“Silence is suffocating. Her story deserves to be told. And if I don’t tell her story, then what is my story gonna look like?”

– Linde

Caregivers of those living with Frontotemporal Dementia will tell you again and again that the healthcare system has failed them. And it has. 

Frontotemporal Dementia (FTD) or Frontotemporal Lobar Degeneration (FTLD) is a progressive neurological disease that falls under the umbrella term “dementia.” The word dementia itself refers to symptoms caused by different diseases that impact the brain and behavior and significantly impact a person’s participation in their daily life. Dementia is not synonymous with memory loss or old age. The disease most commonly associated with dementia is Alzheimer’s Disease, but it is not the only one. FTD is another type of dementia. And the presentation is not consistent with what society expects from someone living with dementia. Those living with FTD are often under the age of 65 and may have little to no memory impairments, especially early on in the course of the disease. Often, healthcare professionals and families don’t recognize the symptoms. Many haven’t even heard of FTD. This leads to significant hardships, including delays in diagnosis and care, financial loss, loss of social support, and more. 

But even for a family familiar with the disease, getting help can be nearly impossible. 

Allison grew up in White Bear Lake, Minnesota, one of four children. She attended a small liberal arts college in Minnesota, where she graduated with her Bachelor’s in health sciences and then eventually went on to get a Master’s in physical therapy. She worked primarily for a physician in a family practice and ran the Physical Therapy department there. She loved her job. She was proud of the fact that she had her Master’s degree, and she always wanted to help others. No matter what the emergency was, she stepped in, whether that meant assisting a neighbor after a knee surgery or creating a gumball costume at the last-minute for her daughter. Allison’s greatest joy in life was being a mother to her three daughters.

It was Allison’s middle daughter, Linde, who spoke with me and detailed the story of her family’s experience with FTD.

But it didn’t start with Allison.

After Linde’s younger sister was born, Allison’s mother (Linde’s grandmother) began displaying symptoms of what was then generally known as Pick’s Disease. Pick’s Disease is another name for Frontotemporal Dementia. Allison became her mother’s power of attorney and was her caregiver until she passed away in 2006. An autopsy confirmed Frontotemporal Dementia. 

“One thing about my mom with my grandmother is that she was the most aggressive caregiver, advocate, would not stand for anything less for my grandma,” Linde shared. “My grandmother at the end of her disease became very contracted. My mom cut all of her clothing up the back and put velcro on it so that from the front, it still looked like a nice shirt or blouse, but then they could get it comfortably onto my grandmother without it causing her pain or discomfort.” 

Linde recalls her mother’s commitment to care for her grandma was deeply representative of her personality. “Everything was always just done 110%. If she was gonna commit, she was gonna give it every ample amount of her energy.”

Linde graduated from college in 2010 and around that time, Allison began experiencing changes in her language and her sleep. “Nothing really noticeable to us simply because we weren’t trying to diagnose anything because there were no behavioral changes.” Then behavioral changes began and, by 2012, “it was becoming very apparent to myself, my siblings, and some of her closer friends that there was just something not right, that something was just kind of off. She was making really outlandish comments. [She] would show up randomly at people’s houses, not seeing anything wrong with that. [She] had no idea of what a boundary was or what was appropriate.” 

Linde’s mother and father were divorced, and Allison became very focused on her ex-husband. “It was just very odd, but I didn’t know necessarily that it was FTD at that time. I just knew there was something wrong and that we had this family history because my grandmother had the disease. And my mom was 50 at the time, so she was not really fitting the mold for what I assumed would be that age for dementia.” 

Despite this, Linde proactively contacted her mother’s longtime doctor and let the nurse at his office know that there were changes occurring in her mother’s behavior. She told them, “I’m concerned, family members are concerned, and friends are concerned.” But the office did not think there was a reason for concern and the doctor never followed up.

“In 2014, we had all these people emailing us, texting us like, ‘There’s something not right with your mom,’ like, ‘What’s going on? We think it might be Alzheimer’s. You girls need to do something,’ which is easier said than done because my mom was a vehemently independent person. She did all sorts of research on preventing dementia. She had probably fifteen different books on [it]. She was doing all these things to prevent it.” But Allison didn’t realize she was symptomatic. She was simply doing these things because her own mother had dementia and she wanted to prevent it. 

