JIMD reports | 2019
Psychosocial Functioning in Parents of MPS III Patients.
Abstract
BACKGROUND\nMucopolysaccharidosis 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.\n\n\nMETHODS\nTwenty-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).\n\n\nRESULTS\nMothers met the criteria for clinically relevant anxiety (50%) and depression (34.6%) more frequently compared to reference mothers (p\xa0=\xa00.001). Fathers more often met the criteria for clinically relevant depression (36.8%) compared to reference fathers (p\xa0=\xa00.022). Clinically relevant distress was highly prevalent in mothers (84.6%) and fathers (68.4%) of MPS III patients compared to reference parents (p\xa0<\xa00.01). 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\xa0<\xa00.001 and p\xa0<\xa00.05).\n\n\nCONCLUSIONS\nWe 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.