Ehlers-Danlos syndrome

Recommendations for monitoring are based on expert opinion. Each patient is judged individually.

Pain management should be reviewed regularly to determine the efficacy and tolerability of medication. Those who do not respond adequately may require cognitive behavioral therapy (CBT). Follow-up after any physical rehabilitation program and/or CBT should be at 6 and 12 months after therapy. Earlier reassessment is unlikely to have allowed sufficient time to elicit positive effect.

Review at 6 to 12 weeks after an acute injury is advised to ensure that the injury is settling and unlikely to become a chronic problem.

Introducing therapies for the management of autonomic dysfunction should be assessed regularly until the clinician is confident that the therapy is well tolerated and efficacious. Thereafter, a 6-month review is recommended. Periodic echocardiogram to evaluate for mitral valve prolapse and to measure the aortic root is advised, especially for children and adolescents, but the optimal frequency of this investigation has not been determined.