Non-Vascular Ehlers- Danlos Syndrome and Pregnancy: What are the Risks?

1. Non-Vascular Ehlers-Danlos Syndrome and Pregnancy: What are the Risks? EDNF Learning Conference August 10-11, 2012

2. NV-EDS & Pregnancy • Case reports of OB complications : • Abnormal fetal presentation at delivery • Incompetent cervix • Joint dislocation during delivery • Standing erect becoming increasingly difficult • Uneventful

3. Question • What is the obstetrical experience of women with non-vascular Ehlers-Danlos syndrome? • Aim #1: Identify the obstetrical complications women with non-vascular EDS experience • Aim #2: Compare the observed rate of obstetrical complications in women with non-vascular EDS to the • General population • Vascular EDS population

4. METHODS

5. Recruitment • Participants recruited through the EDNF • Monthly electronic newsletter • EDNF homepage • EDNF Facebook page • Study packet distributed at annual national education meeting • July 21-23, 2011 • 2011 meeting had >500 members in attendance • Inclusion criteria: • Woman with a diagnosis of NV-EDS • Had at least one pregnancy • At least 18 years of age

6. Questionnaire • 22 questions - 6 sections: • Demographics • General pregnancy questions • Prenatal care • Pregnancy • Maternal health during pregnancy • Labor and delivery • All sections other than demographic could be answered one time per pregnancy, for up to four pregnancies • Open comment boxes provided throughout

7. Data Analysis • Descriptive statistics for demographics and complication rates • Means, frequencies, percentages • One-tailed binomial test • To compare observed complication rate to published rates when available • Open-ended responses were categorized and tabulated

8. RESULTS

9. Responses • Response method: • Received by mail: 34 • Received online: 484 • Total # received: 517 • Excluded responses • Type of EDS: 40 • Vascular: 1 • Don’t know: 34 • Skipped question: 5 • Never pregnant: 6 • Incomplete questionnaires: 34

10. Included Population • # of surveys: 437 (84.5%) • # of first trimester miscarriages: 61 • Included second trimester miscarriages and stillbirths in analysis of complications • Final # of participants included in obstetrical complication analysis: 376

11. Demographic Information *Two participants did not report their year of birth

12. Pregnancy Information

13. 1st Pregnancy Outcomes a Buss et al., 2006 b MacDorman & Kirmeyer, 2009c Kochanek et al., 2012

14. Timing of Miscarriage a Cunningham et al., 2010, chp 9 $ Five people did not report when miscarriage occurred % One person did not report when miscarriage occurred & Four people did not report when miscarriage occurred

15. Pregnancy Complications: NV-EDS *Rate is significantly higher than the general population a Martin et al., 2006 b Beilin et al., 1998 c Snow & Neubert, 1997 d ACOG Practice Bulletin, Number 76, 2006 e Goldenberg et al., 2008

16. Pregnancy Complications: Classic EDS *Rate is significantly higher than the general population a Martin et al., 2006 b Beilin et al., 1998 c Snow & Neubert, 1997 d ACOG Practice Bulletin, Number 76, 2006 e Goldenberg et al., 2008

17. Complications: Hypermobile EDS *Rate is significantly higher than the general population a Martin et al., 2006 b Beilin et al., 1998 c Snow & Neubert, 1997 d ACOG Practice Bulletin, Number 76, 2006 e Goldenberg et al., 2008

18. Pregnancy Complications * Rate is significantly higher than the general population a Martin et al., 2006 b Beilin et al., 1998 c Snow & Neubert, 1997 d ACOG Practice Bulletin, Number 76, 2006 e Goldenberg et al., 2008

19. Accounting for Fetus’ EDS Status *Rate is significantly higher than the general population a Martin et al., 2006 b Kochanek et al., 2009 c Goldenberg et al., 2008

20. Accounting for Fetus’ EDS Status *Rate is significantly higher than the general population a Martin et al., 2006 b Kochanek et al., 2009 c Goldenberg et al., 2008

21. NV-EDS vs. Vascular EDS *Rate is significantly lower than the vascular EDS population a Pepin et al., 2000 b Yen et al., 2006

22. Other OB Complications

23. Additional Complications Provided

24. Study Limitations • Self-report • General population rates from published literature • No control group collected • Ascertainment bias • Vocabulary of the survey • Premature rupture of membranes • Hemorrhaging versus excessive bleeding

25. Directions for Future Research • Replication of findings • Control group • Confirmation of diagnosis & complications via medical records • Examine additional complications mentioned by participants • Placenta • Amniotic fluid levels • Maternal blood pressure • Correlation studies: • Genotype-phenotype correlations • Complications in previous pregnancy predict future complications • Research on start and duration of complications

26. Conclusions • Results suggest the pregnancy outcomes for women with non-vascular EDS do not differ from those of the general population: • Miscarriage • Still birth • Premature delivery

27. Conclusions • Results suggest women with non-vascular EDS may be at a higher risk than the general population to experience the following obstetrical complications: • Fetal malpresentation, regardless of fetus’ EDS status • PROM, regardless of fetus’ EDS status • Premature delivery, if the fetus is also affected • Incomplete epidural efficacy • Joint dislocation • Uterine hemorrhaging/heavy bleeding

28. Conclusions • Results suggest women with non-vascular EDS may have a lower risk than the vascular EDS population for: • premature delivery, if hypermobile EDS • a during-delivery or post-partum arterial rupture

29. Acknowledgements • The Ehlers-Danlos National Foundation • Participants • Statistical Sciences Core, Center for Clinical Investigation, Case Western Reserve University This work has been supported by the Jane Engelberg Memorial Fellowship Student Research Award to Krista Sondergaard, provided by the Engelberg Foundation to the National Society of Genetic Counselors, Inc.