Associations Between Hyperphagia, Symptoms of Sleep Breathing Disorder, Behaviour Difficulties and Caregiver Well-Being in Prader-Willi Syndrome: A Preliminary Study

Jessica Mackay; Gillian M. Nixon; Antony R. Lafferty; Geoff Ambler; Nitin Kapur; Philip B. Bergman; Cara Schofield; Chris Seton; Andrew Tai; Elaine Tham; Komal Vora; Patricia Crock; Charles Verge; Yassmin Musthaffa; Greg Blecher; Daan Caudri; Helen Leonard; Peter Jacoby; Andrew Wilson; Catherine S. Choong; Jenny Downs

doi:10.1007/s10803-021-05265-5

Associations Between Hyperphagia, Symptoms of Sleep Breathing Disorder, Behaviour Difficulties and Caregiver Well-Being in Prader-Willi Syndrome: A Preliminary Study

Jessica Mackay
, Gillian M. Nixon
, Antony R. Lafferty
, Geoff Ambler
, Nitin Kapur
, Philip B. Bergman
, Cara Schofield
, Chris Seton
, Andrew Tai
, Elaine Tham
, Komal Vora
, Patricia Crock
, Charles Verge
, Yassmin Musthaffa
, Greg Blecher
, Daan Caudri
, Helen Leonard
, Peter Jacoby
, Andrew Wilson
, Catherine S. Choong

Jenny Downs^*

^*Corresponding author for this work

Pediatrics

Telethon Kids Institute
University of Western Australia
Royal Perth Hospital
Monash Children’s Hospital
Monash University
Canberra Hospital
ANU Medical School
The Sydney Children's Hospitals Network
The University of Sydney
Queensland Children's Hospital
University of Queensland
The Children's Hospital at Westmead
Women's and Children's Hospital Adelaide
Hunter New England Health
University of Newcastle
Hunter Medical Research Institute
Sydney Children's Hospital
University of New South Wales
Logan Hospital
Perth Children's Hospital
Curtin University

Research output: Contribution to journal › Article › Academic › peer-review

4 Citations (Scopus)

Abstract

Prader-Willi syndrome (PWS) is a rare genetic disorder characterised by neurodevelopmental delays, hyperphagia, difficulties with social communication and challenging behaviours. Individuals require intensive supervision from caregivers which may negatively affect caregiver quality of life. This study used data collected in the Australasian PWS Registry (n = 50, mean age 11.2 years) to evaluate associations between child behaviours and caregiver mental well-being. Symptoms of sleep-related breathing disorder, child depression and social difficulties were associated with poorer caregiver mental and physical well-being. Growth hormone therapy use was associated with better caregiver mental and physical well-being. Optimising management of problematic behaviours and sleep disturbances have the potential to support caregivers who are the most vital network of support for individuals affected by PWS.

Original language	English
Pages (from-to)	3877-3889
Number of pages	13
Journal	Journal of Autism and Developmental Disorders
Volume	52
Issue number	9
DOIs	https://doi.org/10.1007/s10803-021-05265-5
Publication status	Published - Sept 2022

Bibliographical note

Funding Information:
We acknowledge the generous contribution of all families who participated in this study. We acknowledge the funding provided for this study. This research was supported by funding from the Perth Children’s Hospital Foundation to begin the Australasian Prader-Willi Syndrome Database to which participants in this study were recruited. We also acknowledge funding received from the Prader-Willi Association USA 2016 research grant.

Funding Information:
Gillian Nixon Has grant funding from ResMed Foundation; All renumeration has been paid to her department. Antony Lafferty: Has been on advisory boards in the past of Abbvie Australia, Medtronic Diabetes, Kyowa Kirin Australia and Chiesi Australia. Investigator in a clinical trial with MSD. Helen Leonard: Consultancy for Marinus, Newron, Anavex, GW Pharmaceuticals and AveXis; Clinical Trials with Newron and Anavex; All remuneration has been made to her department. Catherine Choong: Investigator in Clinical Trial OPKO Health & Pfizer Pharmaceuticals. Jenny Downs: Consultancy for Marinus, Newron, Anavex, GW Pharmaceuticals and AveXis; Clinical Trials with Newron and Anavex; All remuneration has been made to her department. Each of these disclosures relate to subject matter not contained in this manuscript. Jessica Mackay, Cara Schofield, Geoffrey Ambler, Nitin Kapur, Philip Bergman, Chris Seton, Andrew Tai, Elaine Tham, Komal Vora, Patricia Crock, Peter Jacoby, Charles Verge, Yassmin Musthaffa, Greg Blecher, Daan Caudri and Andrew Wilson report no disclosures or conflicts of interest.

Publisher Copyright:
© 2021, The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.

