Complications of Pregnancy in Myotonic Dystrophy Nicholas Johnson
- Slides: 14
Complications of Pregnancy in Myotonic Dystrophy Nicholas Johnson, MD Assistant Professor of Neurology, Pediatrics, Pathology University of Utah On behalf of: JD Franklin, E Nasser, E Luebbe, J Hilbert, KA Hagerman, JW Day, E Ciafaloni, M Hung, CR Heatwole
Background • Myotonic Dystrophies are multisystemic disorders due to a CTG repeat (DM 1) or CCTG repeat (DM 2) • Prior reports of pregnancy complications include: – DM 1: • abortions, polyhydramnios, ectopic pregnancy, placenta previa, preterm labor – DM 2: • preterm labor
Methods • Survey included demographics, obstetrical history, and Myotonic Dystrophy Health Index Short Form (MDHI-SF) • Measured QOL prior, during, and after 1 st pregnancy with: – Overall MDHI-SF score – Impact score (mean Likert x prevalence) • Distributed to DM 1 and DM 2 women between the ages of 18 -50 through the NIH Registry and MDF Patient Registry
Results: Demographics • • 151 women responded (39. 7%) Mean age 38. 7 (SD 6. 9) Disease duration 14. 4 years (9. 5) 70. 6% have DM 1 – CTG repeat length 330. 5 (SD 222. 5) • 19. 3% have DM 2 – CCTG repeat length 12414. 3 (7299. 0) • 388 pregnancies
Assistive Reproductive Technology Use Item 1 st pregnancy 2 nd pregnancy and greater Maternal Age 27. 6 (SD 5. 5) 30. 7 (SD 4. 6) Assistive Technology In-vitro fertilization 17. 2% 3. 9% Hormone therapy 10% 1. 4% Pre-implantation diagnosis 6. 6% 1. 9%
Pregnancy Complications in DM 1 and DM 2 Complications 1 st pregnancy 2 nd pregnancy and greater Pre-eclampsia (%) 11. 9 2. 0 Low birth weight (%) 16. 5 2. 3 Polyhydramnios (%) 19. 2 2. 8 Pre-term labor (%) 26. 5 2. 8 Miscarriage (%) 3. 9 2. 0 Stillbirth (%) 3. 3 0. 3 Peripartum hemorrhage (%) 13. 2 2. 2 Infection (%) 1. 3 . 8 None (%) 40. 4 10. 5 Mean gestational age 36. 4 (SD 6. 3) 37. 1 (SD 5. 1)
1 st Pregnancy Complications (DM 1 and DM 2 specific) Complications Mothers with DM 1 Mothers with DM 2 Pre-eclampsia (%) 9. 8 20. 0 Low birth weight (%) 18. 0 12. 0 Polyhydramnios (%) 23. 8 0 Pre-term labor (%) 29. 5 12. 0 Miscarriage (%) 2. 5 12. 0 Stillbirth (%) 2. 5 0 Peripartum hemorrhage (%) 16. 4 0 Infection (%) 1. 6 0 None (%) 35. 2 56. 0
Pregnancy Outcome 1 st pregnancy 2 nd and greater pregnancy Child has myotonic dystrophy 47% yes, 28. 2% unsure 30. 9 yes, 38% unsure Neonatal complications in Mothers with DM 1 Breathing problems 33. 1 5. 5 Needed ventilator 25. 4 5. 2 Feeding problems 33. 6 6. 0 Needed a feeding tube 36. 1 6. 0 Hypotonia 32. 8 7. 4 Clubfoot 14. 8 3. 0
Pregnancy does not affect overall DM quality of life • Change not significant • Subgroup analysis did not identify increased scores with DM type, age, number of pregnancies, ART, or presence of affected child 16 Average MDHI-SF Score 14 12 10 8 6 4 2 0 Prior Pregnancy During Pregnancy After Pregnancy
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yt Vi bu in al in Pa * ue tig es * s* m iti tiv Fa ac le n* tio la ob pr do m te st o ro in st un m Ga ilit ab ar am sio Iss u n ica pr es tio ob l Sle n d em De ep iffic s cr im ult e pa ies De ase E ir d m cr ot me ea sat n io isf se na t* ac d C pe t og l is su rfo ion n es rm in s itiv e o an ce cial issu es sit in u so cia atio n ls itu s at Ch Br io e ok M. . . a th in y ot g o in on g rs di ia w al fficu lo He win lties ar gi in g d ssu iff es icu lti es *=p value <0. 05 Co m In nd or ya nd Ha ilit ob M Impact Score Symptom Impact of Pregnancy DM 2 2 1. 8 1. 6 1. 4 1. 2 1 0. 8 0. 6 0. 4 0. 2 0 Theme 6 mo prior During 6 mo post
Conclusions • Increased rate of pregnancy complications in DM 1 and DM 2 • High rate of potential congenital DM outcomes in respondents • Specific symptoms may worsen with pregnancy
Thank You • Supported by Myotonic Dystrophy Foundation • Collaborators – University of Utah • Jeremy Franklin • Man Hung – University of Rochester • • Chad Heatwole Emma Ciafaloni Elizabeth Luebbe James Hilbert – Stanford University • Katherine Hagerman • John Day – Myotonic Dystrophy Foundation • Eriko Nasser
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