Katie Brandt and Dr. Brad Dickerson: Understanding Frontotemporal Dementia | Live Talk

okay hello everyone welcome to being patient brain talks I'm Deborah Khan founder of being patient since Bruce Willis and his family announced that he was diagnosed with frontal temporal lobe dementia a lot of you have written to us you've commented um asking whether or not your loved one might have the same or you have questions around treatment and care so we thought this was an opportune time to ask um I and frankly in my opinion the world's leading experts on FTD please join me in welcoming Dr Brad Dickerson he heads the FTD unit at Mass gen and Katie Brandt whose husband sadly passed away from FTD but now leads the care services with Dr Dickerson's team so thank you both so much for joining us pleasure to be with you today Deborah thank you for the invitation thank you Deborah so Dr Dickerson let me start with you because you know once the Willis family so bravely came out and announced that um Bruce was indeed diagnosed with FTD we had so many questions come in about could this be the same for my loved one you know um I'm seeing these types of symptoms or um my loved one was diagnosed with Aphasia does this mean that um he he's going to get frontal temporal lobe dementia so let's just like back up a little bit and say at what point I think there's a lot of confusion around here um at what stage does one have to be in to get an accurate diagnosis of FTD well uh FTD is much less common than Alzheimer's disease but it's in the same family of conditions that uh develop in the brain as a neurodegenerative disease that gradually begins to cause symptoms in a person probably some number of years after the disease begins in the brain and depending on exactly what type of disease it is and where it is in the brain it can produce a variety of different symptoms and so with FTD it tends to be a younger age of onset condition for reasons we really don't understand then Alzheimer's disease and it tends to strike the front parts of the brain rather than the back parts of the brain which in general is what Alzheimer's does and when people uh for whatever reason develop their ill their disease in the brain in most people on the left side if they're a right-hander they may start out by having language symptoms and so their initial symptoms may be best described as Aphasia which gradually progresses over time other patients develop changes in personality and behavior that often are very distinct from those of patients with Progressive Aphasia and those are because the disease starts in a different part of the brain Define what aphasia is and you know obviously the Willis we we heard first that um Bruce Willis was diagnosed with a type of aphasia we knew that that type of aphasia often leads to FTD but it wasn't until a period of time where it was conclusively FTD so what is aphasia and why does this one type turn into FTD in a lot of cases yeah and as Katie and I have discussed extensively it's so important for the community of people living with this rare disease for someone like Bruce Willis and his family to disclose this diagnosis so it really matters for a lot of people out there which I know Katie will get into in more detail but uh Aphasia is a problem with communication it can be a problem with expressing yourself linguistically in language or a problem with understanding what other people say to you or a mix of both and so when when a neurologist typically thinks about a patient with Aphasia they think the patient might have had a stroke or they might have had a brain injury but those are usually pretty sudden what we know is a lot of people may develop a gradually Progressive difficulty expressing themselves in words and this isn't just coming up with someone's name many of us have that and we don't have Aphasia it's more than that and it affects Communication in a greater way and so for some number of years it may be completely isolated and may be gradually Progressive Aphasia which is often referred to as primary Progressive Aphasia because the main problem is language but sooner or later in almost everyone with that condition other cognitive or behavioral symptoms will develop and that turns into a more General type of dementia and sometimes it's caused by FTD and sometimes it's caused by Alzheimer's Disease an atypical form and sometimes it's one of the related diseases okay and Katie let's talk a little bit I think it'd be really helpful for those of you um uh for those people who are joining us who don't know your husband's um story um he was very young I mean Dr Dickerson just um said it tends to impact people who are younger um 29 years old was when your husband was um showing symptoms tell us what some of those first symptoms looked like well my husband was a little bit different than than what we suspect Bruce Willis is living with right we've heard of those early uh symptoms about Aphasia and as Dr Dickerson just mentioned when it happens in another part of the brain you can see early personality changes and that's what I saw so my sweet husband who is very outgoing became very withdrawn he was making a lot of impulsive decisions and just generally seemed like a different person one thing that really struck me when I heard about the you know Bruce Willis's announcement was that they had a difficult Journey themselves with diagnosis and how I brought my husband to eight different medical professionals and it wasn't until we were with the chief of Neurology at Beth Israel in Boston when we received an accurate diagnosis uh Dr Albert Galla Berta and he said to me you really need to be with Brad Dickerson's group because they're experts in FTD and I think that whether we're talking about the medical supports that you get the community supports the care supports understanding your loved one specific diagnosis so you can get expert care that's specific can really just make the journey so much easier to navigate for everyone and so that actually exam Ines um Medical Care as do presumably the Willis family um why like write the