Thirsa Conijn1, Stephanie C M Nijmeijer1, Hedy A van Oers2, Frits A Wijburg3, Lotte Haverman2. 1. Department of Pediatric Metabolic Diseases, Emma Children's Hospital and Lysosome Center 'Sphinx', Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands. 2. Psychosocial Department, Emma Children's Hospital, Academic Medical Center, Amsterdam, The Netherlands. 3. Department of Pediatric Metabolic Diseases, Emma Children's Hospital and Lysosome Center 'Sphinx', Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands. f.a.wijburg@amc.uva.nl.
Abstract
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.
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 IIIpatients 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 IIIpatients 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 IIIpatients 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 IIIpatient 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.
Authors: Jessica Warnink-Kavelaars; Hedy A van Oers; Lotte Haverman; Annemieke I Buizer; Mattijs W Alsem; Raoul H H Engelbert; Leonie A Menke Journal: Am J Med Genet A Date: 2020-10-09 Impact factor: 2.802
Authors: T Conijn; C De Roos; H J I Vreugdenhil; E M Van Dijk-Lokkart; F A Wijburg; L Haverman Journal: Orphanet J Rare Dis Date: 2022-09-02 Impact factor: 4.303
Authors: D Roorda; A F W van der Steeg; M van Dijk; J P M Derikx; R R Gorter; J Rotteveel; J B van Goudoever; L W E van Heurn; J Oosterlaan; L Haverman Journal: Orphanet J Rare Dis Date: 2022-09-11 Impact factor: 4.303