True Health Is True Wealth!!: Alzheimer's patients follow different paths to a final debilitation

Untangling Alzheimer's – Part 2 of 3

Post-Gazette.com: Sitting in a small office on the fourth floor of UPMC Montefiore, Mary Lewellyn eagerly awaits a question from someone trying to assess her weakened mind's abilities.

Mary Lewellyn, 59, of Robinson has the "early-onset" version of Alzheimer's. (Andy Starnes, Post-Gazette)

"Do you know what day it is?" she is asked, a standard test question for Alzheimer's patients.

Lewellyn had come in from outside just minutes earlier, on a glorious October afternoon with the sun shining and the temperature reaching 70 degrees. She smiles beneath her frizzy hair, leaning forward in an earnest attempt to answer this once-simple question.

"December thirty-three -- is that what it is?" she responds, bright-eyed.

Eight years ago, Mary Lewellyn worked as a bookkeeper, helped her husband renovate their Robinson home and dreamed of retiring with him someday to Colorado Springs.

10 warning signs of Alzheimer's

1. Memory loss that affects job skills.
2. Difficulty performing familiar tasks.
3. Problems with language.
4. Disorientation to time and place.
5. Poor or decreased judgment.
6. Problems with abstract thinking.
7. Misplacing things.
8. Changes in mood or behavior.
9. Changes in personality.
10. Loss of initiative.

Four years ago, she could no longer compute numbers easily and was forgetting normal household chores. A neurologist suspected she was in the early stages of Alzheimer's disease.

By the time of her recent evaluation, at the Memory Disorders Clinic of the University of Pittsburgh's Alzheimer Disease Research Center, the 59-year-old woman could no longer tell the time of year or what city she was in. Her ability to add two numbers or write a coherent sentence had vanished, just like her job, her retirement plans and her role as equal spouse to Jim Lewellyn, her husband for 18 years.

Jim, patient and mild-mannered, told clinic coordinator Lori Smith Macedonia at the end of Mary's test of mental functioning that she seemed healthy as could be in some ways -- dancing at a wedding, roller-skating with relatives, singing songs from "Oklahoma" loudly in good pitch as he played them on a Hammond organ at home.

Mary scored but 8 out of 30 points, however, on the 30-minute Mini-Mental State Examination, a key tool to monitor dementia patients' ability to think, memorize and follow instructions. She had correctly answered twice as many questions a year earlier. That explains why Jim has to tend to most of her needs, from helping her dress to preparing her meals.

"A lot of people seriously impaired have very good physical states," Macedonia, a physician's assistant, explained afterward. "She could get up and run a race but she is very severely impaired."

Macedonia and Jim agreed to increase Mary's dosage of Exelon, a new drug intended to help preserve cognition, but there was no certainty it would help. The three drugs approved specifically for treatment of Alzheimer's sometimes help treat symptoms and sometimes don't. They are far from a cure even for the patients who respond best.

The Lewellyns will continue periodic visits to the memory clinic, but they hold no illusions it will free Mary of the disease.

Deciphering the signs

The first sign of Alzheimer's can be imperceptible.

Forgetting a name, misplacing car keys or unintentionally missing an appointment happens to most people from time to time, especially as they age. It doesn't mean they're diseased.

The time to be concerned is when short-term memory problems occur frequently. If a family member recognizes a pattern of forgetfulness in a parent or spouse, a medical evaluation may be in order if the person hasn't volunteered for one.

In Mary Lewellyn's case, Jim realizes now, the first signal was when he was working in the back yard on hot days, and Mary would offer to get him a drink. She'd go into the house, and he wouldn't see her again for hours as she busied herself with other tasks, forgetting about him sweating outside.

"I wasn't being mean," she says now, with a laugh, though she'd give anything to relive those days when she could accomplish menial chores.

For some people, the first difficulty they notice is disorientation while driving. Others with a lifelong hobby requiring concentration, such as bridge, can no longer grasp the key details. Husbands telegraph the problem by repeatedly forgetting to mow the lawn, and wives lose track of where they parked the car while shopping.

Donald Helton remembers the day in 1993 his grandmother left the stove on unattended in their Elizabeth Township home, a wake-up call that she had a problem requiring attention in order to avert a disaster such as the house burning down.

"Oh, boy, wouldn't that be awful," 86-year-old Mary Gonzales says while listening now, as though her grandson were talking about some other person. "God forbid."

Short-term memory is the first casualty of Alzheimer's, but it takes more than memory loss to be diagnosed with the disease. Someone with that difficulty alone is usually considered to have mild cognitive impairment, which is often but not always a sign that Alzheimer's is ahead.

By the time they are diagnosed with Alzheimer's, individuals typically will have also lost some reasoning ability or language skills or undergone changes in behavior, such as apathy or agitation. That's often several years after the appearance of the memory problems, which may take on significance only in retrospect.

