"Rates of death are higher for autistic individuals compared to the general population"

It's world autism awareness day today (2 April). The theme this year (2019), according to the United Nations, is "Assistive Technologies, Active Participation", something rather timely given the recent realisation that quite a few people diagnosed with autism are not properly represented [1] in various fields. Indeed, it continues a theme on the under-represented and under-studied populations within the autism spectrum being highlighted today of all days (see here)...

To mark this years autism awareness day, I'm turning to a topic that is probably about as important as it gets with regards to autism or any label: premature death. Specifically, highlighting how premature premature death can actually be when autism is diagnosed. I appreciate that this is not great PR for autism. But it's real-life for too many; and needs to be talked about as much as possible so people sit up and (hopefully) do something about it...

So...

The quote heading up this post - "Rates of death are higher for autistic individuals compared to the general population" - taken from the findings reported by Ye In (Jane) Hwang and colleagues [2] is perhaps not altogether accurate. A more precise meaning is required: as a group, those diagnosed with autism or autism spectrum disorder (ASD) are at some elevated risk for a premature (untimely) death than the non-autistic population (see here). Not exactly great dinner party conversation but vitally important nonetheless.

This is a topic that has cropped up time and time again in the peer-reviewed research literature and beyond (see here and see here). The grim trend crosses geographical boundaries and seems to cover the entirety of the autism spectrum. This time around New South Wales in Australia provided the source data with the aim "to report the rates and risk factors for mortality and cause of death in individuals on the autism spectrum (n = 35,929 age range 5-64) with and without concurrent intellectual disability (ID)."

The results: "Mortality rates for those on the autism spectrum were 2.06 times that of the general population." In line with other data (see here), those who also presented with intellectual (learning) disability alongside autism were at a higher risk of death. Epilepsy is also reported as being an important issue too (see here). A few other details are highlighted in the Hwang paper including the observations that "injury and poisoning" were some of the top-ranked causes of death as per other data (see here and see here).

What's more to say on this topic? Another call to action (see here)? Some more big news headlines (see here)? Another 'long-term plan' (see here)? All well-and-good, but what's actually being done to tackle such frightening statistics here and now and actually improve and extend lives in the here and now? What's currently helping autistic people to 'age well' [3]? And just in case you're of the inclination that epilepsy and intellectual (learning) disability are some how not a 'core' part of some autism, you're probably wrong in many cases (see here and see here)...

If the Hwang and other data aren't enough to satisfy you on this topic, another research paper with another (similar) set of grim findings has also recently been published [4]. Faced with such data, discussions about a puppet depicting one face of autism (see here) for example, don't seem all that important do they? Indeed, if half as much time was dedicated to highlighting the mortality stats associated with autism and making sure this makes headlines - over a 20 year period "6.4% of individuals died at an average age of 39 years" - may be more would be done to tackle them, including providing "adequate access to health care for individuals with autism spectrum disorder."

Food for thought, and hopefully important fuel for action...

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[1] Russell G. et al. Selection bias on intellectual ability in autism research: a cross-sectional review and meta-analysis. Molecular Autism. 2019; 10: 9.

[2] Hwang YIJ. et al. Mortality and cause of death of Australians on the autism spectrum. Autism Res. 2019 Feb 25.

[3] Hwang YI. et al. Aging Well on the Autism Spectrum: An Examination of the Dominant Model of Successful Aging. J Autism Dev Disord. 2018 May 2.

[4] Smith DaWalt L. et al. Mortality in individuals with autism spectrum disorder: Predictors over a 20-year period. Autism. 2019. Feb 28.

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Suicide risk and autism: data from "Utah over a 20-year period"

The findings reported by Anne Kirby and colleagues [1] are not for the faint-hearted but continue a vitally important theme in autism research and practice circles looking at suicide risk and autism (see here and see here). The 'value-added' bit to the Kirby results to distinguish them from other study in this area was the reliance on data across a 20-year period from a place that has some important autism research history: Utah in the United States (see here).

So: "Four sources of existing data were utilized for this study: URADD statewide autism surveillance data, statewide suicide surveillance data collected by the Utah Office of the Medical Examiner (OME), the UPDB, and Utah's Indicator‐Based Information System for Public Health (IBIS‐PH)." From such data sources, researchers accessed information for nearly 17,000 people diagnosed with an autism spectrum disorder (ASD) "alive at the beginning of 1998 and at least 5 years of age in 2013." Most were male and most were white. They similarly determined that nearly 9,000 people in their total population - not just those diagnosed with ASD - died by suicide between 1998 and 2017. Again, most were male and most were white. The data were combined and interrogated "to calculate the incidence (in 5‐year intervals) of suicide deaths in people with ASD over a 20‐year period (1998 to 2017) in total, as well as by sex, and compared suicide risk in people with versus without ASD." Researchers also looked at other important variables such as "sex, race, death age, occupational status, marital status, and manner of death" across the groups.