playbook for us Dr Dickerson what do you ask patients to determine if it's FTD or not and what can a scan tell us can can a simple MRI or a CAT scan give us any indication as to um whether or not it is FTD yeah so I think that uh like anything in medicine the um symptoms and signs that a person is experiencing of their illness are best recognized by someone that's very experienced in dealing with that illness as a medical professional so so much of what's involved is is the experience of the practitioner and many neurologists might have seen one maybe two patients with FTD or Progressive Aphasia in their entire careers it's a rare disease and so uh when when you are not gaining access to someone that's a specialist it can be difficult to figure out if that's the right diagnosis or not many patients and families will advocate for themselves to try to gain access to a specialist but as we know there aren't enough of us so uh you know I think that typically a good history of finding out what's going on with the patient's symptoms in their daily life and a good assessment in the office with testing and examination and so forth and usually a brain MRI scan might be plenty to make a diagnose gnosis confidently of FTD early in the course of the illness or you might need more specialized testing like a pet scan in order to figure it out but a lot of those things are you know best recognized by an experienced specialist that knows what FTD looks like so what are the questions I want to ask this from two perspectives what are the questions you're asking patients and then Katie what do you recommend patients should be and their families should be asking to help aid a a more accurate diagnosis so I I like something that Dr Dickerson has said in the past which is that especially with the very young you know people that have early behavioral and personality symptoms it can look like a psychiatric condition but then it keeps getting worse over time and I think that's when you want to go back to your medical provider and the one thing I could say is I brought my husband to eight different medical professionals he spent a week in a secure psychiatric unit and so many people told me he had depression or severe depression and it just it didn't feel right and I just want to say to every you know family member out there if it doesn't feel right just I want to empower you to keep going and to you know really ask to join that medical appointment with your loved one and if you can't join the medical appointment you can communicate with your loved one's medical provider HIPAA prevents Healthcare professionals from sharing information it does not prevent them from receiving information so really everyone should hear that like think about you know Dr Dickerson can receive an email or a note from a concerned family member that he can read and think about is this relevant during my evaluation that's not you know um breaking any confidentiality so when you talk about like in like different behaviors depression maybe or what is that how does it manifest Dr Dickerson could it be a whole spectrum of is it is it someone who typically had never experienced depression before suddenly was chronically depressed is it inappropriate behavior I mean the frontal cortex of our brain is often judgment and behavior right so if it's impacting that part of our brain what what types of symptoms in terms of behavior manifest yeah so I mean I think that depression major depressive disorder is a disorder of frontal systems as well as other systems of the brain so it's not surprising that some of the symptoms might look in some ways similar but with FTD I I think of uh three main types and there's a lot more Nuance to this but there's the non-fluent type of progressive Aphasia and that's where people have trouble getting words out in grammatically correct sentences so they or their family members and it's always important to get someone else's perspective on this because many patients may have complete insight into their symptoms and others may only have partial awareness of what's going on but the patients with the non-fluent type of progressive Aphasia will often say I can't get the words out in a in a normal way and that that flows like a sentence and you can usually hear a problem in the way they're speaking which is often more monotone or more choppy and fragments of sentences and that's typically in a expressive Aphasia patients with the semantic variant of progressive Aphasia don't understand what less common words mean and often have a lot of trouble figuring out how can they get the word out that they really want to get out and that's a lot different than just most of us having taking longer to come up with the word that we specifically want to use it's more of a situation where someone might say does a cork float in water and the patient might say what's a cork I don't even really know what that is it's really quite striking when you see it but at the beginning it can be subtle and then the third type which probably is most common is the behavioral variant and that's really what you were just asking about dab I think which is where people often will do things that may not be appropriate to the situation they're in we call that disinhibition loss of filter is what a lot of family members will refer to it as they may become what looks like obsessive compulsive even though when you ask them why are you doing those things they'll often not even realize that they're collecting or organizing or hoarding things in the way that their family member reports on and other people may become may look like they have substance abuse or an eating disorder type problem because they have a very specific fixation on certain kinds of foods or on drinking drinks including alcohol but sometimes including other kinds of drinks so the as you can imagine it's much more common for people to have psychiatric illnesses that present with these types of symptoms so it's reasonable that many professionals think of that first because it's a lot more common even though when you're trained and