Anosognosia is a term that refers to lack of recognition of one’s own condition. It is the result of changes to the brain and it is often the reason why a person living with dementia might say something like, “There isn’t anything wrong with me.” They might blame others for items they’ve misplaced or get frustrated with family members when appointments seem to come up at the last minute, even if they were actually booked months in advance. Often, family and friends think the person living with a form of dementia is being stubborn, but they physically cannot recognize what is happening. Their sense of reality shifts to accommodate the events that don’t make sense to them.

 In FTD, anosognosia can prevent the person living with the disease from understanding what is happening. So it is up to families to not only support their loved ones when they may be quite resistant to it, but to advocate for them in scenarios ranging from going shopping to attending doctor’s appointments. 

“We always just knew there was something wrong, but we never could explain it fully to people. I was 22, starting my career as a nurse. My sister had two children by this point. My younger sister was just starting college.”

Amidst Allison’s progressive behavioral changes, life had to continue. Linde got married. Her mom arranged the flowers for her wedding. “She did a great job at that. But she was really inappropriate at my wedding showers and my baby showers, and … would make really odd comments. And it was just kind of uncomfortable. Like those events that you want your mom at, I didn’t want her at.”

In 2015, Linde and her sisters noticed that Allison seemed to be “getting worse. Her judgment was just really off … At this point, I was pregnant with my first daughter … We kind of just got to this point where we said, ‘Hey mom, you know, you can’t babysit the grandkids anymore until you go see a neurologist. And just prove us wrong. Have them tell us that there’s absolutely nothing wrong and then we can move on with life.’”

Unfortunately, this did not have the desired outcome. 

“She went to a neurologist, she had an MRI, she did the neurocognitive testing. And then [we] had a family sit down meeting, where we could kind of voice our concerns.” The family let the doctor know that they suspected FTD because that was what their grandma had.

“The neurologist said, ‘No, it’s not.’ She said, ‘It is adjustment mood disorder and OCD … and clinical depression. So [she] put her on depression medication. And she said she was just failing to adjust to life after divorce, and she was just hyper-fixated on that.” 

With two different medical professionals informing the family that this was not FTD, Allison’s young family was left thinking, “What more can we do?”

Linde and her sisters desperately wanted to help their mom. “[Allison’s] advocacy for my grandmother [when she was living with FTD] was one reason why I felt so [strongly] about caring for her because that is how she deserved to be cared for.” 

But each time they reached out for help, they were met by a system that was not built for them and healthcare professionals who did not understand the situation. “We kind of got failed by the system.” No doctor would diagnose Allison with dementia, and due to her lack of awareness of her own deficits, she did not understand why she needed her daughters’ help. When Linde and her sisters tried to provide assistance, they were met with resistance. When they sought help, they were told their concerns about their mother were unfounded.

At this time, Allison had not held a stable job in over three years. “I think from 2012 to 2017, she had 18 different jobs. She couldn’t hold a stable job. She could get an interview. She could do well in the interview because it just was such … parroted conversation. But when it came to learning new tasks, she couldn’t do it, and she was just so rigid and resistant to learning anything new, so she would just argue and not see that there was anything wrong with that.”

In November 2018, just before Thanksgiving, Linde reached out to her sisters to ask if anyone had heard from their mom. She hadn’t shown up to Linde’s house, hadn’t called or texted in a while, and this was not like her. This was, as it turned out, because Allison was in jail due to driving away from a police officer who was trying to pull her over for running a stop sign. 

“I was like, ‘How do you get ahold of somebody at jail? Like I don’t know how to do this. I’ve never been in jail. I’ve never had to get somebody from jail. What is happening? My mom’s not– This is not her.’ Nobody called us. Nobody contacted us.”

Linde received a letter from Allison, casually informing her daughter that she was in jail. “I mean, it’s the weirdest letter to receive.” And she also received a letter from Allison’s cellmate, expressing concern: “I’ve been trying to, you know, keep her safe … She doesn’t want to shower. I’m going to try my hardest to get her to shower. I’m gonna let her use some of my soap. And you know, she shouldn’t be in here. She has dementia.” 

“I mean, her cellmate could diagnose her,” Linde reflects, “And so that was kind of one of our bargaining chips to get her out of jail was that we had an appointment with the neurologist in December. And so they let her go on the contingency that she would follow up with a physician, and she had to be cleared by the courts. So not only are we doing neurology appointments we have to do all these court appointments.”

In December 2018, after at least eight years of symptoms, the diagnosis of FTD finally came.

