PSP In the News

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PSP In the News

Baltimore Sun Article: December 15, 1997, Monday,

FINAL EDITION

SECTION: TELEGRAPH (NEWS), Pg. 1A

LENGTH: 1819 words

BYLINE: K. Sugg, SUN STAFF

Tortured journeys lead to nowhere with rare disease;

PSP hard to diagnose, has no cure; some die never

knowing cause

Their words emerge as slurred whispers. They often

lose their balance and fall backward, grabbing for

towel fixtures and smashing into walls. Their faces

freeze in anguished expressions. Their eye muscles are

so weak that they can't look down. One patient, Bill

Scruggs, can't even see his grandchildren playing near

his feet. They, in turn, shy away from him. The

collective symptoms are so distinctive that diagnosis

would seem easy.

But this neurodegenerative disease, progressive

supranuclear palsy, or PSP, is rare. That fact forces

Scruggs and other patients into one of the most

tortured journeys in medicine.

Because their symptoms are unfamiliar, neighbors shun

them and doctors dismiss them. Victims wander for

years before finding out what went wrong, and some die

never knowing. Commonly misdiagnosed, they wind up

taking the wrong medicines and becoming misfits in

support groups for ailments that aren't their own.

They find themselves praying that someone famous will

come down with the same thing, to boost awareness and

research dollars.

With PSP and other rare illnesses, those who

ultimately reach the right diagnosis discover it leads

nowhere. There are no cures and few treatments.

Across the country, these circumstances are spurring

the formation of dozens of advocacy groups for rare

illnesses, as well as expansion drives among older

groups.

The Society for Progressive Supranuclear Palsy, based

at s Hopkins Hospital and founded in 1990, has

nearly doubled its membership in the past year, from

3,000 to 5,200. The Society recently received a $

400,000 donation from a man whose wife died of the

disease. One relative is so determined to attract

attention to PSP that in a few weeks, he will attempt

to climb a frozen mountain peak in South America and

unfurl a PSP flag at its summit.

This momentum is hitting a grim reality.

Fewer than a fifth of the 20,000 Americans believed to

have PSP have been diagnosed. Many people have been

mistakenly told they have Alzheimer's or Parkinson's,

two related diseases.

All three are conditions in which brain cells

gradually die. In PSP, those cells are in a few tiny

but important places in the base of the brain. It

usually strikes people in their 60s, causing trouble

with vision, swallowing, speaking, walking and

balance. Patients say they feel as if they're moving

through molasses. The course of the disease is faster

than Parkinson's, leading to death in about seven

years.

And unlike Alzheimer's, whose main problems involve

memory and language loss, mental impairment is mild.

Someone with PSP can consider their plunge into

helplessness. " Everyone feels very remote, very alone.

Many of our friends and neighbors shied away, were

embarrassed, " said Gualandi, whose husband, Gino,

died in May 1996. The New Jersey woman formed a

support group. " No one knows what this is. They know

what Alzheimer's is, cancer is, AIDS is, but this is

the lonely orphan. "

Cross-country trip

Bill Scruggs and his wife, Audrey, expected an active

retirement when he left his government job in 1983. He

turned 55 on a Thursday, retired on Friday and flew to

Florida with Audrey on Saturday. He lifted weights

daily, danced with his wife, traveled around the

world. Ten years later, on a cross-country trip, he

first noticed feeling dopey and fatigued.

Doctors suspected the andria, Va., man might have

Parkinson's disease. The neurologist put him on one of

the most successful treatments for it, dopamine. It

had no effect even after the dosage was doubled and

increased again.

Scruggs then got another diagnosis: multisystem

atrophy, another degenerative disease related to

Parkinson's. He tried other medicines, acupuncture and

chiropractors. They even sought out a psychiatrist.

Without knowing why, Scruggs was giving up pieces of

his life. He stopped driving, ballroom dancing,

playing bridge, cracking open crabs while watching

football games. Finally, in March, more than three

years after his first symptoms, Scruggs was diagnosed

with PSP. At 69, with no cure and few treatments, he

was in for a cruel finale.

There are prescriptions to help Scruggs sleep nights

and stay awake days. There is a therapist to teach him

techniques to better swallow and speak. Mostly,

though, there is the unspoken, the fear of what is to

come.

New chair

His handshake is strong but stiff. His eyes seem

locked on the picture window. His favorite blue

corduroy chair has been pushed into the corner,

replaced by a mechanical one with a seat that rises to

help him get out of the chair.

During the past year, Scruggs' falls have become more

frequent. The casualties: chairs, a glass tabletop, a

sink, a wall in the upstairs hallway. Many other

patients have had broken ribs, stitches, hospital

stays. So far, Scruggs has escaped that. At night,

he's so tired that by 8, his wife helps him to bed.

She finds him increasingly needing her help. She tries

to encourage him to do things himself. " I'm so afraid

that if I don't push, it's too easy then to say, 'It's

too hard,' " she said. " Once that happens, I'm afraid

it will be the end of it all. " But because certain

parts of the brain are affected, the disease literally

causes apathy, said Dr. G. Reich, assistant

professor of neurology at Hopkins medical school. In

the cafeteria of a Virginia hospital, Scruggs works

with a speech pathologist on techniques to help him

eat. That may prevent common, fatal complications of

PSP -- choking on food or aspirating it into the

lungs, causing pneumonia. His wife sits next to him

during the therapy, with their 18-month old grandson,

Mullett, on the other side. She alternates

between helping the toddler eat a grilled cheese

sandwich and cutting her husband's pork chop,because

he can't look down at his plate. Afterward, Scruggs

muses about why he got PSP.

