A complex syndrome on the autism spectrum is receiving increasing attention from researchers and educators - those with the condition and their families hope that front-line clinicians won't be far behind.
Although it was first identified by psychologist Elizabeth Newson in the 1980s, when I started writing this post, Pathological Demand Avoidance syndrome (or PDA) had received barely any media coverage in more than 30 years, but over the last few months this has begun to change. In addition to two new books on the condition being published, it was highlighted by Channel 4, on its Born Naughty? documentary strand, and will see more people made familiar with the condition thanks to this week's Girls With Autism film on ITV, which follows the work of Limpsfield Grange, a specialist state-run school for girls close to my home in Surrey.
PDA is the name of a complex and relatively rare syndrome on the autism spectrum, and it's one with which my own seven-year-old daughter was diagnosed last year. Newson's foundational work recognised a group of children that presented with autistic traits, and who were often being un-satisfyingly diagnosed as having "atypical autism". These children shared several defining features uncommon to others on the spectrum, but in common with each another. The most distinctive of these, and the one from which the condition takes its name, is an overriding compulsion to control situations and avoid demands made by other people, due to exceptionally high levels of anxiety.
Identification is essential. Jane Sherwin, author of the recently published Pathological Demand Avoidance Syndrome - My Daughter is Not Naughty knows this firsthand. "Having one child with Asperger's and one with PDA has enabled me to see first-hand the huge differences both in the outward profile of the conditions, and in their successful management." Those with PDA do not generally respond well to the educational techniques, such as rewards-based systems, used to teach others with autism or with behavioural difficulties, and their condition is frequently misinterpreted.
Although sharing the social impairments, restricted behaviour patterns and intense special interests of other autistic people, those with PDA are often socially outgoing (indeed their fixations are frequently on people, which can prove problematic), generally have good "surface" communication skills such as eye contact and body language, and possess an ability to socially mimic others.
This surface sociability, coupled with often highly charismatic personalities (charm is, after all, a useful tool for avoidance), can mask the debilitating difficulties those with PDA have to contend with, and result in misdiagnosis and dismissal of difficult behaviour as being that of a willful or naughty child, rather than symptomatic of social confusion and levels of anxiety even greater than those felt by others on the spectrum, or with the conduct disorders they may have been mislabeled with.
Other defining criteria for PDA include sudden switches in mood (often accompanied by aggression), impulsiveness and an over-active imagination, where the line between fact and fiction can become blurred.
According to Judith Gould, who, along with the late Lorna Wing detailed the "triad of impairments" that have come to define all autism spectrum disorders (ASDs), the development of the concept of the spectrum itself included the PDA pattern of behaviour. Unfortunately, until recently there has been limited professional interest in the syndrome and a lack of awareness of its presentation amongst clinicians, despite the huge challenges faced by those with PDA and their families, friends and carers.
In the UK, thankfully, this is improving slowly. The "education and handling" guidelines produced by Newson and colleagues at Sutherland House School, a special school maintained by autism charity Autism East Midlands (previously NORSACA), have been adopted as part of the Department for Education's National Autism Standards for schools. These differ from those recommended for other autistic children in important ways, advocating a child-led, choice-driven approach.
"Awareness is growing all the time," says Phil Christie, consultant clinical psychologist at Autism East Midlands' Elizabeth Newson Centre and co-author of Understanding Pathological Demand Avoidance Syndrome in Children and the recently released Can I Tell You About Pathological Demand Avoidance?. It is Christie himself who is behind much of this increase in awareness as he has gone on to develop Newson's original work. "Understanding lags behind though," he says, "and clinicians have been the group that has been hardest to attract to conferences and training events."
Gould agrees. She presented a talk at the fifth PDA conference held by the UK's National Autistic Society (NAS) in November 2014. Her talk with Jacqui Ashton Smith focused on the presentation of PDA and other forms of autism in girls and women. Gould outlines a strong correlation between several of the diagnostic traits of PDA and other types of "high-functioning" autism in girls and women, such as social mimicry, withdrawal into fantasy worlds, and even demand avoidance itself.
Interestingly, unlike for other ASDs, which are weighted towards males, PDA diagnoses are split equally between the sexes. As is being increasingly acknowledged, Gould and other autism experts are aware that many women and girls with autism are currently being missed entirely or misdiagnosed by health professionals, often with mental health issues. She suggests it's possible that the differentiating factor between the list of shared behaviours of the more general female presentation of autism and of PDA is intensity, and adds that, "it is important to describe the PDA pattern, as the management and support needed differs from that of a straightforward autism spectrum disorder."
