Our journey with Behavioural Frontotemporal Dementia (BvFTD)

By Beth Sunshine Farrell

IT didn’t happen all at once. There was no single moment where I realised something was wrong. Instead, it was small things that I could rationalise or explain away. A change in how he spoke to people. A reaction that didn’t quite fit. Another nap needed during the day. A faux pas here and there. A sense, impossible to name, that the man I thought I knew so well was beginning to feel just slightly out of reach.

What was happening didn’t look like memory loss, confusion, or anything people commonly associate with dementia. It looked like not always caring about the people around him. A gradual retreat from wanting to socialise. Random actions that didn’t match his ethics. Moods that didn’t fit his personality. But worst of all, it looked like his behaviours were his choice.

My husband, Mark, is still here, but he is now a shell of the man I married. Long before I had a name for it, I was living with something I didn’t understand. There was an inchoate sense that something wasn’t right. A feeling I couldn’t yet articulate but couldn’t ignore either. I would say, ‘Who are you and what have you done with my husband?’ He would shrug, nonchalantly.

Our daughters began to feel an emotional distance from a once loving and doting dad. He seemed indifferent to their needs. He was there, but he wasn’t there. Our family was being irreparably damaged right in front of my eyes, yet I couldn’t step back far enough to see what was happening. Everything unfolded so gradually, I couldn’t make sense of it at the time.

It didn’t seem like something that warranted medical attention, until subtle cognitive issues began to emerge. Then I went looking for answers. Two years later we finally received a diagnosis of Behavioural Frontotemporal Dementia.

I read the list of symptoms, and with hindsight, everything became crystal clear. The slow and steady changes reached back more than a decade.

I read ‘loss of empathy’ which was suddenly so obvious. But at no earlier point could I have recognised that my husband had lost his empathy. I had no concept that this could happen to person. The emotional absence, not through choice or intention, fully explained the way he had treated his daughters.

I read ‘loss of social etiquette’ and remembered pointing out to him that he hadn’t used the words please, thank you, or sorry, for years. I didn’t know what to make of that realisation at the time. I had received the nonchalant shrug that was becoming his typical response. There had also been countless embarrassing moments where his social filter went missing.

I read ‘apathy’ which helped to interpret why he had let go of his successful tree surgery business and since struggled to hold down a job or solidify his ideas for new initiatives. This had been deeply out of character for a man who had always been highly intelligent, capable and motivated. It also clarified my confusion as to why Mark’s lifelong passion for his martial art, ninjutsu, had quietly slipped away.

At the time, all this had looked like a loss of motivation. Therefore, it was incorrectly diagnosed as depression, despite his insistence that he was not depressed or sad. Mark said he felt numb when asked about his feelings. While numbness can be interpreted as depression, this ‘emotional blunting’ is a sign of FTD.

One important factor that hindered an accurate diagnosis was that Mark truly didn’t believe anything was wrong with him, or his behaviour. He couldn’t see that his words and actions hurt and humiliated people. He genuinely thought he was always right. Everything was just fine in his world. This symptom is called ‘anosognosia’.

While this was frustrating at the time I now see it as the silver lining of the dark cloud that is BvFTD, because Mark still doesn’t know he is ill, despite being in the later stages of the disease. This leaves him blissfully unaware, making ‘anosognosia’ a sweet form of protection for him.

I could never have described what was happening during those early stages. I simply didn’t have the awareness or the language for it then. This is another barrier to diagnosis. The disease was elusive to me, to Mark, and to the practitioners we consulted. So why am I telling this very personal story now?

RAISING AWARENESS

Because I want to spare another family the turmoil we went through. I want to raise awareness about early symptoms so the next family might recognise the signs and seek help sooner. If we had known such a disease existed, we could have been spared years of confusion, heartache, and upheaval that devastated us in so many extraordinary ways.

Equally, if our GP had known about BvFTD, I believe there could have been better outcomes for us all. GPs often struggle to identify early-onset dementia, which is commonly misdiagnosed as mental health issues. This is precisely why medics need to know more about the potential symptoms of FTD.

GPs are also the gatekeepers to other services that could uncover the truth behind a patients’ problems. Their referrals are crucial to the diagnostic process. Mark’s anosognosia meant he presented well at appointments, highlighting the importance of taking family members seriously when they are expressing concern about a loved one.

We have been altered by this journey in profound ways. While we are beginning to bounce back, there will always be a deep sadness and a shared bond with every family who has experienced the ambiguous loss and grief that comes with dementia. However, there is something uniquely difficult about BvFTD.

When we finally received a diagnosis, learning that Mark’s challenging behaviours were not a matter of choice or personal intention, he was already losing his communication skills and comprehension of language. He had masked this degeneration very well.