Results: "In the first 15 years of the study (1998–2012), we did not observe differences in suicide cumulative incidences between the ASD and non‐ASD populations." This means that when the groups were compared as a function of death by suicide, the figures for those with autism were not significantly different from those without autism for this time period. By saying that I don't want to belittle the fact that between 1998 and 2012 for example, 2 people out of 5,202 autistic people died by suicide or that 1,671 out of 1,928,484 non-autistic people died. Each of these figures was a person with a life and with a family, and that's something that should never ever be forgotten.

The pattern however changed when researchers looked at the period between 2013 and 2017: "For the most recent time interval (2013–2017), the cumulative incidence of suicide death in the ASD population was 0.17%, which is significantly higher than the non‐ASD population cumulative incidence of 0.11%." This percentage (0.17%) represented 28 deaths from an autistic population of 16,907 and 2,791 deaths from a non-autistic population of 2,630,221. Although a sideline point, I'll also bring to your attention how the autistic population numbers changed over the 5-year blocks of study in the Kirby paper: 1998-2002: 5,202 people; 2003-2007: 8,722 people; 2008-2012: 13,890 people; 2013-2017: 16,907 people.

A few other details were observed by Kirby et al: "In comparison with non‐ASD + suicide cases, ASD + suicide cases had significantly younger average death ages (32.4 years vs. 41.8 years; t = −3.8, P < 0.001)." Also: "Combined, 73% of the ASD + suicide cases used methods for suicide considered to be violent; the remaining 26% used nonviolent methods." This again, is important information.

Researchers also mention how across the 2013-2017 period, another important trend was observed: "suicide risk in females with ASD was over three times higher than in females without ASD (relative risk (RR): 3.42; P < 0.01)." They contrast this with the finding that "there were no documented cases of suicide death among females with ASD during the first 15 years of the surveillance period" and what this could mean when it comes to possible explanations of suicide risk in relation to autism.

There's quite a bit to learn from the Kirby findings. Although there are limitations attached to the study design - "inadequate data on intellectual ability was available to examine the influence ID may have on suicide risk in individuals with ASD" - the study was a good one because of its population-wide focus and the pretty good autism-related resources that Utah has (and has had for many years). It demonstrates once again that the difficult topic of suicide and autism should remain a research priority in order to identify who might be most at risk and why, alongside the ways and means that such risk *might* be mitigated (see here for one example).

And for those who might need someone to text / email / talk to, there are always options (see here for services in the UK or see here for those elsewhere).

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[1] Kirby AV. et al. A 20-year study of suicide death in a statewide autism population. Autism Res. 2019 Jan 21.

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"Health inequalities in relation to people with learning disabilities": LeDeR reports.

LeDeR mentioned in the title of this post, refers to the Learning Disabilities Mortality Review, an English initiative designed to "make improvements to the lives of people with learning disabilities." Part of that 'make improvements' statement covers the important area of health inequality and some truly shocking early mortality risks that seems to follow a diagnosis of learning (intellectual) disability. To quote: "Today, people with learning disabilities die, on average, 15-20 years sooner than people in the general population, with some of those deaths identified as being potentially amenable to good quality healthcare." Remind me what century we live in again...

I've covered early mortality in the context of learning disability before on this blog (see here and see here) and how various factors seem to 'converge' to place someone diagnosed with a learning disability at some significant disadvantage when it comes to living a healthy life or being faced with an early death. So things like an acknowledgement that various physical conditions seems to be 'over-represented' alongside learning disability and the idea that diagnostic overshadowing is quite prevalent are some key points to consider (see here). Minus any sweeping generalisations, I should also mention that the quality of the care received (or sometimes not received) by some people diagnosed with a learning disability is also sometimes part of the picture too (see here).

