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John L. Digges, MD, PhD, MPH, FAAP
(Fellow of the American Academy of Pediatrics)
Behavioral Pediatrician

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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  • new!Implications of FASD for learning and behavior in 2nd grade student

Question:

I am a second grade teacher and we have a boy who has been diagnosed with Fetal Alcohol Syndrome. He continues to be non-compliant in class and display outbursts of emotion (which are usually verbal but can sometimes escalate to physical acts). We have gathered behavioral data and implemented many strategies in order to reduce the frequency and severity of his outbursts. Although these efforts have been somewhat successful, our focus has been on behavior management and keeping him and the other students safe.

Could you provide us with information about FAS? Also, are there any medications or strategies you can recommend that might help us to help him access the curriculum and experience more academic success?


Answer:

Thank you for your questions. The DCN behavioral pediatrician and clinical psychologist have prepared a joint response to your question. We will begin by considering the ways in which the consumption of alcohol by a pregnant woman might contribute to the problems you identify in your student.

Alcohol appears to be the most toxic of the substances used by expectant mothers (even more so than heroin, cocaine and methamphetamine). Damage is believed to result from any of a number of possible mechanisms, which include: direct teratogenicity during embryonic development; alterations in the ability of the fetus to synthesize proteins, prostaglandins, and hormones; reduction in nutrient and oxygen delivery to developing brain cells; and alteration in neurotransmitter levels in developing brain neurons. Each of these mechanisms can cause changes in brain structure and function.

How much damage occurs and what functions are affected are determined by a complex (and incompletely understood) interaction between multiple factors: the amount, pattern, timing, and duration of the exposure; genetic factors; mother’s general nutritional and health status, usage of other substances known to be toxic to developing brain tissue, and exposure to high levels of stress and or trauma during her pregnancy. The potential variability implicit in this complex interplay helps account for the broad spectrum of effects seen in children exposed to alcohol prenatally.

Diagnostic labels used to describe the effects of prenatal alcohol exposure have evolved over the last 3 decades. Terms used currently for diagnosis include Fetal Alcohol Syndrome (FAS), Partial Fetal Alcohol Syndrome (pFAS), and Neurobehavioral Disorder-Associated with Prenatal Alcohol Exposure (ND-PAE, which was previously known as Fetal Alcohol Effects (FAE) or Alcohol Related Neurodevelopmental Disorder (ARND)). The diagnosis of FAS means that a child was exposed to alcohol early enough in the gestation to result in changes to the developing face and skull as well as to the developing brain. The FAS diagnosis further specifies that the child will display reductions in stature, weight and head circumference; along with the classic findings of a smooth philtrum, thin upper lip and reduced distance between the medial and lateral canthi (inner and outer edges) of the eye. The last 3 features involve midline facial structures, the presence of which is highly correlated with underlying brain malformation and dysfunction. Reduced palpebral fissure length in particular has been shown to reflect a defect in forebrain development, and it is the forebrain which ultimately develops into the cerebral hemispheres (overall cognitive processes), hippocampus (learning and consolidation of memory), basal ganglia (time perception and cause-and-effect relationships), thalamus (efficient sensory processing), hypothalamus, (regulation and control of such bodily functions as temperature, hunger, thirst and rage), corpus callosum (integration between right and left hemispheres, e.g., processing visual and verbal input, emotions and logic), and frontal lobes (self-reflection, impulse control, planning, and working towards the completion of goals).

The great majority of children exposed to alcohol prenatally will not display the full features of FAS, but can manifest any degree of brain injury along the spectrum from mild to severe. If they have a confirmed history of prenatal exposure to alcohol and one or two of the three facial criteria (with or without growth deficiency) they can be diagnosed with pFAS. A third diagnostic category which has been proposed is Neurodevelopmental Disorder associated with Prenatal Alcohol Exposure (ND-PAE, which is currently coded as ICD-10-CM F88 “other specified neurodevelopmental disorder”/neurodevelopmental disorder associated with prenatal alcohol exposure). For convenience, all of the previous diagnostic entities (FAS, pFAS and ND-PAE) can be subsumed under the “umbrella” term Fetal Alcohol Spectrum Disorder (FASD). Since we are not certain of the specific features present in your student; and the neurodevelopmental challenges are not distinguishable between the various groups, we will use the term FASD throughout the remainder of this response.