" The only thing I can come up with is that God wanted

me to die with no pain, " he said. " There is no pain in

this disease. "

There is emotional pain

People with PSP sometimes behave inappropriately,

laughing or crying at the wrong times or talking too

loudly. A man might rise as a woman approaches the

dinner table, forgetting his risk of falling. Over

time, they acquire a nagging cough, a mask-like look,

eyes that seem faraway and sometimes hostile. All of

it makes people uncomfortable.

Some friends won't let their children near, fearing

needlessly that PSP might be contagious. One man's

staggering gait made people mistake him for a drunk.

Another man taking his dog for a walk was brought home

by a neighbor, who scolded the wife for allowing him

out of the house.

In some cases, the appearance of a PSP patient leads

people to assume that the person has lost his or her

mental abilities. But unlike those with Alzheimer's,

with severely affected memory and thought processes,

people with PSP often retain mental faculties.

ta Hunt, a Washington state woman whose husband

died from PSP in 1993, said she was chastised for his

presence at worship services. " So many people I know

said, 'Why is she bringing him to church? He can't

understand,' " she said. " But he knew what was going

on. "

Some PSP patients do develop dementia, but Reich said

it is difficult to track because of a patient's

trouble moving and talking.

Even Bill Scruggs' wife, Audrey, has troubled

deciphering his mumbled speech. She asks him to repeat

once, twice. Then she gets up from the couch and bends

near, asking him again what he said. Finally, she

gives up. He does, too.

Later, he asks her, " How many grandchildren do we

have, nine? "

" No, " she replies quietly. " Seven. "

Eventually, these patients won't be able to talk, or

walk, or even blink. Caregivers sometimes have to

manually open or close eyelids that get stuck.

Families say humor keeps them going. Gualandi's

husband was 6 feet tall and weighed 180 pounds. When

he fell in the bathtub, she couldn't lift him. So she

turned on the faucet and floated him out. The night

before Gino Gualandi died, she took him to a casino in

Atlantic City, N.J., where he perched on a stool at a

slot machine, pulling the lever. He fell backward. " We

got to laughing so hard, " she said. " He was just lying

like a turtle on his back. "

At the National Institutes of Health in Bethesda, Dr.

Irene Litvan has been trying to find treatments for

PSP. She is now testing an Alzheimer's drug that

increases the amount of a brain chemical involved in

memory and thinking. It may also help balance. But,

she said, for complex reasons, she often can't test

the drugs she believes have the most potential.

The origin of PSP is unknown. Dr. Lawrence I. Golbe,

Associate Professor of Neurology at the Wood

School of Medicine, believes the most likely

culprit is a naturally occurring chemical in food, air

or water. Genetics also are being investigated.

But physicians say nowhere in the United States is a

researcher working full time on PSP.

Almost 10 years ago, the National Commission on Orphan

Diseases published findings that experts say still

hold true: Research on rare diseases is uncoordinated

and inadequately funded. Information on the diseases

is lacking and poorly disseminated among patients,

families, physicians, insurers and scientists.

Insurers aren't covering needed services, such as

medicine, durable medical equipment and special foods.

" How far have we come, with no money and no authority?

We haven't come far, " said Abbey Meyers, president of

the National Organization for Rare Disorders.

All told, experts estimate that roughly 20 million

Americans -- or one in 12-- suffer from 5,000 rare

diseases. The NIH Office of Rare Diseases provides one

focal point, trying to link patients with each other

and with researchers.

Say hi to D-Dad

Meanwhile, Bill Scruggs is fading away. His wife,

Audrey, 71, carries on,running after their

grandchildren, urging the younger ones, and

2-year-old Vaughan, to go to their grandfather,

whom they call " D-Dad. "

" Can you go say hi to D-Dad? Go show that to D-Dad, "

she tells them. They ignore her, playing with each

other. then runs to her grandmother, hugging her

legs. " We love you, Grandma! " Bill Scruggs watches

from his chair. He leans forward and manages a

" Hi. " When the children run past him, he tries to pat

them, but can't quite reach.

Later, after several false starts, the toddlers stand

by his chair for a few minutes. Scruggs holds a small

book, reading as loud as he can: " Cup, cap, dish,

bottle, chair. "

For the first time in hours, his stiff facial muscles

allow his expression to match how he feels inside. He

smiles.

For information about PSP, call the Society at

or

1-. For information on rare diseases, see

the NIH's Office of Rare Diseases Web site at

http://rarediseases.info.nih.gov/ord/.

______________________________________________________________________

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[Guest guest]

Pam,

Did you note the date of that article (1997)? The odds are that he is gone

now. Note also that he had the same symptoms as many MSA patients.

Take care, Bill Werre

---------------------------------------------------------

Pam Bower wrote:

> http://www.psp.org/press_room/archive/10000001.asp

>

> http://www.psp.org

>

> PSP In the News

>

> Baltimore Sun Article: December 15, 1997, Monday,