School-Related Medical Issues Archive 2012
John L. Digges, MD, PhD, MPH, FAAP
Dr. Digges practiced general and behavioral pediatrics in Oklahoma and California for 14 years. For ten of the past 12 years, he has served as the Forensic (Child Abuse) Pediatrician for Kern County, California; and he has had a private practice limited to ADHD consultations for the past 12 years. He has been a CME surveyor for the Institute of Medical Quality (CMA) since 2000, and is a recent past-President of the Kern County Medical Society. Dr. Digges has been at the DCN since August, 2008.
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Prader-Willi Syndrome and Aggressive Behavior
Hello,I’m wondering about the onset of aggressive behavior with a 6 year old SDC kindergarten student. This student has average to low average cognitive ability. He has had significant speech and language therapy to address expressive and receptive language deficits. He’s made good progress in this area; receptive language is stronger however there is a latency of response. He kicks, hits, slaps, and pulls hair when instructed by adults. The behavior serves work avoidance and attention seeking motives. Extensive behavior support planning is in effect with use of visual strategies, social stories, break time, structured choices. He continues to engage in the target behaviors about once a week.
In your experience, how common is this scenario for students with Prader-Willi Syndrome? If so, what is the typical age of onset? What positive behavior support interventions have you found most effective, or is the biological component of this a significant, mitigating factor? When he’s not aggressive, he is loving and quite charming.
Quail Run Elementary School
Since your question has both medical and mental health components, our pediatrician and clinical psychologist have collaborated to provide a response. Although there can be wide variation in the number and severity of symptoms that students with Prader-Willi Syndrome (PWS) display and these can change over time, the features your student has demonstrated are certainly consistent with patterns seen in children with PWS.
About Prader-Willi Syndrome
Prader-Willi (pronounced “prah-dur VIL-ee”) Syndrome is a congenital disorder caused by an absence of some genetic material from the long arm of the 15th chromosome (deletion15q 11-13). The several deleted genes happen to be involved with coding for the proteins that are necessary in order for the hypothalamus to develop and function normally. Through both neural and endocrine mechanisms, the hypothalamus plays a central role in maintaining homeostasis through various feedback mechanisms, typically involving the release of hormones which exert either inhibitory or excitatory effects. Abnormalities involving the hypothalamus may compromise the body’s ability to self-regulate such functions as appetite, thirst, sleep, body temperature, heart rate, blood pressure, and emotional responses.
PWS is estimated to occur in 1 of every 12-15,000 births, and the symptoms present in two distinct stages. At birth and throughout the first year of life, infants characteristically have reduced muscle tone, weak sucking reflex, and slow weight gain. They often have dysconjugate gaze (crossed eyes), excessive fatigue, impairment in their ability to respond appropriately to environmental stimuli, and difficulty transitioning between sleep and wakefulness. These aspects predominate through the first year of life, and then are superseded by the symptoms which characterize the second stage of PWS.
The second stage of PWS evolves throughout early childhood (anywhere from 1-8 years of age). Insatiable food cravings and the associated weight gain become the primary features of this stage. The malfunctioning hypothalamus continues to signal these children that they are starving, regardless of how many calories they consume. Due to decreased muscle mass, they do not efficiently metabolize the calories they take in, and so their weight gain trajectory accelerates. Food craving becomes paramount, and any delay in their achieving gratification may be met with an explosive behavioral outburst. With your student, if his insatiable appetite has just begun to emerge, then it may account in part for some of his recent displays of aggressive behavior.
PWS is considered to be a spectrum disorder, so that not every symptom will be displayed by any single child, and each symptom may vary from mild to severe. Mild to moderate cognitive impairment is typically seen, as are learning disabilities. Speech onset is often delayed until two years of age, and poor articulation may persist throughout childhood.
The typical cognitive profile for children with PWS involves relative strengths in visual organization and perception, so they often do relatively well with reading and vocabulary. Spoken language is characteristically at a lower level than their comprehension. They experience significantly greater difficulty with auditory information processing, sequential processing, arithmetic, writing skills, both auditory and visual short term memory, and auditory attention span. Although some improvements in these areas may be seen over time, significant deficits will typically persist throughout childhood.
