Abstract
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.
Thanks
Thanks to ‘Geleceğin Yıldızları Company’, Metabolizma Vakfı (METVAK) and all participiants and their families.
References
- 1. Ford S, O’Driscoll M, MacDonald A. Living with Phenylketonuria: Lessons from the PKU community. Mol Genet Metab reports. 2018 Dec;17:57–63.
- 2. van Wegberg AMJ, MacDonald A, Ahring K, Bélanger-Quintana A, Blau N, Bosch AM, et al. The complete European guidelines on phenylketonuria: diagnosis and treatment. Orphanet J Rare Dis. 2017 Oct;12(1):162.
- 3. Hofman DL, Champ CL, Lawton CL, Henderson M, Dye L. A systematic review of cognitive functioning in early treated adults with phenylketonuria. Orphanet J Rare Dis. 2018 Aug;13(1):150.
- 4. Stone WL, Basit H, Los E. Phenylketonuria. In: StatPearls [Internet]. StatPearls Publishing; 2019.
- 5. El-Metwally A, Yousef Al-Ahaidib L, Ayman Sunqurah A, Al-Surimi K, Househ M, Alshehri A, et al. The Prevalence of Phenylketonuria in Arab Countries, Turkey, and Iran: A Systematic Review. Biomed Res Int. 2018;2018:7697210.
- 6. Feillet F, MacDonald A, Hartung Perron D, Burton B. Outcomes beyond phenylalanine: an international perspective. Mol Genet Metab. 2010;99 Suppl 1:S79-85.
- 7. Crone MR, Van Spronsen FJ, Oudshoorn K, Bekhof J, Van Rijn G, Verkerk PH. Behavioural factors related to metabolic control in patients with phenylketonuria. J Inherit Metab Dis. 2005;28(5):627–37.
- 8. Vegni E, Fiori L, Riva E, Giovannini M, Moja EA. How individuals with phenylketonuria experience their illness: an age‐related qualitative study. Child Care Health Dev. 2010;36(4):539–48.
- 9. Regnault A, Burlina A, Cunningham A, Bettiol E, Moreau-Stucker F, Benmedjahed K, et al. Development and psychometric validation of measures to assess the impact of phenylketonuria and its dietary treatment on patients’ and parents’ quality of life: the phenylketonuria – quality of life (PKU-QOL) questionnaires. Orphanet J Rare Dis. 2015;10(1):59.
- 10. Feldmann R, Osterloh J, Onon S, Fromm J, Rutsch F, Weglage J. Neurocognitive functioning in adults with phenylketonuria: Report of a 10-year follow-up. Mol Genet Metab. 2019 Mar;126(3):246–9.
- 11. Moyle JJ, Fox AM, Arthur M, Bynevelt M, Burnett JR. Meta-analysis of neuropsychological symptoms of adolescents and adults with PKU. Neuropsychol Rev. 2007 Jun;17(2):91–101.
- 12. Gentile JK, Ten Hoedt AE, Bosch AM. Psychosocial aspects of PKU: hidden disabilities--a review. Mol Genet Metab. 2010;99 Suppl 1:S64-7.
- 13. Brumm VL, Bilder D, Waisbren SE. Psychiatric symptoms and disorders in phenylketonuria. Mol Genet Metab. 2010;99 Suppl 1:S59-63.
- 14. Ambler O, Medford E, Hare DJ. Parenting a Child with Phenylketonuria: An Investigation into the Factors That Contribute to Parental Distress. JIMD Rep. 2018;41:91–100.
- 15. Morawska A, Mitchell AE, Etel E, Kirby G, McGill J, Coman D, et al. Psychosocial functioning in children with phenylketonuria: Relationships between quality of life and parenting indicators. Child Care Health Dev. 2020;46(1):56–65.
- 16. Neville AR, Moothathamby N, Naganathan M, Huynh E, Moola FJ. “A place to call our own”: The impact of camp experiences on the psychosocial wellbeing of children and youth affected by cancer–A narrative review. Complement Ther Clin Pract. 2019;36:18–28.
- 17. Fegan-Bohm K, Weissberg-Benchell J, DeSalvo D, Gunn S, Hilliard M. Camp for Youth With Type 1 Diabetes. Curr Diab Rep. 2016 Aug;16(8):68.
- 18. Kim S-S. An effect of the health camp program for promoting self-efficacy in juvenile diabetes mellitus patients. J Korean Acad Community Heal Nurs. 1997;8(1):102–15.
