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Abstract
Background: The neuronal ceroid lipofuscinoses, collectively known as Batten disease, are rare pediatric neurodegenerative disorders resulting in blindness, progressive motor impairment, dementia, and premature death. This study identified services and support accessed by parents of children with Batten disease and assessed parental marital quality. Methods: Parents completed a survey to identify services their affected children received, and their own uses of social support, information, and online resources. We used the Dyadic Adjustment Scale to evaluate parental marital quality. Results: 29 parents of 23 Batten-affected children completed the survey. 18 children (78%) received receiving in-home nursing or paraprofessional care. Over 90% of parents used the internet and social media to learn or talk about Batten disease; their preferred learning method was from other parents of children with Batten disease. Batten parent marital quality was significantly lower than that reported in studies of the general population or parents of children with chronic, but non-neurodegenerative, disease. Conclusions: Parents expressed an interest in and preference for parent-to-parent communication for support and information about Batten disease. Further work is needed to understand factors associated with the significantly lower marital quality among Batten parents.
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Copyright (c) 2017 Elizabeth J Cozart, Erika F Augustine, Jonathan W Mink, Alyssa R Thatcher, Heather R Adams
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References
- NCL Mutation and Patient Database [http://www.ucl.ac.uk/ncl/mutation.shtml].
- Dolisca S, Mehta M, Pearce D, Mink JW, Maria B: Batten disease: clinical aspects, molecular mechanisms, translational science, and future directions. J Child Neurol;28:1074 -1100.
- Labbe EE, Lopez I, Murphy L, O’Brien C: Optimism and psychosocial functioning in caring for children with Battens and other neurological diseases. Psychol Rep;90:1129-1135.
- Labbe EE: Emotional states and perceived family functioning of caregivers of chronically ill children. Psychol Rep;79:1233-1234.
- Adams HR, Mink JW, and the University of Rochester Batten Center Study Group: Neurobehavioral features and natural history of juvenile neuronal ceroid lipofuscinosis (Batten disease). J Child Neurol;28:1128-1136.
- Cousino M, Hazen R: Parenting stress among caregivers of children with chronic illness: a systematic review. J Pediatr Psychol;38:809-828.
- Gavidia-Payne S, Stoneman Z: Marital adjustment in families of young children with disabilities: associations with daily hassles and problem-focused coping. Am J Ment Retard;111:1-14.
- Horton T, Wallander J: Hope and social support as resilience factors against psychological distress of mothers who care for children with chronic physical conditions. Rehabil Psychol;46:382-399.
- Jones J, Passey J: Family adaptation, coping and resources: parents of children with developmental disabilities and behavior problems. J Dev Disabil;11:31-46.
- Lambrenos K, Weindling A, Calam R, Cox A: The effect of a child’s disability on mother’s mental health Arch Dis Child;74:115-120.
- Nabors LA, Kichler JC, Brassell A, Thakkar S, Bartz J, Pangallo J, Van Wassenhove B, Lundy H: Factors related to caregiver state anxiety and coping with a child's chronic illness. Fam Syst Health;31:171-180.
- Raina P, O’Donnell M, Schwellnus H, Rosenbaum P, King G, Brehaut J, Russell D, Swinton M, King S, Wong M, et al.: Caregiving process and caregiver burden: conceptual models to guide research and practice. BMC Pediatr;4:1-13.
- Woolfson L: Family well-being and disabled children: a psychosocial model of disability-related child behavior problems. Brit J Health Psych;9:1-13.
- Baker JK, Seltzer MM, Greenberg JS: Behaviour problems, maternal internalising symptoms and family relations in families of adolescents and adults with fragile X syndrome. J Intellect Disabil Res;56:984-995.
- Baum LS: Internet parent support groups for primary caregivers of a child with special health care needs. Pediatr Nurs;30:381-388, 401.
- Kersh J, Hedvat TT, Hauser-Cram P, Warfield ME: The contribution of marital quality to the well-being of parents of children with developmental disabilities. J Intellect Disabil Res;50:883-893.
- Laurvick CL, Msall ME, Silburn S, Bower C, de Klerk N, Leonard H: Physical and mental health of mothers caring for a child with Rett syndrome. Pediatrics;118:e1152-1164.
- Leonard H, Slack-Smith L, Phillips T, Richardson S, D'Orsogna L, Mulroy S: How can the Internet help parents of children with rare neurologic disorders? J Child Neurol;19:902-907.
- Kingsnorth S, Gall C, Beayni S, Rigby P: Parents as transition experts? Qualitative findings from a pilot parent-led peer support group. Child Care Hlth Dev;37:833-840.
- Gundersen T: 'One wants to know what a chromosome is': the internet as a coping resource when adjusting to life parenting a child with a rare genetic disorder. Sociol Health Illn;33:81-95.
