Talking Dementia: What is Frontotemporal Dementia?

Colm Cunningham: 0:12

Hello to you and welcome once again to the Dementia Podcast. It's so great to have your company. And in this episode we continue our series Talking Dementia and focusing on Frontotemporal Dementia. Or FTD, as it's often referred to. Frontotemporal dementia is the second most common cause of dementia in younger people. Although it's a rare condition, over 50,000 Americans and over 11,000 Australians are estimated to have this type of dementia. Because it's not common, as you will hear from our guests, many people with FTD are misdiagnosed. In fact, the Australian Frontotemporal Dementia Association highlight that people are often treated for psychiatric disorders, depression, or Alzheimer's disease. FTD in the past, when first diagnosed, was described as Pick's disease. In fact, I'm showing my age in that as a student nurse, that is a term I would have been familiar with. But today, we absolutely don't use that term. Not a lot of people are necessarily aware of FTD because we know the words dementia, and Alzheimer's disease, often are the dominant terms. It's why we think these episodes are so important. FTD is a very important type of dementia for us to know about in order to understand what can often be specialized support that people may need. I'm joined again by Head of Clinical Services at HammondCare's Dementia Centre, Professor Steve Macfarlane, as he talks us through what FTD is, and some of the important issues we need to consider in care. Later in the episode, I'll be in conversation with Lynne Sewell. Lynne's husband, Rick has FTD. And we'll be hearing from Lynne about her experiences with Rick, after he received that diagnosis, and their many challenges to find suitable care. So their story may be upsetting to listen to. And it may be useful to be with a friend. And of course, we will include links to organizations that can also offer you support. But let's begin by hearing from Steve. Steve, thanks again for joining us.

Steve Macfarlane: 2:22

Oh, it's always a pleasure, Colm.

Colm Cunningham: 2:23

Steve, Frontotemporal dementia, we've obviously talked about that umbrella term that is used for dementia. And in fact, people often then get really confused, because they don't know what we're talking about, are we talking about Alzheimer's, and lots of organizations are called Alzheimer's organizations. So you can really see that it's something that people often have to navigate to understand what we're talking about. So I guess my first question is, where does Frontotemporal dementia sit? Is that under that umbrella term?

Steve Macfarlane: 2:52

It's certainly a form of dementia, but it differs from the other forms, specifically Alzheimer's disease in a number of key ways. Firstly, the pathology is somewhat different. When you look at a brain under a microscope that's affected by Alzheimer's disease. There's two proteins present called amyloid and tau. In Frontotemporal dementia, there's only the tau protein present. So it's a different pathology. It also tends to affect different age groups. We talk about young onset dementia, certainly as being under the age of 65. But if you go down to the younger age groups, like the 40s, and 50s, frontotemporal is probably more common than Alzheimer's in that age group, it becomes less common as you get older and probably accounts for about 5% of all dementias compared to Alzheimer's, which sits about 70%. The the symptoms are also significantly different, at least in the early stages of the condition. And when we're taking a history to try and determine what sort of dementia somebody might have. We pay very close attention to firstly behavioral and second language symptoms as opposed to the memory problems that might predominate in early Alzheimer's disease.

Colm Cunningham: 4:03

And why is that? Is there a particular parts of the brain that are being more impacted in frontotemporal?

Steve Macfarlane: 4:08

Absolutely, certainly, in the early stages, in Alzheimer's, for example, it's the structures of the medial part, the inner part of the temporal lobe called the hippocampi, that tend to get affected first, and they're much very much involved in short term memory, and new learning. The frontal lobe is the most affected part early on in Frontotemporal dementia. And the frontal lobe does an awful lot of things. It's the controlling side of personality, if you like, it controls our behavior, our ability to control impulses, our emotional expression, and also our spoken language. So those are the areas when we're taking a history where we look for symptoms and signs first on rather than memory problems. And one of the traps with frontotemporal is that people's memory can actually be quite good. And as people in the street we tend to judge people's cognition by how well they they converse with us and whether they seem to remember what we've said, Yeah. And it's a trap for young players that you've got to look for Frontotemporal dementia to actually find it because memory can often be well preserved until quite late.

Colm Cunningham: 5:12

So Steve, what would be some of the typical signs that we tip people off that they may need help?

