Adults with Loeys-Dietz syndrome and vascular Ehlers-Danlos syndrome: A cross-sectional study of life satisfaction

Abstract

Objective: To explore life satisfaction among adults with Loeys-Dietz and those with vascular Ehlers-Danlos syndrome. Design: Postal survey in 2018. Participants and methods: Persons with molecularly verified Loeys-Dietz syndrome or vascular Ehlers-Danlos syndrome were recruited through the National Resource Centre for Rare Disorders in Norway. The study used the Life Satisfaction Questionnaire 11, Hospital Anxiety and Depression Scale, Fatigue Severity Scale, and questions about physical activity and disease burden. Descriptive statistics were conducted with Bonferroni corrections. Results: The response rate was 74%, 52 participants, age range 18–68 years, and 58% were women. Only half of the participants were satisfied with their lives as a whole. Participants reported dissatisfaction with vocation, somatic health, and sexual life in particular. Participants with vascular Ehlers-Danlos syndrome (n = 18) were satisfied with more areas of life than those with Loeys-Dietz syndrome (n = 34). Low overall satisfaction was significantly associated with severe fatigue (p = 0.002) and symptoms of anxiety (p = 0.001). Conclusion: This study provides important information about living with Loeys-Dietz syndrome and vascular Ehlers-Danlos syndrome. Future studies should identify factors that reduce/increase life satisfaction. Professionals in welfare systems need more information about the unique challenges of living with these diagnoses. Guidelines for research and clinical measurements of life satisfaction should be updated. Lay Abstract The aim of this study was to explore life satisfaction in adults with Loeys-Dietz syndrome and those with vascular Ehlers-Danlos syndrome. In 2018, the Life Satisfaction Questionnaire 11 and questionnaires including psychological distress, fatigue, physical activity, and disease burden were sent to adults who were registered at the National Resource Centre for Rare Disorders in Norway. The response rate was 74%, and 52 people aged 18–68 years participated. Participants who had vascular Ehlers-Danlos syndrome were more often satisfied in most areas of their lives than those with Loeys-Dietz syndrome. Both groups indicated the greatest satisfaction in relation to family life, relationships with their partners, and activities of daily living, while satisfaction with vocations, somatic health, and sexual lives were less common. Low overall life satisfaction was associated with higher levels of fatigue and greater symptoms of anxiety. Future studies should be conducted to gain greater knowledge about factors that reduce and increase their life satisfaction.

Keywords

• life satisfaction
• Loeys-Dietz syndrome
• postal questionnaire
• vascular Ehlers-Danlos syndrome