ARF: When we think of a person with AD, we typically have a different image in our mind. We picture someone whose world has shrunk, who needs help dressing etc., whose suffering is obvious. Must we change our concept of Alzheimer disease?
RB: Yes. I think the biggest reason we must change the concept is that there are many people who could be helped if physicians recognized their problem and diagnosed it earlier than they do now. As you said, it becomes obvious even to the non-expert. But I hear of doctors not diagnosing the disease even once it's obvious, much less trying to diagnose the very earliest stages.
Someone at a reputable center for the treatment of Alzheimer's told me that doctors don't like to tell people they have Alzheimer's too early because they fear the patient will commit suicide. To be fair, they told me that they recently had a case where someone had been given the diagnosis and committed suicide.
ARF: Jack Kevorkian's first patient was a woman with early-onset AD in her fifties. Her psychiatrist told me recently she felt the decision was made hastily and without the doctor's input.
RB: Now, that is absolutely horrible. On the other hand, I have also met many people who told me that it was the biggest relief when a doctor finally told them that what was wrong with them was Alzheimer's. They finally had an explanation for what was causing their symptoms—the confusion, the changes. It can be very disconcerting to notice that you are not the same person you always were and have no idea what is causing it. Also, would a doctor not give someone a cancer diagnosis because he or she feared the person might commit suicide? I don't think so.
I have talked with people who were having symptoms, went to one doctor, and were told, "I don't see what the problem is." Then they went to another doctor, and were told the same thing. But they were having problems. Finally, the third doctor does take them seriously, gives them the AD diagnosis, and, most important of all, starts them on Namenda, Aricept, or one of the other drugs like it. Some of these people have been taking these meds several years and not seen significant decline. Do you want to hear the damnedest Catch-22 of all? Sometimes people who've been doing well because they are taking the meds then are told, "Oh, well, you can't possibly have Alzheimer's if you are still functioning," and the doctor wants to take them off of the AD drugs! People live in fear of deteriorating when they hear these words. I've been to conferences where people with dementia were speaking, and they said that if they were not on these meds they wouldn't be there speaking to the audience that day. I know from my own experience that I function much better now than before going on these medications. That's the number one reason, in my opinion, why we need to change our perception. Because if we don't, doctors are depriving multitudes of people of the opportunity to live meaningful, productive, and capable lives for much longer than they are now.
A brilliant researcher named Leon Thal of UC San Diego passed away last February. On January 8, 2007, a Business Week article quoted him:
The massive funding pumped into HIV research led to predictable results: AIDS was transformed from a death sentence into a manageable disease in just 10 years. Scientists say the same could happen with Alzheimer's if there were a similar national will. "Look, if we doubled our research output it would halve the amount of time it will take to find a cure," asserts Dr. Leon J. Thal, director of the Alzheimer's Disease Research Center at the University of California, San Diego.
While right now there is no "cure" for AIDS, the advent of the protease inhibitors changed this disease from being the death sentence it was back in the 1980s. Why is there not a similar "national will" about Alzheimer's? A leader in the AD field said to me: "It is ageism." Many people think of Alzheimer's as a natural part of aging, and, in doing so, they are basically writing people off, saying, "Well, too bad, you got old, that's what happens." Of course, people don't realize that Dr. Alzheimer's "patient Zero," Auguste D., was 51 years old. I have friends with early onset who are in their forties and fifties. I think that is one of the reasons why it is important for those of us who are in the early stage and with early onset to speak up because that might be what it takes to get people to realize that something can be done and needs to be done.
ARF: When did you notice changes for the first time?
RB: In June 2004, the auditory hallucinations began. I was later told that at least one-third of persons with Alzheimer's have them. My psychiatrist put me on Risperdal, a
drug that's often used in people with AD.
ARF: At first, what did you think might be wrong?
RB: Honestly, I had no idea.
ARF: What was getting your diagnosis like?
RB: I saw the psychiatrist right away and the symptom disappeared with the Risperdal. But then strange things began to crop up. I was getting confused. For example, we have received bottled water all of my life. When a five-gallon bottle is empty, I lift it off and replace it with a full one. Been doing that for a long time—it's so simple. One day, I took off the empty bottle, lifted up the new full water bottle, and instead of emptying it into the carafe on the stand, I dumped it down the sink. The next time, I took off the empty bottle, picked up the new bottle, and dumped it in the wastebasket. The next time, I took off the empty bottle, picked up the new bottle, and poured it on the floor. Anyplace but where it was supposed to go. A connection was missing.
Other things happened, too, like losing my place doing routine tasks and probably about 100 things that I'm forgetting to write about right now. When I told these things to my doctor, he said to stop taking the Risperdal. But I did not want to go back to hearing things that weren't there and my psychiatrist advocated for keeping me on it. This went on.
