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.
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Acknowledgments
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.
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Communicated by: Roberto Giugliani, MD, PhD
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Synopsis
Mothers and fathers of MPS III patients have an impaired psychosocial functioning, demonstrated by increased levels of anxiety, depression, distress, and a remarkably high prevalence of PTSD.
Details of Author Contributions
Thirsa Conijn and Stephanie Nijmeijer were involved in conception and design of this study, in analyses and interpretation of the data, and drafting the article. Lotte Haverman, Hedy van Oers, and Frits Wijburg were involved in the conception and design of this study, in analyses and interpretation of the data, and critically revising the article. All authors are in agreement with submission of this draft to JIMD reports. Frits Wijburg is the guarantor for this article.
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F. A. Wijburg
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This study was funded by grants from the private foundations Zabawas, Zeldzame Ziekten Fonds, and Kinderen en Kansen, the Netherlands. The authors confirm independence from the sponsors; the content of the article has not been influenced by the sponsors.
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The study was conducted in compliance with ethical standards.
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Informed consent was obtained from the participating parents.
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This article does not contain any studies with animal subjects performed by any of the authors.
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Conijn, T., Nijmeijer, S.C.M., van Oers, H.A., Wijburg, F.A., Haverman, L. (2018). Psychosocial Functioning in Parents of MPS III Patients. In: Morava, E., Baumgartner, M., Patterson, M., Rahman, S., Zschocke, J., Peters, V. (eds) JIMD Reports, Volume 44. JIMD Reports, vol 44. Springer, Berlin, Heidelberg. https://doi.org/10.1007/8904_2018_119
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DOI: https://doi.org/10.1007/8904_2018_119
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