experienced in this area you recognize that the nature of the symptoms the person is experiencing and often their inability to really talk to you about them differentiates a person with FTD from a person with a primary psychiatric Disorder so do you do you does everyone who comes into your office to get a diagnosis do you give them a scan do you give them an MRI or a CT scan and if you do what can you see yeah we think that it's important if we're concerned about an organ in the body like the brain to get a look at it uh so it's it's unfortunately surprising that people don't always think that way as medical professionals but if we think that the person might have a brain disease that is causing their symptoms we get a brain MRI very routinely no problem insurance pays for it it's completely reasonable and if they have FTD or Alzheimer's disease or another neurodegenerative disease you may be able to see shrinkage of certain parts of the brain that a neurologist would typically expect to be abnormal in a person with the particular kind of symptoms they have many times early in the in the illness the MRI may not show very much and in that case we may need to go to What's called an fdg pet scan to get a measure of the functioning of the brain that's generally a more sensitive test but is less readily accessible for people even though it's paid for by Medicare routinely in an evaluation for a patient suspected of having one of these diseases so I do they have plaques in their brain like in Alzheimer's beta amylase plaque like what are the Hallmarks or is it just shrinkage is it atrophy yeah so the disease in the brain is different from Alzheimer's even though one of the proteins is the same Protein that's affected so these are all normal proteins in the brain that have important regular functions and something goes awry with them and they start to basically rust and twist and Tangle and aggregate within the cells with FTD one major type is called the FTD Tau and that's the same Tau protein that you've talked about that's involved in Alzheimer's along with the amyloid plaques there are Tau tangles in Alzheimer's but what exactly goes wrong with the Tau in the brains of people with FTD is different than in Alzheimer's with the Tau but there's no amyloid the other type of FTD is called tdp43 so FTD tdp43 that was only discovered in 2006 and it also is the same protein that builds up in the brains of people with ALS so FTD is one of these examples of the neurodegenerative disease like some others where there are clear linkages or relationships to some of the other diseases that may be thought of as completely distinct but probably at a biological level have important overlaps okay there's a lot of questions coming in so I'm I want to get to to some of them um so Maria asks um why is my mom passing out more often especially in the bathroom on the toilet it's um three times since December and presumably she's has had a diagnosis of FTD is passing out um a symptom well uh if she has a diagnosis of FDD it used to be the case that uh in the old diagnostic criteria from 1999 blood pressure lability meaning ups and downs of blood pressure that are otherwise unexplained were part of the the supportive diagnostic criteria that was removed because it's not super common in people with FTD but it's common enough that it was in those criteria and that's because some of the parts of the brain that regulate blood pressure the parts that control the autonomic nervous system the fight-or-flight part of our bodies can be affected by FTD and so some people with FTD do pass out for reasons that are unexplained probably because the brain systems are not functioning properly and they end up causing the blood pressure to drop for unexplained reasons there are treatments for that they don't always work when it's a central problem and coming from the brain as opposed to a problem in the nerves which is more common so it deserves medical evaluation because there may be other things that can be done about it there may be other reasons for it Katie how did the presentation of symptoms um occur within with your husband were you did they change rapidly or was it more like a a degenerative process where um initial symptoms got even worse definitely initial symptoms got worse but I think that what is so difficult about FTD is that it strikes people at the prime of life so if you think of Mike and I just like many other families with FTD we had a mortgage to pay we had student loans he had a small business that he was running he was a teacher I had a full-time job and we had a new baby so when you built your life expecting that you're in a partnership with two incomes and then all of a sudden one person in the partnership doesn't seem like Mike became like kind of apathetic about work his work product was not of the high quality that his customers were used to he wasn't able to do all the tasks I didn't suddenly feel comfortable leaving him alone with our son so I didn't have a co-parent I didn't have a co-income earner so what happens is in the early stages like all the scaffolding of your life falls apart and I think later you build new scaffolding right for Medical Care Home Care maybe you connect with a community-based memory program but it's in those early days where so many things are unknown and there are resources right if you get a diagnosis you can get Social Security Disability very likely maybe your loved one has a Disability Policy at work and it matters if they just quit their job because they're no longer interested or if you have that medical evaluation that says no this person needs to leave work because of a medical condition so early and accurate diagnosis can affect the day-to-day lives of families and it definitely did that for us and I think in particular with FTD because it does impact younger people you know no one's really thinking dementia um when you're younger right I I think a lot of people um Freda writes to us my husband seemed to show all types of the Aphasia non-fluent Etc is this reasonable to not just have one aspect of