Compulsive behaviors and increased anxiety are manifested frequently by children with PWS. They characteristically display extreme stubbornness, temper tantrums, obsessions and compulsions (e.g. repetitive behaviors designed to reduce the anxiety caused by recurring intrusive thoughts). Psychotic symptoms and depression are seen much less frequently, affecting about 5-10 % of young adults with PWS.
Children with PWS seem to spend the majority of their time riding a behavioral and emotional roller-coaster, as they attempt to get their needs met. They always seem to be dealing with states of under-stimulation or over-stimulation, but rarely are able to achieve any sense of balance or harmony.
Although children with PWS often display a higher than normal pain threshold, their capacity for tolerating frustration may be very low. Consequently, it would be helpful to remain vigilant for early signs of frustration, as the behavioral outburst may occur in a very short period of time following the onset of frustration. It would also be helpful to consider whether any of the behavioral outbursts were associated with adults acting in a way which precluded your student from getting a basic need fulfilled.
The pattern of behavior you describe in your letter is certainly consistent with the behavioral pattern often observed in students with PWS. It sounds as though you and your colleagues are doing a very good job identifying your student’s needs and providing appropriate supports and services. Children with PWS characteristically present challenging behaviors which are easily triggered and unusually persistent. Try not to become overly discouraged by the fact that, despite all your efforts, your student continues to engage in problematic behaviors. It is quite likely that he is in the process of moving from stage 1 to stage 2 of the syndrome. If so, his behaviors, and the functions underlying those behaviors, can be “moving targets.”
Many of the most common behavioral difficulties associated with PWS revolve around food. Because those with PWS may eat to the point of gastronomic distress and still feel as though they are “starving,” they tend to engage in extreme behaviors to obtain desired food items, including manipulation, lying, and stealing food items.
However, dysregulated appetite is just one of many types of neurologically-based dysregulation experienced by children with PWS. While food-oriented behavioral difficulties are nearly universal, there is a wide range of individual variation in the severity and types of dysregulation experienced and behavior manifested by children with this syndrome.
Children with PWS frequently engage in reactive and volatile behaviors due, at least in part, to brain differences which compromise their ability to modulate sensations and emotions in response to both external and internal stimuli. Hypothalamic dysregulation is a prominent feature of Prader-Willi and impaired functioning of the hypothalamus disrupts the internal “thermostat” that helps modulate sensations and feelings in order to produce a comfortable state of equilibrium. Children with PWS rarely experience anything in moderation, but rather they tend to experience extreme states of stimulation and emotion in response to events or changes in their environment. Children with PWS can be extremely sensitive to stimuli around them and highly reactive to even minor changes in settings and routines. They can rarely maintain a restful state or experience any sustained sense of well-being, as such states depend upon the ability to maintain balance and stability.
These extreme states are often reflected in their behavior. Students with PWS are typically exceedingly reactive, irritable, excitable, and easily “set off” (with very low thresholds), and many of them display frequent and intense meltdowns. Their behaviors probably represent their attempts, usually unsuccessful, to achieve some degree of sensory and emotional equilibrium. Since these children have very limited capacity to adapt and can be obsessive, they may rigidly insist on doing things the same way every time.
Children with PWS are likely to perceive themselves to have very limited control over their own experience and to feel “at the mercy” of unpredictable elements in the environment and their own potent physical and emotional reactions to those elements. They crave predictability and often have a strong drive to try to control their environment, which includes the people with whom they interact. Their behaviors often interfere with successful adaptation to the school setting and limit their availability for learning. Although there is a strong biological component to his behaviors, environmental interventions can often reduce their intensity.
It is important to periodically remind everyone who works with the student that children with PWS are often highly impulsive and reactive, and their behaviors are most often spontaneous rather than being planned or deliberate attempts to disrupt or do harm. Following behavioral outbursts, the student may feel remorse and have the desire, but not the ability, to explain their actions or repair any damage caused by their behavior.
Recognize that behavioral outbursts are generally triggered by:
1) some stimulus in the student’s environment which is perceived to be unpleasant,
2) the student’s failure to get his way, receive the desired attention, or obtain the desired response from others. It may help to view the student as having to select from a limited array of marginally effective options in order to help him get his way or be able to exert control over his own life.
