The family’s journey to a diagnosis of FTD.
Stage I. Something is wrong
Whether initial symptoms are word-finding difficulties, a change in energy level, a few lapses in judgment, or atypical or odd behavior, the earliest changes are subtle and difficult to recognize. More to the point, when viewed in isolation or as an infrequent occurrence, all of these are things that any person might – and most of us do – experience occasionally. What person doesn’t feel the need to be a couch potato once in a while? Indulge oneself with an impractical purchase? Forget a word? Make a decision that turns out to be imprudent? One of the greatest ironies of FTD is the fact that, as it begins to steal the very aspects of a person that make them the unique human being they are, it is that very uniqueness that masks the fact that something has begun to go wrong.
It is also a fact that many families, reflecting back in search of signs of when the disease began, discover that friends and co-workers noticed some of the earliest signs, but were reluctant to mention anything. Again, there is some irony in the fact that it is often informal social rules – we are reluctant to point out when a co-worker does something wrong or embarrassing; we don’t want to insert ourselves into a marriage that may be having troubles – that prevent earlier recognition that there may, truly, be something wrong with a person we know.
After we shared the diagnosis of FTD with her co-workers, stories began to emerge, about mishandled clients, lost documents, and missed deadlines. No one had said anything at the time.
After my husband’s diagnosis, two friends who I was estranged from came forward and shared nearly identical stories, about how he had made sexual overtures to each of them. Rather than tell me, they just disappeared from our lives.
I wish I had known at the time. Though it would have been painful, it would have been reassuring to know that I wasn’t the only one who was noticing a change.
Stage II. Maybe there is a medical problem
At some point it occurs to someone – most often the spouse – that perhaps these strange changes in behavior are not willful, but rather that there may be a brain disorder. Occasionally it is the person him or herself who initiates the search for a cause for these incipient changes. But because the symptoms are fairly intangible, combined with the typical younger age of onset, and the fact that many general physicians, or even community neurologists and psychiatrists, are not familiar with FTD, often the family is sent home without a specific diagnosis.
I knew something was seriously wrong, but it was almost impossible to make other people understand the most troubling changes. They thought I was crazy.
Mom was complaining of word-finding problems, and there were increasing pauses in our phone conversations. How to explain that this didn’t feel like a “normal” memory problem?
The doctor was hearing two different stories: Mine was one of bizarre changes, frustration, and confusion; his was one of a nagging wife, dissatisfied with midlife. Which of us would you believe?
I was several states away and couldn’t seem to marshal the local resources to get Dad to a doctor who would understand what had changed.
Stage III. A series of medical professionals and opinions/diagnoses
Many marriages and other family relationships do not survive this far. Those that do face additional hurdles in this stage of the disease – one in which: individuals with the disease most likely do not recognize they have changed; co-workers and friends who experience the symptoms may be reluctant to point them out and, instead, start to distance themselves; and medical professionals, practiced in diagnosis of psychiatric conditions or Alzheimer’s disease, either conclude that there is no diagnosis or prescribe treatment for the wrong condition. It takes a tenacious caregiver, confident they know the affected person best, convinced there is something truly wrong, to keep making appointments, to keep telling their story, and to stay in the relationship.
It took three visits to our general practitioner before I could convince him that something was really wrong with Mary.
They tested for cognitive issues. They tested for memory. They did a brain scan, looking for tumors. But every test came back negative – there was nothing wrong.
I encouraged him to go to the doctor, who put him on meds for low testosterone. A few months later, with no relief from his strange behaviors, I encouraged him to go back and this time the doc said, “depression,” put him on meds, and said to come back in a year. I knew the year recheck wasn’t right so I found a psychiatrist that he began seeing, who upped his depression meds. Still no improvement.
It took a year and a half to get Clark’s diagnosis. The first five physicians that we went to had little or no knowledge of FTD. I myself have been an RN for seven years and I had never heard of FTD either.
Stage IV. Referral to a physician familiar with FTD
Certainly, not all families have such an extended path to the diagnosis. Some are lucky enough to have a family doctor who is familiar with the disorder; others live sufficiently near a major dementia center and referral to a medical team familiar with FTD occurs rapidly. Many others discover the diagnosis themselves, on the internet. But often this circuitous route to diagnosis has consumed the last, best years the patient and family have together.
June of last year his boss gave him a letter stating that if he didn’t improve (his interpersonal skills, teamwork, follow-up, etc.) he was going to lose his job. This letter was an eye-opener because it confirmed that his behavior and personality changes were noticed outside of our home, as well. We went back to our GP, who finally referred us to a neurologist at a dementia center.
When I typed her list of symptoms for an internet search, up popped FTD. It was like fireworks going off: I went right down the page, checking off every feature listed on the website.
