Parenting a Child with Phenylketonuria (PKU): an Interpretative Phenomenological Analysis (IPA) of the Experience of Parents

Carpenter, Katie; Wittkowski, Anja; Hare, Dougal Julian; Medford, Emma; Rust, Stewart; Jones, Simon A.; Smith, Debbie

doi:10.1007/s10897-018-0227-7

Cited by 39 publications

(50 citation statements)

References 58 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…In PKU, there is an association between parent stress, anxiety and depression [3]. Some studies report a higher incidence in parents (particularly mothers) of children with PKU compared with parents of healthy controls, negatively impacting on quality of life and psychosocial wellbeing [15], others report no significant difference [16,17].…”

Section: Discussionmentioning

confidence: 99%

“…Parents must ensure that their children consume a non-negotiable and weighed amount of natural protein together with a bitter tasting protein substitute in prescribed amounts each day. Parents understand that the neurological outcome of their child depends on their ability to successfully implement diet therapy, and this can have a significant impact on stress and quality of life [1][2][3][4].…”

Section: Introductionmentioning

confidence: 99%

“…The process of weaning a child with PKU is likely to enhance parental anxiety [3], and whilst anxiety is a normal emotion that prepares the mind and body to respond to a threat, when it persists over long periods, it can lead to a number of negative medical and psychological outcomes including the development of anxiety disorders [5]. The guilt, grief and disappointment of not having a healthy baby is prominent, and they must face administering a severely restrictive diet in order to control their child's blood Phe levels at a time when they are still emotionally vulnerable [3,5]. There are many milestones that must be reached (e.g., introduction of a measured quantity of Phe from solid food each day, tolerance of a second-stage spoonable protein substitute and acceptance of special low-protein foods), which require extensive parental organisational skills and tenacity.…”

Section: Introductionmentioning

confidence: 99%

“…There are many milestones that must be reached (e.g., introduction of a measured quantity of Phe from solid food each day, tolerance of a second-stage spoonable protein substitute and acceptance of special low-protein foods), which require extensive parental organisational skills and tenacity. Any food refusal, rejection of protein substitute, child illness, inability to access special dietary products, social situations involving food (parties, eating out) and sub-optimal blood Phe control is likely to heighten parental anxiety [1,3]. Consequently, there may be conflict between maintaining blood Phe levels within the target range and allowing dietary deviations aimed at meeting child and parent psychosocial needs and the desire for family normality [5].…”

Section: Introductionmentioning

confidence: 99%

“…Thereby, parents of children with PKU require a diverse range of complex skills and additional time in order to manage and control dietary treatment, as well as the stress associated with adherence, social isolation and regular blood Phe monitoring [1,3,6,7]. Metabolic and psychological outcomes are likely to be influenced by parental coping strategies and the ability of the family to alter their lifestyle to accommodate the demands of a complex dietary regimen [2][3][4][5]. However, it is uncertain what coping mechanisms exist in the parents of children with PKU, and how this might compare with parents of children with other health conditions or those of normal healthy children.…”

Section: Introductionmentioning

confidence: 99%

See 4 more Smart Citations

Mealtime Anxiety and Coping Behaviour in Parents and Children during Weaning in PKU: A Case-Control Study

et al. 2019

View full text Add to dashboard Cite

Solid food introduction may create anxiety for parents of children with phenylketonuria (PKU) due to the burden associated with protein substitute (PS) administration and natural protein restriction. In a longitudinal, prospective study, 20 mothers of children with PKU and 20 non-PKU control mothers completed 4 questionnaires (mealtime emotions, feed-time, Beck's anxiety inventory and the coping health inventory for parents), examining parent/child mealtime emotions, anxiety, stress and coping strategies at child ages: weaning start, 8 months (m), 12 m, 15 m, 18 m and 24 m. Overall, mothers of children with PKU cope well with solid food introduction when applying a low-phenylalanine diet, with comparable low levels of stress and anxiety reported in both PKU and non-PKU groups. However, mothers of children with PKU reported peak scores in anxiety for emotive/cognitive symptoms at a child age of 15 m, and higher use of coping strategies at 15 m and 24 m (p < 0.05) of age. Generally, there was a trend that maternal anxiety regarding child rejection of PS increased with time, peaking between 12-24 m. In PKU, a child age of 12-18 m is identified as a key period when mothers feel most anxious/stressed with feeding, coinciding with raised blood phenylalanine levels probably associated with teething, illness and developing independence. Health professionals should be conscious of this vulnerable period and be prepared to offer more directional support as required.

