Journal of Inherited Metabolic Disease

, Volume 36, Issue 2, pp 281–291 | Cite as

Parental social support, coping strategies, resilience factors, stress, anxiety and depression levels in parents of children with MPS III (Sanfilippo syndrome) or children with intellectual disabilities (ID)

  • Sheena Grant
  • Elaine Cross
  • James Edmond Wraith
  • Simon Jones
  • Louise Mahon
  • Michelle Lomax
  • Brian Bigger
  • Dougal Hare
Original Article


Mucopolysaccharidosis type III (MPS III, Sanfilippo syndrome) is a lysosomal storage disorder, caused by a deficiency in one of four enzymes involved in the catabolism of the glycosaminoglycan heparan sulphate. It is a degenerative disorder, with a progressive decline in children’s intellectual and physical functioning. There is currently no cure for the disorder. To date there is a paucity of research on how this disorder impacts parents psychological functioning. Specifically, research in the area has failed to employ adequate control groups to assess if the impact of this disorder on parents psychological functioning differs from parenting a child with intellectual disability (ID). The current study examined child behaviour and parental psychological functioning in 23 parents of children with MPS III and 23 parents of children with ID. Parents completed postal questionnaires about their child’s behaviour and abilities and their own psychological functioning. Parents of children with MPS III reported fewer behavioural difficulties as their child aged, more severe level of intellectual disability, and similar levels of perceived social support, coping techniques, stress, anxiety and depression levels as parents of children with ID. Both groups of parents scored above the clinical cut off for anxiety and depression. Parents of children with MPS III rated themselves as significantly less future-orientated and goal directed than parents of children with ID. Services should develop support packages for parents of children with MPS III that incorporate an understanding of the unique stressors and current-difficulty approach of this population. Future research should examine gender differences between parental psychological functioning, using mixed qualitative and quantitative approaches, and utilise matched developmental level and typically developing control groups.


Down Syndrome Intellectual Disability Intellectual Disability Challenging Behaviour Supplementary Material Table 


Competing interests

This work was completed as part of a large-scale project with Dr Elaine Cross, Dr Louise Mahon and Ms Michelle Lomax, as part requirements of the Doctorate in Clinical psychology, at the University of Manchester.

Supplementary material

10545_2012_9558_MOESM1_ESM.doc (80 kb)
ESM 1 (DOC 80 kb)


