In 2006 a case report was published about a 6-year-old girl, Ashley, who has profound developmental disabilities and was treated with oestrogen patches to limit her final height, along with a hysterectomy and the removal of her breast buds. Ashley's parents claimed that attenuating her growth would make it possible for them to lift and move her more easily, facilitating greater involvement in family activities and making routine care more straightforward. The ‘Ashley treatment’ provoked public comment and academic debate and remains ethically controversial. As more children are being referred for such treatment, there is an urgent need to clarify how clinicians and ethics committees should respond to such requests. The controversy surrounding the Ashley treatment exists, at least in part, because of gaps in the literature, including a lack of empirical data about the outcomes for children who do and do not receive such treatment. However, we suggest in this paper that there is also merit in examining the parental decision-making process itself, and provide empirical data about the reasoning of one set of parents who ultimately chose part of this treatment for their child. Using the interview data, we illuminate some important points regarding how these parents characterise benefits and harms and their responsibilities as surrogate decision-makers. This analysis could inform decision-making about future requests for growth attenuation and might also have wider relevance to healthcare decision-making for children with profound cognitive impairment.
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The Ashley treatment has been the subject of intense debate1 ,2 and continues to be controversial.3 Despite this, we are aware of two similar cases in New Zealand and further treatment requests in other countries.4 ,5 Whether and when this form of treatment should be available to children with profound cognitive impairment (PCI) continues to be relevant and a challenge to clinicians. One of the best ways to guide decision-making is to collect empirical evidence through systematic studies of treated and untreated children. However, as there are no large-scale studies to draw on, case studies of individuals receiving such treatment are an opportunity for preliminary investigation. In this paper, we augment the existing literature by providing a detailed account of the way in which one set of parents reasoned about growth attenuation (GA) treatment for their child. In doing this, we aim to illuminate some of the important views and values underlying their decision and reflect on their wider relevance.
Anna (a pseudonym) is a 6-year-old girl with multiple profound disabilities related to neonatal hypoxic ischaemic encephalopathy. She has severe quadriplegic cerebral palsy, microcephaly, profound global developmental impairment, and complex epilepsy. Developmentally, Anna functions at around a 3–4 month level, requiring care 24 h a day, and adult assistance for every task. She is unable to speak, feed herself, sit unaided or roll over, but smiles responsively on occasion and sometimes giggles when tickled. She seems to recognise regular carers and situations and appears to enjoy movement, swimming, face-to-face communication and certain television programmes. She does feel pain and discomfort, relaying this by grizzling, crying or screaming. She has made minimal developmental progress over the last 6 years, suggesting that further significant progress is unlikely.
Anna's parents are both fit and well and were both in employment before her birth; since Anna was 18 months old, they have been paid as her carers through a New Zealand scheme that compensates those affected by accidents (Anna's condition has been attributed to medical misadventure in the perinatal period).
Anna is her parents’ first child, and, although they both express a desire to have several children, they delayed subsequent pregnancy until Anna reached school age, in order to provide appropriately for both children.
Clinical and ethical review process
Anna was 4 years old when her parents requested GA therapy at a routine clinic visit with their paediatrician. Their request was influenced by a number of factors in addition to the main concern that she would become increasingly difficult to move as she grew bigger and heavier without making developmental progress in other areas. Firstly, Anna had recently entered precocious puberty (a recognised complication of her neurological disorder); secondly, her parents were aware of reports detailing ‘the Ashley treatment’; finally, they knew of a similar case in New Zealand.
Anna and her parents were referred to a paediatric endocrinologist who, recognising the controversial nature of the treatment, consulted widely with peers and requested ethical review. The case prompted much deliberation, but ultimately GA was deemed ethically acceptable in Anna's case, and treatment was started in 2012, at age 5 years.
Anna's parents were aware of the controversial nature of the treatment, and although preferring their family not to be identified, they consented to both interview and publication as a way of advancing thinking. They were interviewed together by NK in the family home. The interview followed the chronology of the parent's decision, beginning with what treatment they requested and why, treatment effects, and their perspective on who should decide.
This structure enabled the parents to talk about what was important to them, and to reflect on the whole process, including the values that underpinned their thinking. While the schedule guided the interview, other issues that arose spontaneously were followed-up and probed further. The interview was audiotaped and transcribed verbatim.
