prolonged feeding times (more than 30 minutes) • Underweight • Congestion following meals • Arching back/flexing neck with feedings • Worsened behavior at mealtimes • Multiple swallows required • Drooling or loss of food • Pocketing of food in mouth or cheeks • Scoliosis or poor positioning Nasopharyngeal reflux may be associated with frequent sinusitis or nasal infections. Persons with significant dysphagia may have an absent or poor gag reflex or a hyperactive gag (Tuchman, 1989). These persons may be noted to flex or extend their head during meals. This flexion or extension may be a manifestation of neuromotor instability and increase the risk of aspiration, or it may be an attempt to facilitate swallow in a person with poor oral phase skills. Frequent bouts of pneumonia or bronchitis also may reflect recurrent aspiration. The NP may actually need to watch a feeding, because gross aspiration that is readily apparent to those who have experience with persons with disabilities may be considered to be just a quirk of behavior to those with less experience. A referral to a speech/ language pathologist or occupational therapist may be necessary for a complete assessment of swallow. Gastroesophageal reflux with subsequent esophagitis is another common finding. Persons with neuromuscular problems, such as cerebral palsy, Down
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PH ORIGINAL ARTICLE C syndrome, and other sources of developmental delay are at higher risk for persistent reflux (Herbst, 1996; Tuchman, 1989). Questions to ask include the following: • Does worsened behavior occur around mealtimes or at night/when lying down? • Is there effortless vomiting/gagging/ rumination/food on the pillow in the morning? • Is coffee-ground emesis or melena present? • Does handmouthing (ie, frequently putting hands, fingers, or the forearm into the mouth) occur? • Does the patient have halitosis? • Does the patient have a chronic cough/cough toward end of feeding/bronchitis/pneumonia? • Does the patient have frequent nocturnal arousals? • Does the patient reside in a residential facility? Do others at the facility have a history of Helicobacter pylori? • Does the child have Sandifer’s syndrome/unusual head and trunk posturing? • Does hematemesis occur? • Does the child refuse food?
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onstipation can cause
discomfort, poor appetite, and behavior problems, in addition to more serious concerns such as bowel obstruction.
Salivation is a mechanism that protects the esophagus from gastric reflux by stimulating peristalsis. Chewing may stimulate salivation; therefore, handmouthing (ie, frequently putting hands, fingers, or the forearm into the mouth) may be an indicator of reflux and subsequent esophagitis. Rumination, or bringing food up from the stomach into the mouth, also may be associated with esophagitis. Rumination is also a risk factor for dental erosion. Bad breath,
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finding partially digested food on the pillow in the morning, refusal to eat, and inappropriate behavior when lying down or toward the end of and following meals may be symptomatic of reflux and esophagitis (Herbst, 1996; Tuchman, 1989). Proujansky, Shaffer, Vinton, and Bachrach (1994) found that H. pylori gastritis was associated with recurrent emesis in 3 of 12 patients with severe neurologic impairment; they also found guaiac positive stools or gastrostomy drainage in 3 of 12 patients, refusal to eat in 3 of 12 patients, and irritability during feedings, again in 3 of 12 patients. More than half of the patients with H. pylori lived in a residential institution, making residence in an outof-home setting a risk factor that should be identified. Constipation is another significant gastrointestinal problem for persons with neuromotor problems. Dysphagia and reliance on others for feeding may lead to inadequate fluid intake. This can be complicated by neurogenic bowel and immobility (Staiano & Del Giudice, 1994). Unfortunately, although it may be healthier and more comfortable for the person with disabilities to have a soft bowel movement every day or every other day, caregivers may find it much more convenient to toilet or clean persons who have firm stools on a less frequent basis. Therefore, caregivers may not perceive constipation as a problem. However, constipation can cause discomfort, poor appetite, and behavior problems, in addition to more serious concerns such as bowel obstruction. The history needs to include an assessment of the following: • Frequency of stools • Quality of stools; whether stools are firm/infrequent or whether firm stools alternate with diarrhea • Usual bowel management regimen • Discomfort associated with stooling • Melena, rectal bleeding, or prolapse • Dysphagia/poor oral intake, particularly of fluids • If generally continent, episodes of encopresis • Inappropriate behavior is cyclical/ improves following bowel movements • Abdominal distension or abdominal masses noted • “Gassiness” • Indications that the constipation may have other causes, such as hypothy-
roidism, especially in persons with Down syndrome Caregivers may deny that constipation occurs and complain instead of diarrhea. However, diarrhea may occur in the presence of fecal impaction if liquid stool passes around the impacted stool. It should be remembered that persons may be significantly constipated despite a history of daily or neardaily stools. Neuromotor difficulties may prevent the bowel from being completely evacuated, leading to gradually worsening constipation. Information about daily fluid intake and a diet history may be helpful as well. Cow’s milk (Loening-Baucke,1998) and decreased fiber intake (McClung, Boyne, & Heitlinger, 1995) also may be associated with refractory constipation. A history of behavior problems, gas, abdominal distension, and diarrhea shortly after ingesting milk may indicate lactose intolerance. Children with gastrostomy formula feeding are also at risk for dumping syndrome, in which nausea, vomiting, diarrhea, and palpitations are associated with too-rapid emptying of the stomach (Eicher, 1997), which may also lead to wide swings in blood sugar levels.
Obstructive Sleep Apnea Obstructive sleep apnea (OSA) is known to be associated with behavior problems (Owens, Opipari, Nobile, & Spirito, 1998). Sleep apnea is also a frequent finding in persons with Down syndrome, Prader-Willi syndrome, and craniofacial syndromes such as mandibular hypoplasia and Apert’s syndrome (Yantis, 1999). Obesity and poor muscle tone are risk factors. Clues to pursue include the following: • Obesity/overweight • Hypotonia • Craniofacial abnormalities • Snoring/gasping/apneic episodes • Frequent nocturnal arousals • Breathing through the mouth • Morning headache or worsened behavior in morning • Unusual sleep positions • Daytime sleepiness or naps • Nocturia OSA may cause morning headache (Sheldon, Spire, & Levy, 1992), which may manifest itself as inappropriate behavior upon arising. Nocturia may also be present, although in significantly impaired persons, this may be mere-
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PH ORIGINAL ARTICLE C ly considered further evidence of their primary disability. Sleep apnea may also be associated with failure to thrive, elevated systemic blood pressure, aggression, and behavioral abnormalities (Sheldon et al., 1992).
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ssessment of
orthopedic history should include questions regarding grimacing or behavior problems associated with movement or during transfers, frequency and tolerance of range of motion, and any swelling of joints or extremities.
Headache Headache may be particularly difficult to identify in persons with significant mental retardation and limited communication abilities. A family history of headache, particularly migraines, raises the index of suspicion. Headache, particularly migraine, may also be indicated by the following: • Scalp tenderness • Behavior improves in a dark quiet room or after a nap • Photophobia associated with inappropriate behavior • History of car sickness • Anisocoria/hemiparesis • Vomiting • Color changes (pale/flushed) • Headbanging • Rubbing eyes/head • Inappropriate behavior that may last 1 to 3 hours Problem behaviors may subside in a dark quiet room or after a nap. Migraine headaches typically last 1 to 3 hours,
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although they may continue as long as 24 hours (Haslam, 1996).
Urinary Tract Neuromotor problems affect the renal system as well. Neurogenic bladder dysfunction may lead to incomplete bladder emptying, providing a breeding ground for bacteria, and thereby leading to a UTI (Liptak, 1997). Dyssynergia may lead to vesicoureteral reflux, which also predisposes to UTI or kidney damage. Obtaining a history of frequency of urination in a person who wears incontinence briefs or diapers may be difficult. However, discomfort may be noted with voiding. The urine is sometimes observed to have a foul or strong odor, and blood may be noted in the diaper. UTIs are more common in the presence of constipation because the presence of hard stool in the lower pelvis can impair bladder emptying (Loening-Baucke, 1997, 1998). The NP may ask the following questions to investigate urinary problems: • Is there a history of UTIs? • Does the patient have neuromotor problems/neurogenic bowel and bladder? • Is the patient incontinent? • Is the patient constipated? • Does the patient use incontinence briefs? • Is escalation of behavior associated with urination? • Does the patient have hematuria? • Does the patient have foul-smelling urine? • Does the patient have fever/vomiting/chills?
Abuse Persons with disabilities are at significant risk for abuse, including sexual abuse (Madansky, 1995; Newberger, 1995). Therefore, it may be useful to know whether skin rash or lesions, vaginal bleeding, or vaginal drainage have occurred, or whether behavior is worse around specific caregivers. Unusual bruising or fractures should be noted. Hematuria may be related to trauma. Other perineal concerns may include the possibility of diaper rashes or Monilia infections related to diapers or incontinence briefs. Severe or refractory rashes may be associated with inadequate perineal care on the part of the caregiver.
Musculoskeletal System The musculoskeletal system can also be a source of significant discomfort. Spasticity puts strain on muscle, joints, and bones. The hips seem to be a particular source of discomfort, as adductors tend to pull the hips out of the socket, gradually leading to dislocation, dysplasia, and pain, particularly with transfers or position changes (Cooperman, Bartucci, Dietrick, & Millar, 1987). Contractures may develop. Fussiness with fever, swelling, and diaphoresis may indicate a fracture, especially in the presence of osteopenia. Likewise, osteomyelitis or a septic joint may be a consideration. Assessment of orthopedic history should include questions regarding grimacing or behavior problems associated with movement or during transfers, frequency and tolerance of range of motion, and any swelling of joints or extremities.
