Guanfacine

Cite this article as:
Mary Hardimon. Guanfacine, Don't Forget the Bubbles, 2017. Available at:
https://doi.org/10.31440/DFTB.13727

On 22nd August 2017, guanfacine hydrochloride (current sole brand name Intuniv) was accepted as a new chemical entity by the Therapeutic Goods Administration in Australia.1 Whilst new to Australia, guanfacine has been available within the United States of America and Europe since 20102 and 20153 respectively. The development of generic brands within these countries has seen increased uptake of this medication as an alternative to stimulant medications.

 

So what is it?

Guanfacine is an alpha 2 agonist. Unlike clonidine (which is non-selective and shows high affinity for all 3 subtypes of alpha 2 receptors – A, B and C), guanfacine has preferencial affinity for alpha 2A receptors. Stimulation of these receptors in the prefrontal cortex mimics noradreline/norepinephrine actions in this region, with current ADHD (Attention Deficit Hyperactivity Disorder) causal theories demonstrating noradrenergic dysfunction as underlying the cognitive and behavioural manifestations of ADHD.4

https://www.priory.com/psychiatry/clonidine.htm

 

Indications and Usage

Under current licensing in Australia, guanfacine is indicated for “the treatment of attention deficit hyperactivity disorder (ADHD) in children and adolescents 6-17 years old, as monotherapy (when stimulants or atomoxetine are not suitable, not tolerated or have been shown to be ineffective) or as adjunctive therapy to psychostimulants (where there has been a sub-optimal response to psychostimulants).”

The use of guanfacine “must be used as part of a comprehensive ADHD management programme, typically including psychological, educational and social measures.1

These stipulations encourage prescribers to manage ADHD within a bio-psycho-social context, understanding that in the vast majority of cases, the condition whilst caused by a neurotransmitter imbalance may be attenuated by psychological and social strategies.

Shire Australia (whom is licensed for its distribution) recommends its use in children and adolescents 6 – 17 years of age.6 There are very limited studies7 surrounding guanfacine use in children younger than 6 years and whilst efficacy has been suggested, caution would be suggested in making ADHD diagnoses particularly in toddler years due to a great variety in “normal” in this period.

 

Dosage and Administration

Guanfacine will be released in 1mg, 2mg, 3mg and 4mg modified release tablets with administration once per day orally. Tablets should not be crushed or dissolved. Guanfacine should not be taken with high fat meals as this significantly affects absorption.

The recommended initial dose is 1mg (when used as either monotherapy or co-administered with stimulants). Dose adjustments are recommended by no more than 1mg/week with a target dose range (or based on therapeutic effect should it occur prior to this dose) of 0.05 – 0.12mg/kg/day. Doses exceeding 4mg (co-administration) and 7mg (when used as monotherapy) have not been evaluated.8

 

Recommended target dose range for maintenance therapy when guanfacine is sole agent8

Weight Target dose range (0.05 – 0.12 mg/kg/day)
25.0-33.9 kg 2-3 mg/day
34.0-41.4 kg 2-4 mg/day
41.5-49.4 kg 3-5 mg/day
49.5-58.4 kg 3-6 mg/day
58.5-91.0 kg 4-7 mg/day
≥91.0 kg 5-7 mg/day

 

Adverse effects

The most common adverse effects9 include:

  • Somnolence
  • Sedation
  • Abdominal pain
  • Dizziness
  • Hypotension
  • Dry mouth
  • Constipation

In contrast to stimulant medication, weight gain (mean of 0.5kg) is seen in patients using guanfacine.

Less common (although clinically significant) side effects10 include:

  • Atrioventricular block
  • Asthenia and chest pain
  • Increased ALT
  • Convulsion
  • Increased urinary frequency
  • Hypertension
  • Pallor

 

Drug interactions

CYP3A4 inhibitors (such as ketoconazole) and CYP3A4 inducers (such as rifampin) may affect guanfacine blood levels and subsequent clinical response.10

 

Cost

Since being introduced onto the PBS 1st September 2018, the previously prohibitive costs are now more affordable for families (although still quite expensive without a health care card) – ~$40 regular PBS price ~$6 concession PBS price.

 

References

1 https://www.tga.gov.au/prescription-medicines-registration-new-chemical-entities-australia

2 https://www.drugs.com/newdrugs/shire-announces-fda-approval-once-daily-intuniv-guanfacine-extended-release-adhd-children-1599.html

3

4 https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3676929/

5 https://stahlonline.cambridge.org/content/ep/images/85702c17_fig24.jpg

6 https://www.guildlink.com.au/gc/ws/zi/pi.cfm?product=zipintun10817

7 https://onlinelibrary.wiley.com/doi/10.1002/%28SICI%291097-0355%28199723%2918:3%3C300::AID-IMHJ6%3E3.0.CO;2-Q/abstract

8 https://www.ebs.tga.gov.au/ebs/picmi/picmirepository.nsf/pdf?OpenAgent&id=CP-2017-PI-02254-1&d=2017110516114622483

9 https://www.accessdata.fda.gov/drugsatfda_docs/label/2009/022037lbl.pdf

10 https://pi.shirecontent.com/PI/PDFs/Intuniv_USA_ENG.pdf

Additional useful websites:

  1. https://dailymed.nlm.nih.gov/dailymed/drugInfo.cfm?setid=b972af81-3a37-40be-9fe1-3ddf59852528
  2. https://pi.shirecontent.com/PI/PDFs/Intuniv_USA_ENG.pdf
  3. https://www.cms.gov/Medicare-Medicaid-Coordination/Fraud-Prevention/Medicaid-Integrity-Education/Pharmacy-Education-Materials/Downloads/stim-pediatric-factsheet11-14.pdf
  4. https://www.guildlink.com.au/gc/ws/zi/pi.cfm?product=zipintun10817

The 8th Bubble Wrap

Cite this article as:
Grace Leo. The 8th Bubble Wrap, Don't Forget the Bubbles, 2017. Available at:
https://doi.org/10.31440/DFTB.12183

With millions upon millions of journal articles being published every year it is impossible to keep up.  Every month we ask some of our friends from the world of paediatrics to point out something that has caught their eye.

