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Classical Ehlers–Danlos syndrome with joint instability, postural orthostatic tachycardia syndrome and heavy m

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HardEhlers-Danlos SyndromeClassical Ehlers–Danlos syndrome with joint instability, postural orthostatic tachycardia syndrome and heavy menstrual bleedingDFSRH

A 32-year-old nulliparous woman with genetically confirmed classical Ehlers–Danlos syndrome requests highly effective reversible contraception. She reports heavy, painful menstruation and wants a method likely to reduce bleeding. Her haemoglobin is 98 g/L, and pelvic ultrasonography shows no fibroids or uterine-cavity distortion. She also has postural orthostatic tachycardia syndrome. Six weeks ago, she lost consciousness while standing after cervical screening; no arrhythmia or structural cardiac disease was identified, but her cardiologist has not considered the risks of intrauterine contraception. During the cervical screening examination, forced hip abduction caused a sacroiliac-joint subluxation. She reports that a semi-recumbent, minimally abducted position is usually tolerable. Pregnancy has been reasonably excluded, and assessment identifies no genital infection or other contraindication to intrauterine contraception. Her EDS team has not yet advised on the suitability or setting of insertion. She asks whether a lower-dose or non-hormonal device would be safer because of her connective-tissue disorder. Which management plan is most appropriate?

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Correct answer: D — Plan 52 mg LNG-IUD insertion after EDS and cardiology advice on suitability and setting, using hydration precautions and patient-directed positioning.

A 52 mg LNG-IUD best integrates her need for highly effective contraception with treatment of heavy menstrual bleeding and dysmenorrhoea. Lower-dose LNG-IUDs are effective contraceptives, but the evidence supporting treatment of heavy menstrual bleeding relates to 52 mg devices. A copper IUD may increase menstrual blood loss and is poorly aligned with her symptomatic iron-deficiency anaemia. EDS is not an automatic contraindication to intrauterine contraception. However, its phenotypic heterogeneity, possible tissue fragility and joint hyperlaxity mean that FSRH recommends discussing suitability and the insertion setting with the patient’s EDS specialist. Her previous joint subluxation also makes patient-directed, minimally abducted positioning essential. Most insertions in people with PoTS are low risk when hydration, salt intake and postural awareness are addressed. Her recent postural syncope is the important exception: cardiology advice is required because hospital insertion may be recommended. Therefore C is premature despite incorporating some precautions. A addresses procedural planning but selects a method likely to aggravate bleeding. B incorrectly assumes that a lower levonorgestrel dose is safer in EDS and sacrifices the established heavy-menstrual-bleeding indication. E overstates the risk: an implant remains an alternative if she prefers it or specialist assessment finds intrauterine contraception unsuitable, but neither EDS nor PoTS-related syncope categorically prohibits intrauterine contraception.

Reference: FSRH Clinical Guideline: Intrauterine Contraception — section 7.1.13, Ehlers–Danlos syndrome (Published March 2023; amended 21 January 2025) — https://www.fsrh.org/Common/Uploaded%20files/documents/fsrh-clinical-guideline-intrauterine-contraception-mar-23-amended.pdf FSRH Clinical Guideline: Intrauterine Contraception — section 7.1.14.3, postural orthostatic tachycardia syndrome (Published March 2023; amended 21 January 2025) — https://www.fsrh.org/Common/Uploaded%20files/documents/fsrh-clinical-guideline-intrauterine-contraception-mar-23-amended.pdf FSRH Clinical Guideline: Intrauterine Contraception — sections 5.5.2 and 9.1 (Published March 2023; amended 21 January 2025) — https://www.fsrh.org/Common/Uploaded%20files/documents/fsrh-clinical-guideline-intrauterine-contraception-mar-23-amended.pdf