Hyperthyroidism/Hypothyroidism Hyperthyroidism may be associated with aggressive or self-injurious behavior because it can cause excitability, nervousness, emotional instability, and mood swings (DiGeorge & LaFranchi, 1997). Unfortunately, many of the concurrent signs and symptoms, such as anorexia, gastrointestinal problems, muscle weakness, and tremor also are frequently associated with such neuromotor problems as cerebral palsy and may not be appreciated by the caregivers. Heat sensitivity and muscle wasting also may be present. Hypothyroidism may be associated with constipation, which can cause discomfort. Hypothyroidism also is associated with cold intolerance, achy muscles, easy fatigability, and increased need for sleep (Gregerman, 1999). Skin becomes dry and scaly. Children with Down syndrome are at particularly high risk for hypothyroidism. To assess problems related to hyperthyroidism or hypothyroidism, the NP should ask about the following: • Recent unintended weight loss or gain • Increased irritability or somnolence/ lethargy • Change in hair (either more coarse or finer) • Problems with tolerance of heat or cold • Down syndrome
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PH ORIGINAL ARTICLE C • Changes in bowel status (either constipation or diarrhea)
Seizures Seizure activity, particularly complex partial seizures, may also be correlated with inappropriate behavior. In addition, drug therapy to treat seizures frequently provokes or enhances behavior problems. Seizure disorders are common in persons with significant physical and mental disability. Complex partial seizures impair consciousness and may be accompanied by semipurposeful activity (Vedanarayanan, 1999). The NP should note that purposeful or directed activity is not consistent with a diagnosis of seizures. Likewise, true seizure activity would be expected to have an abrupt onset and end. There may be a variable period of sleepiness or confusion, including nondirected postictal aggression. Questions to include to evaluate for the presence of seizure activity include the following: • Is there a diagnosis of seizure disorder? • Is inappropriate behavior random, with abrupt onset and end? • Is the patient sleepy after being aggressive or injuring himself or herself? • Is inappropriate behavior preceded by/accompanied by lip smacking, fumbling of hands, moaning, or verbal perseveration? • Is inappropriate behavior stereotyped or repetitive? • Is there excess saliva on the patient’s pillow in the morning? • Is there a sudden onset of grimacing/laughing/screaming/drooling?
Dermatology Persons with disabilities also may have common problems that may be the source of discomfort, such as atopic dermatitis. A history of atopic dermatitis, asthma, or allergies may help explain self-injury such as scratching or skin picking. Just plain dry skin also may lead to perseverative scratching. Prader-Willi syndrome is also associated with skin picking, particularly in the rectal or vaginal areas (Holm et al., 1993). In addition, some disabilities, such as spina bifida, are associated with impaired sensation, increasing the risk of skin breakdown. Other persons, such as those with cerebral palsy, may have
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adequate sensation but lack the ability to change position by themselves, or even indicate the need to change positions. Likewise, hand splints or braces such as ankle foot orthoses increase the risk for skin breakdown. When assessing for skin problems, be sure to ask the following questions: • Is there a history of skin breakdown? • Is there a family history of atopy? • Is there a personal history of asthma or atopic dermatitis? • Is any scratching, skin picking, or rectal digging noted? • If the person uses a wheelchair, how often does he or she get out of the chair? • Is the child able to change positions by himself or herself, or does he or she need assistance? • Are any splints or braces used? How long are they on at one time? • For persons who use either wheelchairs or orthoses, is there any redness? How long does it take to fade? Taking more than 20 to 30 minutes to fade is an indication that the orthoses are not fitting well.
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eizure disorders are
common in persons with significant physical and mental disability. Sensory Sensory problems, such as visual and auditory impairments, are common comorbidities in persons with developmental disabilities. In one survey of patients treated for self-injurious behavior, blindness was present at significantly higher rates than normally found in children in residential treatment facilities or in the general population (Hyman, Fischer, Mercugliano, & Cataldo, 1990). Likewise, hearing impairment was present at a rate similar to that of children referred for developmental delay and somewhat less than that of children in residential facilities. The rates of sensory impairment can be quite high in the population of those with developmental disabilities. For
example, children with cerebral palsy may have rates of visual disorders ranging from 50% to 90%, and the incidence of hearing disorders ranges from 10% to 60% with certain etiologic antecedents (Palmer & Hoon, 1994). A child who cannot hear instructions is not likely obey them. Of even more concern are the recent findings that late detection of hearing loss can have a lifelong impact on cognitive abilities, as well as communication (YoshinagaItano, Sedey, Coulter, & Mehl, 1998). Both blindness and deafness can contribute to developmental delay in their own right (Scheiner, 1995; Schlesinger, 1995). Behavioral observations to identify hearing loss are suspect because the child may be reacting to visual cues rather than auditory cues (Schlesinger, 1995). Questions to include in the assessment of sensory status include the following: • Is there a family history of hearing difficulties or speaking difficulties? • Does the child react to/awaken to loud noises? • Does the child pay attention to parents’ speech? • Does the child babble? Blindness is defined as visual acuity in the corrected eye of not more than 20/200, or a significant defect in the visual field (Scheiner, 1995). Blindness may be cortical, in which the vision system is intact and the pupils react to light, but there is no blink to threat (Goetz and Pappert, 1999), and the person is unable to comprehend the input in a meaningful way. In either case, a diagnosis of blindness does not mean the person is unable to react to visual stimuli. Questions that may raise the concern of blindness (Diamond, 1999) include the following: • Does the parent or caregiver have any concerns about vision? • Are there any concerns about strabismus of nystagmus? • Does persistent staring occur? • Does the patient show inattention to objects? • Is there alertness to visual stimuli presented 12 to 18 inches from the eyes? • Does the patient track silent objects? • Does the patient reach for objects? Deafblindness, a combination of both significant visual and hearing impairment, is less common and more than the sum of deafness and blindness indi-
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PH ORIGINAL ARTICLE C vidually. Children with deafblindness need expert intervention.
PHYSICAL EXAMINATION The physical examination should be thorough. Visible problems such as otitis media, abscessed teeth, eroded teeth related to reflux or rumination, or skin problems that may lead to skin picking or scratching need to be ruled out. Observation of the skin also may provide evidence for other pathology, such as neurofibromatosis or tuberous sclerosis; therefore, odd skin lesions or discolorations should be noted. Persistent skin picking and rectal digging may be associated with Prader-Willi syndrome, particularly in an obese, short-for-age child (Holm et al., 1993; Prader-Willi Syndrome Association, 2000). Location, number, and age of bruises may give some indication of the presence of selfinjury. The level of self-injury can be impressive. Nyhan (1994) cites bruising and biting that causes tissue loss, including partial amputation of the fingers, and hypertrophy of consistently abused areas as a result of self-injurious behavior. Lovaas and Smith (1994) described a child whose self-injurious behavior included headbanging, scratching her face and hands, and setting her hair on fire. The child’s scalp was described as “covered with scar tissue.” A disability is a risk factor for child abuse (Davis & Carrasco, 1992). Therefore, the NP must consider whether the history and the pattern of injury would more likely be consistent with physical abuse rather than self-injury. An injury inconsistent with the history, a vague account of the history, frequent changes or contradictory histories, and histories not consistent with the child’s developmental abilities are all red flags (Davis & Carrasco, 1992). Bruises in areas where the child cannot easily reach, such as the posterior thorax or buttocks, upper arms, and thighs may be indicators of abuse. Patterned lesions or cigarette burn lesions also point to the probability of physical abuse. Forensic techniques can identify the likely perpetrator of a bite mark if there are concerns. However, the NP must bear in mind the topography of the selfinjury, because a person who flings himself or herself back against the wall, floors, or other objects may indeed have some unusual bruising patterns. Calluses or areas of hypertrophy on hands
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or forearms are secondary skin lesions and may be associated with chronic skin manipulation, such as frequent hand mouthing or biting (Cohen, Davis, Mallory, & Zitelli, 1992). Other sores on the skin may be associated with significant scratching or skin picking, which may be a result of atopic dermatitis or other skin disorders.
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history of atopic
dermatitis, asthma, or allergies may help explain self-injury such as scratching or skin picking.
Firm stool in the rectal vault or abdominal masses may be palpated despite assurance that the child has recently defecated. However, the absence of abdominal masses or firm stool in the ampulla does not rule out the presence of constipation, because there can be an extraordinary amount of soft stool in the colon as well. Watch for rumination (the ability to bring food back up from the stomach). Carefully assess for dislocated joints (especially in the hips), joint swelling, or bursae. Hand wringing, especially with a late-onset microcephaly and developmental delays, may be associated with Rett syndrome, a disorder seen primarily in girls (Trevathan & Naidu, 1988). Dysmorphic features or low muscle tone may be indicative of the need for a genetic evaluation. High muscle tone or spasticity may be associated with discomfort and contractures. Growth curves may also help provide information about possible genetic abnormalities, particularly short stature and microcephaly (Jones, 1997).
Multifactorial Considerations Having a developmental disability does not preclude other common illnesses of infancy, childhood, and adolescence. Indeed, developmental disabilities are frequently associated with other comorbidities. Changes in behavior may be the only indicator that the child is in
need of medical care. Complicating this is the awareness that behavior problems may be a communicative response to activities in the environment as well. If the history and physical examination do not raise flags for the presence of a physiologic problem, and/or the history indicates the environment may be playing a significant role, a referral to a behavioral psychologist who is able to perform a functional analysis of behavior and develop an individualized treatment program is the logical first step. A functional analysis that reveals an automatic or intrinsic (internal environment) function may indicate the need for further medical workup. If the history raised concerns about possible medical pathology, those concerns should be pursued with an appropriate medical workup. If inappropriate behavior is not fairly clearly maintained by environmental causes, some simple, relatively noninvasive first steps may include a urinalysis to rule out UTI; a complete blood cell count, again to rule out infection or anemia that might be related to occult blood loss associated with esophagitis or gastritis; stool guaiac and guaiac of gastric contents (if gastrostomy or emesis is present) to assess for possible gastrointestinal bleeding; possibly a serum uric acid determination to rule out Lesch-Nyhan syndrome in males (Barabas, 1993); thyroid-stimulating hormone (TSH) or reflex TSH with free T4 if abnormalities are noted in the TSH; H. pylori antibodies; and an abdominal anterior-posterior radiograph to assess the amount of stool in the colon.
