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. 2020 Oct 19;15(1):293.
doi: 10.1186/s13023-020-01476-8.

Coping with Wolf-Hirschhorn syndrome: quality of life and psychosocial features of family carers

Sarah Berrocoso  1 , Imanol Amayra  2 , Esther Lázaro  2 , Oscar Martínez  2 , Juan Francisco López-Paz  2 , Maitane García  2 , Manuel Pérez  2 , Mohammad Al-Rashaida  2 , Alicia Aurora Rodríguez  2 , Paula Maria Luna  2 , Paula Pérez-Núñez  2 , Raquel Blanco  3 , Julián Nevado  4
Affiliations

Coping with Wolf-Hirschhorn syndrome: quality of life and psychosocial features of family carers

Sarah Berrocoso et al. Orphanet J Rare Dis. .

Abstract

Background: Wolf-Hirschhorn Syndrome (WHS) is a rare, congenital disease characterized by a distinctive facial phenotype, seizures, intellectual disability and developmental delay, and pre and postnatal growth requiring lifelong care. The psychosocial status of the family caregivers of children diagnosed with WHS is unknown. This study aims to characterize the sociodemographic and psychosocial profile of WHS caregivers and analyze how these variables impact their quality of life (QoL) and well-being.

Results: The sociodemographic and clinical profile of 22 Spanish caregivers of children with WHS and the characteristics of those affected have been described. Significant relationships were found between sociodemographic and psychosocial characteristics among caregivers. The impact on the parents' QoL and negative relationship with the symptomatology were assessed. The use of engagement strategies such as problem focused coping was associated with improved psychological QoL and social support.

Conclusions: WHS caregivers share similarities in their profile and needs with caregivers of children with other rare diseases. Pychosocial support groups involving parents caring for children with the same disease could improve caregivers' well-being and QoL by strengthening their social support network and using positive coping styles.

Keywords: 4p deletion; Caregivers; Coping; Depression; Quality of life; Social support; Spirituality; Wolf-Hirschhorn syndrome.

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Conflict of interest statement

The authors declare that they have no competing interests.

Figures

Fig. 1
Fig. 1
Summarized conceptual model - Mechanisms involved in caregiving processes. Solid lines represent positive correlations and dashed lines negative correlations. SRPB = spirituality, religiousness and personal beliefs
Fig. 2
Fig. 2
Average profile of WHS caregivers in Spain. This figure shows the data findings of this study and its similarity to other published research: [–61]. The symbols before number references represent the following interpretation: a) > more than in references; b) < less than in references; c) = comparable to references
See this image and copyright information in PMC

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