The doctors recommended that the family sit down with a social worker, but “[the social worker] just started printing things off of the internet and couldn’t answer any of our questions … She’s just google-searching and then printing a document.… I looked at her and I said, ‘I can just do this at home, thank you.’ She gave me the wrong social security disability application, so that took a year to rectify and multiple appeals with the office before there was any source of income to pay for anything for my mom. At this time, the courts had deemed her incompetent to stand trial, so no lawyer would assign power of attorney because my mom’s signature was essentially null and void. So I’m like, well then how do we gain control? She’s not gonna willingly give this.”

In July 2019, someone informed Linde that Allison was pushing other children out of the way at the fourth of July parade to collect candy for her grandchildren. 

“Obviously I’m mortified for myself and for her because that’s not her. This is 100 percent the disease, but [it was that moment that we realized] she can not be [alone] anymore. She was at this point living at home on her own because I had no way to help her.” Additionally, Allison’s financial situation had become dire. “She had spent all of her retirement … She had been a victim of financial exploitation. She had fallen for all those internet scams.”

So Linde contacted the county crisis line. “I just said, ‘I am desperate. I don’t know what else to do. I don’t know how to handle this.’” They suggested she petition for emergency guardianship. Linde went to the courthouse to find out how to do this and was told that most people receive a lawyer’s help to fill out the necessary forms. “I said, ‘I can’t afford to get a lawyer right now.’ So I printed the paperwork off and I handed in my petition for emergency guardianship, filed it with the court, and then  days later, went in front of a judge and petitioned on my own behalf for emergency guardianship. After that was granted, we were then able to get permanent guardianship.”

In 2019, Linde and her family found out that some forms of FTD are genetic. “My mom was eligible for testing because of her mother having it and then her having symptoms. And I believe at this point, my aunt was also exhibiting symptoms. And so they did genetic testing, and she was found to be a positive carrier for the MAPT genetic variant.” 

Microtubule-Associated Protein Tau (MAPT) is one genetic mutation associated with FTD and carriers may have at least a 95% chance of becoming symptomatic (Takada, 2015). The gene is autosomal dominant, meaning a parent who possesses the mutation has a 50 percent chance of passing it on to their child (Ghetti, et al., 2015). 

“So then you sit down with the genetic counselor. They explain to you … the process of here’s how you can go about getting diagnosed if you want. It’s gonna cost you $5,000 out of pocket. You’re gonna have to be cleared by a psychiatrist. You better hope you don’t have any history of depression or anxiety because then you won’t qualify because they’re gonna be concerned you’ll make life-ending decisions. Obviously all of us had diagnoses of anxiety and depression because I don’t think you can [provide care for your mother living with FTD] in your 20s without having that.” So Linde and her sisters did not get tested. 

But they continued to search for resources to support Allison. As it turned out, even with a diagnosis, resources were unbelievably limited or entirely absent. “I tried everything. I mean, I would call the Alzheimer’s Association or I’d call our county resources. I would look at the AFTD, I would call [the doctor]. There was nothing. And I think that was the hardest part was … I would spend an hour on the phone [for them to say], ‘Oh, your mom is not 65 yet? Okay, so she does not qualify for an elderly waiver, which means none of these services are applicable.’ There was always an excuse or reason or, ‘Sorry, we don’t have any way to help you.’ And I think that was the hardest part. It [would have been] one thing if someone could say, ‘Oh, we’re so sorry, we’re not your direct resource, but please call this person.’ But there was no one else. There was no other person.”

“I quit two different jobs because the burden of taking care of her was filtering over into my work.”

In January 2020, it was decided that Linde’s sister would move in with Allison. A nursing home, says Linde, was not an option. “For my mom, you would never have guessed that she had [dementia], so bringing her into a nursing home, she was adamantly against. She goes, ‘I am not going into a nursing home. They’re all old. I am not. I don’t have dementia. There’s nothing wrong with me.’”

But the family still toured nursing homes just to see if there was any chance one might be a good fit for Allison. 

“When we  were looking at nursing homes, because of her physical therapy background, we … found out that she had trespassing orders at one of the nursing homes because she had gone there to get a job and they didn’t know what was happening. And so in this interview, in this tour, the director was being really weird … She pulled me aside and she had tears in her eyes and she said, ‘I just need to apologize … We actually put orders against your mom for trespassing because she kept showing up and she wouldn’t listen … I’m so sorry we did not treat her the way she should have been treated and didn’t recognize there was something wrong.’“

“[This was one] of many interactions she had where she was not treated the way she should have been … I didn’t always treat her the way she should have been either because that is FTD. It’s just hard … I appreciated her honesty because it was a huge learning point for me to realize that everyone makes mistakes.” It also made Linde realize just how little awareness there was about FTD, even in the healthcare world. “They’d never had a patient with FTD in this nursing home because there’s just not a good place for people with FTD to go.”