The LeDeR report covering 2017 [1] once again paints a pretty grim picture of how we, as a society, treat some of how most vulnerable members. Some media coverage of the publication of the report can be seen here and other bloggers have dissected some of the observations (see here). The report notes from the period 1st July 2016 to 30th November 2017 there were over 1300 deaths reported to the LeDeR programme. Also: "The median age at death of people with learning disabilities (aged four years and over) was 58 years (range 4-97 years). For males it was 59; for females 56." And: "More than a quarter (28%) of deaths were of people aged under 50 years." More than a quarter of deaths were noted in those under 50 years of age. Let that sink in, as it appears that being diagnosed with a learning disability impacts on how long you're likely to live. Perhaps just as important was another observation made: "People with profound or multiple disabilities had a median age at death of 41 years; those with mild or moderate learning disabilities had a median age at death of 63 years." There appears to be a dose-related relationship between mortality and how disabled a person was.

Details on the cause of death also revealed some important patterns. Respiratory illness was mentioned on the death certificate of some 30% of people - the most frequently cited cause - and diseases of the circulatory system in around 15%. In more detail, these categorisations covered specific diagnoses such as pneumonia and sepsis. I should also mention that epilepsy was also mentioned as a cause of death in around 4-6% of cases.

Where next? Well, the report does make some recommendations to try and reduce/eliminate such health inequalities in the context of learning disability. Obviously, not every death recorded could have been avoided, but there are potential ways and means in which care might (should!) be improved to help lessen the risk of early mortality for some. Outside of things like 'strengthening collaboration and information sharing' noted in the report, I was drawn to a few other recommendations including one stating that "all people with learning disabilities with two or more long term conditions (related to either physical or mental health) should have a local, named health care coordinator" and another that: "There should be a national focus on pneumonia and sepsis in people with learning disabilities, to raise awareness about their prevention, identification and early treatment." I can't disagree with such future directions assuming that is, that the money required to set in motion such recommendations, also follows suit.

I'm becoming increasingly aware that there is a real issue present in the care of many different groups of people, specifically where a learning, developmental or psychiatric diagnosis is mentioned. People with a learning disability are at a heightened risk of early mortality. People diagnosed with a developmental disorder such as autism are at a heightened risk of early mortality (see here and see here). People diagnosed with a psychiatric disorder such as schizophrenia are at a heightened risk of early mortality (see here and see here). It strikes me that the time may be right to set up an independent commission covering these (often, very often, overlapping) diagnostic areas, and look at putting into action new ways of ensuring that such diagnoses are not a gateway to an early death.

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[1] The Learning Disabilities Mortality Review (LeDeR) Programme. 2018. Annual Report December 2017.

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Euthanasia and assisted suicide mentioning autism: inadequate safeguards and 'vulnerability'

By discussing the paper by Irene Tuffrey-Wijne and colleagues [1] (open-access), I'm once again returning to the complex topic of euthanasia and assisted suicide (EAS) where autism is mentioned (see here and see here).

As per my previous blogging entries on some of the peer-reviewed science on this subject, I'll reiterate how sensitive and contentious this topic is. On the one hand is the primary human freedom to choose. On the other, is another human right: the right to live and the sanctity of life. The two viewpoints collide on this topic (see here).

Tuffrey-Wijne et al follow on from the work of Kim and colleagues [2] by focusing on the Netherlands, where EAS is legal. Legal that is, "provided that statutory due care criteria are met, including: (a) voluntary and well-considered request; (b) unbearable suffering without prospect of improvement; (c) informing the patient; (d) lack of a reasonable alternative; (e) independent second physician’s opinion." The authors searched the records of regional review committees (RTE) who provide oversight on "whether the requirements of 'due care' had been observed" in cases of EAS, looking for any mention of intellectual (learning) disability and/or autism spectrum disorder (ASD). They found a small number of people (N=9) who met these diagnostic criteria and set about analysing whether "the EAS due care criteria are applied." Their conclusions: "The Dutch EAS due care criteria are not easily applied to people with intellectual disabilities and/or autism spectrum disorder, and do not appear to act as adequate safeguards."

Authors provide details on the nine cases, including "selected quotes with regards to the patients’ stated characteristics, diagnosis, and due care criteria." I was particularly struck by some of the reports included under the heading 'unbearable suffering without prospect of improvement' which provides not only insight into how lives can be so totally affected by a diagnosis, but also a reflection of how we (society) continue to fail so many people on so many different levels. I know to use the word 'suffering' in the context of autism for example, has the ability to furrow brows in some quarters. But for these nine people there did seem to be genuine misery and suffering. To quote: "For two patients, for whom various psychiatric and somatic conditions were described, the stated suffering appeared to stem from characteristics of autism spectrum disorder itself, rather than from acquired medical conditions." Factors such as a 'loss of control' and the manifestation of issues stemming from autism "that may not be directly understandable to others" are also detailed.