- Malcolm C, Hain R, Gibson F, Adams S, Anderson G, Forbat L: Challenging symptoms in children with rare life-limiting conditions: findings from a prospective diary and interview study with families. Acta paediatr;101:985-992.
- Scambler S: Care in Partnership - The Social Care Needs of Young Adults with Juvenile Batten's Disease and Their Families, seeAbility, Leatherhead, Surrey, UK, 1999.
- Batten Disease Support and Research Association: The bumpy road of Batten’s: a beginner’s guide to getting programs and services [http://bdsra.org/wp-content/uploads/2012/01/Programs-and-Services-Overview.pdf].
- Sawyer MG, Bittman M, La Greca AM, Crettenden AD, Borojevic N, Raghavendra P, Russo R: Time demands of caring for children with cerebral palsy: what are the implications for maternal mental health? Dev Med Child Neurol;53:338-343.
- Spanier G: Measuring dyadic adjustment: new scales for assessing the quality of marriage and similar dyads J Marriage Fam;38:15-28.
- Alderfer MA, Fiese BH, Gold JI, Cutuli JJ, Holmbeck GN, Goldbeck L, Chambers CT, Abad M, Spetter D, Patterson J: Evidence-based assessment in pediatric psychology: family measures. J Pediatr Psychol;33:1046-1061.
- Harper A, Taylor Dyches T, Harper J, Olsen Roper S, South M: Respite care, marital quality, and stress in parents of children with autism spectrum disorders. J Autism Dev Disord;43:2604-2616.
- Graham J, Liu Y, Jeziorski J: The Dyadic Adjustment Scale: a reliability generalization meta-analysis. J Marriage Fam;68:701-717.
- Walker JG, Manion IG, Cloutier PF, Johnson SM: Measuring marital distress in couples with chronically ill children: the Dyadic Adjustment Scale. J Pediatr Psychol;17:345-357.
- Eddy JM: An empirical evaluation of the Dyadic Adjustment Scale: exploring the differences between marital “satisfaction” and “adjustment”. Behav Assess;13:199-220.
- Grossoehme DH, Szczesniak R, Dodd C, Opipari-Arrigan L: Dyadic Adjustment and Spiritual Activities in Parents of Children with Cystic Fibrosis. Religions;5:385-401.
- Skinner D, Schaffer R: Families and genetic diagnoses in the genomic and internet age. Infant Young Child;19:16-24.
- Santelli B, Turnbull A, Higgins C: Parent to parent support and health care. Pediatr Nurs;23:303-306.
- Mohd Roffeei SH, Abdullah N, Basar SK: Seeking social support on Facebook for children with Autism Spectrum Disorders (ASDs). Int J Med Inform;84:375-385.
- Thoren EM, Metze B, Buhrer C, Garten L: Online support for parents of preterm infants: a qualitative and content analysis of Facebook 'preemie' groups. Arch Dis Child Fetal Neonatal Ed;98:F534-538.
- Wittmeier K, Holland C, Hobbs-Murison K, Crawford E, Beauchamp C, Milne B, Morris M, Keijzer R: Analysis of a parent-initiated social media campaign for Hirschsprung's disease. J Med Internet Res;16:e288.
References
NCL Mutation and Patient Database [http://www.ucl.ac.uk/ncl/mutation.shtml].
Dolisca S, Mehta M, Pearce D, Mink JW, Maria B: Batten disease: clinical aspects, molecular mechanisms, translational science, and future directions. J Child Neurol;28:1074 -1100.
Labbe EE, Lopez I, Murphy L, O’Brien C: Optimism and psychosocial functioning in caring for children with Battens and other neurological diseases. Psychol Rep;90:1129-1135.
Labbe EE: Emotional states and perceived family functioning of caregivers of chronically ill children. Psychol Rep;79:1233-1234.
Adams HR, Mink JW, and the University of Rochester Batten Center Study Group: Neurobehavioral features and natural history of juvenile neuronal ceroid lipofuscinosis (Batten disease). J Child Neurol;28:1128-1136.
Cousino M, Hazen R: Parenting stress among caregivers of children with chronic illness: a systematic review. J Pediatr Psychol;38:809-828.
Gavidia-Payne S, Stoneman Z: Marital adjustment in families of young children with disabilities: associations with daily hassles and problem-focused coping. Am J Ment Retard;111:1-14.
Horton T, Wallander J: Hope and social support as resilience factors against psychological distress of mothers who care for children with chronic physical conditions. Rehabil Psychol;46:382-399.
Jones J, Passey J: Family adaptation, coping and resources: parents of children with developmental disabilities and behavior problems. J Dev Disabil;11:31-46.
Lambrenos K, Weindling A, Calam R, Cox A: The effect of a child’s disability on mother’s mental health Arch Dis Child;74:115-120.
Nabors LA, Kichler JC, Brassell A, Thakkar S, Bartz J, Pangallo J, Van Wassenhove B, Lundy H: Factors related to caregiver state anxiety and coping with a child's chronic illness. Fam Syst Health;31:171-180.