In 2021, “My mom was super mobile, still very verbal. [She] had lost language skills, but could talk her way around what she needed, or give you indications on what she was trying to say. We noticed that she was having issues with swallowing liquids, [but there were no other symptoms besides the behavioral changes], whereas my grandma at this point in her disease process was immobile, incontinent, and nonverbal. And we noticed those changes [occurred] specifically when [grandma] was moved out of the house. So we wanted to stave that process off for as long as we could [by keeping Allison in her home].”

In June of 2021, Allison went to the drugstore, where she fainted and hit her head, resulting in a brain bleed. She spent six weeks in the hospital and then passed away in August due to complications of that fall.

Linde shared, “The most peaceful part of her disease was the last 6 weeks … We never had to put her in a nursing home. We dealt with incontinence for two weeks, as opposed to years. She was still able to eat and drink and speak. And so our journey compared to the other endings of FTD was– I’ll take it any day. Because we were able to keep her home. My sisters and I all did her hospice care.”

“I asked her as she was passing … ‘Mom, how’re you doing?’ She just said, ‘I’m good.’” 

Since Allison’s death, through the ALLFTD study, Linde received genetic testing and found out that she has the same MAPT gene mutation as her mother and other members of her family. 

“In finding that information out, I kind of just went on this personal mission like, “Okay, well, if this is my future, what am I gonna do now? Like I can let this kill me now or I can live my life and change the way that the disease affects me down the line.” 

So Linde moved her family to a home with more space and more access to the outdoors, and she also started sharing her family’s story in order to raise awareness and promote change.

“During the disease, my mom was so secretive about it. A way to preserve her dignity was to not talk about it. I realized … Silence is suffocating. Her story deserves to be told. And if I don’t tell her story, then what is my story gonna look like? If I can’t clear her story for her and give her that dignity, the world is not gonna be any different towards me. And my girls are gonna have to [go through] that same experience … We ran into every obstacle we could find. Every door we could find [was closed]. It wasn’t until she was gone that I was finally able to find the window that was open. And so that’s kind of where I am at now is just helping to tell the story.”

Linde remembers the fiercely independent and deeply loving person her mom was and considers the lessons she has learned from her. She explains that Allison always took care of the home and the people in it. “She knew how to tile and grout her floor, our fireplace. She did the painting. She was always outside doing yard work. She just took so much pride in her home and her yard. I know it wasn’t really within their financial means to be living in the place that we did, but she figured out a way to make it work and to make it look the way that she wanted … She just always took care of things and I think I’ve taken that on, which I realize now in hindsight that it’s okay to rely on others and … I wish she could have known that: That it was okay; that she didn’t have to do everything. But we were always her most important thing. Even during her disease … Her family was just so important and the values of being a hard worker and a good person and giving back. And she tried to do that during her FTD. She still found ways to do it.”

For example, Allison connected deeply with her grandchildren through play, which Linde counts as “one of the blessings of her FTD.” When her mother played with her children, it provided Linde with peace of mind that her children could feel their grandmother’s love and experience a part of who she really was. 

As Linde looks towards her future as a mom, as a nurse, as a sister, as a human, and as someone who is living with an MAPT gene mutation, she reflects on the traditions her mother began and a desire to honor them. For example, “Our birthday parties always had themes, and so if it was an animal party, everything was animal themed and the decorations were all animal themed. And that is something that I do with my kids … I have to because it’s her. It was something she did for me… And I want [my kids] to remember this. They need moments like this that they can reflect on during the harrowing parts of caregiving.”

References:

1. Ghetti, B., Oblak, A. L., Boeve, B. F., Johnson, K. A., Dickerson, B. C., & Goedert, M. (2015). Invited review: frontotemporal dementia caused by microtubule‐associated protein tau gene (MAPT) mutations: a chameleon for neuropathology and neuroimaging. Neuropathology and applied neurobiology, 41(1), 24-46.
2. Takada, L. T. (2015). The genetics of monogenic frontotemporal dementia. Dementia & neuropsychologia, 9, 219-229