The authors conclude: "particular caution [is required] in cases of EAS requests from people with intellectual disabilities and/or autism spectrum disorder, with the onus on both physicians and the RTE to demonstrate much more clearly how all due care criteria were met." In other words, countries that have legalised EAS need to do quite a bit more to ensure that vulnerable groups are not unfairly disadvantaged and are supported as much as possible when it comes to making such an extreme decision. I'll also say again (see here), vulnerability is too often seen as a 'dirty word' when it comes to autism, despite there being ample evidence that vulnerability manifests widely across many areas of daily living for those diagnosed. I can think of no more 'final' outcome than EAS where the (supposed implied) recognition of vulnerability needs to be paramount to any decision being made.

As per the opening paragraph to this blog entry, there is a balance to be struck between the sanctity of life and personal choice in this area. But that 'choice' needs to be an informed choice; with the requirement to ensure that any "such [EAS] legislation includes sufficient safeguards to protect vulnerable patient groups."

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[1] Tuffrey-Wijne I. et al. Euthanasia and assisted suicide for people with an intellectual disability and/or autism spectrum disorder: an examination of nine relevant euthanasia cases in the Netherlands (2012–2016). BMC Medical Ethics. 2018; 19: 17.

[2] Kim SY. et al. Euthanasia and Assisted Suicide of Patients With Psychiatric Disorders in the Netherlands 2011 to 2014. JAMA Psychiatry. 2016 Apr;73(4):362-8.

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On unintentional drowning deaths in children with autism

Sometimes science seems to be science for the sake of science. Y'know, findings are reported and published and are met with either 'so what' or 'what does this mean for me?' sentiments, particularly when dealing with potentially abstract concepts.

The findings published by Joseph Guan and Guohua Li [1] most definitely DO NOT fall into such a category. Their covering of a topic which has potential life-limiting implications - unintentional drowning deaths - in the context of autism is worthwhile repeating again and again and again until everyone sits up and takes note. I might add that other previous research from this authorship team similarly evoke such sit-up-and-listen sentiments (see here).

What did the authors do? Well, simply put, they scanned the Lexis-Nexis® Academic database looking for all newspaper entries covering the terms autism, drowning and boy/girl from the beginning of January 2000 until May 2017 in the United States. They analysed the collected data; retrieving specific details such as "time of day and distance from residence" when it came to such reports.

Results: "During January 2000 through May 2017, US newspapers reported a total of 23 fatal unintentional drowning incidents involving children under 15 years of age with ASD [autism spectrum disorder]." Let's just reflect on that a moment. Twenty-three children / young adults with autism who drowned. Twenty-three lives tragically cut short. Twenty-three families left grieving.

Also: "Data about proximity of the water body to the victim’s residence were available for 11 (47.8%) of the incidents, with all of them within 1000 m of the victim’s residence (mean = 290.7 ± 231.5 m)." And also: "The time of day at which victims were reported missing was available for 15 (65.2%) of 23 incidents, with 2 (13.3%) being in the morning (0:00–11:59), 11 (73.3%) being in the afternoon (12:00–17:59), and 2 (13.3%) being in the evening (18:00 PM – 23:59)." And finally: "Wandering was the most commonly reported activity that led to drowning, accounting for 73.9% of the incidents."

I could go on about the limitations of this study as highlighted by the authors - "small sample size and the availability of information reported in newspaper articles" - but really I have to ask 'does it matter?' The answer: no, such study limitation don't really make too much difference to the final - very final - outcomes reported on.

The fact that wandering (elopement if you will) was a feature of many of the cases found is not new news (see here). It reiterates once again that resources aplenty need to be put into reducing incidences of wandering or at least allowing parents and law enforcement and other agencies every opportunity to locate wandering children/adults as quickly as possible. Some might worry about things like civil liberties when it comes to monitoring someones movements. But in current times, when someone can be tracked by their mobile/cell phone use for example, I'd respectively disagree with such 'civil rights are being impinged' sentiments. Imagine if you will, if one of the various 'tracking' devices currently aimed at those on the autism spectrum was given to every child / every family free of charge at the point of diagnosis? A good use of money methinks...