In spring of 2006, my insurance was changing and I had to look for a new doctor. I told my new physician about my symptoms and he listened to me. During that first visit, he said he wanted to order an MRI, a PET scan, and neuropsychological testing. He said he wanted to rule out some things.
The MRI ruled out a brain tumor. But the PET scan showed global dysfunction and atrophy and hypoperfusion in both frontal lobes and temporal lobes and parietal lobe. And what they called "watershed areas." The frontal and parietal lobes were particularly affected, as is typical for AD. My doctor explained that there were areas where there was activity and then right next to it, it was "just dark, nothing going on." I then had 2 days of extensive neuropsych testing. These were more than 8 hours a day for 2 days, not some casually tossed-off Mini-Mental Status Exam. By the way, when I went for my neuropsych testing, I did not yet know the results of my PET scan. I was actually into the second day of my testing and thought that it was going really well, i.e., meaning I thought I was acing these tests. It's not funny but I remember in one part of the test I was asked to explain sayings. She asked me to tell her what the following expression meant: "One swallow does not a summer make." And I said, "Don't eat summer in one gulp." After the test was over and she explained what the real meaning was, it mortified me. I couldn't draw the face of a clock to the time she specified. My scores were frighteningly low. Mind you, I was tested twice when I was young and both times my I.Q. scored about 150.
ARF: People with high IQ tend to be more difficult to diagnose because they compensate so well.
RB: Yes, the "cognitive reserve" again. I take that to mean that people who had a high degree of intelligence, or high degree of functioning, have to lose more for it to either bother them, or become noticeable to the point where someone thinks, "Okay, something is clearly wrong with Joe." I remember when I got my diagnosis, a good friend said, "Well, you were always too smart anyway. Now you'll be just like the rest of us."
That was meant as a compliment, but it's not very comforting. My friend W. and I often talk about how people will say to us, "Oh, you don't have it." He also had a very high-functioning career. So although he notices his deficits and that he is not functioning the way he once did, others don't see it much, because he is not what people have in their mind as the stereotype of a person with dementia.
To get back to my diagnosis, seeing the results of my neuropsych exam was heartbreaking. My reading comprehension was grade-school level. She had to keep giving me simpler and simpler passages to read. I think I read a seventh-grade passage and got every question wrong. And what did I do for a living? I was an editor. Do you think someone would ever allow me to work in publishing again if I couldn't read a simple passage and retain its meaning? Thankfully, no one is testing me after it takes me 10 times to read an article in the newspaper before some small part of it sinks in.
I mentioned working with a company on testing its cognitive training computer program. One of the clear results is that my short-term memory is very impaired. For example, the computer will show me objects or words and then ask me to recall them in some way. When they appear on the screen, I am just guessing because they don't look familiar to me at all.
ARF: You mentioned others in your family having had AD, too? Can you write about that?
RB: The occurrence of it in my family is among relatives in the Midwest, so it has been mostly from afar. We learned upon the death of a cousin of my Mom's that she'd had Alzheimer's. She and my mother had been fairly close. At first we heard, "Jodie [name changed] can't come to the phone." Then when my Mom would visit for family reunions, Jodie wouldn't be there and they'd say she wasn't feeling well. A great-uncle also had it. Basically, he, Jodie, and other relatives weren't seen again. They were kept at home, hidden away from view. I understand there is a stigma with this disease, but having seen how this was handled in my family, I feel it is important to be someone who is not afraid to speak out. There is nothing to be ashamed about. I didn't ask for this. Neither did my great-uncle nor aunt Jodie.
ARF: Is your treatment any different than if yours were a more "classical" case, i.e., diagnosed at a later stage and later age, say, in your seventies.
RB: I don't think so. The greatest hurdle to receiving this treatment is getting the diagnosis at a younger age. I hear over and over again from people with early-onset that they are told it's stress, it's depression, and it gets dismissed (for another example, see ARF interview Diagnostic Odyssey). And if one doesn't advocate strongly or have a family member or friend willing to advocate, then the diagnosis gets deferred until the person is so clearly demented that the meds will likely have little to no benefit. To me, this is criminal.
ARF: The cholinesterase inhibitors (donepezil, galantamine, rivastigmine) are approved for mild to moderate AD, and memantine for moderate to severe AD, not for the early cognitive symptoms you have. They are standard of care, but at the same time there is debate about whether they are worth the cost. In England, the National Institute for Health and Clinical Excellence (NICE) has touched off controversy with its critical stance on these medicines. What's your experience with these drugs?
