What is the best way to skip menstruation for individuals with Ehlers-Danlos syndrome (EDS) who are sensitive to progesterone?

Best Methods to Skip Menstruation for Individuals with Ehlers-Danlos Syndrome Sensitive to Progesterone

For individuals with Ehlers-Danlos syndrome who are sensitive to progesterone, the copper intrauterine device (Cu-IUD) is the most appropriate option for menstrual management and skipping periods. 1

Understanding the Challenge

Individuals with Ehlers-Danlos syndrome (EDS) face unique challenges regarding menstrual management:

• Heavy menstrual bleeding is common in EDS patients 2, 3
• Progesterone sensitivity limits hormonal options
• Connective tissue abnormalities may increase risks with certain interventions
• Quality of life is significantly affected by menstrual symptoms

Recommended Options (In Order of Preference)

1. Copper IUD (First-Line)

• Mechanism: Non-hormonal, does not contain progesterone
• Benefits:
  • Well-tolerated in 83.3% of patients with hereditary angioedema (similar connective tissue disorder) 1
  • Can be used for emergency contraception and long-term management 4
  • No systemic hormonal effects
  • Long-lasting (up to 10 years)
• Considerations:
  • May initially increase menstrual flow before stabilizing
  • Insertion should be done with acute treatment available (though generally well-tolerated) 1

2. Extended-Cycle Combined Hormonal Contraceptives (Second-Line)

• Mechanism: Skip the hormone-free interval to prevent withdrawal bleeding
• Application: Take active pills continuously for 84+ days
• Benefits:
  • Can skip pills for 3-4 consecutive days to induce controlled bleeding when needed 1
  • Provides predictable bleeding pattern
• Caution:
  • Contains estrogen, which may be contraindicated in some EDS subtypes (particularly vascular EDS)
  • Monitor for thrombotic risk

3. Barrier Methods + Scheduled NSAIDs

• Approach: Use barrier contraception methods with scheduled NSAIDs during menses
• Benefits:
  • No hormonal component
  • Barrier methods well-tolerated in connective tissue disorders 1
  • NSAIDs can reduce menstrual flow by 20-40%
• Limitations:
  • Less effective for completely skipping periods
  • Only reduces rather than eliminates flow

Special Considerations for EDS Subtypes

For Vascular EDS (Type IV)

• Highest Risk Subtype:
  • Traditional recommendation was to avoid all IUDs due to theoretical risk of uterine rupture
  • Recent evidence suggests LNG-IUD may be safe under ultrasound guidance 5
  • Recommendation: Copper IUD placement under ultrasound guidance in controlled setting

For Hypermobile EDS

• Higher prevalence of heavy menstrual bleeding 3
• Copper IUD may initially worsen bleeding but typically stabilizes
• Consider adding tranexamic acid during heavy bleeding episodes if not contraindicated

Important Precautions

1. Avoid estrogen-containing methods in vascular EDS due to increased thrombotic risk
2. IUD insertion considerations:
   • Have acute treatment available during insertion 1
   • Consider ultrasound guidance
   • Higher expulsion rate (9.1%) reported in first 21 days for patients with bleeding disorders and EDS 2
3. Avoid cyproterone acetate as it has been reported to worsen symptoms in similar connective tissue disorders 1

Follow-up and Monitoring

• Assess for anemia with baseline complete blood count
• Monitor for signs of tissue fragility at insertion sites
• Regular follow-up at 3 months after initiation of any method
• Consider pictorial blood assessment chart to quantify bleeding 3

The copper IUD provides the most effective option for individuals with EDS who are sensitive to progesterone, offering long-term management without hormonal side effects while respecting the unique connective tissue concerns in this population.