Schrodinger’s PANDAS

Cite this article as:
Henry Goldstein. Schrodinger’s PANDAS, Don't Forget the Bubbles, 2017. Available at:
https://doi.org/10.31440/DFTB.11670

The existence of PANDAS and other Paediatric Acute Onset Neuropsychiatric Syndromes has been controversial for the last two decades.

 

What is PANDAS?

Paediatric Acute-Onset Neuropsychiatric Disorder Associated with Streptococcal infection, is a concept first mooted by Swedo, an American paediatrician in 1998, as a formé fruste (or incomplete form) of Sydenhams Chorea.

Swedo SE, Leonard HL, Garvey M, et al. Pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections: clinical description of the first 50 cases. Am J Psychiatry. 1998 Feb;155(2):264-71.

She postulated that Group A Beta-Haemolytic Strep infection caused sudden onset explosive OCD symptoms with choreiform movements in prepubertal children. The criteria is: (click for quotes from Swedo’s original wording)

1. Must have OCD or Tic Disorder.

Presence of OCD and/or a tic disorder: The patient must meet lifetime diagnostic criteria (DSM-III-R or DSM-IV) for OCD or a tic disorder.”

2. Pre-pubertal.

 “Pediatric onset: Symptoms of the disorder first become evident between 3 years of age and the beginning of puberty (as is generally true for rheumatic fever).”

3. Abrupt/Explosive Onset.

 “Episodic course of symptom severity: Clinical course is characterized by the abrupt onset of symptoms or by dramatic symptom exacerbations. Often, the onset of a specific symptom exacerbation can be assigned to a particular day or week, at which time the symptoms seemed to “explode” in severity. Symptoms usually decrease significantly between episodes and occasionally resolve completely between exacerbations.”

4. Association with GABHS infection.

Association with GABHS infection: Symptom exacerbations must be temporally related to GABHS infection, i.e., associated with positive throat culture and/or elevated anti-GABHS antibody titers. Of note, the temporal relationship between the GABHS infection and the symptom exacerbation may vary over the course of the illness. In rheumatic fever, there is often a delay of 6–9 months between the last documented GABHS infection and the appearance of symptoms of Sydenham’s chorea; however, recrudescences follow the GABHS infections at a much shorter interval, often with a time lag of only several days to a few weeks . It appears that the pattern is similar for PANDAS. It should be further noted that because fever and other stressors of illness are known to increase symptom severity, the exacerbations should not occur exclusively during the period of acute illness. Furthermore, as in Sydenham’s chorea and rheumatic fever, some symptom recurrences may not be associated with documented GABHS infections, so the child’s lifetime pattern should be considered when making the diagnosis.

5. Abnormal neuro exam, but NOT chorea.

”Association with neurological abnormalities: During symptom exacerbations, patients will have abnormal results on neurological examination. Motoric hyperactivity and adventitious movements (including choreiform movements or tics) are particularly common. Of note, children with primary OCD may have normal results on neurological examination, particularly during periods of remission. Further, the presence of frank chorea would suggest a diagnosis of Sydenham’s chorea, rather than PANDAS. It is particularly important to make this distinction, since Sydenham’s chorea is a known variant of rheumatic fever and requires prophylaxis against GABHS; PANDAS does not.”

Think of it as rheumatic fever for the brain; specifically, the basal ganglia.

So, a specific set of patients, with a well-stated mechanism and a clear symptom cluster. That said, this diagnosis and its criteria have been mired in controversy throughout the last twenty years, due predominantly to incongruity, poor external validity and erratic reproducibility in a number of trials.

Given the many billions of words on the topic, there have been some agreed points and areas of controversy. Hence, it is generally agreed that;

  • Children with signs and symptoms compatible with Group A H Beta-Haemolytic Strep (GAS) infections should be evaluated for same.
  • GAS is one of several factors that can exacerbate OCD or tic disorder in a subset of patients.
  • Children with GAS infection and OCD/tic disorder require standard treatments for these problems (regardless of whether GAS and OCD/tic disorder are causally associated)
  • There is no indication for routine administration of the following therapies for children who meet PANDAS criteria; prophylactic antibiotics, steroids, plasma exchange, IVIG.

Controversy exists regarding;

  • It is unclear whether PANDAS is a discrete neuropsychiatric disorder sufficiently different from OCD/tic disorder to be considered a separate entity.
  • The role of GAS as a precipitant of OCD/tic disorders (+/- PANDAS), and whether this is a causal or incidental relationship.
  • The etiology of PANDAS as an autoimmune disorder.
  • The clinical utility of seeking evidence of GAS infection in children with OCD/tic disorders.

In 2010, the PANDAS criteria was redefined and re-labelled to Paediatric Acute Onset Neuropsychiatric Syndrome (PANS). This widened the age range to <17 years, added restricted food intake as an alternative major symptom, and postulated a much broader aetiology, including a larger number of infectious causes including mycoplasma, HIV, VZV, HSV and the common cold.

Chang K, Frankovich J, Cooperstock M, et al. Clinical evaluation of youth with pediatric acute-onset neuropsychiatric syndrome (PANS): recommendations from the 2013 PANS Consensus Conference. J Child Adolesc Psychopharmacol. 2015 Feb;25(1):3-13. doi: 10.1089/cap.2014.0084. Epub 2014 Oct 17.

By 2013, Singer proposed a move away from the term PANS to Childhood Acute onset Neuropsychiatric Syndrome (CANS), further amending the diagnostic criteria to broaden the causality and focus investigations on the exclusion of treatable causes. When none are found a diagnosis of Idiopathic CANS can be made.

Singer, HS., Gilbert DL., Wolf, DS., et al. Moving from PANDAS to CANS. J Pediatr. 2012 May;160(5):725-31. doi: 10.1016/j.jpeds.2011.11.040. Epub 2011 Dec 22.

