My experience of living with nonfluent/agrammatic variant primary progressive aphasia: Adaptations and strategies to improve quality of life

Word-finding and maintenance of vocabulary

My SLP initially designed a program of exercises employing functional compensatory strategies to help with the language impairments she had identified. At that stage, I was having increasing difficulty in coming up with the correct word to say or write, and so I was given traditional word-finding exercises such as those involving flash cards or sentence completion. Recent controlled studies have demonstrated the utility of lexical retrieval interventions designed to restore and maintain the retrieval of important vocabulary items for as long as possible in people with PPA (e.g. Croot et al., 2019; Henry et al., 2019). However, with the approval of my SLP, I have chosen not to persist with these exercises: not only did I find them simplistic, but many of the words from these tasks bore little relevance to my interests or day-to-day needs. With the encouragement of my SLP and medical team, I have chosen instead to maintain my vocabulary by working on a series of writing projects that are meaningful to me and contain words that I wish to utilize in other contexts. Each of these projects has occupied me for several months; all have been accepted for publication. In the process of composing, I frequently end up searching for a word, and so I try to stretch myself by jotting down several options and often speak these words aloud to prompt myself. I want to avoid interrupting my flow by spending too long trying to retrieve the perfect word or puzzling over the way to achieve a smooth transition, so if I am really stuck, I will leave a blank space and carry on writing. Several hours or even days after I have set the work aside and stopped actively thinking about it, the solution will come into my conscious mind in a process involving the hippocampus, a region of the brain that has not been affected by the advancing atrophy of the frontotemporal lobe, whereupon I will immediately jot it down or capture it in a quick recording on my voice recorder, so that I can insert it seamlessly into the text. The fact that the process of writing allows word retrieval to be delayed without adversely affecting the final product means that I can still manage to produce good quality written work over a period of time. In contrast, conversation and other acts of speaking usually demand immediate word recall. In those cases, I have found myself spontaneously closing my eyes and twirling my right index finger as I search for a word; other distraction techniques suggested by my neurologist and SLP could involve rubbing my ear or tapping my fingers. Rather than expend too much time and energy seeking the perfect word, I remind myself that it is acceptable to use an adequate word. However, sometimes even a merely adequate word will elude me when I am speaking, and I will have to resort to substituting the word “thing” or simply state that I cannot think of the word and, if appropriate, ask for help. In that situation, I will make it clear that I am requesting help in seeking a word by providing clues according to one or more of the following approaches: describing the function of the word; stating the initial letter of the word; describing the appearance of an object (color, shape, size, etc.); or making relevant hand gestures. This request for help has always been welcomed by the person with whom I am talking, because they would—quite rightly—otherwise be reluctant to interject when I am searching for a word. If I hear or read a word that I realize I have not used recently, I will seek an early opportunity to reintroduce it to my active lexicon. I have recently also started making running lists of synonyms on index cards. I have continued to follow my SLP’s recommendation to challenge my word-finding abilities by engaging in a brief conversation with a stranger each time I visit the grocery store.

Writing

I have previously described the concept that I have a daily “quota” of words available for use (Douglas, 2014). As my condition has progressed, this quota has diminished. The time logs I have been keeping for my writing projects since the onset of PPA document that the duration of my ability to write at a high level has successively decreased from 30 minutes to 20 minutes, then 10 minutes and now 7–8 minutes. When I have reached that limit, I experience a sudden and marked drop off in ability accompanied by distinct fatigue—in any given session of writing my loss of functionality over time does not follow a linear curve but is sigmoidal. From the early stages of PPA, this loss of ability has been characterized by increased rates of errors in spelling and composition, and even when there are no mistakes, I start to wonder whether words are spelled correctly and whether my sentences are grammatically correct. With the progression of my disease, my writing output also now degenerates into a series of incomplete sentences when my quota has been depleted, with words and phrases that I cannot organize properly in spite of repeated attempts.

It is crucial to find the optimal length for a writing session: the duration which allows me to write to the full extent of my current ability without experiencing the steep drop in function which follows this point. I review this optimal length when an earlier onset of mistakes in a writing session leads me to realize that I am no longer able to write at a high level for as long as I had been. The sudden onset of my marked drop in ability makes it possible for me to recognize when I have reached my limit in a given session of writing. When I have established this, I set a timer in future sessions to serve as a reminder to myself that I need to stop writing as soon as it is starting to go wrong. Not only will I be unable to improve my work during that session, but if I try to continue writing beyond this optimal duration, I will exhaust my entire daily quota of words for all activities, whether writing, reading, or speaking, and be unable to recover function within the same day—recovery is a much slower process than loss of function. It will be possible for me to regain some function that day if I limit my writing sessions to this optimal duration and then spend time in restorative silence. Fortunately, when I return to the page afresh with a new daily quota of words, my aborted efforts will usually crystallize into the coherent sentence I had been striving for.