Dealing with behaviors triggered by something in the student’s environment
- Children with PWS can be extraordinarily sensitive to changes in their environment or routine that seem insignificant to others. They may react stongly to minor variations in their usual environment (e.g. a change in room temperature, a meal served somewhat earlier or later than usual, food prepared or presented slightly differently, altering the usual route taken to go somewhere, unfamiliar people/change in staff, etc.) Any change, even positive ones, may prompt impulsive/negative/explosive reactions.
- Due to their heightened sensitivity to change, they typically crave predictability. Since they often display inflexibility, they are highly dependent on predictable routines, settings, and people. They possess a limited ability to adjust to changes from what they are accustomed to or events which deviate even minutely from their anticipated course.
Some recommended strategies:
- Try to minimize the amount of change the student must respond to at any one time.
- Establish consistent expectations for all adults who work with the student.
- Encourage adults to respond to the student in a consistent manner in order to get consistent responses and improvments in behavior.
- Provide a highly structured learning environment tailored to the student’s cognitive level, as this is the setting most likely to elicit an optimal response.
- Clearly delineate the expected behavior, establish regular routines, and frequently enforce limits with respect to acceptable behavior.
- Use a neutral, matter-of-fact voice and manner when interacting with the student, because voice tone and gestures which convey excessive emotion may be misinterpreted or precipitate an over-reaction.
- Be firm, but, when possible, avoid words (e.g. “No” or “Don’t”) or gestures that may be interpreted as authoritarian or hostile and trigger a power struggle. Being overly sympathetic may prompt the child to infer that their plight is hopeless. Balancing the need to enforce limits consistently against the desire to avoid arguments may require the “Wisdom of Solomon.”
- When a regular schedule must be altered, warn the student and try to help him prepare for the change. When possible, avoid placing him under pressure, especially time pressure.
- Keep in mind his heightened sensitivity, in that when you talk to other people around him and not to him, he may feel slighted. Conversely, talking at length with him may make him feel singled-out or overwhelmed.
- Staff should present a “united front” whenever possible, as open dissension among school staff or others who work closely with the child on a frequent basis may throw the child with PWS “off kilter” or precipitate a “meltdown.”
When dealing with behaviors triggered primarily to get something:
Keep in mind:
- Use extreme caution in order to avert a power struggle. Students with PWS have a high need for some sense of personal control, and exert a great deal of effort to control everything they can about their environment, including the people in the environment. They often experience a constantly shifting set of needs and cravings along with a limited sense of personal control over their lives.
- Avoid words, looks, or gestures, which might be perceived as confrontational; and instead try to redirect the student by suggesting an alternate activity or behavior.
- Provide some choices, but limit the universe of choices to prevent overwhelming the student with too many options.
- Do not overestimate the student’s ability to meet demands. Even for students with PWS who possess higher intellectual ability, social and emotional immaturity often prevents them from exhibiting their cognitive abilities.
- Realize that the student is likely to have particular difficulty applying his abilitities and skills to solving problems or accomplishing desired goals in daily life. It would be unrealistic to expect that a child who functions well during brief tasks in controlled settings could function equally well with more complex tasks in less structured environments.
- Similarly, recognize that a student with PWS who shows some promising language abilities for a brief period under highly controlled conditions (testing), may not be able to demonstrate those same skills in social settings, due in part to being unusually susceptible to interference.
- Be cognizant that children with PWS have difficulty processing environmental stimuli, and their struggle to manage multiple inputs may deplete their finite supply of mental energy.
- Break messages into very small chunks given slowly and sequentially to allow the student to absorb what is being said.
- Accept that students with PWS have a high need for some sense of personal control.
- Don’t assume that your messages are encoded, processed and understood or that the student’s needs have been effectively communicated.
- Realize that the student may converse reasonably well on topics that are familiar, as they will have had a chance to rehearse specific vocabulary and organize their response. When the topic is unfamiliar and linguistically more complex and demanding, the student may become anxious and irritable. He may lack effective ways to organize, link and store novel information.
General recommendations for behavior management:
- All adults who are working with a child who has PWS need to agree upon and use consistent preventive and reactive practices and common instructional and intervention language across all environments.
- Prepare student at a time when he is available
- Use “if… then”…approach.
- Tell student the specific words or phrases adults will use to prompt him.