aphasia but all is is that common not everyone follows the rule books um so uh you know I think that it's common for people to develop very mixed aphasias uh as their illness progresses whatever it may be what we usually think of when we think of these more distinct subtypes is how did it start but some people do start in ways that are not typical and I think um you know that's usually explainable once we understand what's going on in their brain so not everybody is classifiable in the standard way and that's the nature of any of these illnesses Wendy wants to know she said I'd love to hear more about the motor types of FTD including CBS and PSP you're gonna have to translate those for me yeah so you can think of FTD unlike many of these the other related diseases as sometimes affecting people's cognition language is a major cognitive skill sometimes affecting people's socio-affective function or behavior that's the behavioral variant and sometimes affecting their motor function and so uh cortical basal degeneration and Progressive super nuclear palsy are two conditions that are are part of the FTD Spectrum in terms of the diseases that are fall under this umbrella of diseases they used to be thought of as related to Parkinson's because they cause some of the kinds of motor symptoms that are at least reminiscent of Parkinson's but it's the same protein in the brain as what's going on in FTD teleopathies so we now classify them that way and many patients with those conditions all also have cognitive changes like language or other functions or mood and behavior changes like behavioral variant FTD so they can those two conditions can really be a spectrum as well and a couple of celebrities that have been affected by PSP uh are Linda ronstad who just came out about that not too long ago and um the uh sir Dudley Moore who played Arthur back in the day in the movie so there have been a number of celebrities that have come out about PSP Katie what what do families when they come in I'm you're dealing with the whole unit so how how do uh Partners caregivers how do they advocate for um their loved one and what is what what are your top recommendations what do you in hindsight wish you had known um back then that you know now probably so many things that I have learned from the journey but the biggest is that you're not alone I think that when I received the diagnosis from my husband I had actually never heard the term frontal temporal dementia frontal temporal degeneration before and I felt so alone we were the only people in the world going through this rare condition this rare diagnosis and that's just not true we don't have a care today for these conditions but we do have a care for the isolation and loneliness that may come along with the diagnosis so right away I tell people you need to speak with an elder law attorney and then the second is get connected with your disease community so if you have Alzheimer's disease go to the Alzheimer's Association if you have FTD connect with the association for frontal temporal degeneration find a support group and it's okay to shop around so if you go to One support group and you say oh I didn't like that that wasn't for me go to a different one maybe we've had people that have come to One support group and then they don't come back for a year just know that this is a community that is very welcoming and supportive and if I could say one thing to myself a decade ago I would say ask for help earlier because it's just so unrealistic that you could provide all this care and support by yourself that is such a great Point um and so true you know there's a whole village of people out there um impacted and it's much it's a much better journey together than on your own um okay we have a ton of questions so I'm gonna kind of uh pick things up a little bit um Julie is asking about future treatments let's start first with Dr Dickerson what what are FTD patients if anything prescribed with um to manage symptoms and what's what's going on in research are we are we like on the road to find a cure or where are we in that landscape there's been a ton of progress made but the meaningful treatments are still a ways off unfortunately so uh the treatment for patients with FTD is broad-based but is primarily supportive if a person has a progressive Aphasia speech-language pathology therapy is very important speech therapy can really make a difference for people's ability to try to compensate for the problems they're having it doesn't help them recover like it does in patients with stroke related Aphasia but it can help them in their in their communication Partners communicate more efficiently with each other so medications may play a role but for the most part they're treating symptoms and it's all off-label none of them are approved specifically for FTD we're using medications that are typically approved for other types of conditions to treat symptoms that can sometimes really make a difference in people's quality of life in terms of the research working toward more impactful treatments there's a global effort there's something called the global frontal frontotemporal dementia prevention and initiative FTD prevention initiative and that's an effort across multiple countries to work with our industry partners and our academic colleagues to try to really develop the treatments of the future and there are clinical trials going on right now we're just a ways behind Alzheimer's disease in terms of the number of trials that are going on but look for that to change before too long because I feel like there's a real acceleration of our understanding of the underlying biology of FTD and some improvements in biomarkers that's another place where we need to really make some good progress because a lot of what helped the Alzheimer's Community is biomarkers for things like the amyloid plaques and we don't have those yet for FTD do you think but with both of your experience do you think that FTD I mean I'm beginning to think that this is a dementia like Lewy Body that's severely misdiagnosed or people don't get