Education and employment status among adults with Loeys-Dietz syndrome and vascular Ehlers-Danlos syndrome in Norway, a questionnaire based study

PLoS One. 2022 Dec 30;17(12):e0279848. doi: 10.1371/journal.pone.0279848. eCollection 2022.


Objectives: To describe education level and employment status among adults with Loeys-Dietz syndrome and vascular Ehlers-Danlos syndrome, and explore factors related to work participation.

Materials and methods: Cross-sectional postal survey in 2018. Individuals with molecularly verified diagnosis were recruited through a National Resource Centre for Rare Disorders. A study specific questionnaire included topics on disease burden and validated instruments regarding education level, employment, pain, fatigue, psychological distress, and satisfaction with life.

Results: Fifty persons (56% women) aged 18-67 years, participated. Almost 60% reported education level ≤13 years. Two thirds (66%) received disability benefits, 21 (42%) had full-time disability pension. The median age at ending work was 41 years. Full-time employed and students were younger (p = 0.014), less fatigued (p = 0.035), had less sleep problems (p = 0.028) and higher satisfaction with life (p<0.001) than those who received disability pension. A third (32%) were currently or used to be in sedentary work, and 68% currently had or used to be in practical work requiring much standing and walking (23%), much walking and lifting (34%) or heavy manual work (11%).

Conclusions: There is a potential that more adults with these diagnoses can sustain employment for more years. Health and social service follow-up routines and future studies should include details on employment perspectives to reveal those at risk of poor employment and to identify modifiable factors for work participation.

MeSH terms

  • Adult
  • Cross-Sectional Studies
  • Educational Status
  • Ehlers-Danlos Syndrome*
  • Ehlers-Danlos Syndrome, Type IV*
  • Employment
  • Female
  • Humans
  • Loeys-Dietz Syndrome*
  • Male
  • Norway / epidemiology
  • Surveys and Questionnaires

Grants and funding

The author(s) received no specific funding for this work.