Because I aim for the best possible use of my limited functional time, I have to decide which writing activities I want to accomplish on a given day. I then carefully schedule my day so that I tackle the most complex writing task—the task that will stretch me to the very limit of my current ability—when I still have my full daily quota of words and am feeling most functional, and then approach the less complex writing activities later. And in order to make the best use of this word quota, it is important for me to have a clear idea of what I want to convey before sitting down to write. When composing a short note, I spend time planning it in my head prior to committing anything to paper. For example, when preparing a hand-written thank-you letter, I first remind myself of the three-sentence form I conventionally adopt, and think about the person I wish to thank, their particular talents, and what I am grateful for. Then I deliberately set these thoughts aside and spend time in silence or asleep while I allow my subconscious to work on the note. After several hours, or when I wake the following morning, formed sentences will appear in my conscious mind. I then dictate these into my voice recorder before transcribing a draft version, which I review and copy out by hand the following day. A longer letter will take me several days, first jotting down the theme of each three-sentence paragraph and then focusing on one paragraph a day, which I approach in the same way as a short note. It is becoming increasingly common for me to write a simple one-sentence note of thanks or encouragement—no recipient has ever objected.

After my PPA diagnosis, I found it necessary to approach a longer and more complex piece of writing such as a manuscript by preparing a very detailed outline to guide me. However, as my condition has progressed, I no longer have a sufficient quota of words to be able either to compile such an outline or to review it in its entirety. This presents a significant complication, which I have overcome by breaking down the project into manageable “bite-size” parts. When I have decided on a project, I jot down in a dedicated notebook the concepts I wish to include as soon as they come to mind. After spending a couple of months on this stage, I copy each of these points on to a separate index card, color-coded according to the themes that have emerged. For example, in preparing the current manuscript, I have used yellow index cards for concepts related to language and blue cards for concepts related to physical activity. Having chosen the order in which to address these themes (for example, discuss language before discussing physical issues), I can then gradually organize and write one section at a time by sorting through a limited number of appropriately-colored index cards and working from those.

When I was still able to write for 30 minutes and then 20 minutes in a session, I composed longer pieces of writing on my computer. I occasionally sought to improve my productivity by using Dragon NaturallySpeaking voice recognition software, which eliminates the need to think about spelling at the same time as typing. By the stage I could only manage to write for 12 or 10 minutes at a time, I changed to writing in longhand, which was suggested by my neurologist as a way to help improve my fluency as it is a more natural process than typing. I used a nice pen with a good quality notebook and wrote on alternate lines on the right-hand pages only, leaving the intervening lines free for minor revisions and the left-hand pages for major revisions. This strategy worked well for a time, but as my condition has progressed, I increasingly experience a disconnect between what I intend to write and what happens at the motor system level of hand movements that are supposed to carry out those intentions, leading to errors that fall in the general category of agrammatism. Thus, I omit letters or parts of letters, merge letters, or insert letters in words I know how to spell. I have also been writing the wrong letters (e.g. “z” for “x”) or words (e.g. “systems” for “symptoms”). I have therefore reverted to using a word processor in order to circumvent these issues.

Use of a word processor offers obvious advantages over handwriting in overcoming other difficulties that result from only being able to write for eight minutes per session. Now that I am making multiple false starts toward the end of my functional writing time, it is easier to use the incomplete sentences as cues for the next session if they are on the screen rather than on the page of a notebook. I will frequently be unable to fully develop an idea before my quota of words expires, so I type temporary notes of words or rough ideas to help me return to my train of thought later. Moreover, my shrinking functional time also means I am facing a growing need to go back and edit my work. Rather than being able to write a paragraph straight through from beginning to end, I now tend to start by just getting some text—possibly even something destined for the middle of the paragraph—down on the page and will subsequently build upon this by adding sentences on either side and continually refining over a number of days until it takes shape. I undertake composing and editing in separate sessions because these activities employ distinct regions of the brain. These difficulties also mean that it is no longer practical to continue using voice recognition software.