TREATMENT CONSIDERATIONS Treatment is based on the history and physical findings, as well as the result of any laboratory testing or imaging studies. The effects of constipation should not be underestimated. It is not unusual to identify significant constipation by radiograph despite a history of daily soft stools, an empty rectal vault, and a benign abdominal examination. In one case, a child’s aggression decreased and his appetite and communication attempts increased following bowel clean-out with enemas and the implementation of a more aggressive bowel regimen. This child had an unremarkable physical examination and a history of daily soft stools but was noted to have a large amount of
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PH ORIGINAL ARTICLE C stool in the colon on radiograph (Bosch et al., 1997). Many approaches can be used to treat chronic constipation; diet and adequate hydration are the obvious first choices. Do not overlook the use of daily oral laxatives. Often, consumption of fruit and fiber with adequate fluid intake will resolve constipation. However, many persons, especially those with significant neuromotor problems, will require aggressive use of oral laxatives with suppositories and enemas to avoid constipation. Consideration also may be given to a surgical cecostomy, in which an ostomy is created to allow for administration of enemas into the ascending colon to increase the effectiveness of enema administration. Endoscopy is the best method to identify gastroesophageal reflux disease (Claussen, 1999). However, empiric treatment may be appropriate in the presence of a suggestive history. Although empiric treatment generally follows several steps, our anecdotal experience has been that some of the less potent acid blockers may not be as effective in persons with disabilities, perhaps because they are unable to describe symptoms and effectiveness of medication. Phase one treatment, including elevation of the head of the bed, avoidance of large fatty meals before bedtime, and diet modification, is appropriate. Second-phase treatment of gastroesophageal reflux typically adds an H2 blocker and/or sucralfate. However, we at the University of Iowa Center for Disabilities and Development have seen inappropriate behavior decrease substantially when proton pump inhibitors are used and resurface when H2 blockers were initiated. With patients who cannot describe the severity of their symptoms, it may be worth escalating to the more intense levels of therapy much more rapidly than usual. Prokinetic agents, such as metoclopramide, are also used for both phase 2 and phase 3 treatment of gastroesophageal reflux disease. Another possible gastrointestinal problem is pancreatitis, particularly in persons receiving valproic acid treatment for seizures or those with biliary tract stones. If indicated, tests to determine levels of alanine aminotransferase, aspartate aminotransferase, lactate dehydrogenase, amylase, and serum
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lipase may help to rule out pancreatitis (Banks, 1998). If there is concern about dysphagia, a speech pathologist or occupational therapist can perform an oral-pharyngeal evaluation and can recommend a texture swallow study and/or modified barium swallow to assess oral skills. They may make recommendations for feeding, including rate, bolus size, and texture. They also may recommend the placement of a gastrostomy feeding tube if significant aspiration or oral impairment is present. Some persons with aspiration may benefit from oral stimulation to improve their swallowing ability, and improved nutrition may improve swallowing as well, so family members may be reassured that gastrostomy does not necessarily mean an end to all oral feeding. However, gastrostomy feeding has many socioemotional implications, especially when the family perceives that oral feeding is the last “normal” thing the child can do or that the child enjoys oral feeding.
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ruises in areas where
the child cannot easily reach, such as the posterior thorax or buttocks, upper arms, and thighs may be indicators of abuse.
OSA may be identified by polysomnography, although oximetry trending may be a less expensive way of screening for OSA. Weight loss may be one treatment option if the child is obese. Tonsillectomy and/or adenoidectomy may also help. Finally, continuous positive airway pressure may be needed at night if other options are ineffective. Tracheostomy has also been required in some persons. Headache may be very difficult to
identify and therefore to treat. Allowing rest in a quiet, dark room may be helpful, although one does need to be alert to the possibility that inappropriate behavior is being used to escape to a dark quiet room rather than as a result of discomfort. First-line treatments would include acetaminophen or ibuprofen. A UTI should be identified by urine culture and treated with an appropriate antibiotic. Recurrent UTIs may indicate the need for further workup, including a cystometrogram to identify problems with bladder tone, capacity, or synchrony. Renal and bladder ultrasonography can identify hydronephrosis or urine residuals after voiding. A voiding cystourethrogram may provide further information regarding bladder anatomy, function, and the possibility of reflux. Clean intermittent catheterization may provide relief for persons with inadequate bladder emptying. For persons who have recurrent urinary tract infections while being treated with clean intermittent catheterization, the first line of treatment should be evaluation of technique and frequency of catheterizations, rather than going directly to administration of systemic antibiotics. Functional analysis of behavior can identify environmental variables that maintain inappropriate behavior. Two primary functions of inappropriate behavior exist: escape, in which case a task demand is withdrawn (negative reinforcement) because of inappropriate behavior; and attention, in which the child engages in inappropriate behavior that is then rewarded by attention or being provided with a desired object or activity. Functional analysts devise individualized treatment plans based on the person’s pattern of response to environmental variables. A typical aspect of the treatment is functional communication training. The assumption is that the child is engaging in the inappropriate behavior in part because limited communication skills preclude a more appropriate way of asking for help or attention. During functional communication training, more appropriate ways of communicating, ranging from saying “please may I…” in verbal children to a nonverbal child’s use of pictures, switches, or signs, are taught and reinforced.
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PH ORIGINAL ARTICLE C SUMMARY The assessment of a child, adolescent, or young adult with significant developmental disabilities and poor communication skills may be quite complex and pose a considerable challenge for the NP. Self-injurious and aggressive behavior may be an attempt to communicate pain or discomfort. In addition, inappropriate behavior used to indicate discomfort may serve to obtain attention or escape task demands. As a result, children who are
T
he Web site
www.disAbility.gov provides much helpful information about health and disabilities and also has lists of organizations devoted to particular types of disabilities.
presenting with behavior problems must be carefully assessed and examined to rule out physiologic causes of their behavior. Furthermore, any child with disabilities and poor functional communication skills requires a detailed history that specifically probes for behaviors that may indicate the presence of a treatable problem. The NP may require the participation of a multidisciplinary team for complete assessment and treatment of the infant, child, or adolescent with disabilities, including specialists in behavior, education, communication, physical therapy, speech language pathology, audiology, and occupational therapy. In addition to parents or other caregivers, group home staff, in-home care providers, job trainers, and home health nurses may provide helpful information and should be a part of treatment planning. Many of these services can be accessed through local school districts. See the algorithm for assistance
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(Figure) to develop a plan for assessment. Referrals to subspecialists, including specialists in developmental medicine, may be helpful as well. Each state in the United States is home to a University Center for Excellence in Developmental Disabilities Education, Research, and Service that can provide excellent assistance and resources (see Box). The Web site www.disAbility.gov provides much helpful information about health and disabilities and also has lists of organizations devoted to particular types of disabilities.