Raina P, O’Donnell M, Schwellnus H, Rosenbaum P, King G, Brehaut J, Russell D, Swinton M, King S, Wong M, et al.: Caregiving process and caregiver burden: conceptual models to guide research and practice. BMC Pediatr;4:1-13.
Woolfson L: Family well-being and disabled children: a psychosocial model of disability-related child behavior problems. Brit J Health Psych;9:1-13.
Baker JK, Seltzer MM, Greenberg JS: Behaviour problems, maternal internalising symptoms and family relations in families of adolescents and adults with fragile X syndrome. J Intellect Disabil Res;56:984-995.
Baum LS: Internet parent support groups for primary caregivers of a child with special health care needs. Pediatr Nurs;30:381-388, 401.
Kersh J, Hedvat TT, Hauser-Cram P, Warfield ME: The contribution of marital quality to the well-being of parents of children with developmental disabilities. J Intellect Disabil Res;50:883-893.
Laurvick CL, Msall ME, Silburn S, Bower C, de Klerk N, Leonard H: Physical and mental health of mothers caring for a child with Rett syndrome. Pediatrics;118:e1152-1164.
Leonard H, Slack-Smith L, Phillips T, Richardson S, D'Orsogna L, Mulroy S: How can the Internet help parents of children with rare neurologic disorders? J Child Neurol;19:902-907.
Kingsnorth S, Gall C, Beayni S, Rigby P: Parents as transition experts? Qualitative findings from a pilot parent-led peer support group. Child Care Hlth Dev;37:833-840.
Gundersen T: 'One wants to know what a chromosome is': the internet as a coping resource when adjusting to life parenting a child with a rare genetic disorder. Sociol Health Illn;33:81-95.
Malcolm C, Hain R, Gibson F, Adams S, Anderson G, Forbat L: Challenging symptoms in children with rare life-limiting conditions: findings from a prospective diary and interview study with families. Acta paediatr;101:985-992.
Scambler S: Care in Partnership - The Social Care Needs of Young Adults with Juvenile Batten's Disease and Their Families, seeAbility, Leatherhead, Surrey, UK, 1999.
Batten Disease Support and Research Association: The bumpy road of Batten’s: a beginner’s guide to getting programs and services [http://bdsra.org/wp-content/uploads/2012/01/Programs-and-Services-Overview.pdf].
Sawyer MG, Bittman M, La Greca AM, Crettenden AD, Borojevic N, Raghavendra P, Russo R: Time demands of caring for children with cerebral palsy: what are the implications for maternal mental health? Dev Med Child Neurol;53:338-343.
Spanier G: Measuring dyadic adjustment: new scales for assessing the quality of marriage and similar dyads J Marriage Fam;38:15-28.
Alderfer MA, Fiese BH, Gold JI, Cutuli JJ, Holmbeck GN, Goldbeck L, Chambers CT, Abad M, Spetter D, Patterson J: Evidence-based assessment in pediatric psychology: family measures. J Pediatr Psychol;33:1046-1061.
Harper A, Taylor Dyches T, Harper J, Olsen Roper S, South M: Respite care, marital quality, and stress in parents of children with autism spectrum disorders. J Autism Dev Disord;43:2604-2616.
Graham J, Liu Y, Jeziorski J: The Dyadic Adjustment Scale: a reliability generalization meta-analysis. J Marriage Fam;68:701-717.
Walker JG, Manion IG, Cloutier PF, Johnson SM: Measuring marital distress in couples with chronically ill children: the Dyadic Adjustment Scale. J Pediatr Psychol;17:345-357.
Eddy JM: An empirical evaluation of the Dyadic Adjustment Scale: exploring the differences between marital “satisfaction” and “adjustment”. Behav Assess;13:199-220.
Grossoehme DH, Szczesniak R, Dodd C, Opipari-Arrigan L: Dyadic Adjustment and Spiritual Activities in Parents of Children with Cystic Fibrosis. Religions;5:385-401.
Skinner D, Schaffer R: Families and genetic diagnoses in the genomic and internet age. Infant Young Child;19:16-24.
Santelli B, Turnbull A, Higgins C: Parent to parent support and health care. Pediatr Nurs;23:303-306.
Mohd Roffeei SH, Abdullah N, Basar SK: Seeking social support on Facebook for children with Autism Spectrum Disorders (ASDs). Int J Med Inform;84:375-385.
Thoren EM, Metze B, Buhrer C, Garten L: Online support for parents of preterm infants: a qualitative and content analysis of Facebook 'preemie' groups. Arch Dis Child Fetal Neonatal Ed;98:F534-538.
Wittmeier K, Holland C, Hobbs-Murison K, Crawford E, Beauchamp C, Milne B, Morris M, Keijzer R: Analysis of a parent-initiated social media campaign for Hirschsprung's disease. J Med Internet Res;16:e288.