Water safety is another important part of the current findings. There is science out there talking about how learning to swim might have quite a lot of positives when it comes to autism [2]; perhaps the most important being learning water safety skills. Who would argue with that? And if one wanted to be proactive in this area, how about making water safety and swimming lessons a compulsory part of the learning curriculum for everyone diagnosed with an autism spectrum disorder? Again, a very good use of money methinks and you never know, there may be other benefits too.

I'm not saying that there aren't individual circumstances around every one of those drowning deaths discussed by Guan & Li. I'm not saying that every death could have been avoided. What however I do believe is that armed with the knowledge that drowning is a significant cause of premature death in the context of autism, and knowing a little more about the general circumstances around some of those deaths, there are things that can potentially be done to mitigate future risks to the autistic population and potentially save lives.

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[1] Guan J. & Li G. Characteristics of unintentional drowning deaths in children with autism spectrum disorder. Injury Epidemiology 2017; 4: 32.

[2] Alaniz ML. et al. The Effectiveness of Aquatic Group Therapy for Improving Water Safety and Social Interactions in Children with Autism Spectrum Disorder: A Pilot Program. J Autism Dev Disord. 2017 Dec;47(12):4006-4017.

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"What Happens When I Can No Longer Support My Autistic Relative?"

I've kinda touched upon the subject matter examined in the paper by Renske Herrema and colleagues [1] before on this blog (see here). Detailing important results on views and concerns about the future and specifically what will happen to loved ones with autism when families are no longer able to care for or support them, the findings approach a difficult but important question. Part of that questioning surrounds the perception that social and other supports for example, are pertinent and in place to keep loved ones safe, cared for and able to deliver suitable services onward to ensuring a nurturing environment that caters to the individual's needs, wants and wishes.

Drawing on data from "120 family members of autistic adults" (or adults with autism if you prefer), authors asked about "concerns about the future for their relative" via an online survey. Several key themes emerged from their inquiry on things like concerns for individual needs not being met, the happiness of their loved one and the question of who will care for them as and when primary caregivers are not able to or are not around to care for them. These concerns were things that quite regularly featured in the minds of family members according to their online reporting.

The authors talk about the need for planning to start early - 'timely' - when it comes to ensuring that support is both available now and in the future for family members with autism. I would definitely agree with such early planning given the history of almost Herculean efforts that parents/caregivers have had to go through to ensure that their loved ones are provided the same rights as anyone else. Indeed, legacies have already evolved from such planning (see here). I do worry however that there are factors that parents and other family members seem to have to overcome in modern times; where austerity is pushing social care to breaking point (at least here in Blighty) and the availability of social support being more and more reserved for those who cannot live independently potentially at the expense of the 'look like they're managing' masses...

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[1] Herrema R. et al. Brief Report: What Happens When I Can No Longer Support My Autistic Relative? Worries About the Future for Family Members of Autistic Adults. J Autism Dev Disord. 2017 Jul 28.

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Anxiety disorders and mortality risk: implications for autism?

"Anxiety disorders significantly increased mortality risk. Comorbidity of anxiety disorders and depression played an important part in the increased mortality."

So said the findings reported by Sandra Meier and colleagues [1] looking to assess any relationship between the presence of an anxiety disorder and mortality risk. Based on data from one of those oh-so-useful Scandinavian population registries (Denmark this time), researchers reported that: "The risk of death by natural and unnatural causes was significantly higher among individuals with anxiety disorders... compared with the general population." Death by unnatural causes was also linked in quite a few cases to "comorbid diagnoses of depression."

Although making sombre reading, the data from Meier et al provide further evidence [2] that a psychiatric/behavioural diagnosis might have far-reaching implications when it comes to the risk of early mortality, be that based on natural causes or something rather more unnatural such as death by suicide or an enhanced risk of accidental death or death because of illness. This also follows a trend suggesting that severe mental illness also has social implications such as an increased risk of becoming a victim of crime too (see here). Quality of life, health-related or otherwise, is nearly always affected by such diagnoses.

I introduced the 'implications for autism' bit to this post simply because (a) when it comes to comorbidity surrounding the diagnosis of autism, anxiety and depression (various types) pretty much come top with regards to psychiatric labels applied (see here and see here respectively) and (b) enhanced risk of early mortality is also an unfortunate feature when it comes to autism too (see here). Putting these findings together and well, I'm sure you can understand the need for quite a bit more study in this area and in particular, a reiteration of how utterly disabling anxiety and/or depression can be when it comes to autism.

