Myotonic Dystrophy (DM)
Babies born with congenital-onset DM1 have the most complex medical challenges seen in DM. Although the prognosis for these children has improved, the disease still has profound consequences and can be life-threatening, especially in the early months.
The muscles needed for breathing are very weak in congenital-onset DM1, and a baby born with this disorder is likely to need a ventilator for an uncertain period of time. The breathing muscles do generally become stronger over time.
Cognitive impairment and even mental retardation are common in congenital-onset DM1, often requiring early intervention during the preschool years and special education later.
Weakness in the muscles needed for sucking may impair the baby's ability to feed. If the weakness is interfering with nutrition and hydration, a tube can be inserted either down the nose into the stomach (nasogastric tube) or directly into the stomach from outside (gastrostomy tube) until the muscles become strong enough for the child to eat and drink by mouth.
Babies with congenital-onset DM1 are born "floppy," with weak muscles and poor muscle tone. The medical term for this is hypotonia.
Motor milestones, such as sitting, standing and walking, are likely to be delayed, but ultimately are achieved by most children.
Muscle weakness in the feet may cause them to be fixed in a downward-pointing, inward-turning position. This condition is known as clubfoot or talipes equinovarus. This condition may have to be treated by casting or surgery before a child can walk.
To learn more about congenital DM1, read:
- Energy, Dedication, Hope Help Parents of Children with Congenital DM1;
- Great Expectations: Pregnancy and Childbirth with Neuromuscular Disease (see the section called Myotonic Dystrophy: A Special Case and the profile called Baby Born with Challenges);
- The Brain in DM (see the section called Special education in congenital DM1); and
- Building the IEP Puzzle: Tips for building effective IEPs and improving your child's educational experience.