Before her interview, Joanne Douglas spends the day in silence. Hers is not a spiritual practice, but a cerebral one: Joanne needs to conserve her word supply, which she says runs out over the course of the day, leaving her virtually speechless. Joanne has a form of the brain disease frontotemporal degeneration (FTD) known as primary progressive aphasia (PPA), which affects about 20% of FTD patients, and is what makes her often unable to retrieve the words she once produced with ease. Eventually she’ll lose the ability to produce speech completely.
But before that time comes, Joanne makes the most of her verbal times of the day, enjoying life and chronicling her symptoms as they change so that doctors may better understand her enigmatic variant of the disease. Frontotemporal degeneration itself is poorly understood, and is an umbrella term for a handful of diseases marked by deterioration of the frontal and temporal lobes of the brain. The majority of FTD patients experience often-dramatic changes in their personalities, behavior, cognitive capacities, reasoning, and decision-making abilities. The form of the disease that Joanne has, PPA (subtype: progressive non-fluent aphasia, or PNFA), generally spares the areas of the brain which govern behavior and executive function – until much later on, anyway. But the road on which Joanne finds herself, which will eventually lead to silence, can be frustrating and uncertain.
Joanne grew up in the South of England, and earned her first degree in Biochemistry from Oxford, her PhD in Molecular Microbiology from the University of Southampton. She came to the U.S. for a postdoc at the University of Alabama at Birmingham (UAB) where she later became a lecturer and researcher in human gene therapy. The irony of her former profession – involving both highly technical scientific information and the ability to speak gracefully in front of many – is not lost on her. She had to leave her job when her speech became too halting, and scientific papers too difficult to read.
Joanne says that she gets about 40 good consecutive minutes of talking in per day, and after this window her words begin to fail her, as if she has a quota for the day. “I can tell that my speech is changing over time. It’s so exhausting to speak now. I put a lot of energy into being able to compensate for the losses. It sometimes takes a very long time to pull the right words forward through my brain.” Joanne says she’s constantly monitoring her speech, both forward and backward in time. She plans out what she’s going to say in advance, as much as possible, and then after she’s said it, she immediately looks back to see if her speech has made sense, and corrects if necessary. This forward-backward monitoring is time-consuming and wearying.
She says that as her speech deteriorates over the course of the day – and in a larger sense over time – there’s a clear pattern to it. “At first, I can choose the right words fairly well, but as I continue to talk, I realize I’m using suboptimal words. Then, if I continue speaking instead of recharging, I’ll choose a completely inadequate word, and then finally I’ll notice that I will choose absolutely the wrong word. I can see that pattern worsening over time.”
Joanne’s speech is somewhat halting and becomes more so as our interview carries on. The first time we speak on the phone is particularly difficult, due to a number of unfortunate variables, not the least of which is some distracting background noise on my end. “Background noise knocks the words right out of my head,” she says. “It seems to take the place in the brain of what I want to speak, and leaves no room for words.” During our second try, her words come much easier, particularly since she’s “saved up” before the conversation. When we first begin, her eloquence is striking, but equally noticeable is how this all changes in the course of the 45-minute conversation. Towards the end, she begins having more trouble arriving at the words she wants, and points out that she is also stuttering, which she says she never did before she developed PPA.
Interestingly, even when Joanne has used up her word bank for the day, she says she can still understand the spoken word perfectly. She can also think clearly – and in words, which underlines the bizarre specificity of the human brain. Closing her eyes routinely when she speaks and using extensive hand gestures have also become the new norm for Joanne. “My speech pathologist explained that I do these things as compensatory mechanisms. Now I know not to sit on my hands when I speak, but to embrace these behaviors. That has made a huge difference in my functioning.”
Joanne’s high level of cognitive function throughout her life and what was likely a more-extensive-than-average vocabulary has probably put her in a relatively good position for someone with PPA. “The belief of my neurologist is that I had a high cognitive reserve to begin with. I’ve always tended to speak in long, complex sentences with semi-colons and that kind of thing,” Joanne chuckles. Her higher initial reserves may effectively act to slow down her verbal decline.
Unfortunately, her physical reserves were not so great throughout her life, she admits, which could be why her newer physical symptoms – Parkinsonism on her right side, which is not uncommon with the disease – is hitting her harder than her verbal symptoms. “I was never an athlete,” says Joanne,” which is likely why my physical decline has been faster.” She walks with a cane and has had some falls at home. Joanne is frank about the path her disease will likely take in the future, and about the fact that her physical symptoms will probably present the more significant, and possibly life-threatening, changes over time.
Joanne has made a point of chronicling her disease over the years, writing down symptoms as they occur. She hopes that doctors may learn something from this endeavor because so little is known of PPA, and of brain diseases in general. “The word my neurologist used was ‘useless’ to describe how well neuropsychometric testing is able to measure PPA. There really is no testing to quantify the situation. I’m experiencing the disease subjectively: When we spoke yesterday, for example, my speech was horrible, but today I’m quite coherent. I feel that I need to record for myself the way that things happen, so that I can present it to my doctor in a logical way… Doctors are relying on us, the patients, to be aware of the changes that occur, so more can be understood about these diseases.”
What Joanne says she does not want to do is “drift aimlessly” through her illness. “I live intentionally into the disease. I don’t dwell on what I’ve lost, and I’ve lost a lot. I don’t worry unnecessarily about what might happen in the future though I’m very aware of what might happen. And so I’m very focused on the present. But that’s not automatic. For me, it involves handing everything over to God, and because of my beliefs, I feel I’m temperamentally suited to silence. But what works for me might not work for other people… So of course, if I had a choice, I would not have chosen to be in this position. But given that I have it, how am I going to live with it? If I have 40 functional minutes a day, how do I want to spend those minutes? What kind of use do I want to make of that time? I feel I have responsibility to the people coming after me. It didn’t take much time for me to get my diagnosis (I’ve been so grateful for the excellent medical care I’ve had from UAB and Emory University), and I understand this is not always the case. I realize there are people whose diagnoses take many years. My hope is that writing down my symptoms as they progress might help people in the future have earlier diagnosis.”
The idea that Joanne wants to leave people with is that “dementia is not just a disease of the very elderly. People in our 40s can have degenerative brain conditions. As more people are aware of this fact, doctors may more often include brain conditions in their initial differential diagnosis – order MRIs earlier, and be ready to order other tests. Getting an earlier diagnosis means extending the time in which meaningful interventions can be made in a patient’s – I should say, a person’s – life. There may not yet be cures, but there are interventions that can still help improve one’s quality of life. And this is what I wish most for people.
“My life is not the way I would have chosen, but I can choose what I can make of it now. Not everyone is not at this exact point where I am; there may not always be strategies to intervene. But for others, there are. And earlier diagnosis means that we can have more for the future, and get the most from the time we do have.”
Ps. The idea of not being able to speak at all is perhaps one of the most terrifying for those of us diagnosed with a FTD with Primary Progressive Aphasia.