Expert: Mary Gordon - 4/30/2010

Question
My sister is 52 and was diagnosed just under 6 months ago with dementia from early-onset alzhiemers. Her symptoms have progressed rapidly. She is already not able to live on her own and requiring supervision much of the time. She can feed herself but is not able to cook or often cut her own meat. She can go to the bathroom herself and can shower herself but needs help with dressing, especially with shirts and shoes and undergarments. She frequently needs help with finding the words to finish sentences and very often uses incorrect words, or words that aren't even words. She remembers family still but is starting to forget their names when talking about them. She opened her car door going down the highway when trying to unroll her window....and the list goes on and on. Before my sister came to live with my husband and I we were already caring for a man with mental retardation. This was fine at first but it is too much for me to care for both and I desperately want to care for my sister so that she and my family can have as much time with her as we can while she still remembers us. Because of his disability he often comes across as rude and inappropriate and she is becoming much more agitated by this. Making this change would mean a lot of change for a lot of people, which I am willing to do. It would be so much easier to make decisions about the future if I had an idea as to how much longer my sister will be in this stage. Is there an average life expectancy for someone diagnosed with early-onset Alzhiemer's whose symptoms progress so rapidly? Will she likely continue to progress this rapidly and have a shorter life expectancy or does it tend to slow down towards the end? I know no one can give me definite answers but statistically speaking, how long do people generally survive when diagnosed this young and progressing so rapidly?

Answer
Hi Tammy, sorry I didn't answer right away.

With early onset, the person is physically healthy compared to a frail elderly person. What often ends up causing death in an older person is complications from other health problems that can't be treated because of their dementia. Families may decide invasive and unpleasant treatments, surgeries, tests, and procedures are just not worth it in terms of quality of life. So when their dementia advances, they are in poor shape and it doesn't take much to carry them off.

Your sister is very young and probably relatively very healthy. Having said that, her dementia is advancing very rapidly - and that pattern tends to continue - it isn't true of everyone with early onset to have such a dramatic downslide in such a short time. Although it is hard to predict, my bet would be this will continue to escalate quickly. This does not mean she is likely to pass away in the next year or two. It means she is likely to become very, very disabled - and after that, it may be quite a marathon. They basically become like a person who has had a very serious head injury from an accident. They may be totally disabled and helpless, but they can physically live on for some time when they are well cared for.

The thing is - what comes next is truly grueling, and you need to be ready for the reality of it when you consider what the care arrangements will be. She will need supervision and support 24/7 and you won't be able to leave her alone for even a short while. Her agnosia will worsen - this is the loss of the ability to recognize not just people, but places and things. Nothing will look familiar to her, including your home and neighborhood. This is why they start getting lost, even indoors, and more frighteningly, outdoors if they manage to get out unsupervised. Her aphasia will get worse - this is the problem with her speech. She won't be able to find words for what she says, but more heart breaking, she won't be able to understand what is said to her. She will talk less and less, and what comes out will be nonsense, or just a word or two repeated. She will lose the ability to answer even simple questions. She may go though a phase when she is very agitated. They can't express their frustrations and fears, and when things get to be too much, they can explode (it's called a catastrophic reaction) - it's kind of like a toddler tantrum, in that it gets worse when they are tired, hungry or stressed. She will not be able to do everyday things for herself like choosing clothing or dressing herself at all (you will be dressing her like a baby), performing personal hygiene tasks, - and eventually, feeding herself. Part of this is from apraxia, which is the loss of muscular control. This affects all her muscles, so fine coordination is impacted - her fingers won't be able to handle buttons - and eventually, large muscles. They start to shuffle when they walk because their brain can't tell their legs what to do, and their sense of balance is affected. They can start to be in real danger of falls. The loss of muscular control extends to chewing and swallowing - she will not be able to handle solid food, thin liquids or things with mixed textures like cereal in milk, or she will choke. She will start to need finger foods first because utensils will be beyond her, and then you will be feeding her softer and softer foods. She will become incontinent, first of bladder, then bowel - and the bowel incontinence is often the factor that tips the scale in terms of home care.  Eventually, she will stop walking entirely. The care becomes exhausting and relentless - and in a way thankless, because she will not know where she is, or who she is with. I say that not to be cruel, but to make you consider that there may come a day that this is too much for you to manage in a home environment, even if the other gentleman with challenges was to be placed elsewhere.