  1. Affleck G, Tennen H (1993) Cognitive adaptation to adversity: insights from parents of medically fragile infants. In: Turnbull AP, Patterson SK, Behr, Murphy JG, Marquis DL, Blue-Banning MJ (eds) Cognitive coping, families, and disability. PH Brookes, Baltimore, pp 135–150Google Scholar
  2. American Psychiatric Association (2000) Diagnostic and statistical manual of mental disorders (4th edn., text rev). APA, Washington DCGoogle Scholar
  3. Baker BL, Blacher J, Olsson MB (2005) Preschool children with and without developmental delay: behaviour problems, parents’ optimism and well-being. J Intellect Dis Res 49(8):575–590CrossRefGoogle Scholar
  4. Bax MC, Colville GA (1995) Behaviour in mucopolysaccharide disorders. Arch Dis Child 73:77–81PubMedCrossRefGoogle Scholar
  5. Bitsika V, Sharpley CF (2004) Stress, anxiety and depression among parents of children with autism spectrum disorder. Aust J Guid Couns 14(2):151–161Google Scholar
  6. Carver CS (1997) You want to measure coping but your protocol’s too long: consider the brief COPE. Int J Behav Med 4:92–100PubMedCrossRefGoogle Scholar
  7. Code of Practice for Mental Capacity Act (2005). Retrieved on the 10th March 2012 from
  8. Cross E, Grant S et al (2012) An investigation into the middle and late behavioural phenotypes of Mucopolysaccharidosis type-III. Doctoral Dissertation, University of Manchester, UKGoogle Scholar
  9. De Ruijter J, Valstar JM, Wijburg AF (2011) Mucopolysaccharidosis Type III (Sanfilippo Syndrome): emerging treatment strategies. Curr Pharma Biotech 12(6):923–930CrossRefGoogle Scholar
  10. Eyberg S, Pincus D (1999) Eyberg child behaviour inventory & sutter-eyberg student behaviour inventory-revised: professional manual. Psychological Assessment Resources, OdessaGoogle Scholar
  11. Friborg O, Martinussen M, Rosenvinge JH (2006) Likert-based versus semantic differential-based scoring of positive psychological constructs: a psychometric comparison of two versions of a scale measuring resilience. Personal Individ Differ 40(5):873–884CrossRefGoogle Scholar
  12. Glidden LM, Kiphart MJ, Willoughby JC, Bush BA (1993) Family functioning when rearing children with developmental disabilities. In: Turnbull AP, Patterson SK, Behr, Murphy JG, Marquis DL, Blue-Banning MJ (eds) Cognitive coping, families, and disability. PH Brookes, Baltimore, pp 135–150Google Scholar
  13. Glidden LM, Billings FJ, Jobe BM (2006) Personality, coping style and well-being of parents rearing children with developmental disabilities. J Intell Dis Rese 50:949–962CrossRefGoogle Scholar
  14. Goldberg DP, Williams P (1988) The user’s guide to the general health questionnaire. NFER-Nelson, WindsorGoogle Scholar
  15. Goldberg DP, Gater R, Santorius N et al (1997) The validity of two versions of the GHQ in the WHO study of mental illness in general health care. Psychol Med 27:191–197PubMedCrossRefGoogle Scholar
  16. Green SE (2003) “What do you mean ‘what’s wrong with her?”: stigma and the lives of families of children with disabilities. Soc Sci Med 57:1361–1374PubMedCrossRefGoogle Scholar
  17. Heiman T (2002) Parents of children with disabilities: resilience, coping, and future expectations. J Dev Phys Dis 14:159–171CrossRefGoogle Scholar
  18. Hjemdal O, Friborg O, Stiles TC, Rosenvinge JH, Martinussen M (2006) Resilience predicting psychiatric symptoms: a prospective study of protective factors and their role in adjustment to stressful life events. Clin Psychol Psychother 13:194–201CrossRefGoogle Scholar
  19. Hodapp RM, Fidler DJ, Smith ACM (1998) Stress and coping in families of children with Smith-Magenis syndrome. J Intellect Dis Res 42(5):331–340CrossRefGoogle Scholar
  20. Houser R, Seligman M (1991) A comparison of stress and coping by fathers of adolescents with mental retardation and fathers of adolescents without mental retardation. Res Dev Disabil 12(3):251–260PubMedCrossRefGoogle Scholar
  21. Kim HW, Greenberg J, Seltzer M, Krauss M (2003) The role of coping in maintaining the psychological well-being of mothers of adults with intellectual disability and mental illness. J Intell Dis Res 47(4–5):313–327CrossRefGoogle Scholar
  22. Macdonald ME, Chilibeck G, Affleck W, Cadell S (2010) Gender imbalance in paediatric palliative care research samples. Palliat Med 24:435–444PubMedCrossRefGoogle Scholar
  23. Mahon LV, Lomax M, Grant S et al (2012) Assessment of Sleep in Children with Mucopolysaccharidosis Type III under review for J Inherit Metab DisGoogle Scholar
  24. Malcolm C, Forbat L, Anderson G, Gibson F, Hain R (2011) Challenging symptom profiles of life-limiting conditions in children: a survey of care professionals and families. Palliat Med 25:357–364PubMedCrossRefGoogle Scholar
  25. Malcolm C, Hain R, Gibson F, Adams S, Anderson G, Forbat L (2012) Challenging symptoms in children with rare life-limiting conditions (LLCs): findings from a prospective diary and interview study with families. Acta Paediatr 101(9):985-92. doi: 10.1111/j.1651-2227.2012.02680.x Google Scholar
