Family Adjustment to Hereditary Cancer Syndromes: A Systematic Review

Abstract Hereditary cancer syndromes are inherited genetic pathogenic variants that significantly increase the risk of developing cancer. When an individual becomes aware of their increased probability of having cancer, the whole family is affected by this new reality and needs to adjust. However, adjustment to hereditary cancer syndromes has been mainly studied at an individual level and research about familial adjustment remains dispersed and disorganized. To overcome this gap, this review aims to understand how families adjust to genetic testing and risk management, and to what extent the adjustment of the family influences the psychological response and risk management behaviors of mutation carriers. We conducted searches on the PubMed/Med Line, PsycInfo, SCOPUS, and Google Scholar databases and used the Mixed Methods Appraisal Tool (MMAT-v2018) to assess the methodological quality of each selected study. Thirty studies met the inclusion criteria. Most Results highlighted the interdependent nature of adjustment of pathogenic variant carriers and their families. The way carriers adjust to the syndrome is highly related with how family members react to it, particularly partners and siblings. Couples who share their worries and communicate openly about cancer risk present a better long-term adjustment then couples who use protective buffering (not talking about it to avoid disturbing the partner) or emotional distancing. Parents need help dealing with disclosing genetic information to their children. These findings reinforce the importance of adopting a family-centered approach in the context of genetic counseling and the necessity of involving family members in research. Keywords: hereditary cancer syndromes, genetic testing, family adjustment, cancer risk management, decision-making, genetic counselingThis work was supported by the European COMPETE2020 under grant POCI-01-0145-FEDER-030980, Portuguese National funds FCT - Fundação para a Ciência e a Tecnologia, I.P, under grant PTDC/PSI-ESP/30980/2017 and the COST (European Cooperation in Science and Technology), under grant COST Action CA16102