- Guide him toward a venting area and/or ‘heavy bag’ (big punching bag) at the first sign that he is becoming overwhelmed.
- Use fairly concrete teaching materials that are both interesting and at an age-appropriate level.
- Adjust activities to the student’s attention span, and very gradually increase the duration of his active learning periods.
- Model or demonstrate all desired behaviors. Use “think out loud” strategies to rehearse problem solving processes.
- Help identify the student’s “islands of competence,” and use the student’s particular interests to tailor his learning program.
Potential for Medical Intervention
Although there is currently no cure for PWS, medical intervention can sometimes be helpful. Hormone therapy may help lessen some of the more obvious features of growth hormone deficiency and hypogonadism, and thereby reduce some of the social and emotional stressors with which each child must cope. If obstructive sleep apnea is diagnosed, then continuous positive airway pressure may help normalize oxygen saturation levels through the sleep cycle and thereby improve brain functioning, increase the capacity for learning, and decrease agitation during waking hours.
This is clearly a challenging situation for you and your school. It is also quite frustrating for the student and his family. Hopefully, with cooperation between all parties, a near-optimal set of strategies can be identified and implemented. We wish you well in your continued efforts on behalf of this child and his family.
John L. Digges, MD, PhD, MPH, FAAP
Marji Stivers, PhD
Bullying and Students with Special Needs
Dear Ask A Specialist Readers,
Did you know that children with disabilities are at an increased risk of being bullied? Any number of factors: physical vulnerability, social skill challenges, or intolerant environments, may increase the risk. Research suggests that some children with disabilities may even bully others as well.
On April 3, 2012 the U.S. Education Secretary, Arne Duncan, and Health and Human Services (HHS) Secretary, Kathleen Sebelius, unveiled the revitalized Stop Bullying website: www.stopbullying.gov.
The Stop Bullying Website:
“The site encourages children, parents, educators, and communities to take action to stop and prevent bullying, and provides a map with detailed information on state laws and policies, interactive webisodes and videos for young people, practical strategies for schools and communities to ensure safe environments, and suggestions on how parents can talk about this sensitive subject with their children. The site also explores the dangers of cyberbullying and steps youngsters and parents can take to fight it.”
Special Resources to Help Children with Disabilities:
This website also provides special resources to help children with disabilities who are bullied or who bully others. The website illustrates how IEPs or Section 504 plans can be useful in designing specialized approaches for preventing and responding to bullying. Additionally, the website discusses how civil rights laws protect students with disabilities against harassment. That is, when bullying is directed at a child because of his or her disability and it creates a hostile environment at school, bullying behavior may cross the line and become “disability harassment.” Under Section 504 of the Rehabilitation Act of 1973 and Title II of the Americans with Disabilities Act of 1990, the school must address the harassment.
We know that bullying can negatively impact a student’s ability to learn and threaten their physical and emotional safety at school. We know, too, that the best way to address bullying is to stop it before it starts. The Stop Bullying website and the other websites listed in the Resources provide a number of actions school staff can take to make schools safer and prevent bullying.
We here at Ask A Specialist encourage you to do your part to help all students be safe at school.
Submitted by Ann England, Assistant Director, Diagnostic Center, Northern California on behalf of all the Ask A Specialist Contributors
- U.S. Department of Health and Human Services/U.S. Department of Education
- California Department of Education
- Centers for Disease Control and Prevention
- Positive Behavioral Interventions and Supports
- Striving To Reduce Youth Violence Everywhere (STRYVE)
- Surgeon General’s Report on Youth Violence
What is Klinefelter’s Syndrome? I have a student in my class with this disorder and would like to know how does this condition impacts his learning process?
We have a male student in the 8th grade who is tall and slim with a hand tremor, seems easily distractible, daydreams a lot, and does especially poorly in language arts. He’s not super active, in that he seems to tire easily. He appears to be quite shy, has good manners, and tries very hard to please others. Socially, he has difficulty gaining acceptance from his classmates. We have been told that he has a “sex chromosome disorder” called “Klinefelter’s.” Can you provide us with some information about this disorder? Also, how does this condition impact his learning process?