  26. Menezes A (2010) Moments of realisation: life-limiting illness in childhood – perspectives of children, young people and families. Int J Palliat Nurs 16:41–47PubMedGoogle Scholar
  27. Meyer A, Kossow K, Gal A et al (2007) Scoring evaluation of the natural course of Mucopolysaccharidosis type IIIA (Sanfilippo syndrome type A). Pediatrics 120:e1255–e1261PubMedCrossRefGoogle Scholar
  28. Nakagawa S (2004) A farewell to Bonferroni: the problems of low statistical power and publication bias. Behav Ecol 15:1044–1045CrossRefGoogle Scholar
  29. Nidiffer FD, Kelly TE (1983) Developmental and degenerative patterns associated with cognitive, behavioural and motor difficulties in the Sanfilippo syndrome: an epidemiological study. J Ment Defic Res 27(Pt 3):185–203PubMedGoogle Scholar
  30. Patterson J (1985) Critical factors affecting family compliance with home treatment for children with cystic fibrosis. Fam Rela 34:79–89CrossRefGoogle Scholar
  31. Pendaries C (1997) Pilot study on the development of the learning disability Healthcare Resource Groups. Brit J Learn Dis 25(3):122–126CrossRefGoogle Scholar
  32. Raina P, O’Donnell M, Rosenbaum P et al (2005) The health and well-being of caregivers of children with cerebral palsy. Pedia 115:e626. doi: 10.1542/peds.2004-1689 CrossRefGoogle Scholar
  33. Ray LD (2005) Categorical service allocation and barriers to care for children with chronic conditions. Can J Nurs Res Rev Can de Rech en Sci Infirmieres 37(3):86–102Google Scholar
  34. Ruijter GJ, Valstar MJ, van de Kamp JM et al (2008) Clinical and genetic spectrum of Sanfilippo type C (MPS IIIC) disease in The Netherlands. Mol Genet Metab 93:104–111PubMedCrossRefGoogle Scholar
  35. Rutter M (2000) Resilience reconsidered: conceptual considerations, empirical findings, and policy implications. In: Shonkoff JP, Meisels SJ (eds) Handbook of early childhood interventions, 2nd edn. Cambridge University Press, New York, pp 651–682CrossRefGoogle Scholar
  36. Sanders MR (1999) Triple P-positive parenting program: towards an empirically validated multilevel parenting and family support strategy for the prevention of behavior and emotional problems in children. Clin Child Fam Psych Rev 2(2):71–90CrossRefGoogle Scholar
  37. Simmerman S, Blacher J, Baker B (2001) Fathers’ and mothers’ perception of father involvement in families with young children with a disability. J Intellect Dev Disabil 26:325–338CrossRefGoogle Scholar
  38. Singer GHS (2006) Meta-analysis of comparative studies of depression in mothers of children with and without developmental disabilities. Am J Ment Retard 111(3):155–169PubMedCrossRefGoogle Scholar
  39. Steele R (2000) Trajectory of certain death at an unknown time: children with neurodegenerative life-threatening illness. Can J Nurs Res 32:49–67PubMedGoogle Scholar
  40. Stoneman Z, Gavidia-Payne S (2006) Marital adjustment in families of young children with disabilities: associations with daily hassles and problem-focused coping. Amer J Ment Retard 111(1):1–14CrossRefGoogle Scholar
  41. Streisand R, Braniecki S, Tercyak KP, Kazak AE (2001) Childhood illness-related parenting stress: the pediatric inventory for parents. J Pediatr Psychol 26(3):155–162PubMedCrossRefGoogle Scholar
  42. Ucar SK, Ozbaran B, Demiral N, Yuncu Z, Erermis S, Coker M (2010) Clinical overview of children with Mucopolysaccharidosis type III A and effect of Risperidone treatment on children and their mothers psychological status. Brain Dev 32:156–161CrossRefGoogle Scholar
  43. Valstar MJ, Ruijter GJG, van Diggelen OP, Poorthuis BJ, Wijburg F (2008) Sanfilippo syndrome: a mini-review. J Inhert Metab Dis 31:240–252CrossRefGoogle Scholar
  44. Webster-Stratton C, Reid MJ, Stoolmiller M (2008) Preventing conduct problems and improving school readiness: evaluation of the incredible years teacher and child training programs in high-risk schools. J Child Psych Psychiatr 49(5):471–488CrossRefGoogle Scholar
  45. Zimet GD, Dahlem NW, Zimet SG, Farley GK (1988) The multidimensional scale of perceived social support. J Pers Assess 52:30–41CrossRefGoogle Scholar

Copyright information

© SSIEM and Springer Science+Business Media Dordrecht 2012

Authors and Affiliations

  • Sheena Grant
    • 1
  • Elaine Cross
    • 1
  • James Edmond Wraith
    • 2
  • Simon Jones
    • 2
  • Louise Mahon
    • 3
  • Michelle Lomax
    • 3
  • Brian Bigger
    • 3
  • Dougal Hare
    • 3
  1. 1.Division of Clinical Psychology, School of Psychological SciencesUniversity of Manchester Clin. Psy. D. ProgrammeManchesterUK
  2. 2.Paediatric Inherited Metabolic Medicine, Manchester Academic Health Sciences Centre, Genetic MedicineSt. Mary’s HospitalManchesterUK
  3. 3.University of ManchesterManchesterUK

Personalised recommendations