 

So, what are the challenging parts of this diagnosis?

Firstly, it looks like OCD, and OCD is very common. 1 in 60 kids have an OCD diagnosis

CANS is extremely rare by comparison. Actually, it’s unclear the actual incience, but it is less prevalent than either choreiform disorders or Munchausen’s By Proxy, which are around 1 per 100,000.

Secondly, it’s supposed to be foudroyant, which means literally strikes as with lightning, sudden and overwhelming in effect, stunning and dazzling.”

This in itself is a challenge to identify, as everything must start somewhere. It is the point of noticeable symptoms that really matters. To extend the metaphor, we don’t actually have random lightning strikes, as realistically the weather changes, a storm rolls in, thunder is heard in the distance and then… bang.

Thirdly, Sydenham chorea, obsessive-compulsive disorder, and tic disorders share common anatomic areas: the basal ganglia of the brain and the related cortical and thalamic sites. Some patients with Sydenham’s chorea display motor and vocal tics, obsessive-compulsive symptoms, and ADHD symptoms, adding support to the possibility that, at least in some instances, these disorders share a common etiology.

Fourthly, a review by Murphy notes a study of PANS-OCD versus Non-PANS OCD which identifies an association between Non-PANS OCD and a Family history of same.

Murphy TK, Gerardi DM, Leckman JF. Pediatric acute-onset neuropsychiatric syndrome.Psychiatr Clin North Am. 2014 Sep;37(3):353-74. doi: 10.1016/j.psc.2014.06.001.

Murphy and colleagues suggest this could be seen as an association between the infectious/autoimmune trigger for PANS-OCD. The countervailing argument is that this is instead representative of tolerability of OCD symptoms within families. That is, by the time the child is functionally impaired and the diagnosis made, it’s not controversial and there is acceptance of the diagnosis within the family.

Fifthly, it’s worth noting that the data for most studies is from the USA, where entry into the health system is very different to Australian, New Zealand or UK, particularly around self-referral to specialists. I’d speculate that it is much more socially acceptable for a child to have a neurologic than a psychiatric condition. Thus, children with OCD with or without tics are might present to a neurologist and be more likely to receive a CANS diagnosis.

Pragmatically for general paediatricians, the diagnostic dilemma here is two-fold;

  1. How much to investigate?
  2. How can we treat it?

And this is where Schroedinger comes in. Every time this box is opened, unless we are able to do so in an incisive, foudroyant manner, the child risks becoming medicalised. Indeed, we risk leading them on that journey. By simply looking into the box, we’re disproportionately more likely to find PANDAS than a child with OCD.

I don’t know how to prevent this happening. I do know that the best care for patients with suspected CANS could include general paediatrics, psychiatry, neurology and rheumatology. Ideally, these teams would collaborate to consider diagnosis and management on a case by case basis.

Medical treatments for this constellation of diagnoses have included antibiotics, steroids, immunoglobulins and plasmapheresis. Recent recommendations have highlighted the paucity of evidence for any of these therapies.

Psychiatric treatments include clomipramine, SSRI and Cognitive Behavioural therapy, as for obsessive compulsive disorder; I have not yet seen robust evidence with these interventions specifically for CANS (if you have some, please link in the comments!)
In summary, a child with explosive onset obsessions, compulsions with or without tics might have CANS. Although, on balance, they are more likely to have OCD, considering the prevalence in the population. We need to keep an open mind about these presentations.

 

Selected references

Murphy TK, Gerardi DM, Leckman JF. Pediatric acute-onset neuropsychiatric syndrome.Psychiatr Clin North Am. 2014 Sep;37(3):353-74. doi: 10.1016/j.psc.2014.06.001.

Swedo SE, Leonard HL, Garvey M, et al. Pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections: clinical description of the first 50 cases. Am J Psychiatry. 1998 Feb;155(2):264-71.

Pichichero, ME. PANDAS: Pediatric autoimmune neuropsychiatric disorder associated with group A streptococci. UpToDate [Online database]. Accessed 22 Feb 2017.

Chang K, Frankovich J, Cooperstock M, et al. Clinical evaluation of youth with pediatric acute-onset neuropsychiatric syndrome (PANS): recommendations from the 2013 PANS Consensus Conference. J Child Adolesc Psychopharmacol. 2015 Feb;25(1):3-13. doi: 10.1089/cap.2014.0084. Epub 2014 Oct 17.

Singer, HS., Gilbert DL., Wolf, DS., et al. Moving from PANDAS to CANS. J Pediatr. 2012 May;160(5):725-31. doi: 10.1016/j.jpeds.2011.11.040. Epub 2011 Dec 22.

Adolescent Inpatient Psychiatry

Cite this article as:
Henry Goldstein. Adolescent Inpatient Psychiatry, Don't Forget the Bubbles, 2017. Available at:
https://doi.org/10.31440/DFTB.11391

I’ve been lucky enough to be one of the few paediatric registrars allocated to an Adolescent Inpatient Mental Health ward for a 6 month rotation. Although I’d worked in (adult) Psych wards before, I had few well formed ideas about psychiatric theory or practice past how to “do a takedown”. In Shem’s House of God, the future psychiatrist repeatedly states that “Good medicine as doing as much nothing as possible”; and until this term, I wasn’t entirely sure what that was, and less about to expect when it came to the care of young people.

As this opportunity is becoming rarer by the year and the rarefied air of Inpatient Adolescent Psychiatry is far removed from most Paediatric practice; this series aims to help understand the work (and underpinning theories) seen on the inpatient unit, in four parts.

What is it all about?

Medicine is generally about defining a pathophysiological entity and having it go away, either actively or passively, in this regard, psychiatry is similar. And as much as it would be impossible to describe all of surgery, so it is with this series; hence this post considers just a few of the key underpinning ideas and theories of child and adolescent psychiatry.

Rather than presenting with epistaxis, arthritis or dyspnoea, patients present to psychiatry with their own set of symptoms and maladaptive coping strategies.

