What are the most important symptoms to assess in hypermobile Ehlers-Danlos syndrome? A questionnaire study based on the Delphi technique

Abstract

Purpose

This study aims to determine from questionnaires, submitted to patients with Ehlers-Danlos syndrome hypermobile type (hEDS), what symptoms they perceive as having the most impact on their well-being and, according to them, what symptoms should be assessed.

Materials and methods

Three rounds of online questionnaires were conducted following the Delphi method. The first round allowed us to obtain the most important symptoms to assess according to the patients. The second and third round aimed at ranking the categories according to their order of importance. Establishment of a consensus was evaluated using Kendall’s coefficient of concordance.

Results

A total of 118 responses were analyzed for the first round and 87 for the second and the third round. Ten categories were extracted from the first round. Ranking of the 10 categories in the second round did not reach consensus (W = 0.33, p < 0.001) nor did the four most important categories in the third round (W = 0.43, p < 0.001). However, three categories stand out from ranking: “pain”, “fatigue and sleep disorders”, and “musculoskeletal disorders”.

Conclusions

These categories seem to be the most important to assess in patients with hEDS, despite the lack of consensus on this ranking.

Implications for rehabilitation

• Pain, fatigue and sleep disorders, and musculoskeletal disorders should be given high consideration in the assessment of patients with hypermobile Ehlers-Danlos syndrome (EDS).

• The high phenotypic variability in the hypermobile EDS requires individualized assessment for each patient and a multidisciplinary approach.

Keywords:

• Delphi technique
• Ehlers-Danlos symptoms
• Ehlers-Danlos syndrome
• joint hypermobility
• symptom assessment

Acknowledgements

The authors thank the patients who have agreed to participate in this study as well as the patients association SED1+ who participated in the good progress of the protocol and Ms. Elise Dangtran-Ciccarelli and Mr. Thomas Gillard for their precious help in the translation of this work.

Disclosure statement

The authors report no conflicts of interest.

Data availability statement

The data that support the findings of this study are available from the corresponding author, AH, upon reasonable request.