Although all children with FASD will be expected to have some combination of brain deficits, only rarely will a child have impairments in all domains of function. Not surprisingly, the principle problem areas for children with FASD characteristically involve the following functional domains:

•           planning, sequential processing, and awareness of time;
•           learning, memory, and generalization;
•           spatial concepts and spatial memory;
•           social awareness and adaptive behavior; and
•           motor skills, including oromotor control.

Deficits in these areas of brain function will often manifest as challenges with:

•           remembering rules and following multi-step tasks;
•           remembering appointments or assignments;
•           interpreting social cues;
•           observing appropriate interpersonal boundaries;
•           participating in group activities without disrupting them;
•           processing information quickly and accurately; and
•           behaving appropriately with same age peers;
or
•           behaving socially at an age-appropriate level.

Almost all students with FASD will have significant deficits in social and emotional development, and the discrepancy in social skill level between them and their peers will generally increase as they grow older. Despite having deficits in any or all of these areas, students with FASD do learn. Most of them develop basic academic skills during early elementary school and continue to develop their talents and master new skills in their areas of interest throughout their lives. They are often sociable, humorous and fun to be around. However, because most students with FASD have unusual patterns of ability (relative personal strengths and weaknesses), their behavior can seem unpredictable and confusing. It can be easy for adults and peers to lose patience if they do not know what to expect.

Students with FASD often present initially as being more capable and neuro-typical in their development than they actually are. They can acquire reasonably extensive vocabularies and may become competent at telling simple stories and anecdotes. Many have little shyness or fear of strangers, enjoy chatting with people they meet, and demonstrate a relative strength in the area of expressive language.

Only a small percentage of these children will score in the intellectual disability range at an early age. Most will score in the “below average” to “high average” range during their first years of elementary school. If they have repeated testing, their scores tend to decrease over time. Children with FASD typically do not develop complex, abstract thinking and problem-solving skills at the same rate, or to the same extent, as their peers. However, even when they get older, despite experiencing mild declines in their test scores, their cognitive and academic test scores often overestimate their level of functioning and underestimate their level of need for structure and supervision in daily life. Their deficits in social and emotional development make it increasingly difficult for them to adapt appropriately to increasingly complex environments where they are confronted with higher-level demands and expectations.

Students with FASD characteristically have difficulty generalizing and applying skills learned in a specific context to challenges they face in everyday life. Some have trouble with sequential processing, making it difficult to follow a routine unless they have a visual depiction of it in front of them. Sequential processing difficulties make it more difficult to develop a sense of time and to recognize cause-and-effect patterns. They do learn from experience, but often require considerable repetition.

Although they may learn to read and write and to communicate verbally with others; as they grow older, the concreteness and lack of variety or complexity in their communication becomes more apparent. It has been observed that many individuals with FASD seem not to demonstrate (or less reliably demonstrate) “good judgement” or “common sense.” Although these qualities are difficult to define operationally or measure, most observers can recognize the deficits when they are present. Even with explicit teaching, learning to exercise good judgement in their daily lives can be a formidable lifelong challenge.

Deficits in judgement often present as being more severe in individuals with FASD and no intellectual disability (ID) than in individuals with mild ID. Their lack of “good judgement” and “better than expected” superficial communication skills place students with FASD at increased risk of experiencing violence and trauma, being victimized, putting selves at risk of accidental injury or death (e.g., walking in front of traffic without checking), repeated involvement with the legal system, developing mental health conditions, and suicide.

Fortunately, there are resources available to help children with FAS, and many will be eligible for Regional Center Services in California. Most recommended interventions emphasize the need to provide a supportive, non-traumatizing environment which provides a safe place for them to grow and which recognizes and builds upon each student’s individual strengths. Having at least one adult they feel they can depend upon in any stressful situation is viewed as critical to their success. Additionally, identifying one or more “islands of competence” can be exceedingly helpful for them to experience success and develop a positive self-image. These elements can provide a basis upon which to customize a modified curriculum which builds upon the student’s own interests and strengths. Being exposed to supportive and patient adults will be key to achieving the highest attainable degree of success.

Even though there is no “cure” for the symptoms associated with FAS, early diagnosis followed by therapeutic intervention is crucial. Medications can play a role in addressing certain symptom complexes which are often seen in children with FAS. Unfortunately, the ADHD-like symptoms exhibited by students with FAS often do not respond well to stimulant preparations, which represent the mainstay of pharmacological treatment of symptoms in children with ADHD who were not exposed to alcohol prenatally. Non-stimulant preparations such as extended release guanfacine (trade name Intuniv), extended release clonidine (trade name Kapvay) and atomoxetine (trade name Strattera) are possible choices to help reduce their symptoms. A team approach is optimal, and should include a physician with experience in treating the symptoms often experienced by children with FAS. Although these physicians may be found in any community, they will often be associated with medical centers and teaching hospitals.