People with early Alzheimer's often hide it, even from themselves, by sticking to a familiar routine and avoiding new, uncertain situations. They and relatives laugh off some of the potential warning signs and attribute them to age rather than disease, a form of denial thought to be more common among men than women.

Jim and Mary Lewellyn can still enjoy musical evenings at home. (Andy Starnes, Post-Gazette)

"There is more undetected Alzheimer's than you would imagine" said Dr. James T. Becker, a University of Pittsburgh neuropsychologist. "Some of that may still be related to the way we think about the elderly. Part of that is from how people earlier in the disease adapt. You very quietly change your lifestyle, and even your family may not notice."

Diagnosing the disease

I.G., an 80-year-old woman visiting the Memory Disorders Clinic, is able to spell, count and draw without hesitation but is stumped by the most telling part of the exam: a request that she recall three words given to her several minutes earlier.

"Heh, heh, you're kidding," she says with a chuckle. "I forget what they were. I forget very easily. ... Was 'table' one?"

Yes, it was. So were "apple" and "penny," words that evaporated quickly in a mind clogged by the mild-to-moderate stage of Alzheimer's.

By the time she is asked to write a complete sentence, the very dignified I.G., who did not want to be identified publicly, acts put off by this process of assessing her mental functioning. She writes clearly: "I think it's about time to go home."

The first medical visit where Alzheimer's is discussed can be deadly uncomfortable.

If it is in the office of an Alzheimer's specialist, usually the patient himself is the last one who wants to be there, noted Dr. Michael Usman, who operates the private, non-profit Alzheimer Center of Pittsburgh in Robinson. It's one disease with an ironic twist -- the fact that you have it makes you among the least likely to recognize it.

"Usually, if I ask flat-out, 'Why are you here?' the response is 'Because she brought me,'" Usman said, pointing to where a patient's daughter would sit in his office.

Specialists such as Usman say they may spend four hours on an initial examination in order to determine whether Alzheimer's is present. A team approach is used in which the patient may get physical, neurological and psychiatric or psychological evaluations, as well as interviews done by a social worker with the patient and family members. Sometimes, a brain imaging scan is ordered to rule out stroke or other illnesses that could be affecting the mind.

Such full-court scrutiny can diagnose Alzheimer's with 90 percent or more accuracy, but absolute certainty can only be provided by a brain autopsy. Because it's so dreaded a disease, it's essential that physicians avoid bludgeoning people with references to it and instead focus on helping them, said Richard Besdine, director of the Brown University Center for Gerontology and Health Care Research.

"With mention of the word Alzheimer's, the listening capacity [of patients and relatives] falls off dramatically," Besdine said.

Most people with Alzheimer's and other dementia are cared for by their own physicians, which can pose a problem in the managed-care world. Doctors are reimbursed better for brief stints of hands-on medical treatment than the more leisurely conversation and assessments necessary to manage a person's dementia by monitoring medications and advising caregivers.

Alzheimer's specialists routinely gripe that primary care physicians don't diagnose dementia quickly enough, which could be a carryover from days when treatment options were unavailable to them and a diagnosis mattered little. Now the drugs Aricept and Exelon can delay effects of the disease for up to a year in some patients.

Doctors say the population is becoming better educated every year about the disease and the possibility of getting help for it. New patients at Pitt's Alzheimer Disease Research Center are more commonly arriving within a couple of years after symptoms first appear, when it used to take four or five years, said neurologist Dr. Daniel Kaufer.

Sometimes, he can tell families that the patient has only mild cognitive impairment, a relief to them, but one followed by the warning that half of the people in that state are likely to have Alzheimer's within five years.

Less than 20 percent of the time, the extensive team examination determines the dementia of a loved one is caused by a disease other than Alzheimer's. Kaufer said that news can be even worse for families than a diagnosis of Alzheimer's, because less progress has been made in researching vascular, frontotemporal and other dementias. And often, one of those other dementias overlaps with Alzheimer's.

A different path for everyone

The Alzheimer's course can run as long as 20 years from the first symptoms until death, though it's more typically half that. It's usually a shorter, swifter decline for the fewer than 10 percent who, like Mary Lewellyn, get the "early-onset" version of the disease between their 40s and early 60s.

Each person's experience with the disease is different. Each patient carries a different level of awareness of what is taking place inside their troubled brain. Some know they have Alzheimer's, some just know things seem different from before, and some are clueless that there's anything wrong.

Their lack of insight and loss of language skills make it hard for the victims to discuss the disease, even when they know they're being affected.

"It was gradual," Mary Lewellyn says on her home's back deck one day, quietly deferring to her husband until she is asked point blank how she has been affected. "I don't know, really. ... Something happened -- I have no clue."