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Cite this

Mackay, J., Nixon, G. M., Lafferty, A. R., Ambler, G., Kapur, N., Bergman, P. B., Schofield, C., Seton, C., Tai, A., Tham, E., Vora, K., Crock, P., Verge, C., Musthaffa, Y., Blecher, G., Caudri, D., Leonard, H., Jacoby, P., Wilson, A., ... Downs, J. (2022). Associations Between Hyperphagia, Symptoms of Sleep Breathing Disorder, Behaviour Difficulties and Caregiver Well-Being in Prader-Willi Syndrome: A Preliminary Study. Journal of Autism and Developmental Disorders, 52(9), 3877-3889. https://doi.org/10.1007/s10803-021-05265-5

@article{219e78a31f6346379cd1e0c1562e0124,

title = "Associations Between Hyperphagia, Symptoms of Sleep Breathing Disorder, Behaviour Difficulties and Caregiver Well-Being in Prader-Willi Syndrome: A Preliminary Study",

abstract = "Prader-Willi syndrome (PWS) is a rare genetic disorder characterised by neurodevelopmental delays, hyperphagia, difficulties with social communication and challenging behaviours. Individuals require intensive supervision from caregivers which may negatively affect caregiver quality of life. This study used data collected in the Australasian PWS Registry (n = 50, mean age 11.2 years) to evaluate associations between child behaviours and caregiver mental well-being. Symptoms of sleep-related breathing disorder, child depression and social difficulties were associated with poorer caregiver mental and physical well-being. Growth hormone therapy use was associated with better caregiver mental and physical well-being. Optimising management of problematic behaviours and sleep disturbances have the potential to support caregivers who are the most vital network of support for individuals affected by PWS.",

author = "Jessica Mackay and Nixon, \{Gillian M.\} and Lafferty, \{Antony R.\} and Geoff Ambler and Nitin Kapur and Bergman, \{Philip B.\} and Cara Schofield and Chris Seton and Andrew Tai and Elaine Tham and Komal Vora and Patricia Crock and Charles Verge and Yassmin Musthaffa and Greg Blecher and Daan Caudri and Helen Leonard and Peter Jacoby and Andrew Wilson and Choong, \{Catherine S.\} and Jenny Downs",

note = "Funding Information: We acknowledge the generous contribution of all families who participated in this study. We acknowledge the funding provided for this study. This research was supported by funding from the Perth Children{\textquoteright}s Hospital Foundation to begin the Australasian Prader-Willi Syndrome Database to which participants in this study were recruited. We also acknowledge funding received from the Prader-Willi Association USA 2016 research grant. Funding Information: Gillian Nixon Has grant funding from ResMed Foundation; All renumeration has been paid to her department. Antony Lafferty: Has been on advisory boards in the past of Abbvie Australia, Medtronic Diabetes, Kyowa Kirin Australia and Chiesi Australia. Investigator in a clinical trial with MSD. Helen Leonard: Consultancy for Marinus, Newron, Anavex, GW Pharmaceuticals and AveXis; Clinical Trials with Newron and Anavex; All remuneration has been made to her department. Catherine Choong: Investigator in Clinical Trial OPKO Health \& Pfizer Pharmaceuticals. Jenny Downs: Consultancy for Marinus, Newron, Anavex, GW Pharmaceuticals and AveXis; Clinical Trials with Newron and Anavex; All remuneration has been made to her department. Each of these disclosures relate to subject matter not contained in this manuscript. Jessica Mackay, Cara Schofield, Geoffrey Ambler, Nitin Kapur, Philip Bergman, Chris Seton, Andrew Tai, Elaine Tham, Komal Vora, Patricia Crock, Peter Jacoby, Charles Verge, Yassmin Musthaffa, Greg Blecher, Daan Caudri and Andrew Wilson report no disclosures or conflicts of interest. Publisher Copyright: {\textcopyright} 2021, The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.",

year = "2022",

month = sep,

doi = "10.1007/s10803-021-05265-5",

language = "English",

volume = "52",

pages = "3877--3889",

journal = "Journal of Autism and Developmental Disorders",

issn = "0162-3257",

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}

Mackay, J, Nixon, GM, Lafferty, AR, Ambler, G, Kapur, N, Bergman, PB, Schofield, C, Seton, C, Tai, A, Tham, E, Vora, K, Crock, P, Verge, C, Musthaffa, Y, Blecher, G, Caudri, D, Leonard, H, Jacoby, P, Wilson, A, Choong, CS & Downs, J 2022, 'Associations Between Hyperphagia, Symptoms of Sleep Breathing Disorder, Behaviour Difficulties and Caregiver Well-Being in Prader-Willi Syndrome: A Preliminary Study', Journal of Autism and Developmental Disorders, vol. 52, no. 9, pp. 3877-3889. https://doi.org/10.1007/s10803-021-05265-5

Associations Between Hyperphagia, Symptoms of Sleep Breathing Disorder, Behaviour Difficulties and Caregiver Well-Being in Prader-Willi Syndrome: A Preliminary Study. / Mackay, Jessica; Nixon, Gillian M.; Lafferty, Antony R. et al.
In: Journal of Autism and Developmental Disorders, Vol. 52, No. 9, 09.2022, p. 3877-3889.