show abstract

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 3 more Smart Citations

Mealtime Anxiety and Coping Behaviour in Parents and Children during Weaning in PKU: A Case-Control Study

et al. 2019

View full text Add to dashboard Cite

show abstract

Long‐term cognitive and psychosocial outcomes in adults with phenylketonuria

Aitkenhead

Krishna

Ellerton

et al. 2021

J of Inher Metab Disea

View full text Add to dashboard Cite

Previous studies have suggested that cognitive and psychosocial underfunctioning in early-treated adults with phenylketonuria (PKU) may be explained by suboptimal adherence to dietary treatments, however, these studies often employ small samples, with different outcome measures, definitions and cut-offs. Samples have also tended to comprise participants with a limited range of blood phenylalanine concentrations, and often individuals who may not have been treated early enough to avoid neurological damage. In this study, we explore the impact of lifetime dietary control, as indicated by blood phenylalanine concentrations in childhood, adolescence and adulthood, on long-term cognitive and psychosocial outcomes in a large sample of adults with PKU who were diagnosed by neonatal screening and commenced on dietary treatment within the first month of life. One hundred and fifty-four participants underwent cognitive testing, assessing attention, learning, working memory, language, executive functioning and processing speed. One hundred and forty-nine completed measures of psychosocial functioning, documenting educational, occupational, quality of life, emotional and social outcomes which were compared with a group of healthy controls. Many adults with PKU demonstrated cognitive impairments, most frequently affecting processing speed (23%), executive function (20%) and learning (12%). Cognitive outcomes were related to measures of historic metabolic control, but only processing speed was significantly related to phenylalanine concentration at the time of testing after controlling for historic levels. Adults with PKU did not, however, differ from controls in educational, occupational, quality of life or emotional outcomes, or on a measure of family functioning, and showed only minor differences in relationship style. These findings have implications for patient counselling and decisions regarding the management of PKU in adulthood.

show abstract

Parenting a Child with Phenylketonuria: An Investigation into the Factors That Contribute to Parental Distress

2018

Self Cite

View full text Add to dashboard Cite

Phenylketonuria (PKU) is an inherited metabolic condition that can lead to the onset of intellectual disabilities if not strictly managed through a low-protein diet. Parents are responsible for supervising their child's treatment for PKU, which may impact on their experience of distress. This cross-sectional study aimed to identify the factors that contribute to distress in parents who care for a child with PKU, distinct from parents in the general population. Thirty-eight parents of children and adolescents with PKU and 32 parents in the general population completed the questionnaires measuring parental psychological resilience, child behaviour problems, perceived social support and distress. Parents of children with PKU also completed measures of their child's care dependency and behaviour related to developmental and intellectual disabilities. The findings revealed no statistically significant differences in distress between the groups, but parents of children with PKU reported more child behaviour problems. Multiple regression analysis identified that parental psychological resilience and child anxious behaviour explained 35% of the variance in distress for parents of children with PKU. By comparison, parental psychological resilience and generic child behaviour only accounted for 19% of the variance in distress for parents in the general population. This has implications for developing interventions in clinical settings that aim to reduce parents' distress by enhancing their psychological resilience and supporting them to manage child behaviour difficulties, particularly anxious behaviour. Future research should include larger, more diverse samples and use longitudinal study designs.

show abstract

Parenting a Child with Phenylketonuria (PKU): an Interpretative Phenomenological Analysis (IPA) of the Experience of Parents

Cited by 39 publications

References 58 publications

Mealtime Anxiety and Coping Behaviour in Parents and Children during Weaning in PKU: A Case-Control Study

Mealtime Anxiety and Coping Behaviour in Parents and Children during Weaning in PKU: A Case-Control Study

Long‐term cognitive and psychosocial outcomes in adults with phenylketonuria

Parenting a Child with Phenylketonuria: An Investigation into the Factors That Contribute to Parental Distress

Contact Info

Product

Resources

About