diagnosis early enough do you think there's actually more people out there with FTD than we know given the inexperience of diagnosis I mean I would say yes not even being a clinician but from a family perspective I consider myself someone that had resources and the ability to access expert medical professionals in the Greater Boston area there are places in the United States where there are neurology deserts there's not a neurologist for 200 miles so we know that it's not easy to trip across an expert the way it is in downtown Boston that being said the estimates are maybe 50 to 100 maybe even 150 000 Americans which which is you know an order of magnitude or more lower than Alzheimer's but I think part of that may be underestimates because of lack of diagnosis like you're saying Deborah so I still think by most people's effort estimates it's it's quite a bit rarer than than Alzheimer's disease probably more on par with how common ALS is or maybe slightly more common so we have another questions asking what is the end the ND like is it s other dementia is um do they all converge in the end and I mean I I remember I interviewed a pathologist who told me when he does autopsies on dementia patients by the end stage it's often mixed dementias it's different types of dementias right that have kind of converged as as the brain it impacts more parts of the brain um is is this the case with FTD or what is that I mean I I'm sure Katie you can touch on it from you know the caring perspective what does that end stage look like but also Dr Dickerson if you can touch on what's going on in the brain um in the later part of this disease I think for Dr Dickerson to go first okay yeah I mean I was pausing because Katie you've worked with so many people to help guide them toward brain donation and it's such an important process that you go through and and so we've followed many people to that end stage and and to the precious uh unique resource of brain donation for advancing science and gaining closure on the disease I think in many ways the loss of functioning in people is pretty similar by the time they get to the advanced stage of FTD as it is in people with Alzheimer's there are some differences there's often a more focal degeneration in the front part of the brain in FTD even toward the end with remarkable sparing at the back of the brain which some of the things the back of the brain does is help you recognize family members and so you know there may be a little bit of a difference in that in people with FTD but in general the loss of function is such that people are typically unfortunately not able to do anything for themselves and and not really able to interact much with loved ones when we think about okay here I just want to plug in for palliative care and Hospice Services if your loved one is living with moderate to severe stage dementia of any kind reach out to your local palliative and hospice care agencies and ask for an evaluation find out what you're eligible for to help you protect personhood and dignity your goals of Care at every stage for me that was incredibly supportive I felt like it was a gift to our family at the end and then when I made the choice to donate my husband's brain finally it felt like my first power play against the disease that was trying to run everything about our family's lives did you learn anything from did you get a report about the autopsy on his brain what did you learn I did and I remember um sitting in my living room and Dr Dickerson and I were talking on the phone to you know so that he could explain the autopsy report for me but the biggest thing he gave me was the gift of being sure that my that Mike was living with FTD that is what he had I didn't leave any stone unturned I brought him to the best place I did what I could there wasn't the mystery was uncovered through that gift if you're interested in brain donation for your loved one the association for frontal Temple degeneration is hosting a webinar next week about brain donation so you can go to the ftd.org to learn more about it um post we'll post the link to that in our program at MGH we work with our families to do that all the time well and Maria was asking on this topic of hospice and palliative care when is the right time to call so for you when was the right time I mean everyone thinks of hospice as the very very end is that true so my um many people know I'm also a caregiver for my dad I've been a caregiver for my dad living with Alzheimer's for 14 years and he has been on palliative care for a few years because that helps us to make decisions that match our goals of care and I've had him evaluated for hospice and he's not eligible but because he's in palliative care I know that when that time comes it'll be um he won't have to wait that's that's good advice um Karen has written in saying my husband has Aphasia but early on he exhibited hypersexuality that was very uncharacteristic I've heard this comment before actually how common is that symptom hyper I think when we talk about hypersexuality it's important to differentiate um a person who is displaying more sexual behaviors in an otherwise largely normal way as sexual behavior versus a person who is somewhat innocently fascinated or or wanting to interact with another person usually of the opposite gender not always in a in a way that made to the uh the healthy person feel like it's a sexual interest but at the end of the day it's almost more of a disinhibition and sort of curiosity and exploration those things are really different and I think that um you know trying to figure out what what is the person really trying to do uh and what can I do to try to respond to that in a respectful way that recognizes that they have a brain disease that is causing them to behave in a way that would be different than they would ordinarily behave is really the most important thing in general a true hypersexuality is it relatively less common early Behavior or behavior period in FTD but it certainly can happen it's much more common for people to be disinhibited disinhibited in a way