FOCUS ON PKU
LIFE WITH PKU: WORK IN PROGRESS Sharon Buckley BSc, MSc, MBPsS
In this article, Sharon shares memories of bringing up children with PKU and reflects on the difficulties and demands through the years.
Sharon is a lecturer of cognitive psychology with published research in The Journal of Brain and Cognition. She is currently conducting research in psychology and treatment adherence in PKU
Recently, I asked both Charlotte and Adam, what their childhood was like growing up with PKU. The consensus was that, having PKU as young children did not mean very much to them in terms of negative experiences; neither of them could recall feeling excluded or different to other children. I am thankful, that whilst I was frantic with the demands of managing life with PKU (creating and baking low protein substitute foods, ordering/ collecting prescriptions, scouring supermarkets, educating those involved in their care such as for parties, sleepovers, tea at a friend’s, holidays and school trips etc), all that passed them by. Thus, as a parent I
Sharon is a parent to two young adults withPhenylketonuria (PKU); her daughter Charlotte is 20 years old and remains on the diet, whilst her son Adam is 18 years old and is off diet (though he makes valiant attempts to get back on diet).
had been successful in my endeavours to reduce the impact that PKU had on their childhood and, therefore, had enabled them to lead a ‘normal’ life. In a study that examined the parental experience of caring for a young child with PKU, Carpenter et al1 identified that striving for a normal life for a child was a coping behaviour often carried out by parents of young children with PKU. Though the parents experienced immense pressure, the result of watching their child engaging in a normal life, enabled the majority of parents to be accepting of the PKU diagnosis within the lives of both themselves and their child.
Adam’s story When Adam began high school, he shied away from letting people know that he had PKU, he felt different and wanted to be able to eat the same foods as his peers. Subsequently, from around the age of 14, his phenylalanine levels were recurrently beyond the target range. This was not as a consequence of eating high protein foods, but because he would over eat chips, chocolate and crisps (foods that are allowed, but have to be restricted). As his teenage years progressed, Adam gained a substantial amount of weight, though the summer before his 16th birthday he did lose nearly three stone on Slimming World (green days). However, we had not consulted his PKU clinic before commencing the weight loss programme and when we did, the clinic considered that our approach was incorrect, for example, we should not have been giving him the calorific supplements. The clinic told Adam that as he would be going to adult clinic and compliance was an issue for him, that to aid his weight loss he could make the decision to come off the PKU diet. As yet, there is no approved prevention, or treatment intervention for overweight and obese people with PKU.2
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A month after coming completely off the PKU diet, Adam began to gain weight; he was unable to be selective amongst such a wide range of foods that he was now ‘allowed’. Less than 10 months after stopping the PKU diet, Adam had gained three stone. Within a year of being off diet, whilst on a family holiday to Spain, Adam became feared for his safety; he believed he was going to be robbed, kidnapped or blown up. His paranoia
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was so extreme that whilst in Spain an urgent referral to a mental health unit was made and he was assessed as having psychosis and placed on anti-psychotic medication. We also started Adam back on the PKU diet and supplements, though as he became more mentally settled he desisted from the diet. Two years later, Adam’s psychiatrist and mental health team are doubting the initial diagnosis of psychosis as his problems do not fully fit, though there is no doubt that he had extreme anxiety, agoraphobia and social withdrawal. Indeed, anxiety-related conditions are a frequently reported psychiatric complication in adults that cease to follow the PKU diet.3 We have had great intervention from our local mental health team, be it from a psychiatrist, family therapy, cognitive behavioural therapy and an individual support worker. But in terms of support from the PKU clinic, there has been little help, as these services are not integrated within routine metabolic care. Adam is not alone in his story; diminishing adherence with age is sadly a global issue for many individuals with PKU, from around the ages of 16, phenylalanine control and concentrations often ameliorate for patients.4
Charlotte’s story As Charlotte and Adam entered their adolescence years, their thoughts about life with PKU became disparate. Charlotte continued to embrace life whilst living on the PKU diet, never having a problem in telling people that she followed a medically prescribed low protein diet and asking for alternative food if unsuitable choices were presented. Charlotte’s one gripe with the diet was (and still is) the 345 calories contained in her three prescribed supplements. In her early teenage years, weight gain was an issue; no doubt it was a consequence of being too liberal with the protein-free prescription foods. However, with exercise and a calorie-controlled diet, Charlotte successfully lost just over four stone whilst simultaneously controlling her phenylalanine levels within the required range. Currently, Charlotte works as a learning support assistant in further education, she has a positive attitude towards life and living on the PKU diet, although at weekends she would like a little more protein than her daily six exchanges allows. UNDERSTANDING THE INDIVIDUAL FACTORS ASSOCIATED WITH DIETARY ADHERENCE
