non-vascular ehlers- danlos syndrome and pregnancy: what are the risks?

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Non-Vascular Ehlers- Danlos Syndrome and Pregnancy: What are the Risks?. EDNF Learning Conference August 10-11, 2012. NV-EDS & Pregnancy. Case reports of OB complications : Abnormal fetal presentation at delivery Incompetent cervix Joint dislocation during delivery - PowerPoint PPT Presentation

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  • Non-Vascular Ehlers-Danlos Syndrome and Pregnancy: What are the Risks?EDNF Learning ConferenceAugust 10-11, 2012

  • NV-EDS & PregnancyCase reports of OB complications :

    Abnormal fetal presentation at delivery

    Incompetent cervix

    Joint dislocation during delivery

    Standing erect becoming increasingly difficult

    Uneventful

  • 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 theGeneral populationVascular EDS population

  • METHODS

  • RecruitmentParticipants recruited through the EDNFMonthly electronic newsletterEDNF homepageEDNF Facebook pageStudy packet distributed at annual national education meetingJuly 21-23, 20112011 meeting had >500 members in attendance

    Inclusion criteria:Woman with a diagnosis of NV-EDSHad at least one pregnancyAt least 18 years of age

  • Questionnaire22 questions - 6 sections:DemographicsGeneral pregnancy questionsPrenatal carePregnancyMaternal health during pregnancyLabor and delivery

    All sections other than demographic could be answered one time per pregnancy, for up to four pregnancies

    Open comment boxes provided throughout

  • Data AnalysisDescriptive statistics for demographics and complication ratesMeans, frequencies, percentages

    One-tailed binomial testTo compare observed complication rate to published rates when available

    Open-ended responses were categorized and tabulated

  • RESULTS

  • ResponsesResponse method:Received by mail: 34Received online: 484Total # received: 517

    Excluded responsesType of EDS: 40Vascular: 1Dont know: 34Skipped question: 5Never pregnant: 6Incomplete questionnaires: 34

  • Included Population# of surveys: 437 (84.5%)

    # of first trimester miscarriages: 61Included second trimester miscarriages and stillbirths in analysis of complications

    Final # of participants included in obstetrical complication analysis: 376

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

    Frequency (n)Percent (%)Age(n=435)*18-1910.220-295813.130-3918642.940-4912328.250-595412.6Over 60133.0EDS subtype(n=437)Classic10223.3Hypermobility33175.7Kyphoscoliosis30.7Arthrochalasia10.2Dermatosparaxis00Had Genetic Testing for EDS(n=113)DNA analysis87.3Protein analysis3834.9Dont know6862.4Answer missing43.7

  • Pregnancy Information

    Frequency (n)Total # of PregnanciesPopulation as a whole (n=437)1061Classic EDS (n=102)258Hypermobile EDS (n=331)796Kyphoscoliosis EDS (n=3)6Arthrochalasia EDS (n=1)1

    Average # of Pregnancies per WomanPopulation as a whole (n=437)2.4Classic EDS (n=102)2.5Hypermobile EDS (n=331)2.4Kyphoscoliosis EDS (n=6)2Arthrochalasia EDS (n=1)1

  • 1st Pregnancy Outcomesa Buss et al., 2006 b MacDorman & Kirmeyer, 2009c Kochanek et al., 2012

    EDS SubtypeOutcomeFrequency (n)Percent (%)General Pop Rate (%)P-valueNon-vascular (n=437)Miscarriage(

  • Timing of Miscarriagea 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

    Type of EDSTime of MiscarriageFrequency (n)Percent (%)Gen Pop Rate (%)P-valueNon-vascular$(n=432)First trimester (

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

    ComplicationFrequency (n=376)Percent (%)General Population Rate (%)P-valueAbnormal fetal delivery position* 55/34615.95.4a

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

    ComplicationFrequency (n=93)Percent (%)General Population Rate (%)P-valueAbnormal fetal delivery position* 13/81165.4a

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

    ComplicationFrequency (n=279)Percent (%)General Population Rate (%)P-valueAbnormal fetal delivery position* 41/26115.75.4a

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

    Non-vascular EDSTotal N=376Classic EDSTotal N=93Hypermobile EDSTotal N=279ComplicationGen Pop Rate (%)Frequency (n)Percent (%)P-valueFrequency (n)Percent (%)P-valueFrequency (n)Percent (%)P-valueAbnormal fetal delivery position5.4a55/34615.9

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

    Fetus has EDS?Frequency (n)Percent (%)General Population Rate (%)P-valueAbnormal delivery positionYes*(n=161)2918.05.4a

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

    Fetus has EDS?Frequency (n)Percent (%)General Population Risk (%)P-valueAbnormal delivery positionYes*(n=161)2918.05.4a

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

    Type of EDSComplicationFrequency (n)Percent (%)Vascular EDS Pop Rate (%)P-valueNon-vascularArterial rupture at delivery or post-partum*11/3353.38.6 a

  • Other OB Complications

    Non-Vascular EDS(Total N=376)ComplicationFrequency (n)Percent (%)Increase in bone and/or joint pain263/34675.6Difficulty standing >5-10 min210/34560.9Ankle instability 183/34752.7Skin tingling, prickling, numbness 127/33637.8Teeth fragility 118/34534.2Heavy vaginal bleeding139/35439.3Amniotic sac complications, not specified 48/34414.0Excessive bleeding/Hemorrhage (other than uterus) 38/33811.2Blood vessel rupture at any time during pregnancy18/3475.2Cervical cerclage attached3/3460.87Bowel perforation2/3410.58

  • Additional Complications Provided

    Complication (if n>5)Frequency (n)ExamplesMaternal hypertension and pre-eclampsia 40Placental problems28PreviaAbruptionPelvic complications26SymphysisInstabilityCardiac issues and fainting23POTSChange in heartrateSwelling and edema16Oligohydramnios18Gastrointestinal manifestations14GERDDysmotilityHyperemesis gravidum13Emergency c-section12Stalled labor9Gestational diabetes7

  • Study LimitationsSelf-report

    General population rates from published literature No control group collected

    Ascertainment bias

    Vocabulary of the surveyPremature rupture of membranesHemorrhaging versus excessive bleeding

  • Directions for Future Research

  • ConclusionsResults suggest the pregnancy outcomes for women with non-vascular EDS do not differ from those of the general population:MiscarriageStill birthPremature delivery

  • ConclusionsResults 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 statusPROM, regardless of fetus EDS statusPremature delivery, if the fetus is also affectedIncomplete epidural efficacyJoint dislocationUterine hemorrhaging/heavy bleeding

  • 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

  • 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.

  • Tell why we only analyzed first pregnancy: Confounding factor of multiple pregs in one individual**One-tailed binomial DECREASED rates in NV-EDS population

    Arterial ruptures: COL3 vs other types of collagen in arterial wallsPremature delivery: approached significance in classic EDS populationPROM: abnormalities in collagen predispose to PROM, doesnt matter which type of collagen?

    Ruptures resulted in death in the vascular population*Exacerbations of normal EDS symptoms*