REFERENCES Banks, P. (1998). Acute and chronic pancreatitis. In M. Feldman, B. Scharschmidt, M. Sleisenger, & S. Klein, Eds., Sleisenger & Fordtran’s Gastrointestinal and Liver Disease, 6th ed., pp. 809-38. Philadelphia: W. B. Saunders. Barabas, G. (1993). Lesch-Nyhan disease. Matheny Bulletin, 2, 1-10. Bosch, J., VanDyke, D., Smith, S., & Poulton, S. (1997). Role of medical conditions in the exacerbation of self-injurious behavior: An exploratory study. Mental Retardation, 35, 124130. Claussen, J. (1999). Gastroesophageal reflux disease. Clinician Reviews, 9, 69-82. Cohen, B., Davis, H., Mallory, S., & Zitelli, J. (1992) Pediatric dermatology. In B. Zitelli & H. Davis, Eds., Atlas of Pediatric Physical Diagnosis, 2nd ed., 8.1-8.48. Philadelphia: J. B. Lippincott. Cooperman, D., Bartucci, E., Dietrick, E., & Millar, E. (1987). Hip dislocation in spastic cerebral palsy: Long-term consequences. Journal of Pediatric Orthopedics, 7, 268-276. Davis, H., & Carrasco, M. (1992). Child abuse and neglect. In B. Zitelli & H. Davis, Eds., Atlas of Pediatric Physical Diagnosis, 2nd ed., 6.1-6.3. Philadelphia: J. B. Lippincott. Diamond, G. (1999). Evaluation and diagnosis— evaluating vision in preverbal and preliterate infants and children. In M. Yanoff, Ed., Ophthalmology, 1st ed., Section 6, 2.1. London: Mosby International Ltd. DiGeorge, A., & LaFranchi, S. (1997). Hyperthyroidism. In W. Nelson, R. Behrman, R. Kliegman, & A. Arvin, Eds., Nelson Textbook of Pediatrics, pp. 1600-1601. Philadelphia: W. B. Saunders. Eicher, P. (1997). Feeding. In M. Batshaw, ed., Children with Disabilities, 4th ed., pp. 621-41. Baltimore: Paul Brooks Publishing Company. Goetz, C. G., & Pappert, E. J. (1999). Textbook of clinical neurology. Philadelphia: W. B. Saunders. Gregerman, R. (1999). Thyroid disorders. In L. Barker, J. Burton, P. Zieve, & T. Finucane, Eds. Principles of ambulatory medicine, 5th ed., pp. 1066-1095. Baltimore: Williams & Wilkens. Haslam, R. (1996). The nervous system. In W. Nelson, R. Behrman, R. Kliegman, & A. Arvin, Eds., Nelson textbook of pediatrics, pp. 1667-1763. Philadelphia: W. B. Saunders. Herbst, J. (1996). The esophagus. In W. Nelson, R. Behrman, R. Kliegman, & A. Arvin, Eds.,
Nelson textbook of pediatrics, pp. 1051-1056. Philadelphia: W. B. Saunders. Holm, V., Cassidy, S., Butler, M., Hanchett, J., Greenswag, L., Whitman, B., et al. (1993). PraderWilli syndrome: Consensus diagnostic criteria. Pediatrics, 91, 398-402. Hyman, S., Fischer, W., Mercugliano, M., & Cataldo, M. (1990). Children with self-injurious behavior. Pediatrics, 85(Suppl.), 437-441. Iwata, B., Pace, G., Dorsey, M., Zarcone, J., Vollmer, T., Smith, R., et al. (1994). The functions of selfinjurious behavior: An experimental-epidemiological analysis. Journal of Applied Behavior Analysis, 27, 215-240. Jones, K. (1997). Smith’s recognizable patterns of human malformation, 5th ed. Philadelphia: W. B. Saunders. Liptak, G. (1997). Neural tube defects. In M. Batshaw, Ed., Children with disabilities, 4th ed., pp. 529-552. Baltimore: Paul Brooks Publishing Company. Loening-Baucke, V. (1997). Urinary incontinence and urinary tract infection and their resolution with treatment of chronic constipation of childhood. Pediatrics, 100, 228-232. Loening-Baucke, V. (1998). Constipation in children. New England Journal of Medicine, 339, 11551156. Lovaas, O., & Smith, T. (1994). Intensive and longterm treatments for clients with destructive behaviors. In T. Thompson & D. Gray, Eds., Destructive behavior in developmental disabilities: Diagnosis and treatment, pp. 243-60. London: Sage Publications. Madansky, D. (1995). Sexual abuse. In S. Parker & B. Zuckerman, Eds., Behavioral and developmental pediatrics, pp. 278-284. Boston: Little, Brown and Company. McClung, H., Boyne, L., & Heitlinger, L. Constipation and dietary fiber intake in children. Pediatrics, 96, 999-1000. Newberger, E. (1995). Physical abuse. In S. Parker & B. Zuckerman, Eds., Behavioral and developmental pediatrics, pp. 232-238. Boston: Little, Brown and Company. Nyhan, W. (1976). Behavior in the Lesch-Nyhan syndrome. Journal of Autism & Childhood Schizophrenia, 6, 235-252. Nyhan, W. (1994). The Lesch-Nyhan disease. In T. Thompson & D. Gray, eds., Destructive behavior in developmental disabilities: Diagnosis and treatment, pp. 181-197. London: Sage Publications. Owens, J., Opipari, L., Nobile, C., & Spirito, A. (1998). Sleep and daytime behavior in children with obstructive sleep apnea and behavioral sleep disorders. Pediatrics, 102, 11781184. Palmer, F., & Hoon, A. (1994). Cerebral palsy. In S. Parker & B. Zuckerman, Eds., Behavioral and developmental pediatrics, pp. 88-94. Boston: Little, Brown and Company. Perry, A. (1991). Rett syndrome: A comprehensive view of the literature. American Journal on Mental Retardation, 96, 275-290. Prader-Willi Syndrome Association. (2000). Basic facts about PWS: A diagnosis and reference guide for physicians and other health professionals. Retrieved from: http://www.pwsausa.org/ basicfac.htm Proujansky, R., Shaffer, S., Vinton, N., & Bachrach, S. (1994). Symptomatic Helicobacter pylori infection in young patients with severe neurologic impairment. Journal of Pediatrics, 125, 750-752. Rincover, A. (1986). Behavioral research in self-
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PH ORIGINAL ARTICLE C injury and self-stimulation. Psychiatric Clinics of North America, 9, 755-766. Scheiner, A. (1995). Blindness. In S. Parker & B. Zuckerman, Eds., Behavioral and developmental pediatrics, pp. 81-85. Boston: Little, Brown and Company. Schlesinger, H. (1995). Hearing loss. In S. Parker & B. Zuckerman, Eds., Behavioral and developmental pediatrics, pp. 174-179. Boston: Little, Brown and Company. Schroeder, S., & Tessel, R. (1994). Dopaminergic and serotonergic mechanisms in self-injury and aggression. In T. Thompson & D. Gray, Eds., Destructive behavior in developmental disabilities: diagnosis and treatment, pp. 198-210. London: Sage Publications.
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Sheldon, S., Spire, J., & Levy, H. (1992). Pediatric sleep medicine. Philadelphia: W. B. Saunders. Sondheimer, J. (1988). Gastroesophageal reflux: Update on pathogenesis and diagnosis. Pediatric Clinics of North America, 35, 103-116. Staiano, A., & Del Giudice, E. (1994). Colonic transit and anorectal manometry in children with severe brain damage. Pediatrics, 94, 169-173. Taylor, D., Rush, D., Hetrick, W., & Sandman, C. (1993). Self-injurious behavior within the menstrual cycle of women with mental retardation. American Journal on Mental Retardation, 97, 659-664. Trevathan, E., & Naidu, S. (1988). The clinical recognition and differential diagnosis of Rett syndrome. Journal of Child Neurology, 3(suppl.), S6-S16.
Tuchman, D. (1989). Cough, choke, sputter: The evaluation of the child with dysfunctional swallowing. Dysphagia, 3, 111-116. Vedanarayanan, V. (1999). Diagnosis of epilepsy in children. Pediatric Annals, 28, 218-224. Winchel, R., & Stanley, M. (1991). Self-injurious behavior: A review of the behavior and biology of self-mutilation. American Journal of Psychiatry, 148, 306-317. Yantis, M. (1999). Assessing children for obstructive sleep apnea. Journal of Pediatric Health Care, 13, 99-104. Yoshinaga-Itano, C., Sedey, A. L., Coulter, D. K., & Mehl, A. L. (1998). Language of early- and lateridentified children with hearing loss. Pediatrics, 102, 1161-1171.
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Use of Directed History and Behavioral Indicators in the Assessment of the Child With a Developmental Disability
ABSTRACT It can be very difficult to get a complete history and review of systems for children with developmental disabilities and poor communication skills. In addition, many children with developmental disabilities may engage in self-injurious or aggressive behavior. Although the causes of inappropriate behavior are frequently environmental, physiologic components may exist as well, particularly pain or discomfort. History taking must be focused and specific and may need to focus on the child’s behavioral patterns, because the child may not have sufficient communication skills to describe his or her problem and parents or guardians may not realize the relevance of certain behaviors. Gastrointestinal problems in particular may be a source of discomfort and should be reviewed with particular care. Referral to a psychologist who is able to perform a functional analysis of behavior may be necessary to treat problem behavior, especially if medical causes have been ruled out. J Pediatr Health Care. (2002). 16, 170-179.
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Jo n i Ja c o b s e n B o s ch , P h D, A R N P
A
ssessing a preverbal child for certain medical problems can be a diagnostic challenge because the child cannot describe symptoms, and the nurse practitioner (NP) must rely on the history provided by the child’s parents or guardians. The challenge is somewhat simplified by the comparatively limited differential diagnoses that apply to younger pediatric patients. The challenge grows considerably when the NP is faced with an older child, adolescent, or young adult whose communication skills are extremely limited by mental retardation. In addition to mental retardation, many persons in this group have comorbidities such as cerebral palsy, visual and hearing loss, and seizure disorders, and thus the differential diagnosis widens considerably. The NP must be prepared to ask detailed and probing questions to guide the evaluation of the older child, adolescent, or young adult who has no formal communication skills. Taking a history is a standard curricular component for NPs and other clinicians; therefore, this discussion will focus on strategies that may help in the assessment of persons with limited formal communication.
Joni Jacobsen Bosch, PhD, ARNP, is a Pediatric Nurse Practitioner and Family Nurse Practitioner at the Center for Disabilities and Development in Iowa City, Iowa. Reprint requests: Joni Jacobsen Bosch, PhD, ARNP, University of Iowa Center for Disabilities and Development, 100 Hawkins Dr, Iowa City, IA 52240; e-mail: [email protected]. Copyright © 2002 by the National Association of Pediatric Nurse Practitioners. 0891-5245/2002/$35.00 + 0
25/1/120393
doi:10.1067/mph.2002.120393
July/August 2002
PH ORIGINAL ARTICLE C HISTORY An accurate history is usually the single most important part of the assessment. Obtaining an accurate assessment may be complicated by the need to rely on persons who do not know the patient well, such as out-of-home care providers or family members who no longer live with the child or do not see him or her on a daily basis. It also may be complicated by care providers, including parents, who fail to volunteer essential information because they do not realize its value.
Behavior Self-injurious and aggressive behaviors are not uncommon among persons with mental retardation (Rincover, 1986). The incidence of these behaviors increases with decreasing cognitive and communicative abilities. Rates of self-injury and aggression are also higher among persons in out-of-home placements, such as group homes or intermediate care facilities/mental retardation. The causes for self-injurious and aggressive behavior are many and may be multifactorial as well. Assessment of self-injury and aggression is important because self-injury by definition causes tissue damage. Behavioral problems also may provide clues about the child’s physiologic status and assist the NP in ruling in or ruling out physiologic versus environmental functions of inappropriate behavior. Some self-injurious behavior has genetic causes, such as Lesch-Nyhan syndrome (Nyhan, 1976) and Rett syndrome (Perry, 1991). Hormonal changes and neurotransmitter imbalances have been implicated in behavioral abnormalities (Schroeder & Tessel, 1994; Taylor, Rush, Hetrick & Sandman, 1993; Winchel & Stanley, 1991). A strong environmental component may exist as well. Behavioral psychologists have identified environmental conditions, or functions, that may maintain inappropriate behavior (Iwata et al., 1994). When the function of the inappropriate behavior is identified, an individual behavior management plan can be implemented to address the inappropriate behavior. If self-injurious and aggressive behavior results from an attempt to communicate pain or discomfort, treating the underlying cause may reduce the inappropriate behavior to a considerable degree.