that leads them to touch other people or or interact in ways that might feel sexual to the other person but might really not be driven by some kind of intent to pursue sexual behavior Katie um Wendy's asking did Mike have ft FTD mutation so I want you to answer that but then I also want to ask um Dr Dickerson if is this hereditary um do is there an FTD Gene does this mean that parents have to consider um perhaps passing it on to children so first Katie why don't you answer if any genetic component there like I said uh when Mike received his diagnosis that was the first time I had ever heard of FTD so no one in his family had had it and you know of course every family when their loved one is diagnosed with anything right whether it's colon cancer or Alzheimer's disease they worry oh my goodness what is the reality for me and I think Dr Dickerson will speak to the value of meeting with a genetic counselor and I cannot say this enough don't don't listen to your neighbor or your Aunt Mary who who is saying things that could be upsetting speak with an expert learn the facts and that can help you make plans for your own health care decisions and your loved ones so what are the facts in terms of genetics Dr Dickerson what do we know is there a variant that's associated with FTD FTD can run in families most of the time it's what we call sporadic which means it affects the person that comes down with it and there's no history of anyone else in the family that's had similar symptoms and it's important also to recognize that sometimes other people in the same family may have had related conditions that might have been diagnosed as Parkinson's or might have actually been ALS I mean these conditions run in families in ways that are not uh stereotyped it's not necessarily true that the person that might have the same disease in the family had exactly the same symptoms so it's important to I like to talk to people about tell me about your dad what happened to him as he entered the later stages of his life tell me about your mom I really like to go through family history and detail like that and it turns out that probably somewhere around 15 to 20 percent of people with FTD have a family history of something that might be similar and right now about 10 to 15 percent of people have a pretty clear family history from generation to generation of the same type of thing and we can explain many of those cases with one of three major genetic mutations that have been identified over the years that are now being taken advantage of in ways that hopefully will lead to better biological understanding of the disease and ultimately opportunities to try to treat it and maybe even give people treatments before they develop symptoms if they're a member of a family like that just like the a lot of the Alzheimer's studies are looking to re-engineer apoe4 to act more like E2 which is protective rather than risk what are the genes for associated with FTD what do you know um I'm sure you know the variance what what are the names of them there's one called map T it's the gene that makes the Tau protein so if you get mutations in the gene that makes the Tau protein you get FTD you don't get Alzheimer's disease even though interestingly there have been a lot of animal models in the laboratory that have developed been developed using uh mice with the map T mutation to study teleopathy to try to develop treatments for Alzheimer's but that's really an FTD mutation then there's Pro granulin which is on the same gene as as map T they're both on chromosome 17. um and that that leads to the tdp-43 type of FTD and then there's a C9 or of 72 which is just a letters and numbers but that was discovered in 2011 and is the thing that the gene that links families where some members have FTD and some members have ALS and so that's one of the major genetic causes of FTD or ALS or or combinations of of the two anyway and this and this is important that more men than women get FTD is that true I think it might be slightly more but it's really it's very similar in terms of the the effects on gender and then when you were talking about the age span do older people get FTD as well or is it primarily below the age of 60. the majority of people develop symptoms at the beginning of their illness in their 50s or 60s but we've seen people as as you were talking about with Katie who who have been as young as in their 20s and people who have been as old as in their 80s when symptoms first start and they've been documented as having FTD so it's uh generally speaking a younger age of onset illness than Alzheimer's but there are plenty of people in the age range where you think of Alzheimer's that turn out to have FTD and um Don Kirby is on this um video who was a her daughter sadly passed away um but but she says that her daughter Cara who she's been on this on being patient before and I think with with both of you um she said she donated Cara's brain to um but how long does it usually take for the pathology report to come back because obviously they haven't gotten it back so maybe you could talk about that Katie how long did it take to get results and do you have to be proactive and ask or you know I I know there's a lot of uh like clarification that has to come back to really communicating to the loved ones of of the person if they do donate for me it was a year and for many of the families I work with it does follow that timetable um and you know we do appreciate um hearing from the families who've donated their loved one's brain to check in with us we're always happy to update them um and we want to connect with them and I think that those meetings and those interactions they take time and we want to do it thoughtfully and and that's why I think families sometimes have to wait longer than we would like Scott writes that his wife was diagnosed with FTD PPA in January of 2015. her neurologist has indicated she's in the advanced stages of her condition I'd like to get a better idea of where we're at in her journey um reviewing three stage seven stage models reveal no confident prediction