- 19. Semiz S, Bilgin ÜÖ, Bundak R, Bircan I. Summer camps for diabetic children: an experience in Antalya, Turkey. Acta Diabetol. 2000;37(4):197–200.
- 20. American Diabetes Association. Diabetes management at camps for children with diabetes. Diabetes Care. 2012 Jan;35 Suppl 1(Suppl 1):S72-5.
- 21. Jun Y-S, Lee J-E, Lee Y-S, Bae Y-J, Kim M-H, Lee Y-S, et al. Evaluation of nutritional health camp in obese elementary students. Korean J Food Nutr. 2007;20(1):79–87.
- 22. Gibbs A, Moor S, Frampton C, Watkins W. Impact of psychosocial interventions on children with disruptive and emotional disorders treated in a health camp. Aust New Zeal J Psychiatry. 2008;42(9):789–99.
- 23. Lord A, St. Leger LH, Ridge DT, Elisha D. The value of asthma camps for young people in Victoria, Australia. Contemp Nurse. 2001;11(2–3):133–41.
- 24. Epstein I, Stinson J, Stevens B. The effects of camp on health-related quality of life in children with chronic illnesses: a review of the literature. J Pediatr Oncol Nurs. 2005;22(2):89–103.
Abstract
Amaç: Bu çalışmada fenilketonüri kampının fenilketonürili (PKU) adölesanlar üzerindeki etkisinin değerlendirilmesi amaçlanmıştır.
Gereç ve Yöntemler: Bu kohort tipi çalışma, yaşları 12-16 yaş arasında değişen toplam 67 katılımcı ile gerçekleştirildi. Katılımcılar, fenilketonürili hastalar için düzenlenen, hastaların sosyalleşmesini, hastalıkla ilgili bilgi düzeylerini arttırmayı ve çeşitli sosyal aktivitelerle keyifli vakit geçirmeyi amaçlayan PKU kampına katılan PKU hastalarından seçildi. Kampa hastaların yanı sıra, çocuk metabolizma uzmanları, psikolog ve diyetisyenler katıldı. Araştırma anketi kampın ilk ve son günlerinde yüz yüze görüşme ile uygulandı ve 10 adet bilgi soruları sorularak, her katılımcı için doğru cevap sayısına göre bilgi puanı hesaplandı.
Bulgular: Katılımcıların 34’ü (% 50.7) kız, 33’ü (%49.3) erkekti. Yirmi bir (%31.3) hastada fenilketonüri hastası başka bir aile üyesi vardı. Ortalama bilgi puanları PKU kampının başlangıcında da yüksek (ortanca 8, en az 4, en fazla 10 puan) olup, hastaların PKU bilgisi karşılaştırıldığında, kamp öncesi (7.40±1.62) ve sonrasında (7.72±1.80) arasında anlamlı bir fark bulunamadı (p= 0.097). Ancak katılımcıların PKU hastası olmanın zorluğuna ilişkin görüşlerinde kamp sonrası anlamlı bir iyileşme olduğu görüldü (p= 0.039).
Sonuç: Ergenler için düzenlenen PKU kampları, PKU hastalarının algılarını iyileştirmede etkilidir. Ancak, daha iyi sonuçlar elde etmek için PKU kamplarının süresini ve sıklığının arttırması faydalı olacaktır.
References
- 1. Ford S, O’Driscoll M, MacDonald A. Living with Phenylketonuria: Lessons from the PKU community. Mol Genet Metab reports. 2018 Dec;17:57–63.
- 2. van Wegberg AMJ, MacDonald A, Ahring K, Bélanger-Quintana A, Blau N, Bosch AM, et al. The complete European guidelines on phenylketonuria: diagnosis and treatment. Orphanet J Rare Dis. 2017 Oct;12(1):162.
- 3. Hofman DL, Champ CL, Lawton CL, Henderson M, Dye L. A systematic review of cognitive functioning in early treated adults with phenylketonuria. Orphanet J Rare Dis. 2018 Aug;13(1):150.
- 4. Stone WL, Basit H, Los E. Phenylketonuria. In: StatPearls [Internet]. StatPearls Publishing; 2019.
- 5. El-Metwally A, Yousef Al-Ahaidib L, Ayman Sunqurah A, Al-Surimi K, Househ M, Alshehri A, et al. The Prevalence of Phenylketonuria in Arab Countries, Turkey, and Iran: A Systematic Review. Biomed Res Int. 2018;2018:7697210.
- 6. Feillet F, MacDonald A, Hartung Perron D, Burton B. Outcomes beyond phenylalanine: an international perspective. Mol Genet Metab. 2010;99 Suppl 1:S79-85.