The transcript was analysed thematically informed by the predetermined interview structure, an initial transcript review, and the literature. An initial descriptive phase fulfilled the first aim of Interpretative Phenomenological Analysis—that is, to explore the participants’ view of the world and to adopt as far as possible an insider's perspective of the phenomenon under study.2 Subsequently, a further phase of analysis was undertaken in which the initial themes were refined through deeper analysis of the data and our own interpretation or explanation of why the data were taking a particular form.3 These themes have been used to structure the following excerpts from the interview. In order to check the validity of our descriptions and interpretations, draft findings were provided to Anna's parents. We were cognisant of the fact that reading these reports may be confronting or distressing for Anna's parents, but they clearly stated that this was not problematic for them in comparison with the challenges they face as part of daily life. They were keen for the perspectives reported here to be disseminated in the academic environment. As this is a case study, formal Human Research Ethics Committee approval was not sought, but written consent was obtained from the family.
Anna's mother talked more during the interview, but both parents seemed to share similar opinions and perspectives on their daughter's life in general and in relation to GA in particular. They also share much of her practical day-to-day care. All quotes are from Anna's mother unless prefixed with ‘Father’.
The data are reported initially in a fairly descriptive manner, with themes aligned to the chronological course of the treatment decision and the priority given to different issues by the parents (eg, they focused much more on the potential benefits of treatment). In the later part of the Results section, themes reflecting the parental views and values underlying their decision-making are explored and interpreted in more detail.
The treatment these parents requested
Anna's parents were aware of the website run by Ashley's parents and initially viewed GA as part of a package:
all we knew was the Ashley treatment, we just figured it all … had to happen, I mean you read about the Ashley treatment and they say you know, it decreases chances of cancer and rah rah rah, … but on talking to the doctor, he didn't really feel like it was necessary and I tend to trust Kiwi doctors…
However, they ultimately decided not to pursue hysterectomy, sharing the concerns of the paediatric endocrinologist that this procedure was potentially more harmful and less beneficial to Anna, and that less invasive alternatives existed if necessary:
surgery's a whole different thing to oestrogen patches
As they had no family history of breast cancer or large breasts (as was the case for Ashley's family), they also did not request removal of breast buds, preferring to take an expectant approach to breast development. These aspects of the interview highlight the importance of dialogue and shared decision-making between parents and local specialists concerning each particular child. Subsequent discussion will focus on the GA.
Why they requested GA
Anna's parents spoke first of the potential benefits they envisaged, all of which focused on the issue of size:
The reason we were interested in oestrogen therapy is, it was about keeping Anna small, that was really the only, you know the main goal…because, I mean we still hold Anna like our baby
They were aware of mechanical aids that could help them move a larger child, but objected to the way in which such appliances would change the nature of their interaction with their daughter:
We just keep picking her up and that's what we expect we will continue to do, we don't want to put a hoist between us and our daughter, … she is still like our baby, she still sits on our lap and putting a hoist in between that just makes it feel so, what's the word I'm looking for, institutional you know…
Father …exactly, it just puts this big divide between us and her and that's just not nice
They also argued that the time involved in using hoists would preclude the frequent changes of position that were currently part of their daughter's routine:
… transfer her into that and then back out of that and into the next thing … onto our lap for a meal and then down onto the ground for a change of bum so there's, I think … something like 35 transfers in a day as we do things now so … that would have to dramatically reduce…
These parents consider frequent changes of position to be an important component of Anna's quality of life, something that could be overlooked by those without direct experience of caring for a profoundly disabled child. They articulated this more from the perspective of avoiding negative experiences and consequences that might arise through prolonged confinement in one setting rather than direct benefits from the new position:
She doesn't like staying in one position for a long time; we've got a lot of different bits of equipment for her … standing frame, gravity chair, expander chair, Hart walker. There's a lot of transfers that need to be done …
They see the consequences of a reduction in the number of position changes as being quite profound and far reaching. For example, they describe how a small change may lead to a ‘cascade’ of negative effects:
she'd miss out on a lot of activities or she would be stuck in a piece of equipment she doesn't like … if you watch her at school in her expander chair, she gets grumpier and grumpier and they move her out and put her in the bean bag and she has a kick around and that makes her a lot happier. Same thing here if she had to be in the expander a lot more … then she would get grumpier which means her tone would increase and that would set her seizures off more, which means we'd probably end up giving more drugs …
In addition to the benefits of position change, Anna's parents envisage maintaining her small size as a way of avoiding other problems:
… for every centimetre bigger she gets, I notice she gets that little bit more contorted…, so every centimetre we can keep off her, the better and it's not just …how she looks but also how she feels…it can't be comfortable sort of like this (mimics upper limb contractures).