Research output: Contribution to journal › Article › Academic › peer-review

TY - JOUR

T1 - Associations Between Hyperphagia, Symptoms of Sleep Breathing Disorder, Behaviour Difficulties and Caregiver Well-Being in Prader-Willi Syndrome

T2 - A Preliminary Study

AU - Mackay, Jessica

AU - Nixon, Gillian M.

AU - Lafferty, Antony R.

AU - Ambler, Geoff

AU - Kapur, Nitin

AU - Bergman, Philip B.

AU - Schofield, Cara

AU - Seton, Chris

AU - Tai, Andrew

AU - Tham, Elaine

AU - Vora, Komal

AU - Crock, Patricia

AU - Verge, Charles

AU - Musthaffa, Yassmin

AU - Blecher, Greg

AU - Caudri, Daan

AU - Leonard, Helen

AU - Jacoby, Peter

AU - Wilson, Andrew

AU - Choong, Catherine S.

AU - Downs, Jenny

N1 - Funding Information: We acknowledge the generous contribution of all families who participated in this study. We acknowledge the funding provided for this study. This research was supported by funding from the Perth Children’s Hospital Foundation to begin the Australasian Prader-Willi Syndrome Database to which participants in this study were recruited. We also acknowledge funding received from the Prader-Willi Association USA 2016 research grant. Funding Information: Gillian Nixon Has grant funding from ResMed Foundation; All renumeration has been paid to her department. Antony Lafferty: Has been on advisory boards in the past of Abbvie Australia, Medtronic Diabetes, Kyowa Kirin Australia and Chiesi Australia. Investigator in a clinical trial with MSD. Helen Leonard: Consultancy for Marinus, Newron, Anavex, GW Pharmaceuticals and AveXis; Clinical Trials with Newron and Anavex; All remuneration has been made to her department. Catherine Choong: Investigator in Clinical Trial OPKO Health & Pfizer Pharmaceuticals. Jenny Downs: Consultancy for Marinus, Newron, Anavex, GW Pharmaceuticals and AveXis; Clinical Trials with Newron and Anavex; All remuneration has been made to her department. Each of these disclosures relate to subject matter not contained in this manuscript. Jessica Mackay, Cara Schofield, Geoffrey Ambler, Nitin Kapur, Philip Bergman, Chris Seton, Andrew Tai, Elaine Tham, Komal Vora, Patricia Crock, Peter Jacoby, Charles Verge, Yassmin Musthaffa, Greg Blecher, Daan Caudri and Andrew Wilson report no disclosures or conflicts of interest. Publisher Copyright: © 2021, The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.

PY - 2022/9

Y1 - 2022/9

N2 - Prader-Willi syndrome (PWS) is a rare genetic disorder characterised by neurodevelopmental delays, hyperphagia, difficulties with social communication and challenging behaviours. Individuals require intensive supervision from caregivers which may negatively affect caregiver quality of life. This study used data collected in the Australasian PWS Registry (n = 50, mean age 11.2 years) to evaluate associations between child behaviours and caregiver mental well-being. Symptoms of sleep-related breathing disorder, child depression and social difficulties were associated with poorer caregiver mental and physical well-being. Growth hormone therapy use was associated with better caregiver mental and physical well-being. Optimising management of problematic behaviours and sleep disturbances have the potential to support caregivers who are the most vital network of support for individuals affected by PWS.

AB - Prader-Willi syndrome (PWS) is a rare genetic disorder characterised by neurodevelopmental delays, hyperphagia, difficulties with social communication and challenging behaviours. Individuals require intensive supervision from caregivers which may negatively affect caregiver quality of life. This study used data collected in the Australasian PWS Registry (n = 50, mean age 11.2 years) to evaluate associations between child behaviours and caregiver mental well-being. Symptoms of sleep-related breathing disorder, child depression and social difficulties were associated with poorer caregiver mental and physical well-being. Growth hormone therapy use was associated with better caregiver mental and physical well-being. Optimising management of problematic behaviours and sleep disturbances have the potential to support caregivers who are the most vital network of support for individuals affected by PWS.

UR - https://www.scopus.com/pages/publications/85114432689

U2 - 10.1007/s10803-021-05265-5

DO - 10.1007/s10803-021-05265-5

M3 - Article

AN - SCOPUS:85114432689

SN - 0162-3257

VL - 52

SP - 3877

EP - 3889

JO - Journal of Autism and Developmental Disorders

JF - Journal of Autism and Developmental Disorders

IS - 9

ER -

Associations Between Hyperphagia, Symptoms of Sleep Breathing Disorder, Behaviour Difficulties and Caregiver Well-Being in Prader-Willi Syndrome: A Preliminary Study

Abstract

Bibliographical note

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