If and when possible roles for anxiety and/or depression are found to contribute to some of the excess risk of early mortality when it comes to autism, the bright side is that this could have implications for intervention and management and onwards a reduction in mortality risk. I might also introduce the findings reported by Butnoriene and colleagues [3] at this point, who suggested that sex differences might also be relevant to the type of risk factors associated with mortality. Discussions in this area should also probably include discussions on a related topic based on a particularly extreme path being selected by some on the autism spectrum (see here).

Without trying to make connections where none might exist, I'm also inclined to suggest that outside of psychological and pharmacological interventions to tackle anxiety and/or depression comorbid to autism, one might also look to treating certain somatic correlates also potentially exerting an effect (see here). There is potentially lots to examine across such comorbidities as yet again, another very important line of study opens up that intersects with autism.

Finally, dare I also add that other labels such as obsessive-compulsive disorder (OCD) that may also intersect with some autism (see here - yes, this is another Meier paper) might also increase the risk of early mortality when it comes to autism too [4]?

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[1] Meier SM. et al. Increased mortality among people with anxiety disorders: total population study. The British Journal of Psychiatry. 2016; 209: 216-221.

[2] Pratt LA. et al. Excess mortality due to depression and anxiety in the United States: results from a nationally representative survey. Gen Hosp Psychiatry. 2016 Mar-Apr;39:39-45.

[3] Butnoriene J. et al. Metabolic syndrome, major depression, generalized anxiety disorder, and ten-year all-cause and cardiovascular mortality in middle aged and elderly patients. Int J Cardiol. 2015;190:360-6.

[4] Fernández de la Cruz L. et al. Suicide in obsessive-compulsive disorder: a population-based study of 36 788 Swedish patients. Mol Psychiatry. 2016 Jul 19.

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Meier SM, Mattheisen M, Mors O, Mortensen PB, Laursen TM, & Penninx BW (2016). Increased mortality among people with anxiety disorders: total population study. The British journal of psychiatry : the journal of mental science PMID: 27388572

What will happen to my child when I'm gone?

From time to time I cover some uncomfortable topics on this blog as a function of what hand the autism research cards deal. Today is another one of those times as I bring to your attention the paper by Cathy Cox and colleagues [1] and their analysis of death concerns and psychological wellbeing in mothers of children diagnosed with an autism spectrum disorder (ASD).

What they observed based on completion of a "fear of death scale" and "measures of death-thought accessibility, positive and negative affect, depression, and anxiety" by some 70 mums of children with autism and 70 mums of "typically developing children" suggested that more investigation in this area is required. Aside from reporting "worse psychological health" than control mums, the autism mums group "evidenced greater death-thought accessibility" that in turn "mediated the influence of ASD diagnosis on negative affect, depression, and anxiety." In other words: "increased death-thought accessibility among mothers of children with ASD was associated with worse psychological health."

Thinking about one's own mortality and the idea that our time on this dusty rock called home is finite is not an uncommon feature of life. Death is a daily feature of life as any newspaper or news website informs us. Specifically with autism in mind, various viewpoints have been published by parents of children with autism on the topic of death concerns and the important question: what will happen to my child / children when I'm gone?

This is an uncomfortable question to try and answer given the multitude of factors around things like provisions, finances and family circumstances including the role that any siblings may need to play. That also a parents death will inevitably affect the child (or adult) with autism serves to further complicate any response. It's perhaps not surprising that some parents have written some fairly extreme material with titles like 'Why I can never die' when it comes to this topic.

There is no easy way through this important subject. Cox et al talk about how training care providers to "better discuss thoughts of death may help to alleviate stress and foster greater psychological well-being" for parents of children with autism as being one answer. I agree that death needs to figure more in conversations but am slightly unsure as to how talk without positive action and planning is going to put minds at rest and reduce an already heavy burden of stress and risk of adverse psychological health. That there may also be some fairly unique circumstances associated with the presentation of anxiety in some mums [2] (see here for further reading on intolerance of uncertainty) perhaps adds to the requirement for quite a bit more study and action in this important area.

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[1] Cox CR. et al. Death concerns and psychological well-being in mothers of children with autism spectrum disorder. Res Dev Disabil. 2015 Aug 6;45-46:229-238.

[2] Uljarević M. et al. Brief Report: Effects of Sensory Sensitivity and Intolerance of Uncertainty on Anxiety in Mothers of Children with Autism Spectrum Disorder. J Autism Dev Disord. 2015 Aug 9.