Klinefelter Syndrome is named for Dr. Harry Klinefelter, who reported in 1942 on a series of nine men who had symptoms related to underactivity of the testes. It was not until 1959 that the cause of this condition was identified to be an abnormality in the sex chromosomes. Patients with Klinefelter syndrome have the karyotype of 47, XXY; instead of the usual male karyotype of 46, XY. The extra “X” chromosome may result from receiving both an “X” and a “Y” chromosome from the father and a single “X” chromosome from the mother, or from receiving two “X” chromosomes from the mother and a “Y” chromosome from the father.
Whereas the genetic information of one “X” chromosome is typically suppressed when there are two “X” chromosomes (as in the case of a normal “XX” female); the presence of the “Y” chromosome seems to “unblock” many of the genes on the second “X” chromosome, resulting in increased “female” effects. Most 47, XXY males have sparse facial and body hair, wider hips than other boys, reduced muscle bulk, and increased breast development (in about ½ to ¾). They may also have long legs and a short trunk, delayed onset of puberty, and reduced energy levels.
This syndrome is the most frequent abnormality of the sex chromosomes in males, occurring about once in every 500 or so male births. The underdeveloped testes produce lowered amounts of testosterone, which in turn appears to contribute to many of the symptoms noted.
A wide range of IQ’s has been observed, and verbal IQ is often lower than performance IQ. Deficits in auditory memory and auditory processing are believed to contribute to the observed reduction in verbal abilities, which typically affects expressive language skills more than receptive language skills. Many boys with 47, XXY have problems with executive function, and they are diagnosed with AD/HD more frequently than boys with 46, XY chromosomes. They are often described as being friendly, kind and helpful; and tend to be unassertive, sensitive and quiet. They often rate themselves as being more apprehensive and insecure than their peers, and adolescents report an increased incidence of anxiety and depression.
From a medical perspective, it is important to have these children followed by a pediatric endocrinologist, who will oversee testosterone replacement therapy when it is indicated. Adolescents with low testosterone levels may respond to therapy by becoming more masculinized, and looking less different from their peers. This may result in improvements in self-esteem, behavior and mood.
Educational supports are warranted, especially to address any identified speech and language needs. If a child meets the DSM IV TR diagnostic criteria for AD/HD, then both non-pharmacologic and pharmacologic modalities may be helpful to reduce symptoms and improve academic performance. Once a diagnosis of AD/HD has been made, it would be critical to have the child’s care directed by a physician who is both knowledgeable about and experienced in the treatment of AD/HD in children and adolescents.
I hope this is helpful for you.
John L. Digges, MD, PhD, FAAP
Could it be that the social issues (lack of understanding of other peoples feeling) & their need to control issues associated with Aspergers are being made worse by the ADHD medicine? Or could we be giving these kids too much medicine trying to curb the inattention & distractibility due to Aspergers?
I'm in between a rock and a hard place! My son is almost eight years old and is in a regular 2nd grade class. He has been diagnosed as ADHD since before kindergarten. It's also been suggested that he has Asperger’s and/or NVLD, but we're exploring that (the more I read about it the more I'm positive he does).
Here's the problem: He has been taking either Adderall or Dexedrine since kindergarten for ADHD. His medicine does improve his attention some but a few hours after taking either of them he would get irritable, sometimes very violently. It’s like a balancing act to get enough of a dosage to help with attention but minimize the rages. When I talk to his doctor about it I feel like they're just saying, "Oh well.. that's how it works." My son is not the only one with this issue! The interesting thing about these other kids I've met with this problem is that they too have an autistic spectrum disorder as well as ADHD like my son.
Could it be that the social issues (lack of understanding of other peoples feeling) & their need to control issues associated with Asperger’s are being made worse by the ADHD medicine? Or could we be giving these kids too much medicine trying to curb the inattention & distractibility due to Asperger’s in addition to the same due to ADHD (because there are biologically different reasons for the inattention)?
I can't find out any definite information on this problem. Who would know about this? Can you help me understand?
When a child meets diagnostic criteria for both Attention Deficit/ Hyperactivity Disorder (AD/HD) and some disorder on the autism spectrum, treatment can be complicated. A number of non-pharmacologic strategies for children with executive function deficits have been developed*, and these should be initiated as soon as possible. The medication issue remains problematic, but there is some hope.