What a relief to find someone who understood what we were experiencing, and who could put a name to what was going on.
Stage V. The diagnosis is FTD
And so, having finally arrived at a diagnosis of FTD, where does this family find themselves? They are branded with a label they most likely have never heard of before, with little information as to the prognosis, course, and timeline ahead. They are told there are no disease-modifying treatments; there is no cure. Perhaps they are informed that the diagnosis remains somewhat tentative, and will not be confirmed until autopsy. The only solid fact that can be presented to this family is how the journey will end.
The situation is daunting for clinicians as well. A primary goal of clinical medicine is cure or at least treatment to modify the disease progression. Yet at present, we still have no definitive drugs and few research treatment studies for FTD. We try medications developed for other disorders, or suggest non-pharmacologic behavior management strategies, but these techniques have received little systematic study. Unfortunately, owing to the complexities of reimbursement within today’s healthcare system, clinicians are saddled with time constraints, making it difficult to develop individualized plans for patients and families. And yet, there are things you can do that will make a tremendous difference in the quality of this journey for yourself, for the family, and for the patient.
Managing the disease through partnership
The single most destructive aspect of FTD is the isolation it creates for the patient and family. At this point in the journey, despite the lack of approved treatments and definitive prognosis, the mere fact of a diagnosis can bring tremendous relief. Knowing there is a medical name for the symptoms is validation. The connection with a clinician or clinical team that understands the symptoms and their ramifications is comforting. And the realization that there is a broader community out there that has walked this path before brings affirmation and hope.
Despite the lack of traditional tools in a physician’s armament when facing the task of managing a patient with FTD, the primary clinical care team has an opportunity to set a more positive tone from the start. Invite family caregiver(s) to be full partners in any care plan. They are the ones who know the patient best and who are on the front line in terms of reporting troubling symptoms and disease progression. They are the ones who will implement any treatment strategy, and be able to report whether or not an intervention is having effect. Also, be open to the possibility that the patient him or herself may retain the insight and communication skills to play a role in setting treatment goals and strategy.
Building this partnership will necessarily entail listening carefully to what patients and caregivers say, helping them understand the disease process, and referring them for individualized assistance. Despite the lack of specific prognosis, there are themes to this journey with certain predictable factors and challenges the clinician can recognize. As our communal experience with FTD grows each year, there is an ever-expanding world of resources and strategies for the care team to employ. Working with patients, families, and clinicians, the Association for Frontotemporal Degeneration (AFTD) in the USA has developed guidelines to address many of these challenges, including steps to take after receiving an FTD diagnosis, ways to help young children and teenagers cope, and information about the genetics of FTD. A complete list of resources appears in the section Creating a broader community for resources and support, below.
Avoid nihilism after the diagnosis
As patients and families gradually realize FTD is a progressive condition and cannot be cured, they begin to alter their future plans. Clinicians, too, need to adjust their approach to manage behavioral, language, and other cognitive changes associated with FTD. Patients and families want and deserve an honest discussion of the current state of knowledge and lack of treatment options, yet it is important to also communicate what can be done after delivering this distressing diagnosis. Families benefit greatly from a continued “emotional presence” from clinicians knowledgeble about the disease. This commitment prevents feelings of abandonment described by many families who receive the unhelpful message: “There is no treatment, no cure, and nothing more I can do.”
Identify a collaborative team
Facilitate the transition from diagnosis to management by identifying an appropriate care team. For example, specialized FTD programs in university-based settings may elect to coordinate all FTD-related care or may serve as consultants with patients’ primary care providers, speech therapists, nurses, and social workers by clearly documenting their assessments and recommended plans of care. Members of the treatment team then learn from experienced FTD clinicians through these consultations as they provide ongoing management within routine health management.
Families, too, are an integral part of the team, and appreciate clinicians who allow them roles as true partners by carefully attending to their observations as symptoms and behaviors evolve during the disease course. Since patients themselves generally do not recognize behavioral and cognitive changes caused by the illness, families seek ways to alert clinicians about these changes without making patients angry or causing them unnecessary grief. It is often helpful to speak with family members alone for a few minutes while patient vital signs are collected, or ask for a brief written description of observations and concerns. Finally, it can be difficult for family members to convince many FTD patients to agree to follow up with clinicians as they neither feel sick nor believe they have problems. Family feedback reveals that patients agree to appointments because they like going to see their clinicians, particularly when clinicians and patients themselves forge a bond.
Related posts:
Practical management of frontotemporal dementia
The FTD-ALS spectrum
Neuropathology of frontotemporal dementia and related disorders
Functional disability and the impact of frontotemporal dementia in everyday life
Historical introduction to FTD
Overview of frontotemporal dementia and its relationship to other neurodegenerative disorders
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