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 handful of pills with him to take when we had something to eat. He was tired of holding the pills so he threw the entire handful into his mouth and proceeded to dry swallow them. He usually takes his pills in this manner but he usually has something to drink with him. Thanks goodness nothing “dangerous” happened but he was struggling with all of those pills in his throat with no moisture to help push them down. I hurried into the nearby Starbucks and asked for a cup of water. I took it to him and he was able to swallow the pills down with the assistance of the fluid. I was startled at his frivolousness with these pills. It was very clear to me that his executive function was impaired. Kent functions on a high level and works full-time as a statistician. But, it’s interesting to see subtle effects of the DM in his judgement.

It still catches me off-guard when an outsider observes my family and is perplexed by the degree of involvement that my “adult” children have with me. It has become so “normal” and necessary that it is just routine for us. Michael and Chad have a bright yellow Smart car. We like that it is bright yellow because it gets noticed by the other cars around it. I think that it keeps them safer that way. They don’t drive far, only to the grocery store and to our house. Once in a while they need to go across a very busy street at an intersection that has lights. They take their dog to Petco that is across this busy street. They can manage it and they have managed this intersection in the past. But they still ask me to take them. It’s a difficult decision on my part because I want them to be as independent as possible but I also want them to be safe. More often than not I will take the time to take them.

This brings up a question that plays in the back of my mind most of the time. Am I empowering or enabling. Enabling is doing something for someone that they can do for themselves. Empowering is doing something for someone that helps them live as independent as possible for their situation. My decision to drive them borders on both empowering and enabling. It is true that they are capable of driving themselves across this busy street therefore it could be considered enabling. But, at the same time, if it keeps them safe so that they can continue to have their independence and the ability to drive themselves places I am empowering them by assisting in this activity.

Those of us who are caregivers for someone that has cognitive effects from their disease face this kind of decision many times in our day to day activities. I recently took a survey that was created by the myotonic foundation to help us determine if we need to get guardianship/power-of-attorney for our loved one. This helped me clarify the decisions that I have made to act on my family’s behalf. https://www.childneurologyfoundation.org/wp-content/uploads/2017/08/C_SelfCareAssessmentParents.pdf