One of the cruellest aspects of BvFTD is, by the time you work out what’s going on, relationships have been damaged. It can seem too late to make amends. Suddenly, history is altered. Everything must be reframed. It wasn’t really him! It wasn’t his fault! Simultaneously, you are faced with the reality that this condition is life-limiting, progressive, and incurable!

FTD BROTHERS

Part of our recovery has been discovering the FTD Brothers and realising that we are not alone. In that shared space, there is comfort. Not in comparison, but in recognition. For so long, we felt isolated in what we were experiencing. Finding others who understand is helping our daughters begin to make sense of it all, and to feel less alone in carrying the pain.

But the FTD Brothers have an additional burden. After joining their dad and sister in caring for their mom, Geraldine, until she passed away from familial FTD, aged 52, they learned that they’re likely to develop this type of dementia in their 40s. I can only begin to imagine what they went through on discovering their genetic connection to the disease.

However, Jordan impressively decided to embrace this, and now refers to his potential fate as a “Licence to Live!” Cian has joined him in the most incredible work, raising awareness and funds for dementia research. Their sporting feats are phenomenal, and they just don’t seem to stop running.

Jordan ran the recent London Marathon carrying a fridge on his back! The 25kg fridge was an important analogy for the load carried by dementia carers. It was such a strong way to portray the message and garner attention. Which it certainly did. You got it over the line, Jordan! Fantastic!

Our daughter, Iris, has been inspired and has been training to run with the brothers in Mayo since she discovered them and their Irish challenge. Iris has said every time she feels like giving up, she thinks of her dad and their mom, and all the other amazing people who have been lost to dementia. This is what gets her through each run.

Our eldest daughter, Anna, is now travelling from the UK to join the Mayo marathon in support of her sister and the FTD Brothers. Also, Mark’s devoted father, Tom Farrell, who writes to him every week from the UK, will be running for the cause in his hometown. A former Olympian hurdler now aged 93, Tom is extremely keen to raise money and awareness for FTD.

Mark’s devastated parents visit regularly. They still find it hard to accept that this cruel brain disease began stealing their son in his 40s, to be diagnosed aged 54. It crept in like a ninja. Silent, subtle, and so destructive. It took hold before we even realised it was there.

As for me, I’m not a runner, but I wanted to use this opportunity to thank the FTD Brothers for bringing this cruel disease out in the open and uniting people who have suffered their own version of its harm. I saw their sister, Kennedy, say no one will really understand unless they’ve been through it. We felt the same. I hear you, Kennedy! We get it!

Our family has so much respect and admiration for those who care for their loved ones at home 'til the end, like the Adams family did for Geraldine. Our circumstances haven’t allowed for that. We are now so grateful for the kind staff at MacBride’s Community Nursing Unit, where Mark resides in Westport, and to those at Pilgrims Rest Nursing Home, who got Mark back on his feet after the collapse which led to his diagnosis.

We also couldn’t manage without Mark’s amazing personal assistants from the Irish Wheelchair Association, who spend quality time walking, talking, reading, and singing with him when we cannot be there. Their presence brings connection and comfort in ways that are hard to measure.

Social isolation for people living with dementia is an overlooked issue. Healthcare workers and nurses should be afforded more time within their working systems to fully provide the social care essential to a person’s dignity and wellbeing. I would like to see a world where everyone has access to this kind of support and are accepted by their community despite their illness.

So please, be aware that odd behaviours displayed in public could be unintentional, due to a neurological condition. And if something you are seeing in a loved one doesn’t quite add up, don’t ignore it. Trust that instinct and seek help early. I urge everyone, including medical, social, and mental health practitioners, to learn about the full range of FTD symptoms and its different forms. Go to theaftd.org for reliable information.

No two experiences are the same and I’ve only touched on some of the early signs. I hope that by sharing this small part of our story, I encourage self-education of FTD as a precautionary measure. A dear friend of mine was able to recognise the disease in her mother because she knew about Mark’s condition. Her confusion and frustrations could have continued for many more years without that knowledge.

FTD strips individuals of their humanity, emotions, and personality, while tearing families apart. Awareness can alter the course of that journey. It may not change the diagnosis, but it can affect how quickly it is understood, how well it is supported, and how much unnecessary pain is avoided.

If this article resonates in any way, come out to support the FTD Brothers who appreciate cheers, funds, and fellow runners, for all or parts of their many marathons. Jordan needs our encouragement to complete this remarkable challenge!

Visit theftdbrothers.com to find the 10 a.m. start location and route of their marathon in your county town: May 8th in Sligo, May 9th in Mayo, May 10th in Leitrim, May 12th in Roscommon, and May 14th in Galway.