Partners in FTD Care, Fall 2020
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Primary progressive aphasia (PPA) is a neurodegenerative disease that affects the parts of the brain responsible for speech and language, resulting in the gradual loss of the ability to speak, read, write, or understand what others are saying. Researchers divide PPA into three subtypes, including logopenic variant PPA (lvPPA), which is mainly characterized by difficulty with word-finding, resulting in frequent pauses while speaking. People with lvPPA, however, generally recall the meanings of words, unlike other types of PPA. While knowing the differences between PPA subtypes can be scientifically valuable, families living with PPA are better served receiving support and advice for the road ahead. The case of Tami W. demonstrates how ongoing engagement with knowledgeable professionals can help persons diagnosed with PPA and their care partners adapt to symptoms as they progress, as well as teach them how to cope with related challenges and identify means of support.

The Case of Tami W.

Tami W. works at a sports management firm in a major U.S. city, where she is one of its top agents. Never at a loss for words, she is known for her outgoing personality, witty banter, and unique ability to connect with the athletes she represents. But in her early 50s, she began having trouble speaking. Once praised for her knack for speaking off the cuff, even in front of large crowds, she now struggled with finding the right words, resulting in long, awkward pauses in conversations with clients. In public and especially at work, Tami felt increasingly uncomfortable and embarrassed with her language struggles.

While home for the holidays, Tami’s 24-year-old daughter Devon noticed that her mother was speaking with unusual hesitance and frequent mid-sentence pauses, as if she could not find the right word to say next. Devon, a recent college graduate who lives four hours away, did not recall her mom having such difficulties when she visited last year. Devon also realized that Tami, who recently turned 52, had stopped calling her on the phone in recent months, instead choosing to hold conversations via text—were her new language difficulties to blame? When she asked her mom if she was having trouble thinking of the right words during conversation, Tami acknowledged that she had, and that she worried that her problems could affect her job.

Together, Devon and Tami did some research online, which further concerned them. They asked Tami’s partner, Jessie, if she had noticed anything. Jessie was tentative at first—she knew how proud Tami was of her public speaking ability—but soon acknowledged that Tami had seemed less confident in her speech lately, and was at times unable to produce words, instead using placeholders like “thing” or “whatchamacallit” to refer to objects. Tami was not surprised to hear this; she had been worried that Jessie was aware of her recent language difficulties. All three decided that a doctor’s visit was in order.

Devon and Jessie accompanied Tami to her appointment. After some routine tests, her primary care
physician attributed the her language changes to job-related stress coupled with increased anxiety due to menopause. The doctor prescribed an SSRI to help Tami cope with her symptoms, and scheduled a six-month follow-up appointment. While Tami felt her symptoms were caused by more than just stress, she decided to try the medication anyway.

Searching for Answers

Several months went by, and Tami’s language difficulties did not wane. The medicine seemed to make no difference. She felt growing anxiety in her job, which is heavily language-dependent; she feared that co-workers and clients could notice her struggling to find words during meetings. Her conversational skills had long been an asset and a strength—now that talking was becoming harder, Tami experienced feelings of worthlessness, a sense of disconnect from her work, and depression.

Devon, who had started visiting more frequently, noticed her mother’s condition was worsening. Her inability to remember words mid-sentence grounded conversations to a halt, and she had begun responding to questions with “yes” when she meant “no,” and vice versa. Devon knew this was more than stress. Determined to find answers, she decided to seek a second and more specialized opinion. After extensive research, she contacted an institution that uses a multi-disciplinary approach to care, offering a more robust care team to provide her mother the most accurate diagnosis and personalized care.

At her first visit to the center, which occurred approximately two years after the onset of language difficulties, Tami underwent a series of evaluations, including a neuropsychological assessment and an evaluation with a behavioral neurologist. Imaging, blood tests and a detailed medical history were taken to help “rule in” and “rule out” different causes of her language deficits. Neuropsychological testing revealed relatively isolated deficits in language, but preservation of other thinking skills, such as memory. The neurologist found no evidence of stroke, tumor, vitamin deficiency or other potentially reversible causes of her language challenges.

This information, in concert with the family’s description of gradual, progressive loss of language, led her neurologist to make a clinical diagnosis of primary progressive aphasia (PPA). PPA is a syndrome caused by a neurodegenerative disease that currently has no cure. Tami’s language challenges were most consistent with a subtype of PPA called logopenic variant PPA (lvPPA, also known as PPA-L). More specifically, people with lvPPA have difficulty recalling the names of objects and/or thinking of words in conversation, but still understand what those words mean. Persons diagnosed with any PPA subtype can expect to continue losing language skills; because the disease is progressive, it will eventually spread beyond the language areas of the brain, resulting in further changes in cognition and even behavior. Nevertheless, Tami’s diagnosis gave her relief: Finally, a concrete reason for her language struggles.

Establishing a Care Plan

Tami next met with a speech-language pathologist (SLP). However, the SLP, more familiar with stroke aphasia, was uncertain how to help someone with Tami’s condition, so she identified a colleague who was part of a speech and language therapy clinical trial for individuals living with PPA. The trial aimed to identify and understand which communication strategies successfully capitalize on—and compensate for—the remaining language abilities of a person with PPA. It included persons diagnosed with PPA and their care partners, recognizing that communication involves a speaker and a listener—and both would need to learn new communication techniques. After careful consideration, Tami and Jessie agreed to join the trial.

