Psychosocial functioning in children with phenylketonuria: Relationships between quality of life and parenting indicators

Morawska, Alina; Mitchell, Amy E.; Etel, Evren; Kirby, Grace; McGill, James; Coman, David; Inwood, Anita

doi:10.1111/cch.12727

Cited by 17 publications

(24 citation statements)

References 33 publications

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“…As a chronic disease, PKU and the resulting daily implementation of the lifelong dietary treatment challenge everyday life of the affected children and their families. Quality of life and psychological wellbeing of these patients are therefore often jeopardized 2,3,26 . The study presented here aimed to evaluate psychological well‐being of early and continuously treated children and adolescents with PKU.…”

Section: Discussionmentioning

confidence: 99%

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Psychological well‐being of early and continuously treated phenylketonuria patients

et al. 2021

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Background Despite enormous advances in therapy, phenylketonuria (PKU) remains an incurable, inherited metabolic disease requiring life‐long treatment with potential to negatively impact quality of life and psychological well‐being. Therefore, the aim of this study was to screen early diagnosed and continuously treated children with PKU on psychological strengths and behavioral difficulties. Methods Evaluation of psychological strengths and behavioral difficulties in 49 patients with PKU (23f, 2‐17 years) by Strengths and Difficulties Questionnaire (SDQ; self‐report 11‐17 years and parent‐report 2‐17 years). Comparison to age, sex and BMI‐matched healthy controls (n = 98; 46f). Results In patients with PKU and healthy controls median SDQ Total Difficulties Score and median scores of subscales were within the normal range in parent‐ and self‐report, irrespective of sex and age group (children 2‐10 years, adolescents 11‐17 years). No influence of long‐term metabolic control in PKU on SDQ could be revealed. The 2‐ to 10‐year‐old boys with PKU showed significantly higher scores in Prosocial Behavior compared to their healthy peers (P = .032). Likewise, adolescent boys with PKU showed fewer Conduct Problems (parent‐report, P = .006). Adolescent girls with PKU rated themselves more often as abnormal in the subscale Emotional Problems compared to their healthy peers (P = .041). This subscale was also responsible for a significantly different Total SDQ Difficulties Score between patients and their parents' report (P = .008). Discussion SDQ represents a suitable instrument within the care for patients with PKU. Specific aspects, however, require separate consideration and evaluation with respect to this chronic disease. Special attention should be paid on adolescent PKU girls who seem to be at risk to develop emotional problem.

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Section: Discussionmentioning

confidence: 99%

“…Quality of life and psychological wellbeing of these patients are therefore often jeopardized. 2,3,26 The study .393…”

Section: Discussionmentioning

confidence: 99%

Psychological well‐being of early and continuously treated phenylketonuria patients

et al. 2021

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“…It has been stated that, in the treatment of phenylketonuria, going beyond focusing on metabolic control, adopting a holistic approach that supports the psychosocial wellbeing and quality of life of the child and family can help achieve more productive and successful results (14). Healthcare camps, which can be considered as part of the holistic approach, are thought to be beneficial because PKU reduces the quality of life of the patient and his/her family (15).…”

Section: Discussionmentioning

confidence: 99%

Fenilketonüri Kampının Fenilketonürili Adölesanların Bilgi ve Davranışları Üzerindeki Etkileri

Gürbüz¹,

Karaboncuk²,

Sivri³

2021

Turkish Journal of Pediatric Disease

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Objective: This study aimed to determine the effect of a phenylketonuria camp on adolescents with phenylketonuria (PKU). Material and Methods: A small cohort-type study was carried out with 67 participants between the ages of 12-16. Participants were selected from PKU patients who were attended a PKU camp, organized for patients with phenylketonuria, aiming socialization, disease-related information transfer, and providing pleasant time with various social activities. Besides the patients, specialists of pediatric metabolism, psychologist and dieticians attended the camp. The study questionnaire was applied by a face-to-face interview on the first and last days of the camp. The primary outcome Questions were asked about PKU perception, and knowledge scores were calculated for each participant by adding the number of correct answers in the 10-item test. Results: Of the participants, 34 (50.7%) were females, while 33 (49.3%) were males. Twenty-one (31.3%) patients had another family member with phenylketonuria. The mean knowledge scores were already high at the beginning of the PKU camp (median 8, min. 4, max. 10). When the patients' knowledge of PKU was compared, there was no significant difference between before (7.40±1.62) and after (7.72±1.80) the camp (p=0.097). However, it was found that the opinions of the participants about the difficulty of being a PKU patient improved significantly (p= 0.039). Conclusion: A PKU camp for adolescents is effective in improving the perceptions of the participants. However, it would be beneficial to increase the duration and frequency of PKU camps to achieve better outcomes.

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“…Eighteen mothers who participated in a study by Morawska at al reported the highest impact of PKU on their children's anxiety during blood tests on their own HRQoL and guilt related to poor adherence to dietary restrictions and supplementation regimens. Higher scores were shown in the emotional, social and overall impact of PKU [29] .…”

Section: Introductionmentioning

confidence: 95%

Quality of life in children living with PKU – a single-center, cross-sectional, observational study from Hungary

Becsei

Hiripi

Kiss

et al. 2021

Molecular Genetics and Metabolism Reports

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Background Phenylketonuria (PKU) is an inherited error of metabolism, screened at 48–72 h of life since 1975 in Hungary. The patients have to keep a strict lifelong protein-restricted diet, resulting in PKU and its treatment can lead to social and financial burdens. The current study aimed to evaluate the health-related quality of life (HRQoL) of children living with PKU. Patients and methods A single-centre, cross-sectional, observational study was conducted at the Center of Newborn Screening and Inherited Metabolic Disorders of Budapest, Hungary, using the PKU-quality of life (PKU-QoL) questionnaire. Responses of 59 parents and 11 teenagers were collected. Numerous aspects regarding HRQoL were analysed according to clinical compliance and severity. The patients were classified into groups with good or suboptimal adherence based on regular phenylalanine (Phe) values. The online officially translated versions of the adolescent or parental PKU-QoL questionnaire were used and analysed anonymously. Differences in HRQoL were compared - PKU vs. Hyperphenylalaninaemia (HPA) and good vs. suboptimal adherence. Results Twenty-five of 32 examined parameters had no or little impact on HRQoL. The most frequently reported symptom was irritability. Food enjoyment was the most impacted domain, with a major severity score in the adolescent group (median 62,5, IQR: 25–75). The emotional impact was scored at moderate severity by both the adolescents and parents. Classical PKU patients with good metabolic control were more frequently tired than HPA patients (0,0027). The group with poor metabolic adherence showed more frequent tiredness ( p = 0,03), slow thinking (p = 0,018) and anxiety (p = 0,015). Conclusion Overall, our patients showed an excellent HRQoL; most domains (29/36) were reported as little/no impacted. Worse QoL was found in patients with suboptimal metabolic control. Particular attention should be paid to the emotional health of PKU patients.

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Psychosocial functioning in children with phenylketonuria: Relationships between quality of life and parenting indicators

Cited by 17 publications

References 33 publications

Psychological well‐being of early and continuously treated phenylketonuria patients

Psychological well‐being of early and continuously treated phenylketonuria patients

Fenilketonüri Kampının Fenilketonürili Adölesanların Bilgi ve Davranışları Üzerindeki Etkileri

Quality of life in children living with PKU – a single-center, cross-sectional, observational study from Hungary

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