Reading

When I was first diagnosed with PPA-G, I was not employing any specific strategies to support my reading, but I have subsequently had to make a number of adaptations as my condition has advanced and my word quota has progressively decreased. I have found it increasingly hard to sustain the level of critical reading required for nonfiction, whether a scientific article or a book, such as a history or biography. I went from having been able to study many scientific papers in a single session to reading the individual sections of a scientific manuscript on separate days, to covering just a couple of paragraphs per day until I could do no more than skim a scientific abstract without reading the paper itself. This difficulty also applies to my own writing; I can no longer critically review more than one or two paragraphs at a time. Likewise, I went from having been able to read many chapters of a nonfiction book each day down to reading 20 pages a day, then 10 pages and then five. Initially, I sought to make the best possible use of this declining ability by scheduling my reading of nonfiction for the time of day at which I would be most functional. Accordingly, I limited my nonfiction reading to the afternoon, at first stopping before 5 p.m. and then before 4 p.m. However, this adaptation became insufficient to compensate for my deficits and I have had to accept that I can no longer manage to read these materials whatever the time of day. As the portions of text that I can read become successively shorter and less complex, I have continued to optimize my daily reading by scheduling short nonfiction pieces, such as nontechnical essays or news articles, in the early afternoon and then reading a novel later in the day. In addition, I have made increasing use of audiobooks, both nonfiction and fiction, thereby deriving enjoyment from a book without the difficulties associated with reading.

Speaking

My writing and reading skills have been declining more rapidly than my ability to speak, although the deterioration of my speech is more apparent to other people. My speech became progressively more effortful and less fluent, with a decreasing quota of words available for use. I could speak reasonably fluently at the start of a conversation, but as the conversation proceeded, I demonstrated more stuttering and dysarthria (motor speech problems), I increasingly needed to close my eyes to produce speech, I used more hand gestures both to cue and to accompany my speech, and the length of my sentences decreased. My stuttering led my SLP to teach me to break a cycle of repeated dysfluent sounds (e.g. b-b-b …) by silently repositioning my lips and tongue into the exact opposite oral posture. For example, dysfluent lip sounds (b, m, p) should be repositioned into open mouth sounds (o, ah); elevated tongue sounds (d, t) should be repositioned into depressed tongue sound (ah). As my condition progressed, I was able to speak for less than 40 minutes before my lack of fluency, accompanied by fatigue, meant I could no longer remain in conversation. Just as with writing or reading, it is not possible for me to regain fluent speech when it has started to slip, so I need to spend the rest of the day in restorative silence in order to recover. I seek to make the optimal use of my limited quota of words, as I have described previously (Douglas, 2014). In order to try to maximize my ability to communicate when it is most needed, I first have to be selective about the activities involving speech that I choose to engage in; I therefore plan ahead and prioritize accordingly. Before important conversations, I will intentionally spend anywhere from a couple of hours to a whole day in silence. And I support my functional speaking time by making notes of key points beforehand and mentally rehearsing some relevant vocabulary or even entire sentences.

My speech continued to decline until I visited my neurologist the day after an intramuscular injection of a magnesium solution (1 g magnesium sulfate in 2 ml water), an injection that I had been receiving every four weeks starting from the time I was medically diagnosed with magnesium malabsorption more than 25 years ago. My neurologist observed an improvement in my speech that day and hypothesized that this resulted from my recent magnesium boost. At his recommendation, I increased the frequency of injection to 1 g magnesium sulfate every two weeks, and, when that proved safe, to every seven days. My serum electrolyte levels are checked every three to four months because the long-term impact of magnesium infusion is unknown. Under this new regimen, I was able to converse for up to one hour before depleting my daily quota of words. My speech was also more fluent: fewer word-searching pauses, less frequent spontaneous closing of my eyes, fewer hand gestures, and only very occasional stuttering. This significantly enhanced my quality of life, even though I did not regain my premorbid speech level of function and there was no discernable improvement in my reading or writing. These substantial benefits persisted for 30 months before my speech again became noticeably much less fluent. At this point, the effort involved in speaking meant that I found myself closing my eyes to focus from the very start of a conversation. My word-searching pauses are both more frequent and longer than ever, and I am gesturing a lot with my hands to cue and accompany my speech. It is as though the benefits from the increased levels of magnesium turned back the clock on the decline in my speech for almost three years by giving my cognitive reserve a boost, but the inexorable progression of PPA then broke through.

As my PPA progressed, even a quiet conversation with one other person would leave me feeling overstimulated or “wired” for up to 48 hours, which precluded any further social interaction during this recovery period and also made it very difficult to fall asleep at night. However, since increasing the frequency of magnesium injections, I have been free from this overstimulation. This new magnesium regimen has also tamped down the overamplification of every individual background sound that had troubled me and made it problematic to converse in public settings such as coffee shops and restaurants. This means I can now enjoy a meal in a quiet restaurant, although I still seek to minimize distraction by visiting during off-peak hours and sitting at a side table. My preference remains to host individual friends in my home, where I can manage the environment. I cannot socialize in a group because I find it too difficult to switch from conversing with one person to the next.