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First, the NP needs detailed information about the behavior. What type of behavior is exhibited? Is it aggression? Is it directed toward children, adults, or something else? Is there self-injury? What is the topography or type of the self-injury—for example, face slapping, head banging, hand biting, or body slamming? What is the setting in which the inappropriate behavior occurs? This information is important, but it must be remembered that any given inappropriate behavior may have many functions. Children may tug on an ear because they have otitis or because they enjoy tugging on their ear occasionally. Likewise, a child may bang his head because of a headache or to escape an unpleasant task demand. The fact of head banging tells us little about the cause of the behavior. More important are data concerning the antecedents, behavior, and consequences (also known as the ABCs) of the behavior. Observers may or may not be aware of the ABCs of a given behavior. They may report that nothing triggers the behavior. However, a careful observer may have noted that
I
f self-injurious and
aggressive behavior results from an attempt to communicate pain or discomfort, treating the underlying cause may reduce the inappropriate behavior to a considerable degree.
the child was not receiving attention before the behavior occurred and did receive attention after the behavior occurred. Attention itself can be a very powerful behavioral reinforcer for inappropriate behavior (Iwata et al., 1994).
BOX Resources • Local University Centers for Excellence in Developmental Disabilities Education, Research, and Service/University-affiliated Programs: http://www.aauap.org/data/ directory/directory.cfm • Links to various disability related sites: http://www.ninds.nih.gov/health_ and_medical/disorder_index.htm http://www.disability.gov/CSS/ • Parker, S., & Zuckerman, B. (1995). Behavioral and developmental pediatrics: A handbook for primary care. Boston: Little, Brown and Company. • The membership page of the Association for Behavioral Analysis may be searched by name or state: http://www.wmich.edu/cgi-bin/ aba/db.cgi?
The ABCs can give you some clues as to differential diagnoses as well. If inappropriate behavior occurs during high allergen times of the year, environmental allergies may need to be considered as a cause. Cyclical behavior problems in young women occurring on a monthly basis may represent menstrual cycle discomfort (Taylor et al., 1993). Inappropriate behavior just prior to meals may be an indicator of hunger. Likewise, dysphagia, esophagitis, gastritis, or enteric ulcers may lead to behavior problems associated with mealtimes (Bosch, VanDyke, Smith, & Poulton, 1997). Behavior that worsens when lying down, after meals, or at night may be indicative of gastroesophageal reflux associated with painful esophagitis (Sondheimer, 1988). Dysuria resulting from a urinary tract infection (UTI) as well as constipation may cause behavior problems associated with toileting. A good history regarding the ABCs of behavior may help focus a differential diagnosis toward a body system or toward a behavioral diagnosis. Correct identification of the cause is essential for the development and implementation of an appropriate treatment plan. Some questions that may help include the following:
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FIGURE Algorithm for assessment. • Is the behavior worse if the child is being ignored or during busy/noisy times of the day? • Is the behavior worse when the child is asked to complete a task? • Does worsened behavior lead to attention? (What’s wrong? Don’t do that!) • Does worsened behavior lead to an escape from a demand situation (time out)? • Does worsened behavior lead to access to preferred objects or activities?
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Gastrointestinal One study (Bosch et al., 1997) found that 7 of 25 patients being treated for behavioral problems had previously unidentified but significant medical problems that could be expected to cause discomfort or pain. Six of those seven patients had new diagnoses related to the gastrointestinal tract; therefore, the gastrointestinal tract is an important place to initiate a more focused medical history. Neuromotor problems, such as cerebral palsy, can affect the
gastrointestinal tract as well as other body systems. For example, neuromotor problems may interfere with the ability to chew, form a bolus, or correctly and safely swallow, leading to a diagnosis of dysphagia (Eicher, 1997; Tuchman 1989). Likewise, the incidence of gastroesophageal reflux can be as high as 75% in children with central nervous system disease (Tuchman, 1989). Questions related to swallowing difficulties should be detailed and include the following areas (Eicher, 1997): • Neuromotor problems • Recurrent pneumonia/bronchitis/sinusitis • Coughing/choking/gagging with meals • Preference for certain textures, especially soft or puree • Refusal of food • Prolonged feeding times (more than 30 minutes) • Underweight • Congestion following meals • Arching back/flexing neck with feedings • Worsened behavior at mealtimes • Multiple swallows required • Drooling or loss of food • Pocketing of food in mouth or cheeks • Scoliosis or poor positioning Nasopharyngeal reflux may be associated with frequent sinusitis or nasal infections. Persons with significant dysphagia may have an absent or poor gag reflex or a hyperactive gag (Tuchman, 1989). These persons may be noted to flex or extend their head during meals. This flexion or extension may be a manifestation of neuromotor instability and increase the risk of aspiration, or it may be an attempt to facilitate swallow in a person with poor oral phase skills. Frequent bouts of pneumonia or bronchitis also may reflect recurrent aspiration. The NP may actually need to watch a feeding, because gross aspiration that is readily apparent to those who have experience with persons with disabilities may be considered to be just a quirk of behavior to those with less experience. A referral to a speech/ language pathologist or occupational therapist may be necessary for a complete assessment of swallow. Gastroesophageal reflux with subsequent esophagitis is another common finding. Persons with neuromuscular problems, such as cerebral palsy, Down
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PH ORIGINAL ARTICLE C syndrome, and other sources of developmental delay are at higher risk for persistent reflux (Herbst, 1996; Tuchman, 1989). Questions to ask include the following: • Does worsened behavior occur around mealtimes or at night/when lying down? • Is there effortless vomiting/gagging/ rumination/food on the pillow in the morning? • Is coffee-ground emesis or melena present? • Does handmouthing (ie, frequently putting hands, fingers, or the forearm into the mouth) occur? • Does the patient have halitosis? • Does the patient have a chronic cough/cough toward end of feeding/bronchitis/pneumonia? • Does the patient have frequent nocturnal arousals? • Does the patient reside in a residential facility? Do others at the facility have a history of Helicobacter pylori? • Does the child have Sandifer’s syndrome/unusual head and trunk posturing? • Does hematemesis occur? • Does the child refuse food?
C
onstipation can cause
discomfort, poor appetite, and behavior problems, in addition to more serious concerns such as bowel obstruction.
Salivation is a mechanism that protects the esophagus from gastric reflux by stimulating peristalsis. Chewing may stimulate salivation; therefore, handmouthing (ie, frequently putting hands, fingers, or the forearm into the mouth) may be an indicator of reflux and subsequent esophagitis. Rumination, or bringing food up from the stomach into the mouth, also may be associated with esophagitis. Rumination is also a risk factor for dental erosion. Bad breath,
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finding partially digested food on the pillow in the morning, refusal to eat, and inappropriate behavior when lying down or toward the end of and following meals may be symptomatic of reflux and esophagitis (Herbst, 1996; Tuchman, 1989). Proujansky, Shaffer, Vinton, and Bachrach (1994) found that H. pylori gastritis was associated with recurrent emesis in 3 of 12 patients with severe neurologic impairment; they also found guaiac positive stools or gastrostomy drainage in 3 of 12 patients, refusal to eat in 3 of 12 patients, and irritability during feedings, again in 3 of 12 patients. More than half of the patients with H. pylori lived in a residential institution, making residence in an outof-home setting a risk factor that should be identified. Constipation is another significant gastrointestinal problem for persons with neuromotor problems. Dysphagia and reliance on others for feeding may lead to inadequate fluid intake. This can be complicated by neurogenic bowel and immobility (Staiano & Del Giudice, 1994). Unfortunately, although it may be healthier and more comfortable for the person with disabilities to have a soft bowel movement every day or every other day, caregivers may find it much more convenient to toilet or clean persons who have firm stools on a less frequent basis. Therefore, caregivers may not perceive constipation as a problem. However, constipation can cause discomfort, poor appetite, and behavior problems, in addition to more serious concerns such as bowel obstruction. The history needs to include an assessment of the following: • Frequency of stools • Quality of stools; whether stools are firm/infrequent or whether firm stools alternate with diarrhea • Usual bowel management regimen • Discomfort associated with stooling • Melena, rectal bleeding, or prolapse • Dysphagia/poor oral intake, particularly of fluids • If generally continent, episodes of encopresis • Inappropriate behavior is cyclical/ improves following bowel movements • Abdominal distension or abdominal masses noted • “Gassiness” • Indications that the constipation may have other causes, such as hypothy-
roidism, especially in persons with Down syndrome Caregivers may deny that constipation occurs and complain instead of diarrhea. However, diarrhea may occur in the presence of fecal impaction if liquid stool passes around the impacted stool. It should be remembered that persons may be significantly constipated despite a history of daily or neardaily stools. Neuromotor difficulties may prevent the bowel from being completely evacuated, leading to gradually worsening constipation. Information about daily fluid intake and a diet history may be helpful as well. Cow’s milk (Loening-Baucke,1998) and decreased fiber intake (McClung, Boyne, & Heitlinger, 1995) also may be associated with refractory constipation. A history of behavior problems, gas, abdominal distension, and diarrhea shortly after ingesting milk may indicate lactose intolerance. Children with gastrostomy formula feeding are also at risk for dumping syndrome, in which nausea, vomiting, diarrhea, and palpitations are associated with too-rapid emptying of the stomach (Eicher, 1997), which may also lead to wide swings in blood sugar levels.