If we’re honest, the idea of “treating” psychotic, hungry, anxious, suicidal and lonely kids remains unpalatable to most clinicians, let alone wading into toxic family systems with the goal of “fixing” everyone, by “having a chat*”. This is not what we do.

But I’m getting ahead of myself.

In mental health, we don’t see young people as individuals, rather as a representative within a family system. This idea underpins the paediatric mental health model of care; that the young person in our care is the index or named patient, and they are part of a larger their family system.

The approach then, is about understanding the child, the family, how they function both on a “day to day” basis, and under stress, from a biological, social and psychological perspective.

Truthfully, a well functioning family system can hold a huge amount of distress in the community setting. These families spend many hour with our Community colleagues, School Guidance officers and therapists. It is when this system is put under further stress and strain that the young person ends up on the locked mental health ward.

In short, they are stuck. An inpatient mental health admission is, I suspect, rarer than major surgery in childhood, so you have to be pretty stuck to get admitted. And this is what we do: we seek to understand why a young person is stuck – their predicament – by asking three fundamental questions:

  • Why them?
  • Why now?
  • What does it mean?

These questions allow both a Diagnosis and a Formulation for the young person’s presentation.

Appreciating the difference between these two is essential to understanding the role of Psychiatry. A Diagnosis describes a constellation of symptoms and coping mechanisms. It facilitates comparisons and ultimately permits the use of evidence-based-medicine, therapies and science. It provides a label. Diagnosis is why everyone with this label is the same. Formulation is the opposite. It focuses on narrative, influences, attachment and coping styles and the dynamics of a situation. In short, Formulation is why this person is different.

 

How do you even talk to teenagers? What do you ask them?

My peers asked me this on numerous occasions throughout the term, and I’d defer heavily to the benefits of the environment. Young people presenting with full-blown mania or untreated psychosis are almost always keen to talk.

Equally, by the time young people make it to the ward, there’s usually been some time between the awfully risky, intense, angry situation that has seen them arrive via Emergency.

On the ward, we sit in a quiet room with small unobtrusive windows and give the young person our undivided attention. This layer of environmental influence cannot be underestimated. Most young people are keen to talk by the time they meet the inpatient Psych team; they see them as the gatekeepers! That said, the role of the clinician is to help, remain nonjudgemental, understand, facilitate, support, challenge the young person.

Another frequent observation is that “it must be awful to see and hear all of that”, mainly, I suspect because as doctors we are better programmed to cope with lacerations of the flesh than of the mind. One part of understanding this, that is also a component of the therapeutic relationship, is the “Dynamic theory” (to which I will profess general ignorance past this brief explainer).

Essentially, the interview is treated like an operating room.

  • Outside the room is the rest of life.
  • In the room is you and the patient.
  • The only way we can understand the patient is through their words and actions (and metacommunication)
  • But, not everything the patient thinks or feels is in the room.
  • Equally, not everything you think or feel makes it into the room.

Every interaction arouses thoughts and feelings that remind you of your real (outside the room) life. The challenge here is to work out what is your ‘stuff’, and what is the patient’s ‘stuff’.

The additional layer to understanding this is that even when people talk, they can bring forward any number of ‘voices’. By this I mean that we all have an inner child (c) and an inner parent (P). The goal of the interview is to talk to the Adult/adolescent (A/a) as the clinician (*). You need to quiet your inner parent, and take the position of being bigger, wiser, strong and kind.

 

Bigger, wiser, stronger and kind?

The key, of course, is not to ignore the patient’s ‘inner child’ or ‘inner parent’, because the presence of those ‘voices’ in the room is exquisitely powerful. The metacommunication allows you to feel the golden egg/sacred cow/holy grail of Mental Health, counter-transference.

That is, patients will arouse in you feelings. When you talk to people, you feel sad or angry or aggressive or hopeless or miserable or agitated or confused. This is counter-transference – the unconsciously activated reactions to the client – which may often simply mirror that of the patient. By seeking to understand these feelings, you begin to understand what it is to “be” the patient, and therein lies a key route to understanding the child’s predicament. I’ve heard it described that the best way to “see” these feelings is as if you are a third person watching the interview. At least that’s the theory.

Of note, understanding a patient’s words and feelings as they occur both in the interview and afterwards leads to an interesting paradox; if (in this setting) you feel the urge to act, first take no action and secondly consider Why is it that I feel like I need to act?

 

Defenses

No post on psychiatry would be complete without at least one mention of Sigmund Freud, the Ego, SuperEgo and Id. The concept is, of course, generally relevant to understanding psychiatry, but more specifically and pragmatically helpful in understanding the Ego Defenses. These were first described by Anna Freud, and are part of a personality irrespective of mental health or unhealth.

Although an extensive explanation about the individual styles of personality defence are beyond the scope of this post, their particular characteristics are that they usually operate unconsciously (outside of awareness), that they operate to protect self-esteem by keeping unacceptable thoughts, impulses and wishes out of awareness, that they function to protect the person from experiencing excessive anxiety, they are part of normal personality functioning, they can lead to pathology if one or more is used excessively and that they are distinguishable from one another. Examples include projection, passive aggression, reaction formation, dissociation among others.

The key message here is to consider how a particular defensive style might deal with a difficult day, and an impossible day, and how these coping strategies can make things better or worse for the young person.

Further, the relationship between reality testing and personality defenses is important. Essentially, psychosis is impaired reality testing, and depending on a person’s ego defences, this reality testing can become more or less vulnerable to compromise. Often, rather than a sudden, abrupt impairment of reality testing, this decline may occur more gradually as psychotic thought processes emerge then supervene those grounded in reality.

 

Theories

The theories and philosophies I encountered in my time in adolescent psychiatry were also based on some of the work by three prominent psychoanalysts, John Bowlby (Attachment Theory), Donald Winnicott (being a “good enough” parent and the Transitional object) and Melanie Klein (understanding what “Borderline” means). Rather than oversimplify and undersell their work, I’ll let The School of Life explain in three short (and fascinating!) videos.