Stimulant medications constitute the class for which there exist the most persuasive evidence for both efficacy and safety in treating AD/HD. For children “on the spectrum,” however, standard doses may result in an increase in unacceptable side effects, such as you mentioned. An article by Handen et. al. in 2000 concluded that methylphenidate (MPH) can be efficacious in reducing the severity of AD/HD symptoms in children who have both AD/HD and autism. However, they caution that these children appear to be more susceptible to adverse side effects of methylphenidate such as increased irritability and anxiety.
A 2010 posting on the website www.travisthompson.net/ADHD/page25.html cites two additional pertinent studies on methylphenidate. Di Martino et. al. (2004) studied 13 children (average age about 8 years) diagnosed with both PDD and moderate to severe hyperactivity/impulsivity, who were given a single dose of 0.4 mg/kg of short acting methylphenidate. One hour later, 5 of the 13 students exhibited an increase in motor tics, hyperactivity, stereotypy or dysphoria and also were found to be minimally to much worse on the Clinical Global Improvement Scale. These five received no further treatment with MPH. Of the remaining eight students, half were rated as improved and half as unchanged. These eight were then placed on a 12 week trial of MPH. Two were unchanged after the first week, and the MPH was discontinued. The other 6 continued on MPH for the completion of the 12 week period, and displayed significant improvement in hyperactivity and impulsivity, but showed no change in measures of the core symptoms of autism. A study conducted by Santosh et. al. (2006) examined 226 children with AD/HD, some of whom also had ASD. Of the 226 children, 174 were initially evaluated retrospectively and 52 children were subsequently evaluated prospectively. Statistically significant improvements were found in both groups on the measures of hyperactivity, impulsivity, inattention, oppositionality, aggression, and intermittent explosive rage. The AD/HD-only group experienced significant amounts of nausea, headaches, giddiness, and sleep difficulties; while the AD/HD-ASD group experienced only an increase in sleep difficulties.
The two stimulant medications you mention, Dexedrine and Adderall, are chemically very similar. The core compound in Dexedrine is dextroamphetamine, while Adderall is a mixture of two salts of racemic amphetamine and two salts of dextroamphetamine. The dextroamphetamine molecule is 2-3 times as potent as the methylphenidate molecule, so it may be associated with an increase in side effects. I did not find any studies of children with AD/HD and ASD treated with dextroamphetamine.
One hypothesis I have entertained, but not yet tested, is that children on the spectrum may have some genetically predetermined tendency to be slow metabolizers of stimulant medications. This might account for the observation that they tend to respond well to low doses of stimulants, but to have a high incidence of dose-related side effects on even standard doses.
Another option would be to consider any of the three FDA approved non-stimulant medications currently available for the treatment of AD/HD symptoms: atomoxetine (Strattera), Guanfacine (Tenex or Intuniv), and clonidine (Catapres or Kapvay). The website cited previously reviews three pertinent studies. Jaselskis et. al. (1992) evaluated response to oral clonidine versus placebo in a study involving eight autistic male children (average age about 8 years old) with excessive levels of inattention, impulsivity and hyperactivity. They found improvement in teacher ratings of irritability, hyperactivity, stereotypy and inappropriate speech when on clonidine versus when taking a placebo. Posey et. al. (2006) evaluated 16 children on the autism spectrum (7 each with autism or Asperger’s disorder and 2 with pervasive developmental disorder) who averaged about 8 years of age. Improvements of “much” or “very much” on the Clinical Global Impressions Improvement Scale for AD/HD symptoms were noted in 75% of patients receiving atomoxetine (Strattera). In a study involving 12 boys and 4 girls ranging in age from 5-15 years who had ASD and prominent AD/HD symptoms, Arnold et. al. (2006) evaluated children on both atomoxetine and placebo. They found atomoxetine to be superior to placebo in reducing scores on the Hyperactivity subscale of the Aberrant Behavior Checklist and for improving the nine hyperactive/impulsive symptoms contained in the DSM-IV. However, they did not note any significant improvement in the nine inattentive symptoms contained in the DSM-IV diagnostic criteria for AD/HD.
I‘m afraid I did not find any data addressing the specific hypotheses you proposed. Thank you for your question, and I hope that what I could find is helpful.
John L. Digges, MD, PhD, MPH, FAAP
Behavioral Pediatrician, Diagnostic Center North