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Help for my Family by Myotonic Dystrophy Expert Ann S Woodbury

My family (husband and 4 children – now adults) are all diagnosed with Myotonic Dystrophy (DM). DM affects as many or more systems in the body than any other disease. The effects that are usually the most compromising for the individual with DM are the fatigue and the GI system. Usually the severity of these effects relates to the age of onset of DM but not always. Meaning the age that symptoms first started. For instance, my third child Michael has severe GI issues and his age of onset is childhood. But, my second child Andrea also has severe GI issues and her age of onset wasn’t until around the age of 17. However, for the caregiver, me, the effects that are the most difficult are the way that DM affects the brain. Through observation and MRI it has been found that DM creates white matter in the frontal lobe. From my layman’s perspective here are some of the things that I have learned. We all have white matter and it is necessary to form the intertwining connections that join all four lobes of the brain. But, with DM it has been found that there is excessive white matter in the frontal lobe, the front part of the brain that controls our executive functioning. The executive function is the part of our brain that helps us act as “executives” in our lives. Think of what an executive does at a company (the person that puts plans and actions into effect and then manages the outcome). Some of my family’s experiences have been very frustrating and sometimes life threatening. Michael has been in speech therapy his entire life. Speech therapy also includes swallowing therapy. Michael has had several bouts of aspiration pneumonia because he wasn’t paying attention to what he was eating, even though he has had years of training to eat safely. This comes from his inability to monitor and prioritize multiple activities going on at the same time. Impairment of the frontal lobe can create an inability to monitor “important” activities versus less important activities. When Michael eats while other activities are going on he is more vulnerable to choking/aspirating. Even with his training and awareness he still gets caught off guard and does something foolish. We were walking to a football game once and there were vendors along our path. Michael received a chicken nugget from one of the vendors and proceeded to eat it while walking and he had no drink in his hand. He ended up choking, even starting to turn blue before a person walking by successfully gave him the Heimlich maneuver. An ambulance was called and he had to be checked out before we could proceed to the football game. It was very scary. Recently Kent, Warren (our oldest), and I went to California to see the Rose Bowl. Our team, the University of Utah Utes, were playing in the play-off. My husband Kent, has late-adult onset DM. So, he is the least affected of our family with DM. We were waiting in the hotel lobby for Warren. Kent had brought his large h