Christie's original book and the new one are co-authored with Ruth Fidler, an educational consultant specialising in complex autism. Their first book is invaluable, but dense. It's a guide aimed primarily at parents and teaching staff, rather than clinicians.
However, "Getting an appropriate diagnosis matters both to the individual themselves as well as to the people who live and work with them," says Fidler. "For the adults supporting children with PDA a diagnosis is important because it can gain access to services and because it can help organisations or schools to plan strategically, but more significantly getting a diagnosis helps them better understand the child or young person with PDA. Many parents and teachers will have exhausted traditional strategies that they may have found to be highly successful with other children, but which are much less effective with this group."
Speaking from personal experience, securing such a diagnosis is far from straightforward. But there is hope - four new papers published in peer-reviewed journals over the last few years have boosted the clinical literature concerning the condition, as has the first major research study since Newson's formative work. This study, completed last year as part of clinical researcher Liz O'Nions' PhD, was supervised by Francesca Happé, professor of cognitive neuroscience and director of the Medical Research Council's Social, Genetic and Developmental Psychiatry Centre. It was prompted by conversations with Gould and Wing,
"One of the ideas we began with was that PDA might be a female manifestation of autism spectrum conditions. Without a large-scale study comparing males and females with and without extreme demand avoidance, we can't have a definitive answer, but we certainly found plenty of boys with demand avoidance in our research so far.
"One of the biggest motivations for our research was hearing from parents, teachers and clinicians about young people with PDA who were excluded even from specialist autism schools, and who were the hardest to know how to teach despite apparently good intellectual capacity. If the usual ASC-friendly settings and approaches don't work, it's vital to find out what will."
Some progress has been made in this area, using the educational guidelines produced by Sutherland House School, but Happé's point is a crucial one. In Understanding Pathological Demand Avoidance in Children there's a summary of results from a survey Newson and her colleague Claire David carried out in 1999 that makes for a rather gloomy read. It followed up a sample of 18 individuals aged 16 or over who had an earlier diagnosis of PDA. Thirteen of them had been excluded from school, and only one had attained GCSE standard education. (On a brighter note, one young lady had declined to take part as she was too busy studying at university.)
But Christie points out that, "The young people included may not have been fully representative of the condition as a whole. As they had already come to the attention of the Elizabeth Newson Centre it is likely that they were mostly those young people with more significant needs. As the study was some while ago it was also at a time when very little was known, or understood, about some of the teaching and management approaches that are proving more effective."
It can't be overstated how beneficial these techniques can be. Alis Hawkins is a speech and language therapist specialising in autism conditions. She works as a family support worker for the NAS in Wales, and regularly meets families who are operating without professional recognition of their children's needs. Sixteen years on and the statistics are a little better, but still pretty bleak. "Four of the nine children affected by PDA whose families I am currently supporting have been excluded from at least one school," Hawkins explains. "And one is now out of school altogether, having been excluded, finally, from a special school for children with behaviour issues and ASDs."
My own family has been exceptionally lucky that our daughter's infant school has joined us on the journey towards PDA-awareness. Nonetheless, as prepare for her imminent transition to junior school, it's still difficult not to fret about the future.
Our daughter had been seen by paediatric consultants, behaviour support workers, speech & language and play therapists, along with her regular teaching staff, one-to-one special needs assistant and several NHS-prescribed psychotherapists, none of whom could offer us firm hope or recognition. Until one day a pair of teaching staff from our local autism school's Outreach team agreed to observe our daughter in class. They didn't just recognise her difficulties - they recognised that we recognised them, validated our approach, and understood what we'd been going through ever since our daughter had started preschool. Until that happened, we felt like we were shouting into an abyss.
"Of the nine families I mentioned," says Hawkins, "all but one set of parents has been sent on social services-run parenting courses. Without exception, parents tell me that the strategies recommended on the courses made their children's behaviour worse. And without exception, these parents feel at fault, blamed and judged. The attitude of professionals, on the whole, does nothing to dispel these feelings. Often, they have been involved with one professional after another who has told them your child isn't autistic, he just needs firm boundaries or she's just manipulating you and you're letting her, you're colluding with her."
That doesn't just ring true for me, but for every related comment from a parent with a child with PDA that I've come across.
Jane Sherwin agrees. "The first obstacle is getting anyone to believe you and to not automatically assume that your child is completely neurotypical but just extremely naughty, compounded by inadequate and inconsistent parenting."