It's easier physically on us to stretch her… I've noticed that the bigger she gets, the harder it is to actually do the stretches… the harder it is on our bodies to get into the right position to stretch her.
Anna's parents spoke much less of the potential harms of GA therapy, but were aware of both the available data and the prognostic uncertainty. Lack of direct experience with high-dose oestrogen treatment in young children makes the possible adverse effects and risks difficult to assess. Studies involving teenage girls reported mild adverse effects such as nausea, headache and weight gain, and, in combined reports of more than 700 girls, there was one report of a mild deep vein thrombosis in a girl with a strong family history.6 ,7 They also believe the impact of such an event on their daughter may be very different from that on a typically developing child, and should be considered alongside the many other disorders and interventions she has experienced:
… it's just one more thing … when we talked about what the risks would be, you know an increased risk of stroke, blood clots … I'm like well you know she can go into status (epilepticus) with this any second …she wasn't meant to live past one.
A little clot is the least of our worries when we see the potential benefits as being quality of life, you know I mean if she had a stroke and lived through it, what's gonna be different…?
In terms of psychosocial effects, these parents do not see being small as in any way disadvantageous for Anna either in the way other people view her:
for Anna being small is kind of the least of her worries, I mean OK people might look at her and say she's different, anybody could look at her now and say she's different
Or in relation to her own awareness:
…I would say I am 99.9% sure she will never have any idea that she's smaller than other people.
Actual effects of treatment on their child
At the time of interview, Anna had been treated with oestrogen patches for approximately 1 year. As expected, she had experienced an initial increase in size that subsequently slowed as her bone age advanced and she approached the end of her growth.2 There were no treatment complications, but, in this section, we detail how the family experienced the process of their daughter's puberty.
During the year, Anna experienced menstrual bleeding, although only one episode seemed to cause her discomfort:
… she got that first lot of bleeding and went a bit green at school … all we did was bring her home, give her a Panadol and let her sleep and she was fine…She has had a few more bleeds, but none of them were accompanied by her being grumpy or sick or anything
The blood itself caused Anna no distress but was initially unsettling for her father:
Father: A bit bizarre from a male's point of view … seeing this 6-year-old daughter, 5-year-old daughter going through periods.
It did not, however, present a significant practical problem to the family:
It's (the blood) just in her nappy … just whoever was changing had to know that...
Some of the pubertal changes had been evident before the GA treatment progressed:
… her pubic hair was growing beforehand…and that's probably the most obvious thing, and the most difficult thing was just … cleaning blood out of her pubic hair
Some of the effects had not been specifically anticipated—for example, Anna's sleep pattern altered:
She did start to sleep like a teenager, she started to sleep in and we had to keep waking her up for school.
Father: Sleep in late, yeah.
Other effects had been predicted and discussed—for example, Anna had a small amount of breast development:
She's got a wee bit of breast development but it's certainly nothing that anybody would notice…
Father: Most people wouldn't have a clue; it just looks like a little chubby chest.
But this did not cause any problems with wheelchair straps:
Father: We don't strap her in tight enough to have any effect anyway.
… she's miles away from anything like that after one year of treatment.
This family believe that neither menstruation nor breast development have had significant negative effects on Anna's quality of life, supporting their decision to proceed with GA alone.
Parents’ perspective on who should decide
Anna's parents understand why people may see GA as controversial:
we knew it was controversial … we could see … how people could take it the wrong way. To me the people that take it the wrong way, they're applying it to everybody's situation but our situation is quite different … it's something normal families don't even need to think about.
They do not personally consider it to be significantly different from other treatment decisions they have made, or will make, in conjunction with the paediatrician. For example, Anna has undergone tendon releases, adenotonsillectomy and femoral osteotomies, and a gastrostomy is under consideration. Furthermore:
X (paediatrician) has suggested Botox for Anna's dribbling…well how is that so different?
Here they are specifically alluding to the fact that the aim of diminishing Anna's dribbling is not only her comfort but also to make their own lives easier. They have also had significant involvement regarding the dosage and combination of anticonvulsants:
X (paediatrician) might say … this is the epilepsy medication you should be trying, but how's that working for you guys…there's no point in us filling her with medication only to find she's asleep constantly, if we can live with these seizures and we're getting this sort of behaviour and it's nice and everybody's happy, then cool.