The family was also referred to a local social worker for an in-depth psychosocial assessment, which included an analysis of Tami’s living situation and support networks, as well as the ways that she, Jessie and Devon were adapting to Tami’s changing language abilities and overall functioning. Devon, worrying that her presence at home may become overbearing (and beginning to feel the pressure of putting her own career on hold to care for her mother), said she had limited her visits to every other weekend. Meanwhile, Tami and Jessie told the social worker about the strain that PPA was putting on their relationship. Jessie felt frustrated by Tami’s increased dependency; for example, she had become her partner’s crutch in social situations. And both grieved the loss of the way their relationship used to be. Their emotional and intimate bond was noticeably dwindling along with their ability to maintain fluid, back-and-forth conversations.

The social worker answered the questions Jessie and Devon had about PPA symptoms, and told them to visit AFTD’s website for more information. She also referred them to legal counsel to secure Powers of Attorney for healthcare, finances and estate planning; and connected them to online PPA and FTD support groups to help them navigate Tami’s diagnosis and treatment. Being introduced to this wealth of information helped Jessie and Devon cope. Tami, meanwhile, met with her own monthly online support group for persons diagnosed, fostering a sense of community with others living with the same rare disease.

Stepping Away from Work—and Toward a New Purpose

On the advice of her social worker, Tami shared her PPA diagnosis with her boss at work, who agreed that she could and should continue her work as normal. While her language ability was an issue, she had not lost the other skills—notably her ability to relate to others—that had contributed to her storied success. But over the next year, Tami noticed that exhaustion would set in each workday around noon, making it even harder to find the right words while speaking. Her boss, concerned that she may begin losing clients, eventually asked her to step down from her position and assume a part-time administrative role at the agency. While this worked out at first, Tami’s language decline soon extended to reading and writing, making administrative work impossible, and she left the agency.

Tami applied for Social Security Disability Insurance (SSDI)—her social worker told her that PPA is a qualifying condition under Compassionate Allowances—but her initial application was denied. She appealed the decision, and her neurologist wrote a strong letter on her behalf, clearly stating the neurodegenerative nature of PPA and the underlying diagnosis of Alzheimer’s disease or FTD. The appeal succeeded; SSDI benefits were instated and made retroactive to her first application.

Tami has been able to maintain a sense of purpose in her life, allowing her to manage the grief of her diagnosis. Since the COVID-19 pandemic took hold, she has continued to meet with the speech-language pathologist virtually, helping to improve her quality of life. Inspired by her daughter’s persistence in finding an accurate diagnosis—and hoping to find meaning in the years she has left—Tami volunteered with AFTD and was eventually asked to join the AFTD Persons with FTD Advisory Council, a committee of persons living with a diagnosis who advise the staff and Board and raise awareness of the disease. She has also felt empowered through her work to advance research; after a conversation with her doctor, Tami decided to join an observational research program with eventual brain donation in hopes of helping researchers advance treatments and a cure. Through her volunteer advocacy work and participation in research, Tami has been able to not only feel a sense of personal accomplishment, but also a sense of hope in using her experience to help others in their FTD journeys.

Questions for discussion:

1. How did Tami’s insight into her condition contribute to getting a diagnosis and her management of the disease?
Unlike in other forms of FTD, people living with PPA often retain the ability to recognize that they are changing, and can exhibit awareness or concern for the effect their symptoms have on those around them. Tami, for example, was fully aware of what was happening to her. She joined her spouse and daughter in researching her symptoms online and fully participated in doctor’s appointments; later she helped to develop and facilitate her own care plan. Additionally, her self-awareness allowed her to attend and participate in a support group for persons diagnosed. The group helped Tami gain a sense of community and understanding by relating to others living with a rare, debilitating disease.

2. After Tami’s diagnosis, what approach did she take to seeking help?
Tami initially met with a speech-language pathologist (SLP); however, the SLP, more familiar with stroke aphasia, was not sure how to help someone with her condition, so she identified a colleague who was part of a speech and language therapy clinical trial for people living with PPA. The intervention program included both Tami and her partner Jessie, recognizing that communication involves a speaker and a listener—and both would need to learn new communication techniques. After careful consideration, Tami and Jessie agreed to join the trial and participate in its targeted language therapies, which were adjusted as Tami’s condition progressed. Throughout, they referred to the Winter 2016 issue of AFTD’s Partners in FTD Care for more information about maximizing communication success with speech and language therapy in PPA.

3. What role did support groups play in Tami’s PPA journey?
Support groups helped Tami and her family navigate the PPA diagnosis. Tami attended a support group specifically for people living with PPA, where she formed connections with others facing this diagnosis and learned coping skills. Jesse attended a group for caregivers, and Devon joined AFTD’s Facebook group for young adults. Groups that focus more broadly on FTD and Alzheimer’s provide peer support and care management strategies, and may help families become aware of and prepare for behavioral and cognitive symptoms that may appear over time.

See also:

• Ask an Expert: Is Logopenic PPA an FTD Disorder or Alzheimer’s Disease?
• PPA and Depression
• PPA Subtyping: Helping or Hindering the Understanding of PPA?
• From a Caregiver’s Perspective: Music Therapy and Language Retention
• From a Caregiver’s Perspective: The Importance of Speech and Language Therapy in PPA
• What to Do About… Managing Logopenic Variant PPA
• Download the full issue (pdf)