The Flip Side of the Sandwich

I talk quite a bit about being a rare mom, and in many ways for the last 7+ years it has defined me. But the reality is that for the last 5 years I have also been a rare daughter. You read that right, in 2018 my father was officially diagnosed with frontotemporal dementia. FTD, as it is commonly called, is the most common cause of dementia in younger people, but is a rare disease. It has been in the news as of late as Bruce Willis got his official diagnosis just last month. As is often the case with rare diseases, when we look at my father, there were signs earlier, misdiagnoses and when the diagnosis finally came it affected the whole family, not just him. The average length of time between first symptoms and diagnosis is over 3 1/2 years.

Bruce WIllis' wife is again in the news this week asking paparazzi to refrain from yelling at him when out in public, asking how he is, telling him that he is in their thoughts and prayers, and yelling "Die Hard" after his famous movie franchise. It is because of this that I am stepping aside from my son's diagnosis story to talk about this today, to explain to those who may not understand why this is even more harmful to someone with FTD than others with other illnesses, even other forms of dementia or Alzheimers.

I recently became an ambassador with aFTD to begin sharing information with others about frontotemporal dementia and I will refer to their website for facts throughout this post - www.theaftd.org. I encourage anyone who would like more information to reach out to me or go directly to the site for more, as I do not claim to be an expert on this topic - again, just a daughter who cares and loves her daddy.

Like I mentioned above it is the most common form of dementia in younger ages, those under 60. Why is this an important fact? Besides the fact that it throws the life of the patient earlier than other forms of dementia, think about all the other factors it can impact. At 60 (or younger) most adults are still working to support their families, they still may have children at home or young adults that depend on them, they may just be planning their retirement plans, other health concerns may be starting to rear their heads. Also, because this often occurs at younger ages and is rare it makes the diagnostic journey more difficult. These adults are often misdiagnosed with mental health issues or written off completely prior to FTD being diagnosed. And what happens when it is diagnosed?

Like many other rare diseases there are no cures or treatments for FTD, though some medications may help some people with some symptoms sometimes. Frontotemporal dementia is characterized by deterioration of the frontal lobe of the brain, but can affect different parts of that lobe and different brain functions. Therefore there are subtypes of FTD, at first when Bruce Willis was in the news with a medical condition they described him as having aphasia - this falls under the language variant and makes communication more difficult - both verbal and written, both for them to do themselves and to understand . The most common subtype though is the behavior variant - and as the name suggests it changes this person's behavior first. The kind, loving, gentle person may become mean and aggressive, they may lose their social filter or societal boundaries in public, inappropriate comments or humor, compulsive habits, poor decision making and general problems with executive functioning are possible in this type. There are other types, but these are the most common - all are unpredictable and progress at unpredictable rates.

Knowing what I have shared with you now and going back to the Willis family and the latest request. I think about what it would be like for him, understanding that he has the language variant based on the aphasia that was first shared publicly. Language is most likely understood at a very concrete level, with memories being blurred or nonexistent depending on how deteriorated his frontal lobe is at this point of the disease. I then imagine the confusion he has being out in public with people just shouting out and cameras going off, chaos around him. This illness is one where people may do better with structure, routine and going out without chaos can be difficult on them and those with them, as it throws that off balance. So, now on top of that I think about him hearing those words "Die Hard" - and while he may (or may not) know that those were movies that made him famous it may be difficult to make that connection. If he can make that connection in a safe, secure, calm spot in the chaotic condition that is being described it may be more difficult. I ask again that you think of those words without the movie context which he may not have at that moment - and imagine people yelling those words at you. Imagine the confusion, the pain, the questions.

I share this today to help bring awareness to a brutally, awful illness affecting whole families that needs more attention. And while those in the community hope that Bruce Willis and family will do that right now they need to focus on him, on his (and their) well being. It is up to us in the community to explain the illness, to share what this means and to spread awareness beyond the headlines. I ask those who care about what Bruce Willis is going through to share this with others so that more people understand. Again for more information, please visit www.theaftd.org