Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2019:44:33-41.
doi: 10.1007/8904_2018_119. Epub 2018 Jul 7.

Psychosocial Functioning in Parents of MPS III Patients

Thirsa Conijn  1 , Stephanie C M Nijmeijer  1 , Hedy A van Oers  2 , Frits A Wijburg  3 , Lotte Haverman  2
Affiliations

Psychosocial Functioning in Parents of MPS III Patients

Thirsa Conijn et al. JIMD Rep. 2019.

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.

Keywords: Anxiety; Depression; MPS III; Parental distress; Posttraumatic stress symptoms; Psychosocial functioning.

PubMed Disclaimer

References

    1. Basart H, van Oers HA, Paes EC, et al. Health-related quality of life in children with Robin sequence. Am J Med Genet A. 2017;173(1):54–61. doi: 10.1002/ajmg.a.37968. - DOI - PubMed
    1. Bjelland I, Dahl AA, Haug TT, Neckelmann D. The validity of the Hospital Anxiety and Depression Scale. An updated literature review. J Psychosom Res. 2002;52(2):69–77. doi: 10.1016/S0022-3999(01)00296-3. - DOI - PubMed
    1. Bronner MB, Peek N, Vries M, Bronner AE, Last BF, Grootenhuis MA. A community-based survey of posttraumatic stress disorder in the Netherlands. J Trauma Stress. 2009;22(1):74–78. doi: 10.1002/jts.20379. - DOI - PubMed
    1. Carlier IV, Lamberts RD, Van Uchelen AJ, Gersons BP. Clinical utility of a brief diagnostic test for posttraumatic stress disorder. Psychosom Med. 1998;60(1):42–47. doi: 10.1097/00006842-199801000-00010. - DOI - PubMed
    1. CBS (2012) Onderwijsniveau bevolking gestegen. https://www.cbs.nl/nl-nl/nieuws/2013/40/onderwijsniveau-bevolking-gestegen. Accessed Sept 2017

LinkOut - more resources