Thank you for your questions, and we hope your student receives the help he needs.

John L. Digges, MD, PhD, MPH, FAAP
Behavioral Pediatrician
Margaret Stivers, PhD
Clinical Psychologist
Diagnostic Center North
Fremont, California


  • An increase in ADHD symptoms despite continuing on Concerta

Question:

We have a 7 year old male student with ADHD, who showed significant improvement after he was started on Concerta last year. His dose was gradually increased to 36mg over a few weeks, and he has been doing well for several months.

Recently after returning from Winter Break, his old symptoms have returned. We thought he was just having trouble getting back into the school routine, but his distractibility, difficulty initiating work both in school and for homework, and his impulsive behaviors have resurfaced and persisted. Can you offer any possible explanations?


Answer:

When I hear of an ADHD student having experienced improvement on medication followed by a noticeable return of ADHD symptoms, I wonder about any changes which may have occurred. For your student, has there been any significant change in his life (injury, illness, incarceration or death of a family member; loss of employment of one or both parents; having to move residences; etc.)? Has the school situation changed significantly (new teacher, best friend moved away, being bullied, course material overwhelming, etc.)? An acute loss or other stressor can cause substantial disruption in a child’s routine, and may divert their interest and energy away from school work. If discreet inquiry reveals the child has experienced such a loss or stressor, providing the child with access to resources and emotional support to address the loss/stressor can be quite helpful. Is he continuing to take his medications as prescribed? After experiencing success on the medication, children (or parents) sometimes believe they can maintain the improvement without the medication, and stop taking it.

Another possible cause relates to the specific medication he was prescribed. All stimulant preparations used to treat ADHD can be viewed as consisting of first, a molecule and second, a release mechanism; each of which will affect the efficacy of the preparation. The product he was prescribed, Concerta, is the brand name for d,l-methylphenidate, and employs the OROS (osmotic release oral system) delivery method. If the physician writes the prescription so that it allows for generic substitution, there have been 3 preparations from which the pharmacist could choose. There is one generic that uses the same molecule and the same release mechanism, but there are two generic products that use the same molecule but a different release mechanism.

The OROS release mechanism consists of a non-soluble tablet which has a laser drilled hole at one end, 3 equal sized chambers internally, and a coating applied to the outside of the tablet which contains 22% of the total dose. An additional 26% of the total dose is contained in a slurry in the first chamber, while 52% of the total dose is compressed into a slurry in the second chamber. The third chamber contains a non-soluble fiber matrix (“push compartment”).

After the tablet is swallowed, the coating with 22% of the total dose dissolves rather quickly, and the medication then enters the blood stream in about 45 minutes or so. Once the coating has dissolved, the tablet becomes semi-permeable to water, allowing water to enter into the third compartment and interact with the matrix. The matrix then starts to expand at a predictable rate, pushing the medication slurry through the opening adjacent to the 1st compartment. The 26% contained in this compartment leaves the tablet over a ~3-4 hour period, followed by the 52% in the second compartment also being pushed out over a ~3-4 hour period. This elegant arrangement allows for a smooth upslope of medication in the blood for about 6-9 hours after ingestion.

It is this period of “upslope” of the pharmacokinetic curve which is associated with the improvements seen in focus and resistance to distraction. (The reduction in non-goal-directed motor activity excess is attributed to the higher concentration of medicine in the system, so it roughly equates to the middle portion of the curve). Tablet preparations which do not employ the OROS system generally produce a similar upslope of the curve for the first 2 hours, but then produce a plateau for the next 4-5 hours. This results in improved focus for the first 2 hours, but is typically followed by a period where the concentration is high enough to reduce non-goal-directed motor activity excess; but the lack of upslope results in a lack of improvement in the symptoms of inattention, easy distractibility, and difficulty with both initiating and completing work.

In November 2014, the FDA recognized the lack of equivalence between the two non-OROS generics and the brand name Concerta (made by Janssen) or the OROS generic (made by Janssen but marketed by Actavis under a licensing agreement). The FDA changed their therapeutic equivalence code from “AB” (equivalence) to “BX” (data are insufficient to determine therapeutic equivalence). Additionally, the FDA requested the other two manufacturers to either voluntarily withdraw their products from the market, or within six months, provide data to confirm bioequivalence. They did neither, but pharmacists were permitted to continue to fill “Concerta” prescriptions that allowed for generic substitution with the two non-bioequivalent products. In November of 2016, the FDA proposed to withdraw approval of the two non-equivalent products, a process likely to result in only OROS products being able to be sold as bioequivalent to Concerta.