RB: Interestingly, Namenda (memantine) was the first drug my doctor prescribed for me. As I understand it, Namenda is designed to slow down the progression of the disease, and my personal experience is certainly that it is helping me. I don't remember noticing any side effects with the Namenda. After I was on it for 6 weeks or so, he added the Aricept. Once I started on that, I got gastrointestinal distress and insomnia as side effects. Originally, I was taking the Aricept in the morning and at bedtime. My doctor suggested that I take the second dose at about 2 p.m. so it would be less likely to keep me awake at night, and he was right. I put up with the gastrointestinal distress because it's important to me to keep my brain functioning as long as I can.
I have noticed improvements since being on the medications. As you can see from the symptoms that I describe, yes, I have problems. But, for example, whereas before it seemed like every morning when I tried to make breakfast I was getting lost going about this simple task, that happens less frequently now. Or if it does, maybe one event gets confusing but I am able to get back on track again fairly quickly. Life isn't perfect, but it is much, much better. I do know that they say that Namenda is not for "early stage," but I can't think of one early-stage person who isn't on it. I understand this is considered "off label," but in my opinion doctors who put their patients on Namenda and help us stave off the progression of this disease are heroes.
ARF: There also is debate about how long the meds help, how long to stay on them. Any thoughts?
RB: I have been to dementia conferences where persons with early-stage said, "I was diagnosed in 1999 and immediately put on Aricept. And now that I have not progressed significantly in 8 years, my doctor has taken away my Alzheimer's diagnosis." What's worse is when they not only take away the diagnosis, but also want to take away the person's AD drugs. So people have kept the wolf from the door because they took the medications, and now the doctor wants to take them off their medications. What happens if they then deteriorate to the point where the meds won't do them much good once or if they are prescribed again later? To me, this doesn't make sense.
ARF: Everyone agrees we need a new generation of drugs that halt the underlying disease. Have you tried to participate in drug studies of mild cognitive impairment or AD?
RB: Not to date.
ARF: Would you be interested in doing so?
RB: I would need to know more about what is involved. It also would depend on whether I had to go off of other medications.
ARF: Most AD drug studies exclude people younger than 50 or 55 (see ARF essay on clinical trials). Part of the reason why companies are not testing their experimental drugs in people with EOAD is that they think they can't find enough people in that situation. Do you agree? What could be done?
RB: My understanding is that it is estimated that some 5 to 10 percent of the 5.1 million diagnosed with Alzheimer's in the United States have EOAD. It isn't that we don't exist. JoAnn Webster, who is a leading authority in the field of outreach to the EOAD community, just gave a presentation in Chicago where she said that persons with EOAD are often the most vocal advocates (see also ARF comment series).
I am now on the national Alzheimer's Association's Early Stage Advisory Group. There are 14 of us, 12 with EOAD. We're here and we're vocal. I don't agree that it is because they can't find these people. Personally, I think it is a bias that again goes to the issue that Alzheimer's is perceived as a disease of the elderly. Therefore, we who have EOAD are seen as a rarity, an anomaly.
But given that we have been taken out of the work force in the prime of life, we are often diagnosed earlier on in the illness and are more able to talk about what it is like to live with this disease, we would be much better able to articulate what a drug is doing to our bodies. What are its side effects? Do we think our memory is better? Is our cognition better? Who better to ask than the person taking the medication as opposed to a care partner who is giving second-hand information?
As far as what could be done, they need to start doing trials that a) include persons with EOAD, and b) perhaps consider doing trials solely on persons with EOAD because some of the drugs might be more robust if tested on us because we are often diagnosed earlier.
ARF: Many leading scientists agree that drug studies should move toward earlier treatment and eventually prevention, when the brain has not already been ill for years. Do you get the sense from others you know that they want to participate, i.e., be "guinea pigs" for such trials?
RB: The people I know with the disease fall into two camps. The first are people who would like to be part of clinical trials but haven't qualified because they are too young. The second are people who hear positive word-of-mouth about a drug in a certain phase—anecdotal evidence that Drug X is working—and they then want to get into a now-closed study.
ARF: Besides awareness, understanding, and tolerance on the part of your fellow citizens, what do you want to see happen most urgently?
RB: When I was a kid, people said someone had "the Big C"—they didn't say cancer. Or they whispered it: "She has cancer." Unless you talk about something openly, then you pretend it isn't there as if it's going to go away. Well, Alzheimer's isn't going away. I wish there were the leadership in this country to spread the word that this is not an old person's disease or a natural part of aging. Then, "Oh, well, what can you do? It's inevitable," would not be an acceptable stance anymore. Alzheimer's disease doesn't need to happen. We are learning more all the time. When I attend conferences and listen to researchers, they often talk about how they think we will have a cure in 10 years, maybe 15 years. And then I hear that because the government doesn't spend the money on Alzheimer's research, the most brilliant minds are moving on to study other diseases. When I speak, I like to be a message of hope. Frankly, I think if we had the will, this national will, we could conquer this disease. It takes money, will, and commitment, and I don't see that forthcoming. But I want to continue to remain hopeful.