These sections have detailed how Anna's parents characterise the harms and benefits of GA and how they see their role as surrogate decision-makers. Through the lens of their deep and nuanced understanding of Anna's quality of life, they believe the potential positive effects of GA far outweigh any potential harms. They also believe that GA treatment falls within a broad realm of treatment decisions into which they should have significant input.
In addition to these findings, the threads of a broader, superordinate theme, ‘the parent–child relationship’, were evident throughout the interview. These threads are explored further here, in subcategories that arose directly from the data, to assist in understanding why Anna's parents reasoned in the way described, and what values underpin their decision-making.
The parent–child relationship
Anna as a newborn
Anna's parents stated several times throughout the interview that they view their daughter in many ways as a young infant. For example:
we still hold Anna like our baby and …it's like when you've got a newborn, you've always gotta think, assume that the newborn's coming with you, well Anna's still at that stage.
Other aspects of the interview suggest that they don't mean this in an entirely literal or complete sense: they do many things for Anna that would not be appropriate for a young baby, such as sending her to school. What they do seem to mean is that they view Anna's fundamental needs and experiences as being profoundly similar to those of a young baby.
This view leads them to a particular conception of Anna's quality of life, which in turn guides how they interact with their child. Firstly, they place considerable emphasis on the quality of interactions, recognising, as have others in the early childhood and disability fields, the importance of nurturing touch:8 ,9
We just keep picking her up and that's what we expect we will continue to do, we don't want to put a hoist between us and our daughter, you know she is still like our baby.
They also talk about the quantity and type of interactions they consider appropriate:
for her, enjoyment is food, drink, cuddles; she loves face to face stuff and just having attention on her.
Anna's parents also believe that basic discomforts and pleasures may be magnified for her, a theme echoed in the ethics literature on disability.10 For example, we have already discussed how sitting too long in one place may be a very miserable experience for Anna. In contrast, this would generally be considered tolerable discomfort for most 6 year olds, who can understand why and for how long they are expected to remain seated. The consequences of such discomfort may also be worse for Anna, with her parents describing the potential for a cascade of negative effects if she is not moved frequently. Conversely, Anna's parents describe the joy she may derive from what we might consider very simple pleasures, such as lying next to the aquarium: ‘she'll kick and squeal and play like a baby’.
Viewing Anna in this way, with newborn-like experiences and needs, also impacts on the way in which these parents consider their own and Anna's interests. Throughout the interview, it became clear that they do not consider any decision as impacting only on Anna or only on themselves, rather viewing their lives as being inextricably entwined:
We don't really separate ourselves from her because we can't, there's nothing that we can do in our entire lives that doesn't take her into consideration…
I don't think we separate her needs from our needs or anything like that…
These observations seem powerful and ethically significant, particularly in the context of GA therapy. While they recognise that attenuating their daughter's growth will benefit them in some ways, they see this benefit as flowing directly to Anna and find it impossible to view effects in isolation: they very much see a congruence of interests rather than a conflict.
Goals of parenting
Given these beliefs about Anna's apparent newborn-like experiences and needs, it is also not surprising that the overwhelming concern of these parents’ is their daughter's current well-being. They believe that Anna's current experience is all that exists in her universe, much like we imagine it to be for an infant:
…our whole attitude to her life … why we've made every decision we have so far … is that we just want to keep her happy, it's about quality of life, it's not length of life...
This view appears to contrast with standard accounts of parenting, which place significant emphasis on both attempting to maximise current well-being and considering the child's future flourishing.11 ,12 In Anna's case, the ‘here and now’ is clearly of great import for her, and hence for her parents, but, despite this emphasis, plans for her future are not completely subjugated. There is a sense in which Anna's parents try to maximise her later quality of life—for example, providing physiotherapy to preserve or maximise flexibility and muscle tone, allowing greater comfort later in life. Indeed, the very choice of GA therapy reveals that Anna's parents have a concept of maximising her future experiences. However, although Anna's parents attend to her future in some ways, they recognise that it will not consist of the agency-driven choices and opportunities available to most people:
…Anna doesn't really impact on her world, … she's there for the ride and we're just there to make it all happy the whole time … she can't really say this is what I want and this is what I don't want and hey I'd quite like to read this book or I'm really interested in astrophysics, she can't do any of that and she is never going to…
So, while Anna's parents share both of the typical elements of parenting, the balance between the two appears to be shifted, with their concept of ‘future flourishing’ necessarily truncated and constrained.