Obstructive Sleep Apnea Obstructive sleep apnea (OSA) is known to be associated with behavior problems (Owens, Opipari, Nobile, & Spirito, 1998). Sleep apnea is also a frequent finding in persons with Down syndrome, Prader-Willi syndrome, and craniofacial syndromes such as mandibular hypoplasia and Apert’s syndrome (Yantis, 1999). Obesity and poor muscle tone are risk factors. Clues to pursue include the following: • Obesity/overweight • Hypotonia • Craniofacial abnormalities • Snoring/gasping/apneic episodes • Frequent nocturnal arousals • Breathing through the mouth • Morning headache or worsened behavior in morning • Unusual sleep positions • Daytime sleepiness or naps • Nocturia OSA may cause morning headache (Sheldon, Spire, & Levy, 1992), which may manifest itself as inappropriate behavior upon arising. Nocturia may also be present, although in significantly impaired persons, this may be mere-
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PH ORIGINAL ARTICLE C ly considered further evidence of their primary disability. Sleep apnea may also be associated with failure to thrive, elevated systemic blood pressure, aggression, and behavioral abnormalities (Sheldon et al., 1992).
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ssessment of
orthopedic history should include questions regarding grimacing or behavior problems associated with movement or during transfers, frequency and tolerance of range of motion, and any swelling of joints or extremities.
Headache Headache may be particularly difficult to identify in persons with significant mental retardation and limited communication abilities. A family history of headache, particularly migraines, raises the index of suspicion. Headache, particularly migraine, may also be indicated by the following: • Scalp tenderness • Behavior improves in a dark quiet room or after a nap • Photophobia associated with inappropriate behavior • History of car sickness • Anisocoria/hemiparesis • Vomiting • Color changes (pale/flushed) • Headbanging • Rubbing eyes/head • Inappropriate behavior that may last 1 to 3 hours Problem behaviors may subside in a dark quiet room or after a nap. Migraine headaches typically last 1 to 3 hours,
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although they may continue as long as 24 hours (Haslam, 1996).
Urinary Tract Neuromotor problems affect the renal system as well. Neurogenic bladder dysfunction may lead to incomplete bladder emptying, providing a breeding ground for bacteria, and thereby leading to a UTI (Liptak, 1997). Dyssynergia may lead to vesicoureteral reflux, which also predisposes to UTI or kidney damage. Obtaining a history of frequency of urination in a person who wears incontinence briefs or diapers may be difficult. However, discomfort may be noted with voiding. The urine is sometimes observed to have a foul or strong odor, and blood may be noted in the diaper. UTIs are more common in the presence of constipation because the presence of hard stool in the lower pelvis can impair bladder emptying (Loening-Baucke, 1997, 1998). The NP may ask the following questions to investigate urinary problems: • Is there a history of UTIs? • Does the patient have neuromotor problems/neurogenic bowel and bladder? • Is the patient incontinent? • Is the patient constipated? • Does the patient use incontinence briefs? • Is escalation of behavior associated with urination? • Does the patient have hematuria? • Does the patient have foul-smelling urine? • Does the patient have fever/vomiting/chills?
Abuse Persons with disabilities are at significant risk for abuse, including sexual abuse (Madansky, 1995; Newberger, 1995). Therefore, it may be useful to know whether skin rash or lesions, vaginal bleeding, or vaginal drainage have occurred, or whether behavior is worse around specific caregivers. Unusual bruising or fractures should be noted. Hematuria may be related to trauma. Other perineal concerns may include the possibility of diaper rashes or Monilia infections related to diapers or incontinence briefs. Severe or refractory rashes may be associated with inadequate perineal care on the part of the caregiver.
Musculoskeletal System The musculoskeletal system can also be a source of significant discomfort. Spasticity puts strain on muscle, joints, and bones. The hips seem to be a particular source of discomfort, as adductors tend to pull the hips out of the socket, gradually leading to dislocation, dysplasia, and pain, particularly with transfers or position changes (Cooperman, Bartucci, Dietrick, & Millar, 1987). Contractures may develop. Fussiness with fever, swelling, and diaphoresis may indicate a fracture, especially in the presence of osteopenia. Likewise, osteomyelitis or a septic joint may be a consideration. Assessment of orthopedic history should include questions regarding grimacing or behavior problems associated with movement or during transfers, frequency and tolerance of range of motion, and any swelling of joints or extremities.
Hyperthyroidism/Hypothyroidism Hyperthyroidism may be associated with aggressive or self-injurious behavior because it can cause excitability, nervousness, emotional instability, and mood swings (DiGeorge & LaFranchi, 1997). Unfortunately, many of the concurrent signs and symptoms, such as anorexia, gastrointestinal problems, muscle weakness, and tremor also are frequently associated with such neuromotor problems as cerebral palsy and may not be appreciated by the caregivers. Heat sensitivity and muscle wasting also may be present. Hypothyroidism may be associated with constipation, which can cause discomfort. Hypothyroidism also is associated with cold intolerance, achy muscles, easy fatigability, and increased need for sleep (Gregerman, 1999). Skin becomes dry and scaly. Children with Down syndrome are at particularly high risk for hypothyroidism. To assess problems related to hyperthyroidism or hypothyroidism, the NP should ask about the following: • Recent unintended weight loss or gain • Increased irritability or somnolence/ lethargy • Change in hair (either more coarse or finer) • Problems with tolerance of heat or cold • Down syndrome
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PH ORIGINAL ARTICLE C • Changes in bowel status (either constipation or diarrhea)
Seizures Seizure activity, particularly complex partial seizures, may also be correlated with inappropriate behavior. In addition, drug therapy to treat seizures frequently provokes or enhances behavior problems. Seizure disorders are common in persons with significant physical and mental disability. Complex partial seizures impair consciousness and may be accompanied by semipurposeful activity (Vedanarayanan, 1999). The NP should note that purposeful or directed activity is not consistent with a diagnosis of seizures. Likewise, true seizure activity would be expected to have an abrupt onset and end. There may be a variable period of sleepiness or confusion, including nondirected postictal aggression. Questions to include to evaluate for the presence of seizure activity include the following: • Is there a diagnosis of seizure disorder? • Is inappropriate behavior random, with abrupt onset and end? • Is the patient sleepy after being aggressive or injuring himself or herself? • Is inappropriate behavior preceded by/accompanied by lip smacking, fumbling of hands, moaning, or verbal perseveration? • Is inappropriate behavior stereotyped or repetitive? • Is there excess saliva on the patient’s pillow in the morning? • Is there a sudden onset of grimacing/laughing/screaming/drooling?
Dermatology Persons with disabilities also may have common problems that may be the source of discomfort, such as atopic dermatitis. A history of atopic dermatitis, asthma, or allergies may help explain self-injury such as scratching or skin picking. Just plain dry skin also may lead to perseverative scratching. Prader-Willi syndrome is also associated with skin picking, particularly in the rectal or vaginal areas (Holm et al., 1993). In addition, some disabilities, such as spina bifida, are associated with impaired sensation, increasing the risk of skin breakdown. Other persons, such as those with cerebral palsy, may have
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adequate sensation but lack the ability to change position by themselves, or even indicate the need to change positions. Likewise, hand splints or braces such as ankle foot orthoses increase the risk for skin breakdown. When assessing for skin problems, be sure to ask the following questions: • Is there a history of skin breakdown? • Is there a family history of atopy? • Is there a personal history of asthma or atopic dermatitis? • Is any scratching, skin picking, or rectal digging noted? • If the person uses a wheelchair, how often does he or she get out of the chair? • Is the child able to change positions by himself or herself, or does he or she need assistance? • Are any splints or braces used? How long are they on at one time? • For persons who use either wheelchairs or orthoses, is there any redness? How long does it take to fade? Taking more than 20 to 30 minutes to fade is an indication that the orthoses are not fitting well.
S
eizure disorders are
common in persons with significant physical and mental disability. Sensory Sensory problems, such as visual and auditory impairments, are common comorbidities in persons with developmental disabilities. In one survey of patients treated for self-injurious behavior, blindness was present at significantly higher rates than normally found in children in residential treatment facilities or in the general population (Hyman, Fischer, Mercugliano, & Cataldo, 1990). Likewise, hearing impairment was present at a rate similar to that of children referred for developmental delay and somewhat less than that of children in residential facilities. The rates of sensory impairment can be quite high in the population of those with developmental disabilities. For
example, children with cerebral palsy may have rates of visual disorders ranging from 50% to 90%, and the incidence of hearing disorders ranges from 10% to 60% with certain etiologic antecedents (Palmer & Hoon, 1994). A child who cannot hear instructions is not likely obey them. Of even more concern are the recent findings that late detection of hearing loss can have a lifelong impact on cognitive abilities, as well as communication (YoshinagaItano, Sedey, Coulter, & Mehl, 1998). Both blindness and deafness can contribute to developmental delay in their own right (Scheiner, 1995; Schlesinger, 1995). Behavioral observations to identify hearing loss are suspect because the child may be reacting to visual cues rather than auditory cues (Schlesinger, 1995). Questions to include in the assessment of sensory status include the following: • Is there a family history of hearing difficulties or speaking difficulties? • Does the child react to/awaken to loud noises? • Does the child pay attention to parents’ speech? • Does the child babble? Blindness is defined as visual acuity in the corrected eye of not more than 20/200, or a significant defect in the visual field (Scheiner, 1995). Blindness may be cortical, in which the vision system is intact and the pupils react to light, but there is no blink to threat (Goetz and Pappert, 1999), and the person is unable to comprehend the input in a meaningful way. In either case, a diagnosis of blindness does not mean the person is unable to react to visual stimuli. Questions that may raise the concern of blindness (Diamond, 1999) include the following: • Does the parent or caregiver have any concerns about vision? • Are there any concerns about strabismus of nystagmus? • Does persistent staring occur? • Does the patient show inattention to objects? • Is there alertness to visual stimuli presented 12 to 18 inches from the eyes? • Does the patient track silent objects? • Does the patient reach for objects? Deafblindness, a combination of both significant visual and hearing impairment, is less common and more than the sum of deafness and blindness indi-
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PH ORIGINAL ARTICLE C vidually. Children with deafblindness need expert intervention.