 

*In Psychiatry, there is no such thing as “a chat”. A good psychiatric interview might feel – for the patient – like “a chat”, but any interaction where the doctor-patient relationship is in place remains clinical. The clinician remains alert and mindful of their words and can either in situ or retrospectively make an assessment of the patient’s mental state; hence the interaction, irrespective of perceived formality, is essentially a psychiatric interview.

Many thanks for the guidance and mentorship of Dr Jannie Geertsema, Child & Adolescent Psychiatrist in this series.

References:

Alyami H, Sundram F, Hill AG, Alyami M, Cheung G.Visualizing psychiatric formulation.Australas Psychiatry. 2015 Oct;23(5):575-80.

Mace, C & Binyon, S. Teaching psychodynamic formulation to psychiatric trainees Part 1: Basics of formulation. Advances in Psychiatric Treatment (2005), vol. 11, 416–423

Mace, C & Binyon, S. Teaching psychodynamic formulation to psychiatric trainees Part  2: Teaching methods. Advances in Psychiatric Treatment (2006), vol. 12, 92–99

Skynner, R & Cleese, J. Families and How to Survive Them. Vermillion Press, United Kingdom. 1993.

Felluga, Dino. “Modules on Freud: On Psychosexual Development.” Introductory Guide to Critical Theory. Jan 2011. Purdue U. 22/02/2017

Big Picture Paediatrics : Adverse Childhood Experiences

Cite this article as:
Henry Goldstein. Big Picture Paediatrics : Adverse Childhood Experiences, Don't Forget the Bubbles, 2016. Available at:
https://doi.org/10.31440/DFTB.10082

So much of paediatrics, and medicine in general, is focussed on small experimental or observational studies. This series of posts takes the wider view; we’re talking here about some of the biggest and longest running studies that help us frame, measure and understand childhood through time and across the world.

Who & what was studied?

Kaiser Permanente is a large Medical Insurer in the USA; they collected data in two waves in the primary care setting with a view to describing the long-term relationship of childhood experiences to important medical and public health problems. The study initially rolled out in 1996 & 1997.

Felitti, VJ, Anda RF, Nordenberg D et al. Relationship of Childhood Abuse and Household Dysfunction to Many of the Leading Causes of Death in Adults : The Adverse Childhood Experiences (ACE) Study. American Journal of Preventive Medicine. 1998:14, 245–258.

The study aimed to assess – both retrospectively and prospectively – the long-term impact of abuse and household dysfunction during childhood on disease risk factors and incidence, quality of life, health care utilization, and mortality for adults.

Here is the actual questionnaire:

Answer yes or no; all ACE questions refer to the respondent’s first 18 years of life.

Abuse

  • Emotional abuse: A parent, stepparent, or adult living in your home swore at you, insulted you, put you down, or acted in a way that made you afraid that you might be physically hurt.
  • Physical abuse: A parent, stepparent, or adult living in your home pushed, grabbed, slapped, threw something at you, or hit you so hard that you had marks or were injured.
  • Sexual abuse: An adult, relative, family friend, or stranger who was at least 5 years older than you ever touched or fondled your body in a sexual way, made you touch his/her body in a sexual way, attempted to have any type of sexual intercourse with you.

Household Challenges

  • Mother treated violently: Your mother or stepmother was pushed, grabbed, slapped, had something thrown at her, kicked, bitten, hit with a fist, hit with something hard, repeatedly hit for over at least a few minutes, or ever threatened or hurt by a knife or gun by your father (or stepfather) or mother’s boyfriend.
  • Household substance abuse: A household member was a problem drinker or alcoholic or a household member used street drugs.
  • Mental illness in household: A household member was depressed or mentally ill or a household member attempted suicide.
  • Parental separation or divorce: Your parents were ever separated or divorced.
  • Criminal household member: A household member went to prison.

Neglect

  • Emotional neglect: Someone in your family helped you feel important or special, you felt loved, people in your family looked out for each other and felt close to each other, and your family was a source of strength and support.
  • Physical neglect: There was someone to take care of you, protect you, and take you to the doctor if you needed it, you didn’t have enough to eat, your parents were too drunk or too high to take care of you, and you had to wear dirty clothes.

What does this mean?

The ACEs questionnaire accumulates a score from zero to seven based on yes/no responses to the above questions. These results in conjunction with a “Health Appraisal Clinic’s questionnaire” allowed correlation with risk factors such as smoking, severe obesity, physical inactivity, depressed mood, suicide attempts, alcoholism, any drug abuse, sexually transmitted diseases, parental drug abuse and a high lifetime number of sexual partners (>50), as well as the big swingers; mortality and overall morbidity.

The ACE score has been utilised to demonstrate a graded dose-response with more than 40 outcomes. You can see the entire list of publications here.

How good is this dataset?

Although there are almost all of the expected threats to validity from a questionnaire administered to people obtaining health insurance in the USA in the 1990s, the dataset is very good.

Of the 13,494 surveys, there was a 70.5% (9508) response rate, sent a week after standardised medical review. Respondents who did not respond to all questions were excluded from the final analysis. After non-responders and exclusions, a total dataset of 8056 responders was analysed. Alarmingly, more than half of the exclusions were for not answering the question about childhood sexual abuse. This certainly raises some concern for a risk of underreporting, particularly if this was the only question omitted! 

What meaning can be drawn from the results (so far)?

The dataset has lent itself to the associations between adverse childhood experiences and a veritable laundry list of medical, psychiatric pathology as well as social and public health problems.

This is data reports that 1 in 5 were sexually abused, nearly 1 in 4 lived with a “problem drinker or alcoholic” and that around 1 in 6 had a household member who was depressed or mentally ill.

It’s worth remembering that this study paints a picture of the adverse childhood experiences of the older generations in the USA – the mean age of respondents was 56.1 (19-92) years – in a study undertaken just over 20 years ago.