It's a nightmare because it can go on and on. Even my mother in law, who was 79 when she passed, lived a full two years in the last stage - completely helpless. When they reach this level of disability, they really have a limited awareness of their surroundings.

You have to prepare yourself for the idea that your sister may deteriorate rapidly to the point where being in your home is no longer of real benefit to anyone. It's not just the 24/7 nature of it - it's that you are just one person and not Superwoman, either physically or emotionally. Facilities have a lot of specialized equipment to help with the last stage - lifts, special showers, special beds, elevators, safety features, special locks and security  - and most important, they have fresh staff on every 8 hours, so dealing with the 4 am clean up does not mean frustration and irritation and exhaustion the next day. I'm not saying you are anywhere near the point where you would be ready to consider a care facility, but I am saying - give yourself permission to put yourself into this equation. Your real job with your sister is to love her and to make sure she is well cared for. That does not mean you have to be the person doing the actual work. If you wear yourself out, you will start to resent the burden of caring and make yourself sick, which benefits no one.

The way to look at this is you will keep her at home for a while and keep reevaluating, being brutally honest with yourself about the impacts on you and your family - and your husband - your health and sanity and your marriage must be a priority.

Even for right now, you should be looking into care supports so you are ready. Does your sister have any insurance that may pay for some assistance? Are there local programs that you could qualify for? Certainly help at home will help you to cope better and keep her home a little longer. You should start with looking into home supports, like someone to come in a few hours a day to give you a break, or help with domestic chores or even do the yard work.  There may be day programs (sort of like adult daycare) that she could go to. Tap into family, friends, neighbors and your faith community if you have one - don't be ashamed to ask for help. Perhaps someone could take her for an afternoon or an evening, or "babysit" so you can get things done or just go out and relax. If she starts getting up a lot at night (which is extremely common and very disruptive) you can get a night "sitter", whether hired or volunteer. There is also respite care available from many facilities so you can take a weekend off or have a vacation.  You may not want to think about any of these possibilities, but the physical reality is the whole situation is already too much and it gets much more challenging on every front from here on in.

I would also start looking into local assisted living facilities and nursing homes. You need to do your homework now, so you have a plan for going forward. You need to know what the costs are, what she qualifies for, and what is available in your area. You need to check them out and figure out what is best for everyone. Best options are usually places that offer specialized dementia care and have progressive care programs, so she can stay in the same place. What I mean by that is many assisted living places have limits on what they can handle, and will kick residents out if they get beyond a certain level of disability (i.e. if they can't walk). You want a place that can continue to care for her as her illness progresses. Many of the good places have long waiting lists, so even if you are not ready to think about this now, you may be prudent to have checked them out and get her on waiting lists now - so if in 6 months or a years time you are not in crisis because you are at the end of your rope and can't find a good alternative. Having done prep work, and checking out options will greatly reduce your stress and worry, because you will have a plan in your back pocket. Another reason to have a plan is that you can never tell what else might happen - if you or your husband were to become ill or there were other family emergencies, you would be in the soup in short order. I felt horribly guilty putting my mother in law's name on a waiting list - like some sort of awful person. At the time, she was coping pretty well at home with the supports we had arranged. However, by the time they called with a spot, things had changed radically, and we knew we no longer could keep her safe and well cared for in a home environment.  

As to the other man you look after, I'm not sure what the arrangement is. If he has been with you a long time, he likely regards you as his family.
Are there group homes he could go to in the area where he can still see you so he doesn't feel rejected? I wish I had a definitive answer about your sister, but the answer has to be she will get worse and worse and harder and harder to look after, but she is very unlikely to be about to pass away in the next year or two.  

This must be a very difficult situation for you to cope with. I'm not sure what the answer is - but first stop is to find out what extra help you can get to take some of the load on. No rose colored glasses. Planning for dementia care is one area where it pays to be a pessimist because then you will be ready for worst case.

Below my signature I've pasted the last three stages of Alzheimer's (based on Reisberger's functional assessment stages (the FAST scale). My guess is she's in late Stage 5 or early Stage 6. Few people fit neatly into a stage category, but it gives you an idea of what to expect might come next.

I know this is brutal - and it's so unfair. She is so young and it must be heartbreaking to see someone you love go through this. If you have any other questions, come back and ask. Hope this helps.

Thinking of you

Mary G.

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