This case study provides important insights into how one set of parents reasoned about GA therapy for their child. The key findings relate to how Anna's parents weighed up the harms and benefits of treatment and considered this to be the type of decision they should be able to make. Their reasoning is based on the belief that attenuating Anna's growth would maximise her quality of life, by permitting them to maintain a parent-to-newborn-like relationship, focused primarily, although not exclusively, on her short-term well-being.
We acknowledge that this is a single-case report reflecting one couple's views on GA therapy and that these may not be shared by other parents in similar circumstances. Providing such a nuanced and detailed account of these parents’ reasoning precludes exhaustive review of ethical arguments surrounding GA, but these are well documented elsewhere.1 There is a clear need for further work to see whether our findings are replicated, and to consider the views of parents who choose not to access this treatment or who access it for different reasons. However, while the ethical considerations these parents thought important might not be ones other parents think matter, they are viewpoints that can be weighed in the balance and contribute to the quality of ethical argument. In this regard, we believe there are important insights to be gained from this report regarding how these parents characterise benefits and harms and their responsibilities as surrogate decision-makers.
One of these insights relates to how Anna's parents discuss her quality of life. For them, this is their overwhelming concern, and they are clear that what others may consider minor effects could profoundly affect her experiences. We have already alluded to support for this view from the disability literature, and there are also philosophical arguments suggesting that PCI may reduce the overall benefits of life in proportion to the severity of intellectual disability.13 If so, it may well be the case for Anna that any small increment in quality of life represents a large percentage gain in overall quality, and, conversely, small harms or burdens (that could potentially be avoided) may tip the balance sufficiently to move her life from one worth living to one that is intolerable.
No one can be certain about treatment outcomes, particularly for children such as Anna whose interests are ultimately ‘unknowable’ (as the very nature of severe intellectual disability prevents us from accessing the experience of those affected3). What we can say is that these parents clearly describe how basic discomforts and pleasures may be magnified for Anna in a way that may be difficult for anyone not directly and intimately involved in caring for such a child to fully comprehend. The experienced paediatrician involved in Anna's care recognises this in relation to other aspects of her treatment, and we suggest there is also much to learn from Anna's parents about how to weigh and balance benefits and burdens that might otherwise be discounted in relation to GA therapy. Despite this assertion, we acknowledge that parental views can be subject to pressures and influences, and adequacy of social support and other biases should always be explored.
Other insights stem from the parents’ view that Anna is relevantly similar to a young infant. For Anna's parents, it is this view that enables them to provide what they consider to be the most appropriate emotional and practical care for their daughter. It also contributes to the manner in which Anna's interests and her parents intersect, and are hard to separate. In neonatal treatment decisions, this entwining of interests is well recognised: it has been argued that parental interests should be given somewhat more weight in decisions about newborns than for older children. The reasoning underlying this claim relates in part to the way in which therapeutic decisions impact significantly on the parents, but also acknowledges that parental views are likely to directly influence the infant's interests.14 In this way, the view Anna's parents take toward her not only assists them in caring for her, but also aligns with their views regarding the degree of discretion and input they should have into treatment decisions, including GA therapy.
Future research implications
There remains a need to collect systematic data about larger numbers of children whose parents do and do not access GA treatment. Our case analysis also suggests a place for more general empirical work concerning parenting children with PCI, including further exploration of parental views and values that underpin decision-making. Normative analysis exploring the relevance of different parental views, including that their child with PCI is relevantly similar to a newborn, could contribute to thinking about the degree of decisional discretion that should be accorded to parents of children with PCI. Furthermore, while the notion that adults have obligations to nurture the future interests of their children is widely accepted, what this means in the context of PCI is less clear: given the dearth of literature concerning appropriate parental goals in such circumstances, this is also an area ripe for further empirical and normative analysis.
This single-case analysis has provided important insights into how one set of parents reasoned about GA, but has also highlighted key unanswered questions for future empirical and normative ethics research. This research is important not just as a way of furthering the debate around GA therapy, but also as a way of justifying (or not) subtle differences in the degree of discretion that may currently be offered to parents of children with PCI.
Contributors Both authors fulfil criteria for authorship. NK made substantial contribution to design, acquisition, analysis and interpretation of data, and drafting the manuscript. JRM made substantial contribution to the conception and design of the work and revised it critically for intellectual content.
Competing interests None declared.
Patient consent Obtained.
Provenance and peer review Not commissioned; externally peer reviewed.
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