PHYSICAL EXAMINATION The physical examination should be thorough. Visible problems such as otitis media, abscessed teeth, eroded teeth related to reflux or rumination, or skin problems that may lead to skin picking or scratching need to be ruled out. Observation of the skin also may provide evidence for other pathology, such as neurofibromatosis or tuberous sclerosis; therefore, odd skin lesions or discolorations should be noted. Persistent skin picking and rectal digging may be associated with Prader-Willi syndrome, particularly in an obese, short-for-age child (Holm et al., 1993; Prader-Willi Syndrome Association, 2000). Location, number, and age of bruises may give some indication of the presence of selfinjury. The level of self-injury can be impressive. Nyhan (1994) cites bruising and biting that causes tissue loss, including partial amputation of the fingers, and hypertrophy of consistently abused areas as a result of self-injurious behavior. Lovaas and Smith (1994) described a child whose self-injurious behavior included headbanging, scratching her face and hands, and setting her hair on fire. The child’s scalp was described as “covered with scar tissue.” A disability is a risk factor for child abuse (Davis & Carrasco, 1992). Therefore, the NP must consider whether the history and the pattern of injury would more likely be consistent with physical abuse rather than self-injury. An injury inconsistent with the history, a vague account of the history, frequent changes or contradictory histories, and histories not consistent with the child’s developmental abilities are all red flags (Davis & Carrasco, 1992). Bruises in areas where the child cannot easily reach, such as the posterior thorax or buttocks, upper arms, and thighs may be indicators of abuse. Patterned lesions or cigarette burn lesions also point to the probability of physical abuse. Forensic techniques can identify the likely perpetrator of a bite mark if there are concerns. However, the NP must bear in mind the topography of the selfinjury, because a person who flings himself or herself back against the wall, floors, or other objects may indeed have some unusual bruising patterns. Calluses or areas of hypertrophy on hands
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or forearms are secondary skin lesions and may be associated with chronic skin manipulation, such as frequent hand mouthing or biting (Cohen, Davis, Mallory, & Zitelli, 1992). Other sores on the skin may be associated with significant scratching or skin picking, which may be a result of atopic dermatitis or other skin disorders.
A
history of atopic
dermatitis, asthma, or allergies may help explain self-injury such as scratching or skin picking.
Firm stool in the rectal vault or abdominal masses may be palpated despite assurance that the child has recently defecated. However, the absence of abdominal masses or firm stool in the ampulla does not rule out the presence of constipation, because there can be an extraordinary amount of soft stool in the colon as well. Watch for rumination (the ability to bring food back up from the stomach). Carefully assess for dislocated joints (especially in the hips), joint swelling, or bursae. Hand wringing, especially with a late-onset microcephaly and developmental delays, may be associated with Rett syndrome, a disorder seen primarily in girls (Trevathan & Naidu, 1988). Dysmorphic features or low muscle tone may be indicative of the need for a genetic evaluation. High muscle tone or spasticity may be associated with discomfort and contractures. Growth curves may also help provide information about possible genetic abnormalities, particularly short stature and microcephaly (Jones, 1997).
Multifactorial Considerations Having a developmental disability does not preclude other common illnesses of infancy, childhood, and adolescence. Indeed, developmental disabilities are frequently associated with other comorbidities. Changes in behavior may be the only indicator that the child is in
need of medical care. Complicating this is the awareness that behavior problems may be a communicative response to activities in the environment as well. If the history and physical examination do not raise flags for the presence of a physiologic problem, and/or the history indicates the environment may be playing a significant role, a referral to a behavioral psychologist who is able to perform a functional analysis of behavior and develop an individualized treatment program is the logical first step. A functional analysis that reveals an automatic or intrinsic (internal environment) function may indicate the need for further medical workup. If the history raised concerns about possible medical pathology, those concerns should be pursued with an appropriate medical workup. If inappropriate behavior is not fairly clearly maintained by environmental causes, some simple, relatively noninvasive first steps may include a urinalysis to rule out UTI; a complete blood cell count, again to rule out infection or anemia that might be related to occult blood loss associated with esophagitis or gastritis; stool guaiac and guaiac of gastric contents (if gastrostomy or emesis is present) to assess for possible gastrointestinal bleeding; possibly a serum uric acid determination to rule out Lesch-Nyhan syndrome in males (Barabas, 1993); thyroid-stimulating hormone (TSH) or reflex TSH with free T4 if abnormalities are noted in the TSH; H. pylori antibodies; and an abdominal anterior-posterior radiograph to assess the amount of stool in the colon.
TREATMENT CONSIDERATIONS Treatment is based on the history and physical findings, as well as the result of any laboratory testing or imaging studies. The effects of constipation should not be underestimated. It is not unusual to identify significant constipation by radiograph despite a history of daily soft stools, an empty rectal vault, and a benign abdominal examination. In one case, a child’s aggression decreased and his appetite and communication attempts increased following bowel clean-out with enemas and the implementation of a more aggressive bowel regimen. This child had an unremarkable physical examination and a history of daily soft stools but was noted to have a large amount of
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PH ORIGINAL ARTICLE C stool in the colon on radiograph (Bosch et al., 1997). Many approaches can be used to treat chronic constipation; diet and adequate hydration are the obvious first choices. Do not overlook the use of daily oral laxatives. Often, consumption of fruit and fiber with adequate fluid intake will resolve constipation. However, many persons, especially those with significant neuromotor problems, will require aggressive use of oral laxatives with suppositories and enemas to avoid constipation. Consideration also may be given to a surgical cecostomy, in which an ostomy is created to allow for administration of enemas into the ascending colon to increase the effectiveness of enema administration. Endoscopy is the best method to identify gastroesophageal reflux disease (Claussen, 1999). However, empiric treatment may be appropriate in the presence of a suggestive history. Although empiric treatment generally follows several steps, our anecdotal experience has been that some of the less potent acid blockers may not be as effective in persons with disabilities, perhaps because they are unable to describe symptoms and effectiveness of medication. Phase one treatment, including elevation of the head of the bed, avoidance of large fatty meals before bedtime, and diet modification, is appropriate. Second-phase treatment of gastroesophageal reflux typically adds an H2 blocker and/or sucralfate. However, we at the University of Iowa Center for Disabilities and Development have seen inappropriate behavior decrease substantially when proton pump inhibitors are used and resurface when H2 blockers were initiated. With patients who cannot describe the severity of their symptoms, it may be worth escalating to the more intense levels of therapy much more rapidly than usual. Prokinetic agents, such as metoclopramide, are also used for both phase 2 and phase 3 treatment of gastroesophageal reflux disease. Another possible gastrointestinal problem is pancreatitis, particularly in persons receiving valproic acid treatment for seizures or those with biliary tract stones. If indicated, tests to determine levels of alanine aminotransferase, aspartate aminotransferase, lactate dehydrogenase, amylase, and serum
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lipase may help to rule out pancreatitis (Banks, 1998). If there is concern about dysphagia, a speech pathologist or occupational therapist can perform an oral-pharyngeal evaluation and can recommend a texture swallow study and/or modified barium swallow to assess oral skills. They may make recommendations for feeding, including rate, bolus size, and texture. They also may recommend the placement of a gastrostomy feeding tube if significant aspiration or oral impairment is present. Some persons with aspiration may benefit from oral stimulation to improve their swallowing ability, and improved nutrition may improve swallowing as well, so family members may be reassured that gastrostomy does not necessarily mean an end to all oral feeding. However, gastrostomy feeding has many socioemotional implications, especially when the family perceives that oral feeding is the last “normal” thing the child can do or that the child enjoys oral feeding.
B
ruises in areas where
the child cannot easily reach, such as the posterior thorax or buttocks, upper arms, and thighs may be indicators of abuse.
OSA may be identified by polysomnography, although oximetry trending may be a less expensive way of screening for OSA. Weight loss may be one treatment option if the child is obese. Tonsillectomy and/or adenoidectomy may also help. Finally, continuous positive airway pressure may be needed at night if other options are ineffective. Tracheostomy has also been required in some persons. Headache may be very difficult to
identify and therefore to treat. Allowing rest in a quiet, dark room may be helpful, although one does need to be alert to the possibility that inappropriate behavior is being used to escape to a dark quiet room rather than as a result of discomfort. First-line treatments would include acetaminophen or ibuprofen. A UTI should be identified by urine culture and treated with an appropriate antibiotic. Recurrent UTIs may indicate the need for further workup, including a cystometrogram to identify problems with bladder tone, capacity, or synchrony. Renal and bladder ultrasonography can identify hydronephrosis or urine residuals after voiding. A voiding cystourethrogram may provide further information regarding bladder anatomy, function, and the possibility of reflux. Clean intermittent catheterization may provide relief for persons with inadequate bladder emptying. For persons who have recurrent urinary tract infections while being treated with clean intermittent catheterization, the first line of treatment should be evaluation of technique and frequency of catheterizations, rather than going directly to administration of systemic antibiotics. Functional analysis of behavior can identify environmental variables that maintain inappropriate behavior. Two primary functions of inappropriate behavior exist: escape, in which case a task demand is withdrawn (negative reinforcement) because of inappropriate behavior; and attention, in which the child engages in inappropriate behavior that is then rewarded by attention or being provided with a desired object or activity. Functional analysts devise individualized treatment plans based on the person’s pattern of response to environmental variables. A typical aspect of the treatment is functional communication training. The assumption is that the child is engaging in the inappropriate behavior in part because limited communication skills preclude a more appropriate way of asking for help or attention. During functional communication training, more appropriate ways of communicating, ranging from saying “please may I…” in verbal children to a nonverbal child’s use of pictures, switches, or signs, are taught and reinforced.
