Uniwersytet Gdański, Gdańsk, Instytut Psychologii, Zakład Badań nad Rodziną i Jakością Życia.
Gdański Uniwersytet Medyczny, Gdańsk, Katedra i Klinika Kardiologii Dziecięcej i Wad Wrodzonych.
Psychiatr Pol. 2021 Aug 31;55(4):815-834. doi: 10.12740/PP/OnlineFirst/114838.
The purpose of the study was to analyze the functioning of afamily as perceived by a person with Marfan syndrome and to look for relationships between the characteristics of the system and the overall quality of life of the ill.
Participants included 33 individuals with Marfan syndrome and 33 individuals without chronic illness. We used the Family Evaluation Scale - the Polish adaptation of the FACES-IVby D.H. Olson and the Satisfaction with Life Scale (SWLS) by Diener, Emmons, Larson and Griffin.
People with Marfan syndrome perceive their families as significantly less coherent and significantly more disengaged than people without chronic illness. This family system of people with Marfan syndrome can be characterized by low scores on the "Cohesion" and "Flexibility" and high scores on the other four scales showing the level of imbalance of the family as a system, which makes this family profile similar to an ‛unbalanced' system. Life satisfaction of people with Marfan syndrome correlated positively with "Cohesion", "Flexibility" and "Family Satisfaction" as features of family system perceived by them.
The obtained results confirm the importance of supporting families of people with Marfan syndrome and specialist help aimed at dealing with emotional burden related to the health of the patient.
本研究旨在分析马凡综合征患者对家庭功能的认知，并探讨该系统的特征与患者整体生活质量之间的关系。
研究对象包括 33 名马凡综合征患者和 33 名无慢性病患者。我们使用了家庭评估量表——Olson 夫妇的 FACES-IV 量表的波兰语改编版和 Diener 等人的生活满意度量表（SWLS）。
与无慢性病患者相比，马凡综合征患者认为自己的家庭凝聚力显著降低，家庭参与度显著降低。马凡综合征患者的家庭系统可以用“凝聚力”和“灵活性”得分较低，以及其他四个显示家庭系统失衡程度的量表得分较高来描述，这使得这种家庭模式类似于“不平衡”系统。马凡综合征患者的生活满意度与他们所感知到的家庭系统的“凝聚力”、“灵活性”和“家庭满意度”呈正相关。
研究结果证实了支持马凡综合征患者家庭的重要性，以及为应对与患者健康相关的情感负担而提供专业帮助的重要性。
