Research: Supporting pregnant women and newborns with Ehlers-Danlos Syndromes

By Sally Pezaro on 03 September 2018 Midwives Magazine Ehlers-Danlos Syndromes Research

The Ehlers-Danlos Syndromes (EDS) are a group of 13 genetic conditions that affect the connective tissues throughout the body.

The prevalence of EDS may be far greater than previously thought (0.75% to 2%) and remains largely undiagnosed. Those with a diagnosis of EDS report a lack of understanding among healthcare professionals.

Hypermobile EDS (hEDS) and the related Hypermobility Spectrum Disorders (formerly Joint Hypermobility Syndrome) is the most common subtype of EDS and is associated with a number of childbearing complications. Consequently, the aim of this research was to draw upon the existing international evidence, and present evidence-based care considerations for newborns and childbearing women with hEDS.

Women with hEDS can experience higher rates of infertility. Due to this, the genetic nature of EDS and for the provision of existing medical management plans, it is prudent to suggest appropriate preconception medical review. Pregnant women with hEDS are more likely to experience premature labour, increased joint elasticity and dislocations, pain, varicose veins and increased rates of depression and anxiety. As such, this may prompt early referrals to physiotherapy, obstetric, mental health and GP services. Appropriate maternal positioning should also be led by the mother throughout. Furthermore, up to 78% of women with hEDS also experience Postural Tachycardia Syndrome (PoTS) or orthostatic intolerance, the symptoms of which may be exacerbated by the cardiovascular changes seen in pregnancy.

The literature retrieved demonstrated how those with hEDS are at higher risk of a variety of complications in childbearing when compared to the general population. As such, the management of hEDS in childbearing women can be complex. Some of the evidence retrieved suggested that the use of non-tension, non-dissolvable, deep double sutures for perineal repair, left in for at least 14 days, early anaesthetic reviews, a multidisciplinary approach and individualised care plans may improve the quality and safety of care received. Newborns with hEDS can also be given extra joint support to avoid unnecessary dislocations.

While it is beyond the scope of a midwife’s role to diagnose hEDS in childbearing women, the authors suggest that midwives could play a key role in raising awareness and making useful referrals where appropriate. In light of a lack of evidence in this area, the authors also suggest further work to develop robust guidelines for all health professionals caring for this unique subgroup of women. This work forms part of the EDS Clinical Toolkit developed in partnership between the RCGP and The Ehlers-Danlos Support UK, a project led by co-author Emma Reinhold.

Sally Pezaro is a midwife, researcher and lecturer at Coventry University.