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PH ORIGINAL ARTICLE C SUMMARY The assessment of a child, adolescent, or young adult with significant developmental disabilities and poor communication skills may be quite complex and pose a considerable challenge for the NP. Self-injurious and aggressive behavior may be an attempt to communicate pain or discomfort. In addition, inappropriate behavior used to indicate discomfort may serve to obtain attention or escape task demands. As a result, children who are
T
he Web site
www.disAbility.gov provides much helpful information about health and disabilities and also has lists of organizations devoted to particular types of disabilities.
presenting with behavior problems must be carefully assessed and examined to rule out physiologic causes of their behavior. Furthermore, any child with disabilities and poor functional communication skills requires a detailed history that specifically probes for behaviors that may indicate the presence of a treatable problem. The NP may require the participation of a multidisciplinary team for complete assessment and treatment of the infant, child, or adolescent with disabilities, including specialists in behavior, education, communication, physical therapy, speech language pathology, audiology, and occupational therapy. In addition to parents or other caregivers, group home staff, in-home care providers, job trainers, and home health nurses may provide helpful information and should be a part of treatment planning. Many of these services can be accessed through local school districts. See the algorithm for assistance
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(Figure) to develop a plan for assessment. Referrals to subspecialists, including specialists in developmental medicine, may be helpful as well. Each state in the United States is home to a University Center for Excellence in Developmental Disabilities Education, Research, and Service that can provide excellent assistance and resources (see Box). The Web site www.disAbility.gov provides much helpful information about health and disabilities and also has lists of organizations devoted to particular types of disabilities.
REFERENCES Banks, P. (1998). Acute and chronic pancreatitis. In M. Feldman, B. Scharschmidt, M. Sleisenger, & S. Klein, Eds., Sleisenger & Fordtran’s Gastrointestinal and Liver Disease, 6th ed., pp. 809-38. Philadelphia: W. B. Saunders. Barabas, G. (1993). Lesch-Nyhan disease. Matheny Bulletin, 2, 1-10. Bosch, J., VanDyke, D., Smith, S., & Poulton, S. (1997). Role of medical conditions in the exacerbation of self-injurious behavior: An exploratory study. Mental Retardation, 35, 124130. Claussen, J. (1999). Gastroesophageal reflux disease. Clinician Reviews, 9, 69-82. Cohen, B., Davis, H., Mallory, S., & Zitelli, J. (1992) Pediatric dermatology. In B. Zitelli & H. Davis, Eds., Atlas of Pediatric Physical Diagnosis, 2nd ed., 8.1-8.48. Philadelphia: J. B. Lippincott. Cooperman, D., Bartucci, E., Dietrick, E., & Millar, E. (1987). Hip dislocation in spastic cerebral palsy: Long-term consequences. Journal of Pediatric Orthopedics, 7, 268-276. Davis, H., & Carrasco, M. (1992). Child abuse and neglect. In B. Zitelli & H. Davis, Eds., Atlas of Pediatric Physical Diagnosis, 2nd ed., 6.1-6.3. Philadelphia: J. B. Lippincott. Diamond, G. (1999). Evaluation and diagnosis— evaluating vision in preverbal and preliterate infants and children. In M. Yanoff, Ed., Ophthalmology, 1st ed., Section 6, 2.1. London: Mosby International Ltd. DiGeorge, A., & LaFranchi, S. (1997). Hyperthyroidism. In W. Nelson, R. Behrman, R. Kliegman, & A. Arvin, Eds., Nelson Textbook of Pediatrics, pp. 1600-1601. Philadelphia: W. B. Saunders. Eicher, P. (1997). Feeding. In M. Batshaw, ed., Children with Disabilities, 4th ed., pp. 621-41. Baltimore: Paul Brooks Publishing Company. Goetz, C. G., & Pappert, E. J. (1999). Textbook of clinical neurology. Philadelphia: W. B. Saunders. Gregerman, R. (1999). Thyroid disorders. In L. Barker, J. Burton, P. Zieve, & T. Finucane, Eds. Principles of ambulatory medicine, 5th ed., pp. 1066-1095. Baltimore: Williams & Wilkens. Haslam, R. (1996). The nervous system. In W. Nelson, R. Behrman, R. Kliegman, & A. Arvin, Eds., Nelson textbook of pediatrics, pp. 1667-1763. Philadelphia: W. B. Saunders. Herbst, J. (1996). The esophagus. In W. Nelson, R. Behrman, R. Kliegman, & A. Arvin, Eds.,
Nelson textbook of pediatrics, pp. 1051-1056. Philadelphia: W. B. Saunders. Holm, V., Cassidy, S., Butler, M., Hanchett, J., Greenswag, L., Whitman, B., et al. (1993). PraderWilli syndrome: Consensus diagnostic criteria. Pediatrics, 91, 398-402. Hyman, S., Fischer, W., Mercugliano, M., & Cataldo, M. (1990). Children with self-injurious behavior. Pediatrics, 85(Suppl.), 437-441. Iwata, B., Pace, G., Dorsey, M., Zarcone, J., Vollmer, T., Smith, R., et al. (1994). The functions of selfinjurious behavior: An experimental-epidemiological analysis. Journal of Applied Behavior Analysis, 27, 215-240. Jones, K. (1997). Smith’s recognizable patterns of human malformation, 5th ed. Philadelphia: W. B. Saunders. Liptak, G. (1997). Neural tube defects. In M. Batshaw, Ed., Children with disabilities, 4th ed., pp. 529-552. Baltimore: Paul Brooks Publishing Company. Loening-Baucke, V. (1997). Urinary incontinence and urinary tract infection and their resolution with treatment of chronic constipation of childhood. Pediatrics, 100, 228-232. Loening-Baucke, V. (1998). Constipation in children. New England Journal of Medicine, 339, 11551156. Lovaas, O., & Smith, T. (1994). Intensive and longterm treatments for clients with destructive behaviors. In T. Thompson & D. Gray, Eds., Destructive behavior in developmental disabilities: Diagnosis and treatment, pp. 243-60. London: Sage Publications. Madansky, D. (1995). Sexual abuse. In S. Parker & B. Zuckerman, Eds., Behavioral and developmental pediatrics, pp. 278-284. Boston: Little, Brown and Company. McClung, H., Boyne, L., & Heitlinger, L. Constipation and dietary fiber intake in children. Pediatrics, 96, 999-1000. Newberger, E. (1995). Physical abuse. In S. Parker & B. Zuckerman, Eds., Behavioral and developmental pediatrics, pp. 232-238. Boston: Little, Brown and Company. Nyhan, W. (1976). Behavior in the Lesch-Nyhan syndrome. Journal of Autism & Childhood Schizophrenia, 6, 235-252. Nyhan, W. (1994). The Lesch-Nyhan disease. In T. Thompson & D. Gray, eds., Destructive behavior in developmental disabilities: Diagnosis and treatment, pp. 181-197. London: Sage Publications. Owens, J., Opipari, L., Nobile, C., & Spirito, A. (1998). Sleep and daytime behavior in children with obstructive sleep apnea and behavioral sleep disorders. Pediatrics, 102, 11781184. Palmer, F., & Hoon, A. (1994). Cerebral palsy. In S. Parker & B. Zuckerman, Eds., Behavioral and developmental pediatrics, pp. 88-94. Boston: Little, Brown and Company. Perry, A. (1991). Rett syndrome: A comprehensive view of the literature. American Journal on Mental Retardation, 96, 275-290. Prader-Willi Syndrome Association. (2000). Basic facts about PWS: A diagnosis and reference guide for physicians and other health professionals. Retrieved from: http://www.pwsausa.org/ basicfac.htm Proujansky, R., Shaffer, S., Vinton, N., & Bachrach, S. (1994). Symptomatic Helicobacter pylori infection in young patients with severe neurologic impairment. Journal of Pediatrics, 125, 750-752. Rincover, A. (1986). Behavioral research in self-
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PH ORIGINAL ARTICLE C injury and self-stimulation. Psychiatric Clinics of North America, 9, 755-766. Scheiner, A. (1995). Blindness. In S. Parker & B. Zuckerman, Eds., Behavioral and developmental pediatrics, pp. 81-85. Boston: Little, Brown and Company. Schlesinger, H. (1995). Hearing loss. In S. Parker & B. Zuckerman, Eds., Behavioral and developmental pediatrics, pp. 174-179. Boston: Little, Brown and Company. Schroeder, S., & Tessel, R. (1994). Dopaminergic and serotonergic mechanisms in self-injury and aggression. In T. Thompson & D. Gray, Eds., Destructive behavior in developmental disabilities: diagnosis and treatment, pp. 198-210. London: Sage Publications.
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Sheldon, S., Spire, J., & Levy, H. (1992). Pediatric sleep medicine. Philadelphia: W. B. Saunders. Sondheimer, J. (1988). Gastroesophageal reflux: Update on pathogenesis and diagnosis. Pediatric Clinics of North America, 35, 103-116. Staiano, A., & Del Giudice, E. (1994). Colonic transit and anorectal manometry in children with severe brain damage. Pediatrics, 94, 169-173. Taylor, D., Rush, D., Hetrick, W., & Sandman, C. (1993). Self-injurious behavior within the menstrual cycle of women with mental retardation. American Journal on Mental Retardation, 97, 659-664. Trevathan, E., & Naidu, S. (1988). The clinical recognition and differential diagnosis of Rett syndrome. Journal of Child Neurology, 3(suppl.), S6-S16.
Tuchman, D. (1989). Cough, choke, sputter: The evaluation of the child with dysfunctional swallowing. Dysphagia, 3, 111-116. Vedanarayanan, V. (1999). Diagnosis of epilepsy in children. Pediatric Annals, 28, 218-224. Winchel, R., & Stanley, M. (1991). Self-injurious behavior: A review of the behavior and biology of self-mutilation. American Journal of Psychiatry, 148, 306-317. Yantis, M. (1999). Assessing children for obstructive sleep apnea. Journal of Pediatric Health Care, 13, 99-104. Yoshinaga-Itano, C., Sedey, A. L., Coulter, D. K., & Mehl, A. L. (1998). Language of early- and lateridentified children with hearing loss. Pediatrics, 102, 1161-1171.
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