Reproductive System

Reproductive System - DM1

Patterns:

  • The deleterious effects of DM1 on both smooth and striated muscle can complicate pregnancy, labor and delivery.

  • Added to these maternal complications is the possibility that the baby may have congenital-onset DM1, with severe neonatal complications, including respiratory and swallowing abnormalities.

  • Women with DM1 have a higher than average rate of spontaneous abortion and stillbirth, although most can expect to have a normal vaginal delivery.

Symptoms:

  • Mothers with DM1 are more likely than the general population to experience the following:

    • Ectopic pregnancy.

    • Premature delivery.

    • Prolonged labor and delivery.

    • Postpartum hemorrhage.

    • Uterine over distention with polyhydramnios which can lead to preterm labor, inadequate contractions during labor, premature rupture of the membranes or postpartum hemorrhage.

    • Untoward reactions to analgesia or anesthesia during labor and delivery.

    • Diminished ovarian reserve with delayed appearance of human chorionic gonadotropin (HCG) due to gonadal insufficiency.

  • Reproductive history and DM1-related personal and family history, including current DM1 symptoms.

  • Fatigue with more rapid onset than average during labor and increased risk of post-partum hemorrhage (PPH).

Diagnosis:

  • Discuss the following tests with your doctor:

    • Preimplantation genetic diagnosis to determine whether the embryo is affected.

    • Prenatal genetic diagnosis to determine if the fetus has the DM1 genetic expansion (see Genetic counseling).

Treatment:

  • Preimplantation genetic diagnosis can allow selective implantation of unaffected embryos.

  • Prenatal diagnosis by amniocentesis or chorionic villus sampling can allow for termination of an affected pregnancy.

  • High-risk obstetrician (Maternal-fetal medicine specialist) for prenatal care and delivery.

  • Analgesics or sedating anesthetic drugs should be used extremely.

  • Emergency medical alert devices.

  • Pediatric or neonatal specialist at delivery if the mother is affected with DM1.

  • Availability of neonatal intensive care, including possible tube feeding or ventilatory support, for neonates known, or suspected to have DM1.

  • Consulting obstetrician before a decision to induce labor is made.

  • Genetic counseling services and family planning services.

Reproductive System - DM2

Patterns:

  • Pregnancy can exacerbate DM2. A few reports indicate that pregnancy can exacerbate or unmask the onset of myotonia and cause increased pain in DM2. More research is needed to confirm these observations.

  • The effects of DM2 on both smooth and striated muscle can complicate pregnancy, labor and delivery.

Diagnosis:

  • Discuss the following tests with your doctor:

    • Preimplantation genetic diagnosis to determine whether the embryo is affected or prenatal genetic diagnosis to determine if the fetus has the DM2 genetic expansion.

    • Reproductive and fertility/infertility history inculding personal and family history.

Treatment:

  • Family planning.

  • Refer to genetic counseling, family planning and other specialists as indicated.

  • Prenatal and preimplantation genetic diagnosis can allow for termination of the pregnancy or selective implantation of unaffected embryos.

Reproductive System - CDM

Patterns:

  • Women with DM1 can have a complicated pregnancy, labor, and delivery, including premature delivery or prolonged labor that can have impact on the newborn.

  • The baby may be born with congenital-onset DM1, with severe neonatal complications, including respiratory and swallowing abnormalities.

  • Knowing if a pre-neonate has a risk of CDM also enables the parents and the obstetric team to prepare for the birth of a DM1-affected baby.

Symptoms:

  • Babies with congenital-onset DM1 may have the following prenatal and neonatal signs of varying severity:

    • Swallowing difficulties, leading to polyhydramnios during pregnancy and poor feeding at birth, possibly requiring tube feeding.

    • Respiratory muscle abnormalities, possibly requiring mechanical ventilation at birth.

    • Poor muscle tone and lack of fetal movement.

    • Abnormal presentation.

    • Club foot or other joint problems.

    • Abnormal accumulation of fluid in the body.

    • Enlarged ventricles in the brain.

    • Reduced fetal movement.

Diagnosis:

  • Discuss the following tests with your doctor:

    • Preimplantation genetic diagnosis to determine whether the embryo is affected.

    • Prenatal genetic diagnosis to determine if the fetus has the DM1 genetic expansion (see Genetic counseling).

Treatment:

  • High-risk obstetrician or neonatal specialist at delivery if the mother is affected.

  • Availability of neonatal intensive care, including possible tube feeding or ventilatory support.

  • Accces to neonatal and consulting specialists who can manage genetic, respiratory, GI, orthopedic, neuromuscular, neurosurgical, and cardiac issues.

  • Refer to pediatric neuromuscular multidisciplinary clinic on discharge from the neonatal unit to manage the many issues that children with CDM will encounter.

Home / What Dm / How DM Affects Your Body / Reproductive System

© Myotonic. All rights reserved.