Steve Macfarlane: 5:18

Okay, well, Frontotemporal dementia has two broad subtypes. One is a language variant where people struggle to find the right word in conversation, if they're looking for a word for an object, they'll struggle to come up with that word, that's usually something that the person themselves notices. And in that circumstance, they come to me complaining of a language problem. Perhaps more commonly, the person themselves isn't aware that there's a problem, but their family and close ones notice behavioral changes. And one of the reasons that patients themselves aren't often aware of the change is that amongst the other things that the frontal lobe does, it's also the seat of a cognitive function called insight. Insight is our own awareness of something going wrong within ourselves. So you can look at the frontal lobe as being the brain's damaged detector, if you like, when the damage detector itself is damaged, you can get people who have quite striking personality and behavioral and emotional changes, but they're quite unaware of it. But it can be quite distressing to their families. And in that circumstance, it's usually a family member, rather than the person themselves who comes along to seek medical help.

Colm Cunningham: 6:32

Steve, I'm going to be talking to our guest next Lynne about the support she's needed. And my question to you is both pharmacological or non pharmacological, what treatments are out there and equally, what services need to be out there to support people?

Steve Macfarlane: 6:47

Okay, so let's start with non pharmacological treatments. We mentioned there were two broad variants of frontotemporal the first being the language variant. And certainly if people are having difficulty with word finding, there's some good evidence that speech therapy can help with that for a period of time. For the non pharmacological interventions for the behavioral variant to really getting back to the holistic personalized behavioral interventions that we use in the Dementia Centre, which rely on trying to identify particular triggers for particular behaviors, and avoiding and modifying those triggers. One of the tragic circumstances around FTD is that there are no approved drug interventions. There are no treatments that are approved on the market for Frontotemporal dementia. That may reflect it's relatively low prevalence, there's not as big a market for drug interventions as there would be for Alzheimer's disease, for example. There's a paucity of effective treatments. We do use medication based treatments occasionally for some of the behavioral symptoms. It's a known fact, for example, that about 10% of people who are ultimately diagnosed with Frontotemporal dementia, are actually initially misdiagnosed as having schizophrenia. And that's because they can develop psychotic symptoms quite commonly, before the behavioral and cognitive changes appear. So they get prescribed antipsychotics, and if psychotic symptoms are present and they're distressing and intrusive, then yes, they can do respond to anti-psychotic treatments. But by and large, as with all types of dementia, non pharmacological interventions tend to be much more effective, and should be much more widely used.

Colm Cunningham: 8:32

And to challenge a few other misconceptions. You talked about memory being thought as the primary thing with dementia and people assuming that that's the thing to look for. But you've also challenged another thought, which is people can learn things. So you've actually just said that people actually would benefit from speech therapy.

Steve Macfarlane: 8:49

Certainly early on, yes, with this language variant, they can play not so much of memory problems and difficulties with new learning, but with the production of speech. So at the stage of the illness where memory and new learning are intact, they can certainly benefit from psychological strategies and vocal strategies to try and overcome or compensate for their inability to find words correctly.

Colm Cunningham: 9:12

And Steve, we talk typically in Alzheimer's about the number of years that you might see and stages, you might see is there any thing to say about the stages or speed of progression in Frontotemporal dementia typically?

Steve Macfarlane: 9:26

I suppose you can divide all kinds of dementia into mild, moderate and severe stages just on the basis of the symptoms that are present at the time. But at the severe stages of any dementia, all of the symptoms seem to coalesce as the entire brain becomes affected so it's very hard to differentiate things out at the later stages. But in the early stages, mild emotional and emotional and personality changes tend to predominate in the behavioral variant. And as I mentioned, the language variant difficulty in finding words which progressively becomes worse as the illness progresses. As for prognosis, and the course of the disease, it's said that frontotemporal is more rapidly progressive than Alzheimer's, they quote a figure of between seven and eight years from diagnosis to death. But having said that, there's probably a greater delay in people with Frontotemporal dementia in actually receiving a diagnosis because the symptoms, you have to recognize them for what they are. And if you're not looking for frontal lobe symptoms, and just do a standard memory test on people, they'll usually pass as normal in the early stages. And that delay in determining what the symptoms actually mean. We mentioned the 10% of people who get initially misdiagnosed with schizophrenia. That's an artifact that delays the point of diagnosis and perhaps shortens, therefore, the duration between diagnosis and death. So you've really got to take each individual's journey up until the point at which you first see them, and extrapolate beyond that, that's probably your best guide.

