Frontal Temporal Degeneration The impact for sufferers and their often young families. By Cindy Penny
2017 World FTD Awareness Week : September 24 to October 1, 2017
Younger Onset Dementia • Often occurs in 40’s and 50’s, but can occur from 20’s. • FTD most common form of dementia under 65 • Inevitable deterioration in functioning over 2 to 13 years (average is 7) • Affects work and family in a way dementia in older patients does not.
FTD vs Alzheimer's Dementia Characteristics FTD AD Age of Onset 30 to 60 Over 65 Memory Deficits Late Early Executive Function Deficits Early Late Behavioural Disturbance Early Late Brain Imaging Frontal temporal Atrophy Diffuse Atrophy
FTD – 4 sub-types 1. Behavioural variant FTD – “Picks Disease”. 60% of people with FTD. Affects social skills, emotions, personal conduct and self- awareness. 2. Semantic – can speak but lose understanding of the meaning of words
3. Progressive non-fluent aphasia – opposite of Semantic type. Have difficulty producing language fluently, but still know the meaning of words. 20% 4. FTD with Motor Neuron Disease – 15% of patients will also developed MND. Most often, this combination occurs in patients with bvFTD.
Early Signs bvFTD (Pick’s Disease) • Change in usual character and habits • Lack of inhibition or social tact • Obsessive or repetitive behaviour • Weight gain due to dramatic over-eating • Neglect of personal hygiene • Failure at work • Emotional coldness • Apathy
Early signs of Pick’s Disease • Driving style changes. There will be a few near misses at corners. Reaction times slower. • May develop obsessive routines. • Needs a lot more sleep, and day time sleeping. • Simple repetitive movements –finger tapping
Social Cognition The processes by which people understand themselves and other people. This includes: • Awareness of self • Awareness of other’s thoughts, feelings, emotions “theory of mind”. • Knowledge of social norms and expectations (i.e., ethical and moral behaviour)
First Person Accounts Howard, 54 Teacher Lee, 54 Operations Manager Dr Bob Fay, GP Brandt, 54 Director of Dev, General Hospital Diana, 45 Senior Vice President
Howard 54 Teacher “ My filter is going…If I’m with people I can control it more. But, if I’m by myself I’ll say anything….ask a 7-foot guy covered in tattoos why he has a cancer stick hanging out of his mouth”
Lee, 45 with bvFTD, Operations Manager “ My attitude at work had become atrocious. I went in late and left early. I just didn’t want to do anything……I found it difficult to complete my job duties, and would surf the web for hours at a time. “When a colleague came to me to get a copy of a computer program I had written a year or so before I knew I was in big trouble. When I look at the code I had written I couldn’t understand it….I WAS SCARED .
Dr Bob Fay, bvFTD, GP “ It has changed me in subtle ways that outsiders find very difficult to understand, but are all too apparent to my wife and family. It has cut short my career as a GP; it has stopped me from driving; it has caused much grief to my family.” “If I’m contradicted I have the greatest difficulty restraining myself, and can suddenly get very angry” “She feels I am not the person she married.”
Dr Bob Fay GP “The true sufferer, as you may be beginning to see, is not the patient, who is relatively indifferent to it, but it is the carer.” “It is so hard for people to realise that in the early stages one may have a very real handicap, but still seem normal”
Brandt, 55 Director of Development , General Hospital “I just quit caring so much” (Disinhibited behaviour began. Loss of filter) . “I would look back on a week of few accomplishments and vow to do better on Monday, or after vacation.” “I was having explosive outbursts of anger”
Diana , senior vice president, single . “ I was having problems completing tasks at work; I’d lose my focus. I finally decided something had to be done.” “My executive functioning was compromised and I completely lost the ability to multi-task; that’s FTD.” “ I had a high IQ, so I’m ahead of the other dingdongs out there….but in all seriousness, It’s lonely, that the worst part of this disease.
Cognitive Reserve Study • To find out if higher education and occupation attainments may help persons with FTD .
Findings • Education and occupation (higher level of cognitive reserve) successfully compensate with FTD, and need more advanced pathology before they exhibit clinical symptoms • Results suggest that compensatory mechanisms are in action almost 20 years before disease onset, and that they involve different areas moving from preclinical to symptomatic stage, probably due to depletion of scaffolding.
Findings • Cognitively stimulating lifestyle results in greater elaboration of synaptic networks. • Opens new perspectives in terms of symptom prevention and delaying. This is particularly relevant for individuals carrying pathogenetic mutations who will certainly develop FTD at some point in life. • Need research on whether cognitive interventions can delay progression in early stage.
Causes • 10-15 percent - autosomal dominant pattern of inheritance. This means that there is a specific genetic mutation that can be passed from generation to generation. In families like this, each first degree relative of a person with FTD (e.g., sibling or child) has a 50 percent chance of developing FTD.
Early Diagnosis Important 1. Leads to timely caregiver education and family counselling regarding disease 2. Early institution of any evidence-based supportive therapies for bvFTD. 3. Prepares caregivers for the psychological and economic demands.
Urge patients/families to push harder for diagnosis • Often misdiagnosed – depression, bi-polar, Asperger's, schizophrenia, even as hearing problems • Undiagnosed Pick’s is a disaster waiting to happen.
Impact for Family • FTD gradually erodes the qualities that are required for people to enjoy mutual relationships. • Adults often say the person they loved “fades away” • To the outside world the person with FTD may seem normal... • Friends and relatives become uncomfortable spending time with family.
Impact for Family • Grieving goes on for a long time as the person progressively loses abilities. • Financial – usually person was working with commitments such as a mortgage, dependent children, possibly dependent parents. • Roles and responsibilities change
Impact for kid/teenagers • Embarrassment • Anger towards parent with FTD and other parent for marrying them. • Guilt • Resentment – “parent with FTD just does not care”. Other well-parent not as available. • Fear • Sadness
Impact for Kids/Teenagers • Cancer envy • University students begin to hate returning home in holidays. “The worst they will ever be and the best they will ever be”. • Feel isolated and marginalised • Frustration!!
What can help for them? • Speak openly about feelings • Finding others in similar situation • Extracurricular Activities • Memory Book
Caregivers • Can experience loss of emotional attachment to spouses due to behavioural symptoms. • Loss of previous identity • Special attention and support should be offered to caregivers early in disease process to identify coping strategies, provide alternate interpretations and help them articulate the loss of shared meaning and emotional capacities between them and their spouses.
Resources/Support for Families 1. Dementia Auckland 2. Facebook Groups – The FTD Spouse – The Association for Frontotemporal Degeneration – Frontotemporal Dementia Carers Clinical Support Group Australia 3. Other online supports: – www.aftdkidsandteens.org – www.theaftd.org – www.ftdtalk.org – www.ftdsupportforum.com
Further Educative Resources 1. “Failing frontal lobes" - very informative podcast interview with Bruce Miller, director of UCSF Memory and Aging Center. www.bluefieldproject.org/news/ftd- podcast-with-dr-bruce-miller. 2. “Looks like Laury, sounds like Laury" - a documentary about family, friends and FTD. https://vimeo.com/101447352 3. "It is what it is" -18 minute film chronicles the lives of four families confronted with FTD. https://www.youtube.com/watch?v=drgzhKe_YWI
References Serggio c Lanata and Bruce L Miller. 2015 The behavioural variant frontal temporal dementia (bvFTD) syndrome in psychiatry. HHS Public Access . Massimo L(1), Evans LK, Benner P. 2013 Caring for Loved ones with frontal temporal degeneration : The lived experience of spouses . https://www.ncbi.nlm.nih.gov/pmc/articles/PMC38672 67 /
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