Complications of Pregnancy in Myotonic Dystrophy Nicholas Johnson, MD Assistant Professor of...
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Transcript of Complications of Pregnancy in Myotonic Dystrophy Nicholas Johnson, MD Assistant Professor of...
Complications of Pregnancy in Myotonic Dystrophy
Nicholas Johnson, MD Assistant Professor of Neurology, Pediatrics,
PathologyUniversity 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 (DM1) or CCTG repeat (DM2)
• Prior reports of pregnancy complications include:– DM1:• abortions, polyhydramnios, ectopic pregnancy, placenta
previa, preterm labor
– DM2:• preterm labor
Methods
• Survey included demographics, obstetrical history, and Myotonic Dystrophy Health Index Short Form (MDHI-SF)
• Measured QOL prior, during, and after 1st pregnancy with:– Overall MDHI-SF score– Impact score (mean Likert x prevalence)
• Distributed to DM1 and DM2 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 DM1– CTG repeat length 330.5 (SD 222.5)
• 19.3% have DM2– CCTG repeat length 12414.3 (7299.0)
• 388 pregnancies
Assistive Reproductive Technology Use
Item 1st pregnancy 2nd 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 DM1 and DM2Complications 1st pregnancy 2nd 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)
1st Pregnancy Complications (DM1 and DM2 specific)
Complications Mothers with DM1 Mothers with DM2
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 Outcome 1st pregnancy 2nd and greater pregnancy
Child has myotonic dystrophy 47% yes, 28.2% unsure 30.9 yes, 38% unsure
Neonatal complications in Mothers with DM1
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
Prior Pregnancy
During Pregnancy
After Pregnancy
0
2
4
6
8
10
12
14
16
Ave
rage
MD
HI-S
F Sc
ore
Symptom Impact of Pregnancy in DM1 and DM2
0
0.2
0.4
0.6
0.8
1
1.2
1.4
1.6
1.8
2
6 mo prior
During
6 mo postTheme
Impa
ct S
core
*=p value <0.05
Symptom Impact of Pregnancy in DM1
0
0.2
0.4
0.6
0.8
1
1.2
1.4
1.6
1.8
2
6 mo priorDuring6 mo postTheme
Impa
ct S
core
*=p value <0.05
Symptom Impact of Pregnancy DM2
0
0.2
0.4
0.6
0.8
1
1.2
1.4
1.6
1.8
2
6 mo priorDuring6 mo postTheme
Impa
ct S
core
*=p value <0.05
Conclusions
• Increased rate of pregnancy complications in DM1 and DM2
• 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