Colm Cunningham: 10:56

Thank you so much for helping us better understand Frontotemporal dementia today.

Steve Macfarlane: 11:01

You're very welcome Colm. It's an interesting discussion. Thank you,

Colm Cunningham: 11:04

Lynne thank you for taking the time to talk with us today. I know your advocacy on the support Rick, and others with FTD need will be greatly appreciated by our listeners. We've just heard from Steve about what can happen when a person has a diagnosis of FTD. But can we begin by you telling us what were the things you first noticed that were changing for Rick?

Lynne Sewell: 11:28

The things that made me seek help was the doctor where he was had very high IQ. And he was very good at maths and an amazing memory for innocuous information, dates and things. So if you played Trivial Pursuit, he was the person you wanted on your team. And I noticed that he couldn't we were selling a house and buying a house. And we were working out we actually had a line of credit. So like an overdraft rather than a loan. And he couldn't work out what we owed. And I'm like, No. And we almost had a stand up argument over these numbers, even though they were written down in front of him. And I went away from that thinking that's weird. And then there were a few other incidents of where he was getting angry when he was on his work computer at home working from home. And my particular interest is we were living with our daughter in between houses and he was sitting his computer, we were going on a holiday and he was on SeatGuru trying to select our seats for our trip. And he was the one that showed us how to use that site. And he was getting crankier and crankier because he was clicking on seats, and he couldn't get them. And my daughter and I both went when they're all the ones that have taken. It's only those dark boxes that you can click on. And we both just looked at each other and with the other little incidents, and then we just sort of put it all together. And then the numbers were starting to worry me there was just odd incidents of maths and I went and had a chat to the GP. And she asked me if I thought he would come in. And he he did.

Colm Cunningham: 13:08

And Lynne, one of the things that we know with Frontotemporal dementia is it's not always accurately diagnosed. Did the GP pickup properly and some of the symptoms?

Lynne Sewell: 13:17

She no one mentioned the word dementia to me for another maybe 18 months. So she did a small test, which I realized was a dementia test, a generic dementia test, like who's the Prime Minister, etc. What day is today? What day of the week is it? And he could answer most of that I think he did make it's too long ago. This is March 9 2014. And he she did some other stuff. And she just said, look, I think just leave it with me, I need to do some homework. This is quite specific, I need to send you to the right person. She rang me about a week later and referred us to a neuro psychologist to do psychometric testing, I realized my background is early childhood. And I've worked with a lot of children with disability. So I was used to that sort of testing with children. And so we went for seven hours worth of testing. So no one had mentioned the D word at this point in time. And she did five hours of testing on the first day she asked me to sit in on it and I I said no, I don't think that's fair. I think I'll just go away and I'll come back in time to pick you up. And she said to me that it's inconclusive, I think it's something to do with the and she tapped the left side of the head with something to do with the language center of the brain. But she said I need to do some more testing just to make sure and we went back a couple of weeks later, she'd written most of her report and she asked me to stay in for this last two hours. And it was mainly a lot of mental math and memory stuff and I was all I could do not to be upset sitting there and watching my husband fail, basically. And just prior to this IBM, whom he worked for laid off 15,000 worldwide, and Rick was a technical expert and always said he wouldn't go. He'd survived many of these retrenchment culls. And he got the email that said, you're going, I mean, it was done so poorly. And I've always said that he fell off a cliff that day, because cognitively, he started to be very obviously failing. And that's when the neuro psych Jane told me about this. And she told me it was prior nonfluent primary progressive aphasia. So I was telling people, that's what he heard. Until the following June 15. I didn't understand it was dementia.

Colm Cunningham: 16:01

Right. And that's very relevant, because we just had a podcast that's gone out on this very issue of better support and clarity for the person with dementia and their care on that whole process. So you're actually just flagging that, again, I'm very struck by the fact that we were talking before we started the podcast about how yours is met. And it was clear to me that Rick's work, clearly, very important. So that must have been so difficult.