Whilst there is recognition of the hardship of dietary control, there is a lack of studies examining interventions to aid treatment compliance.5 However, there is not a lack of studies that seek to understand the various individual factors that contribute to an individual managing the PKU diet. In order to understand whether knowledge about PKU is a contributory factor to dietary adherence, Witalis et al6 assessed knowledge and attitudes towards the PKU dietary regime in both patients and their parents. It was concluded that PKU dietary knowledge did not negatively impact upon adherence. However, it was found that other influences, such as limiting a child’s independent food choices, growing helplessness in dietary adherence and embarrassment in following the
PKU diet did add to building a negative attitude towards the diet. It was, therefore, concluded that families affected by PKU would benefit from consistent long-term family and individual therapy that would help ‘counteract the effects of learned helplessness’. HELPLESSNESS AND LOCUS OF CONTROL
Individuals with a high external locus of control believe that they are helpless in controlling what happens to them, that promoting change is beyond their power as it is guided by fate, luck or other external circumstances. This is contrary to the belief held by people with a high internal locus of control; individuals possessing a high internal locus of control are confident in their personal abilities to influence change in themselves and their environment. Subsequently, individuals with an internal locus of control are less likely to submit to the www.NHDmag.com May 2018 - Issue 134
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FOCUS ON PKU Figure 1: Locus of control; the extent to which people believe they have control over their own lives
However, in recent years, resilience has come to be understood as being a dynamic process that involves the interaction of personal attributes with environmental circumstances. Consequently, at differing periods throughout an individual’s life, resilience can increase or decrease depending on a person’s ability to deal with the stress of a given situation.12 MENTAL TOUGHNESS
pressures to conform or obey, in comparison to individuals with an external locus of control.7 In a study that examined the association between metabolic control and child attributional style, Antshel et al8 found that locus of control ratings correlated significantly with blood phenylalanine levels; higher blood phenylalanine was associated with a higher external locus of control, suggesting that those children felt powerless over their condition and thus treatment compliance was reduced. Furthermore, it was suggested that children with a high inner locus of control took on more personal responsibility for their treatment and demonstrated better adherence, which thus resulted in better metabolic control.8 PSYCHOLOGICAL RESILIENCE
Associated with the presence of an internal locus of control is the concept of psychological resilience. Resilient individuals possess a strong belief in their own ability to control what happens to them.9 Though there is a lack of consensus as to a definitive definition, the term resilience is useful in understanding the positive manner people adopt when managing stressful life events.10 In the past, a person overcoming an adverse life event was said to possess a hardy personality type.11 Thus, resilience viewed as a personality trait was seen as being innate and fixed. 58
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A personality construct related to favourable stress management is mental toughness (MT). Originating from sports psychology, it predicts outcomes in sports. The attributes thought to be involved in being mentally tough are: resilience, confidence, commitment, self-belief, concentration and the ability to thrive under pressure. Individuals who are deemed to be mentally tough have a high sense of self-belief with a strong faith that they can shape their own destiny.13 Indeed, if I look back at the personalities displayed in my children as they began adolescence: it could be said that Charlotte demonstrated many of the attributes associated with MT. This was evidenced by her ability to maintain focus and concentrate on the end goal (i.e. her ability to lose weight and maintain it); she also thrived in opportunities where she was part of a team, for instance, playing at county level as a Crown Green bowler required her to be a confident and committed team member. Both my children are gentle, warm and considerate people and though Adam is lacking in the traits associated with MT, he does not look negatively upon Charlotte’s success with the PKU diet. CONCLUSION
From a parental perspective, I wonder whether the personality measures that predict success in sports (resilience, confidence, commitment, self-belief, concentration and the ability to thrive under pressure) reflect those that are needed in order to successfully manage the PKU diet. Living each day with the dietary regime of PKU is hard work; whether you are a person on or off diet, or whether you are a carer of someone who has PKU. For everyone involved, daily PKU is work in progress.