Here's the science bit
This is something that those with professional awareness of and involvement with PDA acknowledge. "Parents fears for the future mirror those of parents of children with other ASDs, but may be magnified by the variable recognition and understanding of the condition," says Christie.
Despite the growing understanding amongst parents and within education, lack of clinical recognition is a painful stumbling block, which is exacerbating the difficulties faced by parents of children with PDA. We can only hope that this will improve as more research is published.
As well as holding her post at the MRC, Happé is also past-president on the board of the International Society for Autism Research. She notes that for the moment, PDA is pretty much a European concept.
"When I visit other countries I try to ask what they would call the pattern of extreme demand avoidance we call PDA here. Different places have different sorts of labels, often around either ASD or oppositional defiant disorder (ODD) (e.g., the non-compliant child). For PDA as a term to enter diagnostic manuals, a great deal more research evidence would be needed." This discord is concerning, as there are important distinctions between PDA and the conditions that it often gets confused with (although, it should be stated, can potentially be comorbid with), such as ODD and Attachment Disorder.
Attachment Disorder is a particularly hurtful label for a clinician to suggest to the parent of a child with PDA who has spent their life trying to understand and help their child, and sadly echoes the early days of scientific thinking that laid the blame for autism on "refrigerator mothers".
"Attachment Disorder, or Reactive Attachment Disorder as described in the diagnostic manuals, describes a group of children who show inhibited, emotionally withdrawn behaviour", Christie explains, "and also a persistent social or emotional disturbance. The criteria, though, also include patterns of extremes of insufficient care and are not judged to meet the criteria for autism spectrum disorder."
"She is starting to know how she should behave, but that doesn't mean she is capable."
In contrast to "extremes of insufficient care" our daughter's first few years of life were highly enjoyable for my wife and me as parents, and hopefully for her too. But in hindsight the demand avoidance and drive to control social situations was ever present: rejecting breastfeeding; sleep problems; repetitive, dictated play; an incapability to not be interacting with someone; and a hungry, insatiable curiosity that we were required to feed were all present in infancy.
As for oppositional defiant disorder, Christie notes that, "ODD itself often exists alongside ADHD, and is characterised by persistent negative, hostile and defiant behaviour towards authority. There are obvious similarities here with the demand avoidant behaviour of children with PDA. PDA, though, is made up of more than this, the avoidance and need to control is rooted in anxiety and alongside genuine difficulties in social understanding."
Steph Curtis is the mother of two junior-age daughters. She maintains a popular blog, Steph's Two Girls, where she has detailed some of the difficulties the family has faced in trying to secure a clinical acknowledgement of PDA for Sasha, her youngest. Like me, Steph has one typically developing child, and one with PDA, and the two enjoy a good relationship with one another. But still, even with an older role model, "Social skills are extremely difficult for Sasha," Steph says. "She has always had little awareness of others and what is expected of her. From an early age, she just didn't understand authority figures were in control - so we'd go to toddler groups where everyone else would sit still and listen but Sasha would be oblivious to that. Then at nursery, if they wanted to take the whole class out for a walk, but Sasha wasn't happy, she would refuse to go - in a way where she would become a lead weight or scream.
"She is starting to know how she should behave, but that doesn't mean she is capable of it. For example, because her body temperature always runs high and she doesn't like the feel of clothes on her skin, at home she always strips off to her underwear as soon as we get in from school. If she ever goes on a play-date (rarely, but it does happen!) she will generally ask if it's okay to do this there. She kind of knows she shouldn't, but obviously she feels much calmer if she undresses. Playground time at school is difficult as she can't really join in with the other children's games without adult intervention - she can't stop herself from getting upset over not winning still, or over others not wanting to play by her (made-up) rules which they don't even understand."
Looking to the future
I understand that even for those who have come round to accepting that PDA is a "thing", it's still a pretty complex "thing" to comprehend. Perhaps the best way I can describe what it is, is to give an innocuous example from my day-to-day life: A couple of weeks ago my daughter was in reasonably relaxed, settled mood. We'd been playing together and she was now watching television and munching on a bowl of popcorn. I asked if I could have a piece. She looked at me, appeared to experience some internal battle of wills with herself and then said, "No. You can have two pieces." Trust me, this was not (solely) generosity at play. This was her unable to comply with my request, but caring about me enough and feeling relaxed enough to attempt an accommodation.
