Advertisement

Quality of life in caregivers of young children with Prader–Willi syndrome

  • Shu-Jiong Mao
  • Jian Shen
  • Fang Xu
  • Chao-Chun ZouEmail author
Brief Report
  • 13 Downloads

Abstract

Background

This study aimed to measure quality of life (QOL) in primary caregivers of young childrenwith Prader–Willi syndrome (PWS).

Methods

The caregivers of 32 children aged from 6.1 to 71.2 months completed the Chinese version of the World Health Organization Quality of Life-BREF (WHOQOL-BREF). We also evaluated the social adaption capacity of these children with Infants-Junior Middle School Students’ Social-Life Abilities Scale. Correlation test was used to explore the related factors to caregivers’ QOL.

Results

Caregivers of young children with PWS had significantly lower QOL. The correlation analyses revealed that caregivers' QOL was lower in children with young age, combined diseases or symptoms or poor social adaption, or caregivers having concerns about the child.

Conclusions

Rearing a chilld with PWS may lead to decreased QOL. Psychological status of caregivers should be highlighted and social support should be given to families with PWS children.

Keywords

Caregiver Prader–Willi syndrome (PWS) Quality of life (QOL) Social adaption WHOQOL-BREF 

Notes

Author contributions

SM and CZ are the principle investigators of this manuscript. JS and FX conducted the data collection and analysis. This manuscript was drafted by SM, and critically revised by CZ. All authors have approved submission of the manuscript.

Funding

This work was partly supported by National Natural Science Foundation of China (81371215&81670786), Zhejiang Provincial Program for the Cultivation of High-Level Innovative Health Talents, and Medical Health Science and Technology Project of Health Commission of Zhejiang Province (No. 2019RC239).

Compliance with ethical standards

Ethical approval

Consent was obtained from the parent and the study was proved by the Ethical Committee of the Children Hospital of Zhejiang University School of Medicine.

Conflict of interest

No financial or nonfinancial benefits have been received or will be received from any party related directly or indirectly to the subject of this article.

References

  1. 1.
    Angulo MA, Butler MG, Cataletto ME. Prader–Willi syndrome: a review of clinical, genetic, and endocrine findings. J Endocrinol Invest. 2015;38:1249–63.CrossRefGoogle Scholar
  2. 2.
    Cassidy SB, Driscoll DJ. Prader–Willi syndrome. Eur J Hum Genet. 2009;17:3–13.CrossRefGoogle Scholar
  3. 3.
    Butler MG, Manzardo AM, Forster JL. Prader–Willi syndrome: clinical genetics and diagnostic aspects with treatment approaches. Curr Pediatric Rev. 2016;12:136–66.CrossRefGoogle Scholar
  4. 4.
    Cassidy SB, Schwartz S, Miller JL, Driscoll DJ. Prader–Willi syndrome. Genet Med. 2012;14:10–26.CrossRefGoogle Scholar
  5. 5.
    Chiu VJ, Tsai LP, Wei JT, Tzeng IS, Wu HC. Motor performance in Prader–Willi syndrome patients and its potential influence on caregiver's quality of life. PeerJ. 2017;5:e4097.CrossRefGoogle Scholar
  6. 6.
    Mohapatra S, Panda UK. Behavioral and emotional manifestations in a child with Prader–Willi syndrome. Shanghai Arch Psychiatry. 2016;28:106–8.Google Scholar
  7. 7.
    Rice LJ, Gray KM, Howlin P, Taffe J, Tonge BJ, Einfeld SL. The developmental trajectory of disruptive behavior in Down syndrome, fragile X syndrome, Prader–Willi syndrome and Williams syndrome. Am J Med Genet Part C Semin Med Genet. 2015;169:182–7.CrossRefGoogle Scholar
  8. 8.
    Ihara H, Ogata H, Sayama M, Kato A, Gito M, Murakami N, et al. QOL in caregivers of Japanese patients with Prader–Willi syndrome with reference to age and genotype. Am J Med Genet Part A. 2014;164:2226–31.CrossRefGoogle Scholar
  9. 9.
    Sarimski K. Behavioural phenotypes and family stress in three mental retardation syndromes. Eur Child Adolesc Psychiatry. 1997;6:26–31.CrossRefGoogle Scholar
  10. 10.
    van Lieshout CF, de Meyer RE, Curfs LM, Koot HM, Fryns JP. Problem behaviors and personality of children and adolescents with Prader-Willi syndrome. J Pediatr Psychol. 1998;23:111–20.CrossRefGoogle Scholar
  11. 11.
    Lopez-Bastida J, Linertova R, Oliva-Moreno J, Posada-de-la-Paz M, Serrano-Aguilar P, Kanavos P, et al. Social/economic costs and health-related quality of life in patients with Prader–Willi syndrome in Europe. Eur J Health Econ. 2016;17:99–108.CrossRefGoogle Scholar
  12. 12.
    Zuo Q. Infants-Junior Middle School Students’ Social-Life Abilities Scales. Beijing: Beijing Medical University; 1998.Google Scholar
  13. 13.
    Hao Y, Fang J. The introduce and usage of WHOQOL instrument in Chinese. Mod Rehabil. 2000;4:1127–9.Google Scholar
  14. 14.
    Fang J, Hao Y, Li C. Reliability and validity for Chinese version of WHO Quality of Life Scale. Chin Ment Health J. 1999;13:3.Google Scholar
  15. 15.
    Kayadjanian N, Schwartz L, Farrar E, Comtois KA, Strong TV. High levels of caregiver burden in Prader–Willi syndrome. PLoS ONE. 2018;13:e0194655.CrossRefGoogle Scholar
  16. 16.
    Prader–Willi syndrome Association (USA). https://www.pwsausa.org; Accessed 7th Nov 2018.
  17. 17.
    International Prader–Willi syndrome Organisation. https://www.ipwso.org. Accessed 7th Nov 2018.
  18. 18.
    Prader–Willi syndrome Association (TW). https://pwsa.eoffering.org.tw. Accessed 7th Nov 2018.
  19. 19.
    Prader–Willi syndrome care and support center. https://www.xpwilli.org/m/. Accessed 7th Nov 2018.
  20. 20.
    Descheemaeker MJ, Vogels A, Govers V, Borghgraef M, Willekens D, Swillen A, et al. Prader-Willi syndrome: new insights in the behavioural and psychiatric spectrum. J Intell Disabil Res. 2002;46:41–50.CrossRefGoogle Scholar
  21. 21.
    Sinnema M, Boer H, Collin P, Maaskant MA, van Roozendaal KE, Schrander-Stumpel CT, et al. Psychiatric illness in a cohort of adults with Prader–Willi syndrome. Res Dev Disabil. 2011;32:1729–35.CrossRefGoogle Scholar
  22. 22.
    Yu L, Mo L, Tang Y, Huang X, Tan J. Effects of nursing intervention models on social adaption capability development in preschool children with malignant tumors: a randomized control trial. Psychooncology. 2014;23:708–12.CrossRefGoogle Scholar

Copyright information

© Children's Hospital, Zhejiang University School of Medicine 2019

Authors and Affiliations

  1. 1.Department of Pediatrics, Affiliated Hangzhou First People’s HospitalZhejiang University School of MedicineHangzhouChina
  2. 2.Department of Endocrinology, Children’s HospitalZhejiang University School of MedicineHangzhouChina

Personalised recommendations