Rather than provide a snapshot of what childhood is like today, this data informs us about the childhood of parents of our patients. This gives us some understanding and frameworks by which to consider expectations of childhood from the parental & societal viewpoint – that most parents hope for a rosier childhood with fewer adverse experiences than their own.

With this in mind, and with a critical eye to some of the correlating outcomes, behaviours such as alcohol & drug abuse, smoking, over-eating, and sexual behaviours might alternatively be viewed as both coping strategies and symptoms of the anxiety, anger and depression that is likely co-morbid with high levels of adverse childhood experiences.

Primary prevention of adverse childhood experiences necessitates change at the societal level; with a focus on improving the quality of family and household environments through the childhood years.

Funding for the original study was combined between Kaiser Permanente (San Diego) and the US Center for Disease Control.

Where next?

The Centre for Disease in Childhood has taken over the study and, since 2009, transformed it into a national program across 32 states of the USA, called “Behavioral Risk Factor Surveillance System” (BRFSS). Data from the 2010 BRFSS has been published and includes more than 50,000 respondents. You can see more about the participating states, future timeline and previous data via the CDC website, here.

References:
Felitti, VJ, Anda RF, Nordenberg D et al. Relationship of Childhood Abuse and Household Dysfunction to Many of the Leading Causes of Death in Adults : The Adverse Childhood Experiences (ACE) Study. American Journal of Preventive Medicine. 1998:14, 245–258. 

Centers for Disease Control and Prevention, National Center for Injury Prevention and Control, Division of Violence Prevention Adverse Childhood Experiences (ACEs)”.U.S. Department of Health & Human Services, Atlanta, USA. Accessed 27 September 2016. https://www.cdc.gov/violenceprevention/acestudy

Centers for Disease Control and Prevention, National Center for Injury Prevention and Control, Division of Violence Prevention. “About Behavioral Risk Factor Surveillance System ACE Data”.U.S. Department of Health & Human Services, Atlanta, USA. Accessed 5 October 2016. https://www.cdc.gov/violenceprevention/acestudy/ace_brfss.html

Munchausen by Proxy : Fabricated & Induced Illness

Cite this article as:
Henry Goldstein. Munchausen by Proxy : Fabricated & Induced Illness, Don't Forget the Bubbles, 2016. Available at:
https://doi.org/10.31440/DFTB.9772

I recently attended a superbly insightful presentation by Dr Sue Wilson, the psychiatrist for our Consultation Liaison team here at Queensland Children’s Hospital. Some years ago, I was involved with a case of FII / Munchausen’s syndrome by Proxy, and – as is often the case – the case continues to resonate and evoke strong feelings. She has kindly offered her source material for this post.

Theoretical conceptualizations

Fabrication and Induction of Illness (FII) tends to be conceptualized as a rare/severe form of child abuse. The term Munchausen’s syndrome by proxy is used less in clinical practice, as it places an emphasis on the abuser rather than the victim. We’ll mostly use FII hereafter in this post, although there’s a short explainer about Munchausen himself later on.

An alternative view is that fabricated illness occurs along a broad spectrum that ranges from exaggerated reporting of symptoms by very anxious parents to the actual production of symptoms, with varying degrees of risk. This broader definition includes a far wider range of motivations and behaviours that represent clinical reality.

Here’s a 5 point summary:

  1. MBP/FII is a rare and severe form of child abuse. The behaviours may be considered on a spectrum of induced symptoms.
  2. Focus needs to be on caring for the child, not diagnosing the carer.
  3. Consider FII whenever you come across a perplexing presentation – it’s more common than some of the other, very rare, diagnoses we chase.
  4. A key differentiator is the parental response to a proposed medical change of direction.
  5. If you are even considering FII as a differential diagnosis, make early contact with your hospital’s child protection team – as you would for a consulting service to see an opinion.

Important to remember


The line between volitional and non-volitional processes in the caregiver is difficult to identify. To be clear, volitional means the cognitive process whereby a person decides on and commits to a particular course of action. The harmful effects on the child are very similar, irrespective of the parent’s actions and motivations.

The focus must be on the outcomes or the impact on the child’s health and development and not initially on attempts to diagnose the parent or carer.

Detailed descriptions of the impact of the carer’s behaviour on the child are more useful than diagnostic labels which may distract from the central issue of harm to the child. Recognition of the carer’s difficulties is neither necessary nor sufficient for the diagnosis of FII.

So, who was Munchausen?

Munchausen refers to a satirical character based loosely on Hieronymus Karl Friedrich von Münchhausen, a German nobleman born in 1720. He gained notoriety in German aristocratic society after returning from a number of foreign wars with literally unbelievable stories. An author, so inspired by the Baron’s tales, expanded them into satire and farce and published them widely, much to the rage of Munchausen himself. This last point, to me, serves to reinforce the point above about volition versus non-volitional processes – I’m sure the original Baron von Munchausen did not intend to leave this kind of legacy! The satirical Munchausen features in a number of books and movies, most recently in a 2012 TV movie.

munchausen

Right then, back to it…

Perplexing presentations vs FII

FII are one sub-group within the category of perplexing presentations – FII should be considered as a differential diagnosis when some of the other indicators are present. Think to yourself, “What doesn’t make sense about this child’s presentation? ” What differentiates perplexing presentations from FII is the parental response to a proposed medical change of direction – from investigation to rehabilitation. That is, some parents persist in seeking medical opinions and investigations and decline or do not participate in the rehab process. They find difficulty in enabling their child to function and cope better.

Indicators which should alert professionals to the possibility of FII


Several indicators can give clues to an FII presentation. These include a carer reporting symptoms and signs which are not explained by any known medical condition, physical examination and investigations that do not explain reported symptoms and signs, inexplicably poor response to medication or other treatment, or intolerance of treatment or acute symptoms that are exclusively observed by/in the presence of the carer.

Additionally, on resolution of the presenting problems, the carer may report new symptoms or symptoms in different children.

The child’s daily life and activities may be limited beyond what is expected due to any disorder from which the child is known to suffer e.g, poor or no school attendance; use of seemingly unnecessary special aids.

