Re: Pregnancies and deliveries in patients with Charcot-Marie-Tooth d...

[Guest guest]

They should have studied me. 5 pregnancies, 5 full term babies, no

surgery, no extra bleeding. CMT didn't get any worse actually got a bit better.

In a message dated 10/7/2010 9:41:44 A.M. Pacific Daylight Time,

-owner writes:

Acta Med Croatica. 2010 Jul;64(3):215-20.

Pregnancies and deliveries in patients with Charcot-Marie-Tooth disease

Herman M, Delmis J, Ivanisevi & #263; M, Zupi & #263; T.

University Department of Gynecology and Obstetrics, Zagreb University

Hospital Center, Zagreb, Croatia.

Abstract

Hereditary motor sensory neuropathy (HMSN), also known as

Charcot-Marie-Tooth (CMT) disease, is a spectrum of disorders caused by a

specific mutation

in one of several myelin genes, which results in defects in myelin

structure, maintenance and formation. Affected individuals show progressive

distal

limb atrophy and weakness, often with gait disturbance and deformity of

feet and hands.

There have been few studies on how CMT disease can affect pregnancy, birth

and the newborn. CMT is an independent risk factor for complications

during pregnancy and delivery. Patients with CMT have more operative

deliveries,

malpresentations and postpartum bleeding than the general obstetric

population. It is not clear whether the increased prevalence of malpresentation

is related to fetal disease, although the disorder typically does not

present until later in childhood.

Postpartum bleeding from atony may be related to the disease effect on

uterine adrenergic nerves. Exacerbation of CMT disease can occur in pregnancy,

an effect that may be mediated by increased plasma progesterone level.

Observations in an animal model were consistent with these findings as the

administration of progesterone resulted in a more progressive neuropathy,

while a progesterone antagonist slowed the disease progression.

We treated two patients with CMT (type 5 and type X1) at our Department.

Both of them had normal course of pregnancy until delivery. Emergency

cesarean section was performed in both cases; in one because of

malpresentation,

contracted pelvis and signs of impending fetal asphyxiation during the

second stage of delivery, and in the other one based on neurologist indication.

In the latter, uterine atony with profuse postpartum bleeding occurred

immediately after cesarean section and emergency hysterectomy was performed

according to clinical status.

[Guest guest]

I also had no issues with my pregnancy - normal delivery, correct position of

baby.

 

This is definitely a 'study-worthy' conversation.  I'm glad to see them writing

about it.  However, a research study with just 2 people isn't really a

research study.  It's just two separate case studies, which are not

necessarily representative of the CMT population.  It's great if it opens the

dialogue though and encourages full research studies.

 

From: MommyToJase@... <MommyToJase@...>

Subject: Re: Pregnancies and deliveries in patients with

Charcot-Marie-Tooth d...

Date: Thursday, October 7, 2010, 6:30 PM

 

They should have studied me. 5 pregnancies, 5 full term babies, no

surgery, no extra bleeding. CMT didn't get any worse actually got a bit better.

In a message dated 10/7/2010 9:41:44 A.M. Pacific Daylight Time,

-owner writes:

Acta Med Croatica. 2010 Jul;64(3):215-20.

Pregnancies and deliveries in patients with Charcot-Marie-Tooth disease

Herman M, Delmis J, Ivanisevi & #263; M, Zupi & #263; T.

University Department of Gynecology and Obstetrics, Zagreb University

Hospital Center, Zagreb, Croatia.

Abstract

Hereditary motor sensory neuropathy (HMSN), also known as

Charcot-Marie-Tooth (CMT) disease, is a spectrum of disorders caused by a

specific mutation

in one of several myelin genes, which results in defects in myelin

structure, maintenance and formation. Affected individuals show progressive

distal

limb atrophy and weakness, often with gait disturbance and deformity of

feet and hands.

There have been few studies on how CMT disease can affect pregnancy, birth

and the newborn. CMT is an independent risk factor for complications

during pregnancy and delivery. Patients with CMT have more operative deliveries,

malpresentations and postpartum bleeding than the general obstetric

population. It is not clear whether the increased prevalence of malpresentation

is related to fetal disease, although the disorder typically does not

present until later in childhood.

Postpartum bleeding from atony may be related to the disease effect on

uterine adrenergic nerves. Exacerbation of CMT disease can occur in pregnancy,

an effect that may be mediated by increased plasma progesterone level.

Observations in an animal model were consistent with these findings as the

administration of progesterone resulted in a more progressive neuropathy,

while a progesterone antagonist slowed the disease progression.

We treated two patients with CMT (type 5 and type X1) at our Department.

Both of them had normal course of pregnancy until delivery. Emergency

cesarean section was performed in both cases; in one because of malpresentation,

contracted pelvis and signs of impending fetal asphyxiation during the

second stage of delivery, and in the other one based on neurologist indication.

In the latter, uterine atony with profuse postpartum bleeding occurred

immediately after cesarean section and emergency hysterectomy was performed

according to clinical status.