Background: Mucopolysaccharidosis type III (MPS III or Sanfilippo syndrome) is a lysosomal storage disease resulting in progressive neurocognitive decline during childhood and early demise. Its diagnosis may have a great impact on parents, potentially leading to psychosocial problems such as anxiety, depression, parental distress, and posttraumatic stress.
Methods: Twenty-six mothers and 19 fathers of 34 Dutch MPS III patients completed the “Hospital Anxiety and Depression Scale” (HADS), the “Distress Thermometer for Parents” (DT-P), and the “Self-Rating Scale for Posttraumatic Stress Disorders” (SRS-PTSD). Independent-sample T-tests and chi-square tests were used to assess differences between parents of MPS III patients and reference groups regarding anxiety and depression (HADS), distress (DT-P), and posttraumatic stress (SRS-PTSD).
Results: Mothers met the criteria for clinically relevant anxiety (50%) and depression (34.6%) more frequently compared to reference mothers (p = 0.001). Fathers more often met the criteria for clinically relevant depression (36.8%) compared to reference fathers (p = 0.022). Clinically relevant distress was highly prevalent in mothers (84.6%) and fathers (68.4%) of MPS III patients compared to reference parents (p < 0.01). Finally, the prevalence of PTSD was strikingly higher in both mothers (26.9%) and fathers (15%) than reported in the general Dutch population (respectively, p < 0.001 and p < 0.05).
Conclusions: We report a clinically relevant impact of parenting an MPS III patient on psychosocial functioning, which is demonstrated by high levels of anxiety, depression, distress, and a remarkably high prevalence of PTSD. Structural monitoring of the psychosocial functioning of MPS III parents is therefore essential and may be beneficial for the whole family.
This is a preview of subscription content, log in to check access.
We would like to thank the parents who participated in this study and the Zabawas Foundation, the Zeldzame Ziekten Foundation, and Foundation Kinderen en Kansen for their financial support. Also, we thank Ad Vingerhoets for providing the HADS reference group.
Basart H, van Oers HA, Paes EC et al (2017) Health-related quality of life in children with Robin sequence. Am J Med Genet A 173(1):54–61CrossRefGoogle Scholar
Bjelland I, Dahl AA, Haug TT, Neckelmann D (2002) The validity of the Hospital Anxiety and Depression Scale. An updated literature review. J Psychosom Res 52(2):69–77CrossRefGoogle Scholar
Bronner MB, Peek N, Vries M, Bronner AE, Last BF, Grootenhuis MA (2009) A community-based survey of posttraumatic stress disorder in the Netherlands. J Trauma Stress 22(1):74–78CrossRefGoogle Scholar
Carlier IV, Lamberts RD, Van Uchelen AJ, Gersons BP (1998) Clinical utility of a brief diagnostic test for posttraumatic stress disorder. Psychosom Med 60(1):42–47CrossRefGoogle Scholar
Clarke NE, McCarthy MC, Downie P, Ashley DM, Anderson VA (2009) Gender differences in the psychosocial experience of parents of children with cancer: a review of the literature. Psychooncology 18(9):907–915CrossRefGoogle Scholar
Cleary MA, Wraith JE (1993) Management of mucopolysaccharidosis type III. Arch Dis Child 69(3):403–406CrossRefGoogle Scholar
Cousino MK, Hazen RA (2013) Parenting stress among caregivers of children with chronic illness: a systematic review. J Pediatr Psychol 38(8):809–828CrossRefGoogle Scholar
Grant S, Cross E, Wraith JE et al (2013) 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). J Inherit Metab Dis 36(2):281–291CrossRefGoogle Scholar
Hatzmann J, Heymans HS, Ferrer-i-Carbonell A, van Praag BM, Grootenhuis MA (2008) Hidden consequences of success in pediatrics: parental health-related quality of life – results from the care project. Pediatrics 122(5):e1030–e1038CrossRefGoogle Scholar
Haverman L, van Oers HA, Limperg PF et al (2013) Development and validation of the distress thermometer for parents of a chronically ill child. J Pediatr 163(4):1140–1146.e1142CrossRefGoogle Scholar
Kalkan Ucar S, 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 and Development 32(2):156–161CrossRefGoogle Scholar
Kazak AE, Kassam-Adams N, Schneider S, Zelikovsky N, Alderfer MA, Rourke M (2006) An integrative model of pediatric medical traumatic stress. J Pediatr Psychol 31(4):343–355CrossRefGoogle Scholar
Limperg PF, Haverman L, Peters M, Grootenhuis MA (2016) Psychosocial functioning of mothers of boys with haemophilia. Haemophilia 22(1):e57–e60CrossRefGoogle Scholar
Malcolm C, Hain R, Gibson F, Adams S, Anderson G, Forbat L (2012) Challenging symptoms in children with rare life-limiting conditions: findings from a prospective diary and interview study with families. Acta Paediatr 101(9):985–992CrossRefGoogle Scholar
Marchal JP, van Oers HA, Maurice-Stam H, Grootenhuis MA, van Trotsenburg ASP, Haverman L (2017) Distress and everyday problems in Dutch mothers and fathers of young adolescents with down syndrome. Res Dev Disabil 67:19–27CrossRefGoogle Scholar
Murphy NA, Christian B, Caplin DA, Young PC (2007) The health of caregivers for children with disabilities: caregiver perspectives. Child Care Health Dev 33(2):180–187CrossRefGoogle Scholar
Neece CL (2014) Mindfulness-based stress reduction for parents of young children with developmental delays: implications for parental mental health and child behavior problems. J Appl Res Intellect Disabil 27(2):174–186CrossRefGoogle Scholar
Schepers SA, Sint Nicolaas SM, Maurice-Stam H, Haverman L, Verhaak CM, Grootenhuis MA (2018) Parental distress 6 months after a pediatric cancer diagnosis in relation to family psychosocial risk at diagnosis. Cancer 124(2):381–390CrossRefGoogle Scholar
Shapiro EG, Nestrasil I, Delaney KA et al (2016) A prospective natural history study of mucopolysaccharidosis type IIIA. J Pediatr 170:278–287CrossRefGoogle Scholar
Somanadhan S, Larkin PJ (2016) Parents’ experiences of living with, and caring for children, adolescents and young adults with mucopolysaccharidosis (MPS). Orphanet J Rare Dis 11(1):138CrossRefGoogle Scholar
Spinhoven P, Ormel J, Sloekers PP, Kempen GI, Speckens AE, Van Hemert AM (1997) A validation study of the Hospital Anxiety and Depression Scale (HADS) in different groups of Dutch subjects. Psychol Med 27(2):363–370CrossRefGoogle Scholar
Valstar MJ, Ruijter GJ, van Diggelen OP, Poorthuis BJ, Wijburg FA (2008) Sanfilippo syndrome: a mini-review. J Inherit Metab Dis 31(2):240–252CrossRefGoogle Scholar
van Oers HA, Haverman L, Limperg PF, van Dijk-Lokkart EM, Maurice-Stam H, Grootenhuis MA (2014) Anxiety and depression in mothers and fathers of a chronically ill child. Matern Child Health J 18(8):1993–2002CrossRefGoogle Scholar
van Oers HA, Schepers SA, Grootenhuis MA, Haverman L (2017) Dutch normative data and psychometric properties for the Distress Thermometer for Parents. Qual Life Res 26(1):177–182CrossRefGoogle Scholar