- 7. Crone MR, Van Spronsen FJ, Oudshoorn K, Bekhof J, Van Rijn G, Verkerk PH. Behavioural factors related to metabolic control in patients with phenylketonuria. J Inherit Metab Dis. 2005;28(5):627–37.
- 8. Vegni E, Fiori L, Riva E, Giovannini M, Moja EA. How individuals with phenylketonuria experience their illness: an age‐related qualitative study. Child Care Health Dev. 2010;36(4):539–48.
- 9. Regnault A, Burlina A, Cunningham A, Bettiol E, Moreau-Stucker F, Benmedjahed K, et al. Development and psychometric validation of measures to assess the impact of phenylketonuria and its dietary treatment on patients’ and parents’ quality of life: the phenylketonuria – quality of life (PKU-QOL) questionnaires. Orphanet J Rare Dis. 2015;10(1):59.
- 10. Feldmann R, Osterloh J, Onon S, Fromm J, Rutsch F, Weglage J. Neurocognitive functioning in adults with phenylketonuria: Report of a 10-year follow-up. Mol Genet Metab. 2019 Mar;126(3):246–9.
- 11. Moyle JJ, Fox AM, Arthur M, Bynevelt M, Burnett JR. Meta-analysis of neuropsychological symptoms of adolescents and adults with PKU. Neuropsychol Rev. 2007 Jun;17(2):91–101.
- 12. Gentile JK, Ten Hoedt AE, Bosch AM. Psychosocial aspects of PKU: hidden disabilities--a review. Mol Genet Metab. 2010;99 Suppl 1:S64-7.
- 13. Brumm VL, Bilder D, Waisbren SE. Psychiatric symptoms and disorders in phenylketonuria. Mol Genet Metab. 2010;99 Suppl 1:S59-63.
- 14. Ambler O, Medford E, Hare DJ. Parenting a Child with Phenylketonuria: An Investigation into the Factors That Contribute to Parental Distress. JIMD Rep. 2018;41:91–100.
- 15. Morawska A, Mitchell AE, Etel E, Kirby G, McGill J, Coman D, et al. Psychosocial functioning in children with phenylketonuria: Relationships between quality of life and parenting indicators. Child Care Health Dev. 2020;46(1):56–65.
- 16. Neville AR, Moothathamby N, Naganathan M, Huynh E, Moola FJ. “A place to call our own”: The impact of camp experiences on the psychosocial wellbeing of children and youth affected by cancer–A narrative review. Complement Ther Clin Pract. 2019;36:18–28.
- 17. Fegan-Bohm K, Weissberg-Benchell J, DeSalvo D, Gunn S, Hilliard M. Camp for Youth With Type 1 Diabetes. Curr Diab Rep. 2016 Aug;16(8):68.
- 18. Kim S-S. An effect of the health camp program for promoting self-efficacy in juvenile diabetes mellitus patients. J Korean Acad Community Heal Nurs. 1997;8(1):102–15.
- 19. Semiz S, Bilgin ÜÖ, Bundak R, Bircan I. Summer camps for diabetic children: an experience in Antalya, Turkey. Acta Diabetol. 2000;37(4):197–200.
- 20. American Diabetes Association. Diabetes management at camps for children with diabetes. Diabetes Care. 2012 Jan;35 Suppl 1(Suppl 1):S72-5.
- 21. Jun Y-S, Lee J-E, Lee Y-S, Bae Y-J, Kim M-H, Lee Y-S, et al. Evaluation of nutritional health camp in obese elementary students. Korean J Food Nutr. 2007;20(1):79–87.
- 22. Gibbs A, Moor S, Frampton C, Watkins W. Impact of psychosocial interventions on children with disruptive and emotional disorders treated in a health camp. Aust New Zeal J Psychiatry. 2008;42(9):789–99.
- 23. Lord A, St. Leger LH, Ridge DT, Elisha D. The value of asthma camps for young people in Victoria, Australia. Contemp Nurse. 2001;11(2–3):133–41.
- 24. Epstein I, Stinson J, Stevens B. The effects of camp on health-related quality of life in children with chronic illnesses: a review of the literature. J Pediatr Oncol Nurs. 2005;22(2):89–103.
Details
| Primary Language | English |
|---|---|
| Subjects | Internal Diseases |
| Journal Section | ORIGINAL ARTICLES |
| Authors | |
| Publication Date | March 30, 2021 |
| Submission Date | October 18, 2020 |
| Published in Issue | Year 2021 Volume: 15 Issue: 2 |
The publication language of Turkish Journal of Pediatric Disease is English.
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