Those with PDA aren't entirely incapable of complying; it's just that they find doing so very difficult. Christie and Fidler use a helpful analogy in their books, describing a linked relationship between two dials, one for tolerance levels and one for demands. A person with PDA's ability to deal with demands is variable, and relates to his or her levels of anxiety. It's subject to obvious factors such as tiredness and hunger, but also to how many demands have already been complied with or faced in a given period - this is why to the uninitiated behaviour can appear unpredictable. A panic attack or emotional meltdown is unlikely to occur as a result of an isolated demand like Could you put your shoes on please, but such a demand could easily be the straw that breaks the camel's back.
During the difficult school years, despair and self-pity might seem an easy option for both parents and the PDA-afflicted, but it needn't be. Since our daughter had her autism confirmed and the school has been following PDA guidelines, things are improving in important ways. Incidents of serious aggression are now rare and her academic progress has steadied as a result of being provided with more choice.
Curtis, whose daughter Sasha is also in a mainstream school, feels similarly. "We know there are some tricky times ahead, particularly around the social aspects, but having said that, we think she is coping pretty well so far and definitely still progressing, and in some ways her behaviour has improved as she learns to control parts of it. So it's definitely not all doom and gloom for us. We enjoy life as a family, it's just very different to how it would have been without PDA."
PDA activist Julia Daunt was diagnosed at 12. Now an adult, she's in a happy long-term relationship and campaigns to raise the condition's public profile. She confirms Curtis' optimism. "Self-awareness really is the key," she says. "If you understand why you do something or act a certain way then it kind of takes the pressure off. You no longer feel confused or like a freak. It answers so many questions and allows you the time and space to look deeper and learn ways of coping and, so in turn, modifying some behaviours... It wasn't until I fully understood PDA and how it relates to me that I was able to truly express my empathy. Now I feel it, just as before, but I'm able to show and express that."
Given that PDA makes up such a small portion of all autism diagnoses, awareness is unlikely to reach the levels achieved by other autism spectrum conditions, so what does the future hold?
Christopher Gillberg, who is professor of child and adolescent psychiatry at the University of Gothenburg, acknowledged in a recent commentary in the Journal of Child Psychology and Psychiatry: "PDA is already a very real clinical problem, not just in the United Kingdom, but across the planet. Intervention and treatment currently rest almost exclusively on guesswork, clinical experience and trial and error. It is one of the most difficult-to-treat constellations of problems in the whole of child and adolescent psychiatry. Strategies developed for ASD, ODD or ADHD are often ineffective and parents, teachers and clinicians may be driven half-crazy by the child's stubborn refusal to cooperate by avoidant, manipulative and exhibitionist-style shocking behaviours."
Gillberg followed up the commentary with a co-authored study published in the Journal of European Child and Adolescent Psychiatry, which looked at the total population of 15-24 year olds in the Faroe Islands. All had been screened twice for autism, first in 2002 and again in 2009. The initial research identified that 67 young people - one per cent of the Faroese population - met the criteria for an autism spectrum diagnosis.
For Gillberg's new study, the parents of 50 of the autistic youngsters were interviewed using Gould and Wing's Diagnostic Interview for Social Communication Disorders (DISCO), which has 17 markers for PDA. Nine subjects were judged to meet the criteria for a "possible clinical diagnosis of PDA" in childhood. That's .18 per cent of the population, or just over one in every 625 people. But the study also found that when the subjects reached late adolescence and early adulthood, only one of them still met the full criteria. This sounds encouraging, but there are important caveats concerning the wording of the research and the diagnostic procedure for PDA.
As a parent, my greatest worry for my daughter's future is the potential for the development of mental health issues resultant from how she views herself. As a teacher specialising in autism, Ruth Fidler is aware how genuine a concern this is. "Like any other typically developing children and young people, dealing with growing up, puberty, teenage relationships, and experiences of loss and transition all present challenges," she says. "If we take a moment to consider some of the skills that help most people to navigate these troubled areas, they include self awareness, positive relationships, social responsibility, emotional regulation, problem solving and impulse control. These are all areas that we would not be surprised to find children and young people with PDA struggle with. This means that they face similar challenges but without the same range of strategies to cope with them."
If we can help children with PDA through their childhoods feeling for the most part positive about themselves, and teach them to develop the coping methods that they'll need to get there, then - as Gillberg's study and Daunt's testament demonstrate - once they attain adulthood and secure increased control of their lives, there's little reason that their anxieties won't reduce. In turn the difficulties that they face will decrease and we can all benefit from the distinctive creativity and startling charisma that these individuals possess.
Below, girls from Limpsfield Grange School elaborating on their experiences.
If you're interested in learning more about PDA, take a look at the full interviews on Medium, which were used to inform this post, and visit websites of the National Autistic Society and the PDA Society.
Lead image, Out of Control by Erik Pevernagie.