Many patients with EDS live healthy, unaffected lives. Only vascular EDS (vEDS) is associated with a shortened lifespan.[47]Pepin M, Schwarze U, Superti-Furga A, et al. Clinical and genetic features of Ehlers-Danlos syndrome type IV, the vascular type. N Engl J Med. 2000;342:673-80.
http://www.nejm.org/doi/full/10.1056/NEJM200003093421001#t=article
http://www.ncbi.nlm.nih.gov/pubmed/10706896?tool=bestpractice.com
Patients with chronic pain syndrome and/or severely disruptive recurrent injury and joint dislocation can be immensely challenging to treat. Some patients have been known to excel in recovery to normal daily function with good quality of life, while others remain severely disabled with continued need for medical and psychosocial adjustments.[21]Rombaut L, Malfait F, Cools A, et al. Musculoskeletal complaints, physical activity and health-related quality of life among patients with the Ehlers-Danlos syndrome hypermobility type. Disabil Rehabil. 2010;32(16):1339-45.
http://www.ncbi.nlm.nih.gov/pubmed/20156051?tool=bestpractice.com
Overall, disability in these patients is considered to be clinically significant, but the severity of disability varies between individuals and can be symptom-specific. It is not currently known what factors lead to severe disability and, hence, it is difficult to determine which individuals are at risk of developing chronic complications.[37]Scheper MC, Juul-Kristensen B, Rombaut L, et al. Disability in adolescents and adults diagnosed with hypermobility-related disorders: a meta-analysis. Arch Phys Med Rehabil. 2016 Dec;97(12):2174-87.
http://www.ncbi.nlm.nih.gov/pubmed/26976801?tool=bestpractice.com
Patients with vEDS have a mean survival age of 51 years, with patients succumbing to arterial or visceral rupture.[48]Byers PH, Belmont J, Black J, et al. Diagnosis, natural history, and management in vascular Ehlers-Danlos syndrome. Am J Med Genet C Semin Med Genet. 2017 Mar;175(1):40-7.
http://onlinelibrary.wiley.com/doi/10.1002/ajmg.c.31553/full
http://www.ncbi.nlm.nih.gov/pubmed/8306228?tool=bestpractice.com
Surgery and invasive radiology are best avoided due to the risk of life-threatening complications.[47]Pepin M, Schwarze U, Superti-Furga A, et al. Clinical and genetic features of Ehlers-Danlos syndrome type IV, the vascular type. N Engl J Med. 2000;342:673-80.
http://www.nejm.org/doi/full/10.1056/NEJM200003093421001#t=article
http://www.ncbi.nlm.nih.gov/pubmed/10706896?tool=bestpractice.com
Pregnancy in these patients is hazardous but success is well documented.