Lynne Sewell: 16:34

It was devastating. He'd worked in IT his whole work life. He was technically world renowned. He ran conferences all over the world for HP Compaq, a company called Andial and IBM. He had written a technical journal in 2009. So this was five years before his diagnosis, gone to New York, to do that with three other men from around the world. And he used to deliver papers at conferences, as well as he, from 2009 to 2012. He was in Canberra, which is our capital, as technical support for all the big government purchases of mainframe computers, so he's, his role is to provide the technical requirements or specifications, I guess. Yeah very highly regarded man. And he loved his job. I always used to say, You're so lucky. You love your job. And we've traveled the world because of it

Colm Cunningham: 17:35

Lynne one of the things I know is very complicated is getting the right support. Can you tell me about the experience that you've had, for example of seeking residential or home support to support you and Rick?

Lynne Sewell: 17:49

Look I initially put him there, they're a dementia specific small company that buy houses and do them up with with bedrooms and communal spaces to suit people with dementia. So he was went in for respite initially for three weeks. And they said to me, after two weeks, we don't know how you're managing at home with him on your own. Because he didn't, the biggest thing with Rick was he didn't sleep. He paced and that was from the beginning. And I didn't sleep and I discovered he was going for drives and walks in the middle of the night. So they had they then offered me a permanent place. And then about a few months in when in February 19. August, he moved to one of their smaller, quieter homes that had less staff, there were two staff to seven residents, which included in the seven. Four of those were really high dependency clients with they were blind, incapacitated, wheelchair bound, needed hoisting for everything. And so if you've got two staff managing that the my husband and another very mobile lady used to attack each other. And there was an elderly man that Rick took an instant dislike to and used to just walk up and punch him. I never saw it, but they told me and we didn't see behavior. But so they, the following February said they could no longer provide care for him. I forget the phrase they used was a technical phrase about not being able to meet his needs, basically. So they like they expected me to take him out that day. Wow. So the basic, we're not going to keep him anymore. I was there with my two daughters. One's a chemist, one's a lawyer. We had a little chat out in the hallway and came back and said we want six weeks to find alternatives because I knew there were weren't any we the girls But whilst they're both very busy professionals and young mothers said they'd helped me. And so we set about finding care for him at home, and I had to fix home up to bring him home.

Colm Cunningham: 20:11

Can you tell me then a little bit about Specialist Disability Accommodation, which I believe is part of the story now?

Lynne Sewell: 20:17

Yes. So there's a thing called the National Disability Insurance Scheme. And I had a care worker from Dementia Australia, or as it was known in Alzheimer's, Australia, who pushed me at the end of 2016, to fill out this mountain of paperwork. And I just looked at her and said, I'm not doing any more paperwork. And no one had heard of the scheme. It had been a pilot scheme. It wasn't it was just being launched in parts of Sydney, and she said, You need to apply for this, it's going to be good. She said, I don't know how, but I just know it's going to be good. So she sat with me for two hours, and we filled out the paperwork, and Rick was assessed quite quickly as eligible. And we were given a small bucket of money which provided carers when we were he was at home that took him out to play his golf and gave me a break because I wasn't getting any sleep. And so then after he'd been evicted from care, we had a review of that plan, because the plan that that month, that plan was paying for the 24/7 carers in the home. And a planner did a review. And this is COVID. Right? This is outbreak of COVID. So everything was done. Not even by zoom at that point in time, it was just over the phone, like a conference call. And she said you need SDA and you need this. And so that was the first mention of that. And I sort of became educated on what supported disability accommodation meant. So that was awarded to Rick's plan in May 20. He still isn't in SDA accommodation. I looked at we went on a list, the government puts out spreadsheets, I think monthly. We I have I was the NDIS appointed a specialist support coordinator. I went through a period where the funding wasn't sufficient. And I lodged a formal complaint because of the way this planner had mishandled everything. I rang and lodged a formal complaint, which they acted on very quickly and the person from the NDIA that rang me. So first off, and this is this is 2020. Right? So I'm already six years into the journey. He said, Yeah. Has anyone ever thanked you for looking after your husband? I said no, actually, no. So thank you. And then he said, You need a specialist support coordinator. And you need to find SDA accommodation, and that's going to be her role. And he said you don't need to find her. But them sorry, not her. He said I'm going he said it's a company. And he gave me a name of the company, he said, but I'm calling them and within an hour of hanging up from him. That company called me, specialist support coordinator called me and she's still on board. And her and I have been looking basically for nearly two years for SDA accommodation, right? Because there isn't much for someone in Rick's state of needing a place for one person. It can't be in a group environment, because he can't be trusted to not hurt people. I mean, he's attacking carers and he's attacking us now. So I couldn't sleep at night, if I knew he was hurting other clients or patients, the company she works for have established a relationship with a developer who's a little bit of a philanthropist. He doesn't think he is but I think he is. And he's built an apartment complex in a suburb near where I'm now living. And he's established that six of those apartments are for disability, and the National Disability Insurance Scheme. Um, he won't lose and so and the company that are providing Rick's current care in temporary accommodation, and my specialist support coordinator, they managing that accommodation for him. And when I looked at it 12 months ago, it was a building site. And I was in it was almost finished, and I was told it would be available in May. But COVID COVID got in the way. Oh, it's still it's still not ready to move into it's it's the I think the ninth of March has been set as his date to move into those.

