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. 2021 Jan;185(1):50-59.
doi: 10.1002/ajmg.a.61906. Epub 2020 Oct 9.

Parenting a child with Marfan syndrome: Distress and everyday problems

Jessica Warnink-Kavelaars  1 , Hedy A van Oers  2 , Lotte Haverman  2 , Annemieke I Buizer  1   3   4 , Mattijs W Alsem  1 , Raoul H H Engelbert  1   5   4 , Leonie A Menke  4
Affiliations

Parenting a child with Marfan syndrome: Distress and everyday problems

Jessica Warnink-Kavelaars et al. Am J Med Genet A. 2021 Jan.

Abstract

Marfan syndrome (MFS) is a multisystemic, autosomal dominant connective tissue disorder that occurs de novo in 25%. In many families, parent and child(ren) are affected, which may increase distress in parents. To assess distress, 42 mothers (29% MFS) and 25 fathers (60% MFS) of 43 affected children, completed the validated screening-questionnaire Distress thermometer for parents of a chronically ill child, including questions on overall distress (score 0-10; ≥4 denoting "clinical distress") and everyday problems (score 0-36). Data were compared to 1,134 control-group-parents of healthy children. Mothers reported significantly less overall distress (2, 1-4 vs. 3, 1-6; p = .049; r = -.07) and total everyday problems (3, 0-6 vs. 4, 1-8; p = .03; r = -.08) compared to control-group-mothers. Mothers without MFS reported significantly less overall distress compared to mothers with MFS, both of a child with MFS (1, 0-4 vs. 3.5, 2-5; p = .039; r = -.17). No significant differences were found between the father-groups, nor between the group of healthy parents of an affected child living together with an affected partner compared to control-group-parents. No differences in percentages of clinical distress were reported between mothers and control-group-mothers (33 vs. 42%); fathers and control-group-fathers (28 vs. 32%); nor between the other groups. Distress was not associated with the children's MFS characteristics. Concluding, parents of a child with MFS did not show more clinical distress compared to parents of healthy children. However, clinical distress was reported in approximately one-third and may increase in case of acute medical complications. We advise monitoring distress in parents of a child with MFS to provide targeted support.

Keywords: Marfan syndrome; autosomal dominant; chronic illness; connective tissue disorder; distress; parents.

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Conflict of interest statement

The authors declare that they have no conflict of interest.

References

    1. Biber, S. , Andonian, C. , Beckmann, J. , Ewert, P. , Freilinger, S. , Nagdyman, N. , & Neidenbach, R. C. (2019). Current research status on the psychological situation of parents of children with congenital heart disease. Cardiovascular Diagnosis and Therapy, 9(Suppl. 2), S369–s376. 10.21037/cdt.2019.07.07 - DOI - PMC - PubMed
    1. Conijn, T. , Nijmeijer, S. C. M. , van Oers, H. A. , Wijburg, F. A. , & Haverman, L. (2019). Psychosocial functioning in parents of MPS III patients. JIMD Reports, 44, 33–41. 10.1007/8904_2018_119 - DOI - PMC - PubMed
    1. Cousino, M. K. , & Hazen, R. A. (2013). Parenting stress among caregivers of children with chronic illness: A systematic review. Journal of Pediatric Psychology, 38(8), 809–828. 10.1093/jpepsy/jst049 - DOI - PubMed
    1. Diederen, K. , Haverman, L. , Grootenhuis, M. A. , Benninga, M. A. , & Kindermann, A. (2018). Parental distress and quality of life in pediatric inflammatory bowel disease: Implications for the outpatient clinic. Journal of Pediatric Gastroenterology and Nutrition, 66(4), 630–636. 10.1097/mpg.0000000000001756 - DOI - PubMed
    1. Dietz, H. (1993). Marfan Syndrome In Adam M. P., Ardinger H. H., Pagon R. A., Wallace S. E., Bean L. J. H., Stephens K., & Amemiya A. (Eds.), GeneReviews([R]). Seattle (WA): University of Washington, Seattle University of Washington, Seattle. GeneReviews is a registered trademark of the University of Washington, Seattle.

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