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 ID: 613679
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Slide1
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
HeatwoleSlide2
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 laborSlide3
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
I
mpact
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 Slide4
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 pregnanciesSlide5
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%Slide6
Pregnancy Complications in DM1 and DM2
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)Slide7
1
st 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
56.0Slide8
Pregnancy Outcome
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 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
3.0Slide9
Pregnancy d
oes not affect overall DM quality of lifeChange not significant
Subgroup analysis did not identify increased scores with DM type, age, number of pregnancies, ART, or presence of affected childSlide10
Symptom Impact of Pregnancy in DM1 and DM2 Slide11
Symptom I
mpact of Pregnancy in DM1 Slide12
Symptom Impact
of Pregnancy DM2 Slide13
Conclusions
Increased rate of pregnancy complications in DM1 and DM2High rate of potential congenital DM outcomes in respondentsSpecific symptoms may worsen with pregnancySlide14
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