The Pelvic Disorders Working Group of the International Consortium on Ehlers-Danlos Syndromes (EDS) and Hypermobility Spectrum Disorders (HSD), has collaborated on a comprehensive publication of guidelines tailored for healthcare professionals, and individuals with hypermobile Ehlers-Danlos syndrome (hEDS) and HSD who are either pregnant or planning to become pregnant.
The guidelines are based on an extensive review of existing studies and individual case reports from various countries, considering factors such as risk assessment, symptom management, and outcomes during pregnancy, childbirth, and the postpartum period. The guidelines are divided into four main sections:
- Preconceptual: This section covers planning for pregnancy, including screening and preparing for potential challenges specific to individuals with hEDS/HSD.
- Antenatal: Focuses on managing the pregnancy itself, addressing risks, miscarriage, gastrointestinal issues, mobility concerns, and overall well-being of both the childbearing person and baby.
- Intrapartum: Deals with the process of childbirth, offering guidance on risk assessment, birthing options, pain and mobility management during labor, and anesthesia choices.
- Postpartum: Provides advice on wound healing, pelvic health, newborn care, infant feeding challenges, pain management, mental health, and nutrition.
Key Points from the Guidelines:
Pregnancy-Related Complaints:
- During pregnancy, complaints such as hypermobility, pelvic issues, and joint pain may worsen. These symptoms can take longer than average to normalize postpartum.
Wound Healing and Tissue Scarring:
- People with hEDS tend to experience poorer wound healing and complex tissue scarring, such as atrophic scars or keloids.
- Recommendations for wound treatment:
- Use durable, absorbable sutures and additional glue for fragile skin.
- Close all wounds without tension, using interrupted sutures.
Pregnancy Risks:
- No known increased risk of uterine rupture/torsion, preeclampsia, and stillbirth in hEDS.
- No increased risk of clinically significant mitral valve prolapse or aortic root dilatation in pregnant individuals with hEDS.
- Conflicting evidence on the risk of preterm labor and cervical insufficiency. No evidence supports the use of prophylactic cerclage.
Anesthesia Considerations:
- People with hEDS are less sensitive to local anesthesia; a higher dose may be needed. Allow ample time for the drug to take effect.
Optimal Positioning During Labor:
- Careful positioning of the head, neck, shoulders, back, hips, knees, and ankles is crucial due to increased risk of pain and dislocation.
- Use positioning aids (e.g., wedges, exercise balls, pillows, bed adjustments) as needed.
Infant Feeding Support:
- Additional education, logistical, and physical support may be required to avoid unnecessary pressure on joints and muscles.
Overview of the Comprehensive Guidelines:
The guidelines provide detailed information for different stages of pregnancy and postpartum care, including:
Preparation for Pregnancy:
- Discusses screening processes and considerations specific to individuals with hEDS/HSD who are thinking about starting a family.
Pregnancy Management:
- Focuses on the journey through pregnancy, including assessing risks, managing potential complications like miscarriage or termination of pregnancy, addressing gastrointestinal issues and mobility challenges, and ensuring the overall health and well-being of both the childbearing person and the developing baby.
Childbirth:
- Offers guidance on navigating labor and delivery, including risk assessment, making informed decisions about birthing options, managing pain and mobility during labor, and considering anesthesia choices.
Postpartum Care:
- Provides advice on wound healing, pelvic health, newborn care, and challenges related to breastfeeding and infant feeding. It also discusses strategies for managing pain, supporting mental health, and maintaining proper nutrition during the postpartum period.
The guidelines also address common comorbidities associated with hEDS/HSD, such as dysautonomia and mast cell diseases, providing strategies for managing these conditions alongside pregnancy and childbirth. These guidelines aim to empower healthcare professionals and individuals with hEDS/HSD by offering evidence-based recommendations and practical insights for navigating the unique challenges of pregnancy and childbirth within the context of these connective tissue disorders.
This paper is open access thanks to The Ehlers-Danlos Society. To review it in the PLOS ONE journal please follow this link.