It is possible, then, that the child’s increase in ADHD symptoms may reflect a change in the preparation he received from the pharmacy at his most recent refill. The Concerta-equivalent product will have either Janssen or Actavis on the label, and the tablet will have “ALZA” imprinted on it. If the label has another company listed and the tablets do not have “ALZA” imprinted on them, then the tablet is not bio-equivalent with Concerta.

I hope this is helpful, both for your student and potentially for other students that have been affected by the substitution of non-bioequivalent products for Concerta.

John L. Digges, MD, PhD, MPH, FAAP Behavioral Pediatrician, Diagnostic Center North, Fremont, California


  • Making sense of a child with features of ASD, ADHD and Williams Syndrome (WS), but none of the facial features typically associated with WS

Question:

We have an 8 year old student who functions cognitively at about the kindergarten to first grade level. His strengths seem to be in rote memory recall and expressive language. He has been diagnosed with Attention Deficit/Hyperactivity Disorder (ADHD) and also has some autistic-like features. He is very hyperactive, easily distractible, unable to complete assigned work, and seems oblivious that the ringing bell means it’s time to line up. He has enormous difficulty transitioning from one activity to the next, and he is quite impulsive. He also perseverates a lot, often displays echolalia, flaps his hands frequently when excited, tantrums and screams loudly when he doesn’t get his way, swats at others when annoyed by them, and seems to exist in a world of his own. He typically spends time outside engaging in sensory-stimulating activities, such as repetitively throwing small pieces of bark into the air and watching them fall to the ground. While inside, he appears enthralled by watching the sand fall from the top portion of the container to the bottom through the narrowed orifice of the hourglass timer.

Despite having features consistent with autism, he seems to be very social. He will point to preferred objects to attract the attention of others, appears to enjoy sharing with others and sometimes talking with peers or adults, but often he just seems to be talking to no one in particular. These comments that are unrelated to the task at hand are very disruptive in the classroom, and comments appear to contribute to his being ignored by his peers or being referred to as “weird.”

His pediatrician believes he has Williams Syndrome and not autism, but confirmatory genetics studies have not yet been completed. My concern is that when I Google Williams Syndrome, the description provided seems to fit our student, but he does not look anything like the pictures they show as being characteristic of Williams Syndrome. Could you help us make sense of this confusing picture?


Answer:

Perhaps I can. Children with Williams Syndrome (WS) are missing variable portions of a particular region located on the long arm of the 7th chromosome (7q11.23). This region is suspected of coding for about 20 or so genes. The variable presentation of WS is believed to reflect which specific genes are missing (deleted) from the normal 7th chromosome. For example, the absence of the gene known as GTF2IRD1 is believed to be responsible for producing the characteristic facial features of WS: broad forehead, prominent “starburst” or white lacey pattern on the colored portion of the eye (iris) in blue and green eyed children, shortened distance between each corner of the eye (short palpebral fissure), puffiness around the eyes/full cheeks, small upturned nose, depressed nasal bridge, prominent/full lips, an open mouth, and small chin. Children missing a portion of 7q11.23 but still retaining GTF2IRD1 could have WS but lack the characteristic facial findings.

Features such as strong rote memory skills, well developed spoken language, outgoing and engaging personality coupled with displaying an extreme interest in other people are all consistent with WS. Children with WS will often manifest mild to moderate intellectual disability, learning problems, hyperactivity, distractibility, impulsivity, anxiety and phobias. Fine motor skills and spatial relations are often weak in children with WS.

Other characteristics frequently seen in children with WS include some type of heart or great vessel problem, elevated calcium levels in the blood, feeding difficulties in early childhood, abnormal dentition, problems involving kidney structure or function, hernias, joint laxity, and ultra-sensitive hearing (hyperacusis, or painful sensations associated with certain sound frequencies or intensity). Many children with WS, in addition to their extreme sociability and politeness, will often display heighted interest in or ability involving music.