Occasionally there is objective evidence of fabrication – history from different observers in conflict or being biologically implausible; test results (toxicology or blood typing); covert video surveillance (this is a minefield and we heavily caution against this course of action, even if you loved The Sixth Sense!!).
Sometimes a carer expressing concern that they are under suspicion of FII or relatives raising concerns about FII may be an indicator, as is a carer seeking multiple opinions inappropriately.

Characteristics


Characteristics of parents who fabricate or induce illness in their children should be applied with caution – many of them are also true of many parents. Additionally, they should not be used to confirm or deny the existence of FII and ultimately the identification of characteristics consistent with parents or carers fabricating or inducing illness in children may add to suspicions during the investigation process but do not constitute a profile. Nonetheless, we know;

  • Typically carried out by women, specifically mothers (95%)
  • Usually the child’s primary carer
  • Often present initially as “good” carers

(Yes, the three above points are also true of a very, very high proportion of carers…)

  • Usually accomplished liars and manipulators
  • Usually the only ones consistently present or associated with the onset of the child’s symptoms (when the carers are absent, symptoms or illnesses are not reported or may begin to improve)
  • They may have a history of self-induced symptoms/illness exaggeration, falsification or induction
  • They may have mental health evaluations indicating they are “normal” – psychiatric disturbance may be well-concealed from the observer
  • They may have no prior involvement with child protection services
  • They may appear to be overanxious, overprotective, mistaken or deluded
  • They may have a background in the health profession (14-30%) or an unusual degree of knowledge about health
  • They may seek publicity or attention from a variety of people

These parents or carers do not necessarily stop their behaviour towards the child when under suspicion or caught, but change tactics by:

  • Changing health professionals.
  • Denying all or part of what they have done, even in the face of overwhelming evidence.
  • Accusing their accusers, and shifting blame onto those who are aware of their behaviour. 

Risk factors of mothers for creating Abnormal Illness Behaviour in children

Remote or longstanding risks include;

  • Loss or separation from parent
  • Abuse/neglect
  • Foster care
  • History of lying in adolescence
  • History of self-harm

Recent or acute risks include;

  • Current somatoform disorder
  • Current factitious disorder
  • In receipt of disability living allowance
  • Child missing school
  • Frequent visits to doctors (symptoms unexplained)
  • Frequent moves of house and GP
  • Parent requests disability living allowance for child

Psychopathology of Fabricators

There is no clear relationship between any mental disorder and abusive behaviour towards children.  Many mothers with Borderline Personality Disorder (BPD) or history of abuse do not abuse their children in this way. Such a history may be a trigger to look more closely but doesn’t constitute proof; it’s important to remember that FII is a behaviour to be identified, not a medical or psychiatric diagnosis.

There are a number of associated conditions for those parents/caregivers whom fabricate;

72% somatoform disorders
55% self harmed

21% misused alcohol &/or drugs
89% personality disorder especially Borderline (by interview)
23% personality disorder (by self-rating scale)

Additionally, symptoms of depression and anxiety are common, as well as a high prevalence of somatising and factitious disorder. It’s worth noting that Factitious disorder and FII in children can co-occur; Somatoform disorder in the mother indicates some abnormality of illness behaviour and relationships with health professionals. Detection of factitious disorder in a mother of young children should provoke a search for FII in her offspring.

Fabricators are classically highly persuasive and have a tendency to split between staff (the idealisation of some, whilst devaluing others). FII involves all social classes (not just Barons). There may be a history of significant lying behaviours and deception dating back to childhood. One study notes 1 in 4 abusing carers had a history of being victims of child abuse, whilst another found high rates of deprivation, childhood abuse, significant loss or bereavement in the mothers, however FII is not necessarily associated with young, inexperienced parents or deprivation.

Possible explanatory mechanisms and motivations


The motivations of FII are complex and vary from case to case. However, it can be noted that extreme anxiety leading to exaggeration of symptoms and signs to encourage the doctor to rule out or identify any treatable disorder may play a roles. As can the need to confirm (false) beliefs about the child’s health (e.g, developmental disorder, food allergy) including beliefs held by caregivers with ASD and rarely with a delusional disorder. There may also be a wish for attention or deflection of blame for the child’s (usually behavioural) difficulties. FII also maintains closeness to the child and may invoke a material gain e.g. carer’s benefit. There may be an underlying hostility to doctor or even the child themselves.

In one study motivation for the induced illness in children was unclear in 2/3 of cases.

Intergenerational transmission of abnormal illness behaviours


There appears a common theme amongst caregivers that there is a past use of illness behaviours in relationships with other individuals, including health professionals. FII may at times represent extensions and distortions of childhood patterns of behaviour whose function was to obtain comfort and protection from others, with clinicians now placed in the caregiving role.

This adaptive use of deception develops early in life, and becomes entrenched over time and further distorted by subsequent losses and traumas. From early childhood some caregivers report feigning symptoms in order to avoid beatings or to prevent contact visits with abusive parents/carers. This makes sense if “playing sick” saves you from physical or other abuse.

When parents have been exposed to significant loss and trauma their behaviour is likely to be motivated by trauma-related triggers in situations where they feel threatened or perceive their children to be threatened; it’s worth noting that these ways of thinking and behaving are not always accessible to conscious reflection.

Disturbed attachment

 It may be more useful to see FII as a function of a disturbed mother-child attachment bond, influenced by mother’s own attachment experiences; insecure attachment is associated with higher levels of somatisation. Indeed, a study of attachment models in mothers who fabricated or induced illness found high levels of insecure attachment and unresolved bereavement. This may, in turn, sensitise individuals to see others as more sick than they really are.

Mother-child relationship

Remember, the mother may appear to have a close and caring relationship with the child (may not be so), with presence of separation anxiety and over protectiveness noted.
FII has been described as a “symbiotic bond”, although symbiotic infers mutually beneficial, and in FII it’s pretty hard to see any benefits for the child.  However, illness is the way for this child to maintain a relationship with his/her parent and perhaps preserve the parent’s mental equilibrium.