At a support group of the local Alzheimer's Association, individuals in the early stages of the disease are better able to discuss the turmoil in their heads and lives. Each month, they share insecurities about strange situations, mishaps at home and hardships they create for relatives.

"I don't think anybody can understand what you're going through. It's like somebody dropped a bomb on you, and it blew up," Phyllis Herrup, 71, of McKeesport, said at one meeting as some of the seven other heads around the table nodded.

One day, her husband found her taking apart pieces of the washing machine. She didn't know why. Her memory's been slipping since 1994, and it's a source of daily frustration for the college graduate who has served on McKeesport's school board and development authority.

"If I put down a book or magazine and then look for it, I can't find it," she said. "It just drives me up the wall."

Irene Vellella of Bellevue, who also attends the support group, doesn't see herself as handicapped. Diagnosed with Alzheimer's a year ago, the 77-year-old woman still lives in her own apartment but has given up driving and control of her financial affairs to her children. She says those decisions weren't necessary but make life easier for her, and she faults the memories of friends and relatives as much or more than her own.

"[Patients] often don't understand what's happening, even at the early stage," said Dr. Amelia Gennari, the UPMC Shadyside geriatrician who diagnosed Vellella and encouraged her to give her checkbook to her son. Doctors often take on the "bad guy" role for families and deliver tough advice to patients in denial of their shortcomings.

"The biggest problem often in the early stage is depression, and fortunately, we do have good treatment for that," Gennari said.

About one-fourth of Alzheimer's patients suffer depression, studies suggest. At least that many have delusions or paranoia, and twice as many become easily agitated. Managing Alzheimer's long term often involves treating such personality difficulties with specific drugs and with advice to caregivers, just as much as addressing the patient's loss of memory and reasoning.

A final, debilitating decline

The physician's assistant testing Mary Gonzales' abilities at the Memory Disorders Clinic taps the wrinkled woman's knee, soliciting her attention.

"Mary, what year is it?"

"Oh, 19-about-53 or something, I don't know," the 86-year-old woman responds. "I usually know, but I've had all these things on my mind."

Those things are the pigs and cows that surrounded her in childhood, the two deceased daughters she thinks are still alive, and her parents' origins in Czechoslovakia, all of which she discusses repeatedly without prompting.

Hers is a restless, nonsensical mind masked by a pleasant demeanor and fit body. When leading Gonzales out the door after a half hour, her gentle interrogator is careful to mention she did very well.

"Oh, did I?" Gonzales says sweetly, quite willing to believe it.

Researchers are still trying to identify all of the environmental and biological factors that target some individuals for Alzheimer's and spare others. One thing they have deduced is that education and intelligence help delay the effects of the disease.

Smart people aren't spared the mental mayhem of Alzheimer's, but they appear to have reserves of brain power that help them function close to normal for longer periods.

"If you have a big vocabulary and you can't think of the word you need to use, you'll think of another one easier," said Dr. Judith Saxton, a University of Pittsburgh neuropsychologist. "It doesn't stop you from getting the disease, but maybe intelligence does what the medication is designed to do -- it slows things down."

At a recent Alzheimer Disease Research Center consensus conference where the staff collectively reviewed the status of patients, the doctors commented on the mental tenacity of a 57-year-old teacher with a master's degree. She was diagnosed with mild cognitive impairment nearly two years ago, but she kept awakening three hours early to prepare herself for classes so she could continue working.

Last month, the doctors downgraded her to Alzheimer's status, based on declining test scores and functioning. She has quit teaching. She's given up control of the family finances and can't drive 150 miles to visit relatives.

"I'm amazed she's hung on this long," a neurologist commented, recalling how the woman seemed on the verge of Alzheimer's in 1998.

Dr. Steven DeKosky, the center's director, recognized when he started seeing patients in the 1980s that his more educated patients held steady longer. "If the disease strikes you at 75 and you're highly educated, the sanding from the disease may never get down to the bare wood before you die," he said.

More commonly, however, someone goes from Irene Vellella's stage of functioning independently within a careful routine, to Mary Lewellyn's helpless status, to Mary Gonzales' ignorance of her helplessness, which is accompanied by an urge to wander away from home.

A nursing home is often the final stop. More than half of nursing home patients carry some sort of dementia, most commonly Alzheimer's.

Most are oblivious to their condition by that stage, which enables them to focus on activities that give them pleasure for the moment rather than the depressing fate awaiting them, said Dr. Jules Rosen, a Western Psychiatric Institute & Clinic geriatric psychiatrist. On his visits to institutions, he sees end-stage Alzheimer's patients retreat to a fetal position in bed, unable to care for themselves or take directions from others.

"Most people, given a good, supportive environment with caregivers who understand the situation, and with proper medications, their quality of life can persist for many, many years," Rosen said. "Eventually, they just become so debilitated, and the last few years are bad."

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