Expert: Mary Gordon - 1/23/2011

Question
Hello,
My spouse had P.D since 3yrs now his p.d says he is starting lewy body dementia and have put him on a Exelon Patch. I find he has progressed rapidly is this normal?
Will this continue to go this fast? And how do I recognize the different stages? Am I right that this disease is heading to Alzheimer's?
Thank you for you help
Joy

Answer
Joy, I'm so sorry your poor husband has received this diagnosis.

Here is some good general information on this illness
http://www.lbda.org/category/3431/learn-about-lbd.htm
http://alzheimers.org.uk/site/scripts/documents_info.php?documentID=113

So, what your husband has is a specific type of progressive dementia. As you may know, there are many causes of progressive dementia - Alzheimer's disease is another one, as is vascular dementia which is from tiny strokes (it also sometimes gets called multi-infarct). Lewy Body Dementia is the second most common cause of dementia, after Alzheimer's disease.

So the Lewy Body will not "turn" into Alzheimer's, because Alzheimer's is it's own thing. He will go downhill from the Lewy Body itself. It IS possible to have more than one cause of dementia happening at the same time - for example, some people will have vascular dementia on top of their Alzheimer's or Lewy Body, but it doesn't sound like this is what is happening to your husband.

Although it is a different disease process than Alzheimer's, they both end up in the same place - both are relentlessly progressive, incurable and terminal illnesses that cause irreversible brain damage.

Unlike Alzheimer's disease, which usually begins very gradually, Lewy Body often starts quite rapidly with a relatively fast decline in the first few months, although later there may be some leveling off. It does tend to progress faster than Alzheimer's disease and can last 5-7 years, although this will vary from person to person. A lot depends on the person's overall health and frailty.

If your husband has a constellation of other health problems complicating his condition, he is unlikely to survive as long as 7 years. As you can appreciate, as his dementia progresses, it becomes very difficult to detect, much less diagnose or treat health problems as they arise. The person can't answer questions or report symptoms, much less cooperate with tests, procedures and treatments that may be scary, painful or bothersome.  Families often decide that there is nothing to be gained when the person already has a terminal illness in their dementia. Quality of life becomes the driving factor, not length of life. This is why may families opt for palliative care in the final years. They decide to let nature take its course and keep the person as happy, serene, comfortable and free of pain, and not drag a very confused person around to appointments and hospitals that upset them.

Lewy Body does have some somewhat unique features in the fluctuations and the hallucinations, and the Parkinson's features, but on the whole, as the brain damage progresses, all the major dementias follow the same general pattern of deterioration. You will need patience with the fluctuations because they can be very dramatic, and you may be confused by them, or be tempted to think he's doing it on purpose (i.e. he will be able to do something perfectly well one day, and then later the same day or the next day not be able to do it at all - and then the next day, suddenly be able to do it just fine.

You have probably seen the Functional Assessment Staging Tool (referred to as the FAST scale). It was originally created to describe the stages of Alzheimer's, but it is used for all the major progressive dementias because it gives doctors and caregivers a good way to describe a person's functional abilities so everyone can understand where a person is in their illness. It also is very helpful in that it gives everyone an idea of what is likely to happen next as the cognitive impairment proceeds.

I've pasted it below my signature for your reference, so you can get an idea of where your husband is, and what is likely to come. People rarely fit perfectly neatly into a category  - not just because everyone is different, but because other health problems can complicate things. It really describes the trends for how abilities are lost.

You should be aware that your husband's illness will make him acutely sensitive to certain drugs (neuroleptics) that are commonly used to treat behavioral and mood problems in dementia patients. You have to make all doctors aware of what he has, because if they prescribe any of these drugs, it will make him a lot worse, and can be quite dangerous.

If you have not already done so, I would make sure you have consulted a good estate planning lawyer to make sure all the legal paperwork and planning is order to protect your assets against the day he may need very expensive care. I would also start researching community supports, services and facilities as you will need help with his care - you need to know what all your options are, even if you don't want to think about needing any of it.. I hope you currently live close to family and friends who can provide some assistance. If not, or if your current dwelling is not well adapted to caregiving, you might want to consider a move or renovations. After all, if you end up struggling or stressed to the point of illness, you will not be able to cope. This will not be like a sprint - this will be a marathon, and anything you can do to get help arranged will be good for both of you.

Hope this helps. I know how distressing this is. I'm thinking of you.

Mary

FAST Scale
n 1982 Dr. Barry Reisberg published what was to become the best and most widely accepted description of the stages of progressive dementia. Even today, years later, when experts referto a person being in stage 5 or stage 6, they are referring to Dr. Reisberg's FAST scale of seven stages.