With respect to autistic features, there are numerous reports of overlap between characteristics of WS and autistic spectrum disorder (ASD). Individuals in both groups display language delays, developmental delays, problems with motor skills, hypersensitivity to sounds, perseveration, and the tendency to be “picky” eaters. However, the two groups represent polar opposites when it comes to “social profile,” with the WS children displaying hypersociability and the ASD children characterized by social avoidance. Whereas excessive sociability and an extreme interest in interacting with people represent the core behavioral features of WS, impairments in social functioning and verbal communication are consistently observed in children with ASD. Children with WS typically possess elaborate vocabularies and affect-rich expressive language, whereas children with ASD almost universally manifest a deficit in the “communicative use” of language.

It would appear that your student’s excessively social personality would preclude a formal diagnosis of autism; but together with his developmental delays, ADHD-like symptoms and well developed speech, may very well be consistent with Williams Syndrome. This diagnosis can be confirmed by array CGH (comparative genomic hybridization) testing. Knowing the diagnosis can also alert the physician and family to check for comorbid conditions associated with it.

Thank you for your question, and I hope this helps.

John L. Digges, MD, PhD, MPH, FAAP
Diagnostic Center - Northern California


  • Multiple considerations may affect “correct” dosage of ADHD medications.

Question:

My 9 year old son has been on 50 mg of Strattera for about 2 years. Within the past two weeks, our family and the school saw changes in his behavior.  I took him to his doctor and my son weighed 70 pounds. The doctor changed his dose to 80 mg per day. Is this too much?

Thank you,
Amber


Answer:

Thank you for your question. The answer is not straight forward, as it depends upon the particular circumstances and what is meant by “too much.”

I will assume your son is being treated for ADHD, but I do have a few questions.

Do you know whether he was diagnosed with predominantly inattentive presentation or combined presentation? 

Has he been diagnosed with/treated for any other physical or mental health conditions?

What non-pharmacologic strategies are in place to support your son’s presumed executive function deficits?

Has he been treated previously with any other medications for his ADHD? If so, what was his response?

Is he currently taking any other medications in addition to his Strattera?

Have there been any significant changes in his physical or social environment or his emotional status recently?

Has he experienced a high number of A.C.E.’s (adverse childhood events)?

The 50 mg per day dose he was taking while he weighed 70 pounds (~32 kg) works out to ~1.6 mg/kg/day. An 80 mg per day dose for a ~32 kg boy works out to ~2.5 mg/kg/day. According to the prescribing information available on-line for Strattera (2007), the recommendation from the manufacturer (Eli Lilly) for dosing atomoxetine in children and adolescents is to not exceed 1.4 mg/kg/day or 100 mg, whichever is less. Studies have been conducted using does as high as 2.0 mg/kg/day, with no untoward effects observed on the higher doses. However, there was no additional benefit noted from doses in excess of 1.2 mg/kg/day. A dose of 2.5 mg/kg/day is above the dose for which there are safety data available for children and adolescents.

If your son had co-existing conditions such as bipolar disorder or schizophrenia, or displayed unacceptable side effects to multiple trials of various stimulant preparations, or was unable to tolerate guanfacine; and appeared to have significant symptom reduction only in response to atomoxetine, then it might be reasonable to exceed the recommended dosage guidelines in order to provide symptom relief. I would have recommended a more gradual titration; however, such as 1-2 months on 60 mg/day (~1.8 mg/kg/day). When your son weighs 80 pounds (~36 kg), a dose of 70 mg/day would equal ~1.9 mg/kg/day.

The questions I raised about non-pharmacological strategies in place, environmental stressors that your son may be experiencing, and whether he was exposed to adverse childhood events were to explore whether appropriate supportive strategies were already in place, whether any environmental stressors might be contributing to the behavior changes you and the school observed, or whether exposure to trauma during his early development might be affecting his behavior. If for example, he was not receiving adequate accommodations to support his efforts to deal with his ADHD symptoms, then initiating them might lead to a reduction in his symptoms and improvement in his behavior. If he had been exposed to bullying or had suffered significant emotional loss, then I would suggest that those concerns be addressed and appropriate interventions be directed towards relieving the stress he has been experiencing. If he had indeed experienced a high number of adverse childhood events, then referral to a specialist in this area might also result in improvements in his behavior.

Finally, after appropriate non-pharmacological strategies are in place, and assuming your son did not have contraindications to stimulant medication or guanfacine, then I would recommend that you and your physician enter into a discussion about the possibility of considering either of these classes of preparations instead of trying doses of atomoxetine in excess of those for which we have at least some safety data. I hope these comments are of some help to you and your son.

John L. Digges, MD, PhD, MPH, FAAP
Behavioral Pediatrician
Diagnostic Center North