Colm Cunningham: 24:41

That's a long, long time. And as you say, from diagnosis to know that nearly eight years. Yeah. Lynne, can I ask you for people listening? Some people for the first time may have heard this term Frontotemporal dementia. What what are some of the things that have changed in Rick's needs? And how has that impacted on you saying that he is now, I'm getting the sense that behaviors have changed, which I know is not un-typical. But what that must be very confronting.

Lynne Sewell: 25:15

He lost language first. So that was the so that's the temporal region of the brain. And it wasn't till he had a PET scan in the June of 15, that they, the neurologist, I have to tell you this, the neurologist said, we'll do a PET scan. And she said, It's Alzheimer's. And the PET scan came back and said, No, no question, no question about it. It's not Alzheimer's, we suggest that it's Frontotemporal dementia, because that's where all the damage was. So we went back to see her and she said, Go away and enjoy your life.

Colm Cunningham: 25:49

What does that mean?

Lynne Sewell: 25:50

Tell me.

Colm Cunningham: 25:51

Yeah.

Lynne Sewell: 25:52

And she said, you can come back next week, or you can come back in a month, or you can come back in six months or you can come back in a year. I was so angry, because I said, So what are we supposed to do what she said, there's no treatment, just go away and enjoy your life. I walked up this long corridor at the private hospital where her rooms were fuming. Like, we're coming back next week, I don't care what she says. And tried to do some research. And I'm telling you in the last eight years, the amount of available information today is so much better than what was available back then. And so you're basically dumped. She told me to go contact us. Alzheimer's Australia, and I'm going but you've just told me he's got frontotemporal she said yes, contact Alzheimer's Australia, which was a conflict just in the name. And so I did, and that's how I got to the funding side of things. But what changed for Rick? Well, initially, it was language, he got very lost in conversation. He couldn't say his name. He couldn't ask for directions. But he was still driving and he was driving very well. So he sort of defied everything you read. I sort of slowly weaned him off driving, rather than going through the trauma of the test that said, you're no longer fit to drive in our, our relationship, he'd always done the driving. I'm a good driver, and I drive just as well as him but and driving doesn't faze me, but I was happy to let him do the driving. Yeah. So we changed that role. And he seemed to accept it. So I often wonder whether somewhere up in there, he sort of was acknowledged that maybe he wasn't as good as he should be, right. And then the things I noticed was that I couldn't hold his own in a conversation. He couldn't ask anything, he would still go to the shops for me. But I used to have to write out a little list. And I had to write more than just flour. I had to say self raising flour, and often I would take photos on his phone of things. So he knew visually. And he was going through that retraining program with language

Colm Cunningham: 28:10

And Lynne. You sound like you've got a lot of resilience in you. But how have you managed? I know when we were talking before the recording was hit that? You know, you've been married for 47 years. But how do you look after yourself? And what would you say to others?

Lynne Sewell: 28:32

I think I think the battle for want of a better word keeps me energized. I said to my GP today, I'm stressed by all this, but it's not affecting me. And she took my blood pressure and it's the lowest it's been a very long while. I look after myself by just vegging out. Sometimes I watch some Netflix, I have two very close friends. Neither of them live in Sydney one lives. She's English and lives in Singapore. And the other one's my school friend that I've known since I was 11. And she lives in Melbourne, and I keep in touch with them. They used to come and stay with me. COVID sort of put a two year gap but we keep in touch by phone. Yeah. Um I'm a grandmother and I sorry, I give a lot of time to my grandkids I have two daughters, or we have two daughters. Sorry.