now this is a problem that a lot of people have with all of the dementias how do you know where you're at um in you know the the state the different stages moving from stage to Stage as a lot of people's symptoms appear differently or at different times so what would you say to that I mean I'm I'm happy to hear from both of you because I think you probably have it from a different perspective but starting with you Dr Dickerson is there are there any markers to indicate to people okay this is early mid or or later stage the key is loss of independent functioning so the way I like to think about it is if you can't work anymore because of your mild cognitive symptoms that are making it hard for you to be able to get your job done and you have to take a medical disability you are probably at the very mild stage uh if you have a little bit more trouble and maybe you really are getting lost and it turns out that you can't drive independently any longer in a safe way that's probably at the mild stage but you may still be able to sit and have a pretty reasonable conversation with people about complex topics at the moderate stage people are starting to really have difficulty with what we call instrumental activities of daily living like using uh devices in the kitchen or around the house or dealing with things outside the house independently once you start to have trouble in general and this isn't true for exactly everybody but in general with basic activities of daily living like getting dressed and taking care of your bathroom routine and making sure that you eat that's usually the severe stage and that stage for some people especially younger people can last for years it can last for a lot longer than you would expect which is why it can be tough for families to try to figure out you know how much longer do does this person you know likely have to live because you know they seem so impaired relative to their previous status and we just don't know what the indicators are and so that's where I think uh you know like Katie was saying getting a palliative care consultation can be invaluable in trying to plan ahead and trying to predict what's the likely lifespan that this person may have left and it's always a range we never know for sure but uh it's a long journey even at the end stage for many people yeah and as Chris um points out he said another problem ologists blame your problems on mental illness instead and sunshine and I I'm sure that is true we hear that all the time I was diagnosed with depression as we were talking about so how can people families caregiver you know families um really Ensure if if the neurologist isn't diagnosing right and saying it's depression um go on these antidepressant medications really what what can people do um and Katie you brought up the really good point is go with your gut if something's really not what you think it is keep pursuing but you know any recommendations for all of these people out there saying it may be called something cognitive I mean Katie I know it was your mom who said I think you should get them checked out I remember her saying that to me I think it was AF it was early on the he had an appointment with his primary care and I said to my mother you know there's nothing wrong with his brain he's just being annoying and she said you've got to ask the primary care but you know being in the room with him at that appointment asking that question it made my wheels start to turn I do just want to say one thing because it's so hard I've seen so many families come into clinic and I remember the feeling too you want that answered the day that you have the appointment and I just want to say that it really becomes a partnership between patients caregivers and clinicians and sometimes it is time that helps clinicians really see how does the condition present itself and so for caregivers family members I tell them you know take a quick Journal like instead of vaguely saying how you think your loved one's different if even you had your phone open and you just wrote the date and you know loved one hasn't brushed his teeth in four days loved one forgot to pay the electric bill and he's always paid it on time like just writing those things down and looking for patterns I think can help you communicate to medical professionals why you're so concerned that's a really really good good tip um another question is what advance directive do you recommend um so these diseases don't last for for so many years now I mean there's you know I mean dementia is not classified as um that that gets a little bit complicated but like in terms of advanced directives what should people think be thinking about when they're in the early stage of the disease in the Dickerson lab I'm actually um working on a study about caregiver understanding of the severe stage of dementia and medical decisions that need to be made at that time and we're still recruiting so anyone can send me an email if they want to participate um but really this is where palliative care can come in so if you're worried about medical intervention that may impact your loved one's life in a way that doesn't match your values this is where the palliative care and hospice providers can help and just being very open and direct with your primary care physician and your neurologist about what are your hopes for your loved one's life and yours yeah um Donald has just written in early onset can you explain nighttime disorientation is that common with FTD Dr Dickerson I think it's common with all dementias where people uh really lose track of their sense of time and also often don't really recognize the cues that we all depend on in order to keep track may not be able to really tell time anymore may not be able to recognize that what the what the light or dark outside the window means and so it's very common I think in any kind of dementia I don't think it's particularly more common in FTD than it is in Alzheimer's or Lewy Body dementia but this disruption of people's sense of day and night can be so hard on family members because obviously people