Adapted from Reisberg, B., Ferris, S.H., Leon, J.J. & Crook, T. The global deterioration scale for the assessment of primary degenerative dementia. American Journal of Psychiatry, 1982

Level 1

No cognitive decline - (or Normal Adult). No subjective complaints of memory deficit. No memory deficit evident on clinical interviews.

Level 2
Very mild cognitive decline (forgetfulness or normal older adult). Subjective complaints of memory deficit, most frequently in the following area:
(a) forgetting where one has placed familiar objects;
(b) forgetting names on formerly knew well.
No objective evidence of memory deficit on clinical interview. No objective deficits in employment or social situations. Appropriate concern regarding symptoms.

Level 3
Mild cognitive decline (early confusional or Early AD). Earliest clear-cut deficits. Manifestations in more than one of the following areas:
(a) patient may have gotten lost when traveling to an unfamiliar location;
(b) co-workers become aware of patient's relatively low performance;
(c) word and name finding deficit becomes evident to intimates;
(d) patient may read a passage of a book and retain relatively little material;
(e) patient may demonstrate decreased facility in remembering names upon introduction to new people;
(f) patient may have lost or misplaced an object of value;
(g) concentration deficit may be evident on clinical testing.
Objective evidence of memory deficit obtained only with an intensive interview. Denial begins to become manifest in patient. Mild to moderate anxiety accompanies symptoms. Deficits noticed in demanding employment situations.

Level 4
Moderate cognitive decline (Late Confusional or Mild AD). Clear-cut deficit on careful clinical interview. Deficit manifest in following areas:
(a) decreased knowledge of current and recent events;
(b) may exhibit some deficit in memory of one's personal history;
(c) concentration deficit elicited on serial subtractions;
(d) decreased ability to travel, handle finances, etc.

Frequently no deficit in the following areas:
(a) orientation to time and person;
(b) recognition of familiar persons and faces;
(c) ability to travel to familiar locations.
Inability to perform complex tasks. Denial is dominant defense mechanism. Flattening of affect and withdrawal from challenging situations occur.

Level 5
Moderately severe cognitive decline (Early Dementia or moderate AD). Patient can no longer survive without some assistance. Patient is unable during interview to recall a major relevant aspect of their current lives, e.g., an address or telephone number of many years, the names of close family members (such as grandchildren), the name of the high school or college from which they graduated. Frequently some disorientation to time (date, day of week, season, etc.) or to place. An educated person may have difficulty counting back from 40 by 4s or from 20 by 2s. Persons at this stage retain knowledge of many major facts regarding themselves and others. They invariably know their own names and generally know their spouse's and children's names. They require no assistance with toileting and eating, but may have some difficulty choosing the proper clothing to wear.

Level 6
Severe cognitive decline (Middle Dementia or Moderately Severe AD). May occasionally forget the name of the spouse upon whom they are entirely dependent for survival. Will be largely unaware of all recent events and experiences in their lives. Retain some knowledge of their past lives but this is very sketchy. Generally unaware of their surroundings, the year, the season, etc. May have difficulty counting from 10, both backward and sometimes forward. Will require some assistance with activities of daily living, e.g., may become incontinent, will require travel assistance but occasionally will display ability to orient in familiar locations. Diurnal rhythm frequently disturbed. Almost always recall their own name. Frequently continue to be able to distinguish familiar from unfamiliar persons in their environment. Personality and emotional changes occur. These are quite variable and include
(a) delusional behavior, e.g., patients may accuse their spouse of being an impostor, may talk to imaginary figures in the environment, or to their own reflection in the mirror;
(b) obsessive symptoms, e.g., person may continually repeat simple cleaning activities;
(c) anxiety symptoms, agitation, and even previously nonexistent violent behavior may occur;
(d) cognitive abulla, i.e., loss of willpower because an individual cannot carry a thought long enough to determine a purposeful course of
action.

6a - Requires Assistance dressing
6b - Requires Assistance bathing properly
6c - Requires Assistance with mechanics of toileting
6d - Urinary incontinence
6e - Fecal incontinence

Level 7
Very severe cognitive decline (Late Dementia or Severe AD). All verbal abilities are lost. Frequently there is no speech at all - only grunting. Incontinent of urine, requires assistance toileting and feeding. Lose basic psychomotor skills, e.g., ability to walk, sitting and head control.
The brain appears to no longer be able to tell the body what to do. Generalized and cortical neurologic signs and symptoms are frequently
present.

7a - Speech ability limited to about a half-dozen intelligible words
7b - Intelligible vocabulary limited to a single word
7c - Ambulatory ability lost
7d - Ability to sit up lost
7e - Ability to smile lost
7f - Ability to hold up head lost