Consequences

Half of the patients suffer psychological harm including emotional and behavioural problems, school non-attendance and concentration difficulties, whilst a high percentage are affected by other forms of maltreatment or neglect or a repetition of FII. There are usually  compromised attachment relationships as a result.

Short-term effects include;

  • Self-image of self as sick or disabled
  • School absences
  • Miss normal developmental opportunities
  • Impact on peer relationships
  • Only way to achieve nurture or interaction with parent may be via the sick role
  • Impact of possible collusion in older child
  • Following confirmation, must consider child’s developmental stage, level of attachment, effect of separation from sibs and others

Long-term effects include

  • Impairment of overall development
  • Risk of psychological harm
  • Long term implications for child’s mental health including risks of Factitious disorder
  • Long term implications for attachment – effect on trust
  • Relationships and caring mediated through illness
  • Little research on harm from verbal fabrication

Role of mental health

Since no psychiatric diagnosis is pathognomonic of a perpetrator of this type of abuse, psychiatric assessment should not be used to determine whether FII has occurred, however there is a role for mental health after the behaviour has been confirmed, by way of;
  – assessment: parents, family dynamics, parenting skills, child
  – treatment planning: opinions re possibility of family intervention

The Mental Health team may be asked to assess parents who have a history of psychiatric disorder, especially if it seems that parental anxiety or misinterpretation may be contributing to the presentation. The main role for mental health is providing support for the team behind the scenes and taking part in case discussions; this process can be very stressful for all members of the team!

Given the early life experiences of caregivers, they tend to draw clinicians into close relationships in which boundaries can become blurred; this may be a red flag as well as an issue that can be explored in staff support meetings. There is also the potential to cause splitting in the team and arouse strong feelings (including around diagnosis and methods of investigation).

This is particularly challenging as medical & nursing staff must balance the need to remain engaged with the family as clinicians, whilst also being involved in observation and complex case discussions about the family; the period of growing suspicion and investigation is often the most difficult.

Areas of uncertainty

 FII is an area that has some intrinsic uncertainty, often as cases evolve over time. It’s important to always come back to impact on the child. Additionally, consider is this just an overanxious parent, perhaps exaggerating symptoms? Is there something medical being missed?  How much medical investigation is enough? For the parent?  For the treating team? Could it be conversion or somatoform disorder in the child rather than FII?


In older children, there is also a potential for coaching and collusion.


Plus, it is possible that a child may actually experience symptoms of a psychological nature (e.g. headache) which parent insists must have a medical cause.

Management

 Pragmatically, it is essential to notify your local child safety/child protection organisation. In some jurisdictions, including Australia, this is mandatory for health practitioners. One key message from Dr Wilson’s presentation was that if you are beginning to suspect FII, then making early contact with your hospital’s child protection team – as you would for a consulting service to see an opinion – can facilitate the diagnosis and subsequent management. Generally speaking, psychological treatment is not indicated for individuals who cannot admit their behaviour.

In some cases, reunification is possible eg specialist unit in UK.  Better outcomes are associated with:

  • Acknowledgment of fabrication
  • Less severe abuse
  • Improvement in parent’s psychological functioning and empathy for the child
  • Improved parent-child relationship and child attachment behaviour
  • Change in the family system
  • Therapeutic alliance with the partner and extended family – safety network

Training for Paediatricians

So, how can Paediatricians & Paediatric trainees improve, with the above in mind? Clinical skills in consultations are always  being sharpened; with experience clinicians, become increasingly aware that parents need to be listened to but not always agreed with. Additionally, the skills of managing the potential conflict in the doctor-patient relationship also develop with time. In FII, there is a shift in emphasis so that the child truly becomes the primary client.

In the case of perplexing presentations, exploration of childcare perspective of children’s problems in addition to medical disease model, as well as identification of behavioural and interactional cues may assist in the recognition of FII.

Be mindful of obstacles which stand in the way of paediatricians recognising FII:
  – discomfort with not believing a parent, on whose history paediatricians rely
  – discomfort with not understanding the child’s presentation
  – concern about missing a treatable condition
  – concern about litigation or complaints.

References:

Jureidini JN, Shafer AT, Donald TG.”Munchausen by proxy syndrome”: not only pathological parenting but also problematic doctoring? Med J Aust. 2003 Feb 3;178(3):130-2.

Proops & Sibert (Eds), Fabricated or Induced Illness by Carers (FII): A Practical Guide for Paediatricians. RCPCH, 2009. (Dr Wilson also referenced the 2002 edition of this publication in her talk.)

Bass C, Glaser D. Early recognition and management of fabricated or induced illness in children. Lancet. 2014 Apr 19;383(9926):1412-21.

Kozlowska K, Foley S, Savage B.Fabricated illness: working within the family system to find a pathway to health. Fam Process. 2012 Dec;51(4):570-87.

Kozlowska K.When the lie is the truth: grounded theory analysis of an online support group for factitious disorder. Psychiatry Res. 2014 Dec 30;220(3):1176-7.

Bass C, Jones D.Psychopathology of perpetrators of fabricated or induced illness in children: case series. Br J Psychiatry. 2011 Aug;199(2):113-8.

Bools CN, Neale BA, Meadow SR.Follow up of victims of fabricated illness (Munchausen syndrome by proxy). Arch Dis Child. 1993 Dec;69(6):625-30.

Adshead G, Bluglass K. Attachment representations in mothers with abnormal illness behaviour by proxy. Br J Psychiatry. 2005 Oct;187:328-33.

Adshead G, Bluglass K. A vicious circle: transgenerational attachment representations in a case of factitious illness by proxy. Attach Hum Dev. 2001 Apr;3(1):77-95.

Fish E, Bromfield L and Higgins D. A new name for Munchausen Syndrome by Proxy: Defining Fabricated or Induced Illness by Carers. Australian Institute of Family Studies. 2005; 23.