don't no one does well without a decent night's sleep and and that can be one of the biggest challenges when caring for a person with any type of dementia so I think that that's the kind of thing that is important to talk to your doctor about and there aren't great Medical Treatments for that I think a lot of what Katie and I work on with family members who have that difficulty is trying to figure out what kind of structured routine could they put in place to help keep the person on track in ways that help them recognize when it's time to get ready for bed and and time to stay in bed you you bring up a good point though what are what are some of the from your perspective what are some of the pain points that come up the most um I know there must be a ton of them but you know what do you hear the most that would be beneficial to our audience um of people really wondering how to manage basketball you know uh what are some of the the the things that you you know continually is it sleep is it I mean Behavior what are things that people should know about ahead of time well I'm sure Dr Dickerson will speak about the medical things but I plant the seed that you know your loved one was living likely a rich full life before this diagnosis where they went out in the world they worked they had friends outside of of you their primary caregiver and so helping them to maintain that whether it's through participation in an adult a health program a connection with a companion or a caregiver that understands their condition and can bring them out to engage in activities that are meaningful to them and with purpose um I think that nobody wants to be bored everyone wants to feel that their life has purpose in meeting and that includes people living with a diagnosis of FTD that I I have to interject here one more question um Barbara said how did you explain Mike's condition to young family members and I know you had a young son and that I mean that that's hard how and in the fact that FTD is impacting younger people means children you maybe having the conversation with children how do you go about that it's a lovely book called um Ellie's butterflies that's written about a little girl who has a grandfather with FTD but you know one of the things that I really noticed talking with my son is when he's very young actually he didn't have a lot of questions he just accepted that his dad and his grandfather were as they were that's their they weren't good at remembering things in fact his dad didn't talk for most of his life um but Noah remembered his dad laughing a lot and he just accepted him that way I often think it's children who are Elementary age and above that need more explanation about you know something is going on and daddy or uh Grandma's brain and they need extra support and these are the ways that we can help them because I think kids want to feel like they know they can be helpful too okay and then because I know I'm taking up I'm being very selfish with your time we're almost done but we have so many questions that I just wanted to let's let you know get to all most of them but Dr Dickerson so what do you think from a physician's point of view what are some of the questions that you're being asked over and over again um you know this could possibly help people other doctors who might have FTD patients in the future what are some of the patterns of things that come up all the time well I think uh at at the end of the day one of the things that is most upsetting to most people with with living with these conditions is the disruption of their their relationships with their loved ones and I think that there are a growing number of efforts uh not just pharmaceutical treatments and and the development of new medications but a huge number of ideas that are being developed and worked on for Behavioral intervention strategies and other ways to try to promote better quality of life for a longer period of time even if the disease is continuing to progress and we're seeing a lot more funding from the National Institutes of Health focused on not just Alzheimer's disease but the Related Disorders in FTD is one of the big Related Disorders and so uh that's targeting not just the understanding of the science of FTD but also better evidence supported ways to live with FTD with within families and within communities and and I think that every year we see the funding go up and I think that brings more uh people with creative ideas into the field and I think we're going to see more efforts to really impact people's daily lives even while we're working on trying to develop better treatments of the future okay well I want to say um wholeheartedly and I mean this we talk to a lot of experts there's no better place for FTD than Mass gen and your team and I'm hugely grateful to both of you for giving us so much time I know you know not everybody has access to Quality Care that that mass gen gives um so it's wonderful to even be able to have the opportunity to answer some of the questions that are out there so thank you so much um Dr Dickerson Katie thank you so much for joining us I really can't thank you enough Deborah thank you for the work that you do to help bring the right information to families and to help families raise their voices I really appreciate it thank you so much Deborah we appreciate it okay and so for if you missed any of this interview we're gonna post it on beingpatient.com you can watch it again it's on our YouTube channel um keep sending your questions in it's our job we we want to get answers correct answers from the experts out there to your questions and thanks so much to our guests and thanks everyone for watching we'll see you next

Channel: Being Patient Alzheimer's

Views: 3,510

Keywords: beingpatient, alzheimer's, dementia, caregiver, braintalks, ftd dementia, ftd facts, frontotemporal dementia, frontotemporal dementia patient, frontotemporal dementia 60 minutes, dementia patient remembers, dementia gaming, dementia song, alzheimers disease

Id: Sr1e2fv1RCo

Length: 52min 27sec (3147 seconds)

Published: Mon Mar 13 2023