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Cox CR, Eaton S, Ekas NV, & Van Enkevort EA (2015). Death concerns and psychological well-being in mothers of children with autism spectrum disorder. Research in developmental disabilities, 45-46, 229-238 PMID: 26256841

Sibling death by defenestration: a case report

The case report published by Osman Sabuncuoglu and colleagues [1] (open-access) highlighting the extremes of certain high-risk behaviours potentially associated with autism is the topic of today's brief post.

Detailing the very saddest of outcomes whereby a young boy diagnosed with autism and "aggression, violence and poor behavioral control" threw his 18-month old sister out of a window (defenestration) causing her death, the authors draw attention to several issues tied into the extremes of aggression appearing alongside autism and the legal implications of such behaviour under such circumstances.

The authors describe how due to the gravity of the issues faced by the autistic child (including a degree of learning disability) "the child had no preconception of consequences of his behavior" already with a history of violence towards caregivers before the very unfortunate episode with his sister. The subsequent criminal investigation carried out on this incident was eventually dropped "on the grounds of incompetence due to insanity and being below the age of criminal responsibility."

Treading carefully so as not to make any sweeping generalisations about the very heterogeneous autism spectrum, the Sabuncuoglu report highlights how aggression can manifest alongside [some] autism and brings into focus the plight of quite a few families dealing with such issues day to day. Judging by the lack of peer-reviewed literature on this topic, I am assuming that the extent and effects of the aggression detailed by Sabuncuoglu is thankfully pretty rare(?) (in terms of endangering life for example) but that isn't to say that it hasn't happened before (see here).

"The most distinctive symptoms that led to the death of the sibling seem to be a high level of aggression, low level of impulse control and severe form of disability." Alongside the idea that there may be various strategies that can be employed to potentially off-set such variables (see here and see here for examples), I'd be minded to suggest that further investigations on such factors such be a research priority in terms of improving quality of life for those presenting with such issues and their family and loved ones.

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[1] Sabuncuoglu O. et al. Sibling death after being thrown from window by brother with autism: defenestration an emerging high-risk behavior. Case Reports in Psychiatry. 2015. July 21.

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Osman Sabuncuoglu, Mustafa Yasin IRMAK, Nagehan Ucok Demir, Duygu Murat, Can Tumba, & Yuksel Yilmaz (2015). Sibling death after being thrown from window by brother with autism: defenestration an emerging high-risk behavior Case Reports in Psychiatry

Mortality and autism

I know the topic of this post is not exactly great dinner table conversation, particularly at this time of year during the season of goodwill to all.  Nevertheless I'm posting today on the paper by Deborah Bilder and colleagues* looking at mortality and causes of death in relation to autism spectrum disorder (ASD) simply because there are important lessons which can be learned and applied with relative ease as a result of some of their collected data.

Regular readers might know that one of my interests on this blog is the issue of health inequality when it comes to conditions like autism and indeed beyond. It's a sad fact that whilst autism generates quite a lot of discussion, research, politics and emotion, the message that people with autism are people first, and hence are at least facing the same risk (if not more) of developing health-related complications, can get a little lost in the noise. I'm as guilty as anyone in losing this message with my constant ramblings on the wonders of autism research.

Papers like the one from Bilder et al however reinforce the view that autism, in some cases, might very well place a person at increased risk of early mortality and outside of just seeing a person with autism, we could do a lot by putting the person and their physical health and wellbeing first and foremost.

A few bullet-points from the Bilder paper:

• The study was based in quite an important geographical area for autism research, Utah. Important because as I've blogged about before, Utah has been the focus of some milestone studies on autism, in particular the UCLA-University of Utah Epidemiologic Study of Autism. Indeed some of the original data from that initiative has quite recently become the source of some discussion as part of the autism numbers game and the transition from DSM-III to DSM-IV.
• In particular, the authors sought to follow-up the cases of autism identified during the UCLA-University of Utah study and estimate the mortality hazard rate ratio (HRR) whilst look at the patterns of mortality and what factors were linked to death. The authors define HRR as: "an estimate of the excess risk associated with the identified exposure (i.e. ASD case status) as a constant effect over a specific time period (i.e. interval since case ascertainment) while adjusting for selected covariates (i.e.birth risk factors)".
• Based on a cohort of 305 adults with autism (DSM-III and DSM-IV-TR diagnosed/reclassified participants), 29 people (9.5%) had died by the end of 2011. 
• Most of the deceased were male, and the age at time of death varied between 7 - 46 years old.
• The HRR overall was 9.91 compared to population controls (N=2466). When taking into account the different genders, the HRR for males was 7.92 and for women with an ASD a staggering 20.71 (without covariates).
• As one would expect, there were various causes of death indicated on death certificates, ranging from seizures to cardiac-related disorders. Epilepsy was a prominent feature for quite a few of the cohort as per the range of physical disease comorbidity observed in cases. That being said, and without making any judgements, death listed as a result of an "open wound of hip" in one case invites further investigation. 
• Death by unnatural causes were relatively few although "two individuals died from an adverse event related to medication".