Colm Cunningham: 29:41

Don't say sorry Lynne. It's a testament to your relationship.

Lynne Sewell: 29:44

Well he's my best friend. I mean, his job took us all over the world. We've lived internationally five times. We only had each other for the first half of our married life and and our children. Our kids were with us. Yeah, and we just a partnership, I tried to be positive about that, because we had a fabulous life because of that. And, and I sit here now and, and try to be positive in that we've had this fantastic opportunity for lots of travel as a family, we've had some fabulous times, and a lot of people go when I retire, I'll do this. Well, we never got to retirement age Rick and I with normal work. He was 61, when we first started noticing things going wrong. So I just am grateful that we had this fantastic relationship and this fabulous life that his job gave us.

Colm Cunningham: 30:46

Some things are falling into place now. Obviously, you're still waiting after two years for the accommodation that you need. But what are some of the things that you would call out that they need to have in place so that you don't have to. I do feel you've had to fight for some things. And I think that you're listening to you your personality, you know, that has probably helped you in this. But for those who should expect these things to be in place, you didn't get a clear diagnosis, you didn't get a pathway of support, you have put things in place you've been, you've been told by accommodation that they couldn't care for, I find it hard to accept somebody would give you 24 hours notice. So again, you and your daughters gathered your energy and said Hang about. So there's a lot of strength in what you've done for the but I guess from that what would be the things that you would want to see or call like that should have been there for you that you want to hear for other carers not to have to go through

Lynne Sewell: 31:48

From that diagnosis from the neurologist that that said, Go away and enjoy the rest of your life. I mean, she handed me a brochure on Alzheimer's Australia. So I mean that was totally diametrically opposing, I'm concerned. And I didn't understand FTD or dementia at that point in time. And, and I found myself educating people by saying, you know, dementia is an umbrella. There's Lewy bodies, and there's all these other dementias you they need to the medical profession. To be honest, I don't think they know much about dementia. That's my experience. And I think they're flummoxed by it. And I and they probably to be honest, I don't know where to send us for support. The policymakers, I just think they need to acknowledge that dementia is real. Frontotemporal dementia is very unique as far as I'm concerned. And I'm in a support group for FTD. And there's probably 17 of us in that support group. And no two of those people with dementia with FTD are the same. And, but it's wonderful to have that support. So it's about and now. And so for policymakers that was set up, and we were told, we really should only have access to it for 12 months. And we are a group of very strong, mainly females. There's about four men. And we lobbied hard, and I don't know where they found the funding, but where we're a unique group that we are not wound up and we meet now bi-monthly instead of monthly and on the alternate months, they give us the room to meet independently, which we did last week. So the policymakers need to provide the funding to enable those support groups to happen. Because it is fantastic. We chat to each other via email or WhatsApp. When they hear that someone's going through a tough time like someone's on palliative care or someone's passing away or whatever everyone's there for each other. We're all strangers, but we we hold hands. And so the government needs or the policymakers need to fund those organizations, and we shouldn't have to fight for them to continue. My understanding is that if you join an FTD support group, now you only get 12 visits.

Colm Cunningham: 34:15

And the word fight has come in here a lot the need for people to think about what they're saying to you, when it took years for somebody to actually say thank you.

Lynne Sewell: 34:25

Yes

Colm Cunningham: 34:27

It's significant because we know that the amount of people out there as carers who are supporting people with dementia, and if they weren't doing that, I don't know what we'd do. So I can't thank you enough for sharing your story. That brings us to the end of our episode. Once again, a thank you to Lynne and Steve, for coming on to talk with me today. And thank you our listeners for joining us on this episode of the podcast. FTD will feature later in the year at our International Dementia Conference, as one of our keynote speakers will be sharing about her experience after diagnosis. And you can find out more about the Dementia Conference at dementiaconference.com. And as it's March 2022, you can also submit a paper for the conference and join us in Sydney on the eighth and ninth of September. If you have any questions, comments or feedback, we'd love to hear from you via our email address hello@dementiacentre.com. Bye for now