The Bilder paper does make somber reading when you realise that behind every statistic there was a person and a family. Indeed looking at the list of deceased, their intellectual and autism classification, their cause of death and importantly, their accompanying physical disease, one is struck by the heterogeneity present.

With my cold, dispassionate research spectacles on a few points caught my eye.

It was interesting to note how many cases cite cerebral palsy (CP) for example, as a comorbidity to their autism diagnosis (I counted 6 cases of the 29 deceased). I'm not inferring that CP was the cause of death, even though it was listed as a factor in at least one case, but rather that those cases with comorbid CP might indicate some elevated risk of early mortality as per the literature on excess mortality in CP** alone.

Reports of comorbid Sanfilippo syndrome were also present in more than one case of autism, two actually. Given the rarity with which this syndrome appears - anywhere between 1 in 100,000 to 1 in 1,000,000 in the general population - to see even one case in the current cohort should automatically be a cause of some interest bearing in mind presentation can include autistic features and the syndrome previously being mentioned in the autism research literature*** (2 / 222 cases of autism). Given the poor prognosis of Sanfilippo syndrome, one might reasonably assume some impact on mortality where autism also appears alongside.

I also observed that cancers and neoplasms did not seem to prominently figure among the deceased as a cause of death. One could argue that given the relatively young mean age of the deceased, this might play a role in mortality and indeed the lack of accompanying information on things like cancer treatment and survival among this cohort. But I'm interested in this phenomenon, especially when one considers what the population at large - as in everyone whether with autism or not - generally tend to die of (see this post for more information).

The low prevalence of death by unnatural causes also caught my attention. I've talked fairly recently about the wandering and autism research which was published and how wandering can, in a few cases, lead to the very worst outcome when autism is involved. As far as I can surmise and allowing for the difference in age groups looked at, wandering was not implicated in any of the cases looked at by Bilder. I'm not able to provide an in-depth view of wandering leading to the circumstances of early mortality in autism, but one speculates whether an overall increase in cases of autism might be a factor in the very public recording of death implicating wandering. Same goes for issues like suicide, recently discussed with autism in mind****.

Finally, I can't leave this post without commenting on those two people where death was linked to medication use. In both cases, individuals were in their late 30s-early 40s, and one case also carried a diagnosis of schizophrenia. Accepting that medicines - particularly those with a psychotropic effect - are still a voyage of discovery in terms of their precise effects and mode of action, pharmacotherapy is as much about good medicines management as it is about the effects of the drugs themselves. I note for example one case where poisoning due to exposure to "unspecified" drugs is cited alongside the word 'obesity'. The relationship between these concepts has been discussed before including the important issue of side-effects.

The take-home message from this post is best left to another comment from the paper:

"The elevated mortality risk associated with ASD in the study cohort appeared related to the presence of comorbid medical conditions and intellectual disability rather than ASD itself suggesting the importance of coordinated medical care for this high risk sub-population of individuals with ASD."

I would struggle to disagree.

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* Bilder D. et al. Excess mortality and causes of death in autism spectrum disorders: a follow up of the 1980s Utah/UCLA Autism Epidemiologic Study. J Autism Dev Disord. September 2012.

** Strauss D. et al. Causes of excess mortality in cerebral palsy. Dev Med Child Neuol. 1999; 41: 580-585.

*** Ververi A. et al. Clinical and laboratory data in a sample of Greek children with autism spectrum disorders. J Autism Dev Disord. 2012; 42: 1470-1476.

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Bilder D, Botts EL, Smith KR, Pimentel R, Farley M, Viskochil J, McMahon WM, Block H, Ritvo E, Ritvo RA, & Coon H (2012). Excess Mortality and Causes of Death in Autism Spectrum Disorders: A Follow up of the 1980s Utah/UCLA Autism Epidemiologic Study. Journal of autism and developmental disorders PMID: 23008058