Article on PSP family in Pennsylvania

This long article on a family dealing with PSP was published last week in a Central Pennsylvania newspaper, The Patriot-News. It’s a very realistic portrait of 69-year-old Larry Freeman in the final stages of PSP. He is cared for by wife Sue, son Chuck, and other family members. Larry is on hospice at home. He is mute and moves very little. He was diagnosed with PSP by Johns Hopkins in Baltimore.

It’s also a realistic portrait of Larry’s wife Sue and the overwhelming stress she feels. Part of the stress comes from so many family members living in her home.

Online, there are a dozen photos of the Freeman family as well as a one-minute video of Sue feeding Larry.

There are two points made by the author that I don’t agree with. Families who place their loved ones in care facilities are described as keeping a buffer between “themselves and their loved one’s impending demise.” The only difference in my mind is that the care facility is doing some of the “dirty work.” They certainly weren’t a buffer to my father’s impending demise.

Also, patients who stop taking in nutrition are described as starving to death. There’s a lot of data showing that people whose bodies are shutting down do not experience starvation. … _fami.html

As disease takes over dad, Perry County family crowds together to provide care
Published: Monday, April 11, 2011, 11:50 AM
By John Luciew, The Patriot-News

The mechanical medical bed whirs as the son raises his father’s head so he can eat.

Larry Freeman, once a robust racewalker, is completely immobile now.

His muscles are rubber. His face, a slack mask. His speech, a low, breathy jumble of nearly incomprehensible sounds.
He is on a hospital bed in the middle of the living room of his Newport area home. Five members of his family act as his primary caregivers. Two married couples have moved into the small house to help Larry’s wife, Sue, with his constant care.

Bare-chested, bleary-eyed and barely out of bed, only-son Chuck pads between the living room and the kitchen to take his father’s breakfast order.

It requires a couple of attempts, but Chuck manages to decipher the mumblings of his 69-year-old dad.

Larry wants scrapple.

Chuck asks again, just to be sure.

Scrapple it is.

Sue sets to frying the frozen meat in a skillet. Chuck rounds up the rest of Larry’s breakfast: a bowl of Raisin Bran, a half of a glazed doughnut and a cup of coffee with a bendy-straw.

Ill as he is, Larry’s appetite remains strong.

Because Larry can no longer feed himself, Chuck dishes it out, bite by bite. He uses a spoon, so Larry doesn’t hurt himself champing down on a fork. Chuck goes slow, making sure his father chews each bite before doling out another.

Back when Larry worked on the production line at the Quaker Oats plant in Hampden Township, the finely-tuned weekend athlete was famous for wolfing down his meals. At home, he’d be up from the dinner table and out in the garden before Sue finished her second bite.

But like the rest of Larry’s muscles, the ones that gently move food down Larry’s throat have been ravaged by disease.

Now, no matter how slowly Larry eats, food can get stuck, then back up into his throat.

This ignites prolonged and scary choking sessions, during which Larry is in danger of passing out — or worse.

His body is no longer capable of mustering even one deep, cleansing cough to clear his throat. The disease has taken this away, too.

For the moment, Larry eats his breakfast in peace. Even at the deliberate pace, he makes quick work of a saucer plate-sized portion of scrapple, three-fourths of the Raisin Bran, a bite of the doughnut and several sips of coffee.

Now, it’s time for his medicine.

Sue walks over with a bottle of Ensure and a fistful of pills.

There are so many, Chuck feeds them to his father two at a time. Each dose is washed down with a drink of the Ensure. Larry insists on the thick, calorie-boosting beverage when taking his medicine. Strawberry is his favorite.

With that, another day of caring for Larry has begun in the Freeman household.

Catastrophic illness has come to call on the family in the form of a rare disease with a long name — progressive supranuclear palsy.

When it was finally diagnosed in late 2009 and the prognosis appeared grim, Sue wouldn’t hear of lodging Larry in a nursing home or long-term care facility.

“Being at home is better,” she insists. “Being around family is better.”

So, the Freemans banded together to do battle with Larry’s disease.

Last December, Chuck, 38, moved back home with his wife, Paula. Paula’s sister Kandy and her husband, Bob Little, have since moved in, as well.

It makes for cramped quarters inside the Freeman’s three-bedroom double-wide. But there is strength and support in their numbers.

Family members take shifts caring for Larry, as if they were a team of round-the-clock nurses. They divvy up the cooking and chores. And they pool money for food, as dinners must now feed six.

Most of all, they buoy each other with a combination of emotional sustenance and comic relief essential in such life-and-death struggles.

Sure, there are sisterly squabbles, constant complaints and long, uncomfortable nights in cramped confines. But the
Freemans are finding a different kind of comfort in one another as they care for one of their own.

Still, each long day begins and ends with heavy burdens that never ease.

“I never dreamed I’d be doing this hands-on, 24-7,” a weary Sue says near the end of another seemingly endless day of juggling her school bus route, Larry’s care and her household chores.

“You never know what life is going to give you around the corner,” the 57-year-old shrugs. “Life is very funny that way.”

‘Kryptonite to Superman’
When devastating disease strikes, there are two types of families.

The first prefer to keep disease and death at arm’s length. By choice or necessity, they seek the assistance of professional staffing agencies and the insulation of institutional settings as buffers between themselves and their loved one’s impending demise.

The second insist upon taking on the role of primary caregiver themselves.

The Freemans are the latter.

They have enrolled Larry in Hospice of Central Pennsylvania, and they pay a private-duty caregiver to come in for a few hours a day, mostly when Sue is out making her afternoon van runs for the West Perry School District.

But they are never not involved in Larry’s care.

“It’s a heck of a thing to take on,” says Don Bechtel, a hospice nursing assistant who checks in on Larry for about an hour most weekdays.

“People don’t really know what it takes,” he says with experience of having cared for more than 100 at-home patients. “The biggest shock is that it’s like taking care of a baby. You don’t want to make the patient feel that way, but that’s how it is.”
The biggest adjustment for the Freemans has been how quickly and completely their once-strong and active husband and father has become immobile, bed-ridden and totally dependent upon their care.

For two decades, Larry traveled all over the country as a competitive racewalker.

The trophies he won cram a bookcase. Framed pictures of Larry mid-stride and clad in shorts and tank top, with his race number pinned to his chest, fill his bedroom.

That he excelled in an exacting sport requiring near flawless muscle control seems darkly ironic, because it’s everything his body now lacks.

Race walking is a hip-swiveling, weight-shifting, foot-shuffling study in grace, endurance and controlled speed.

It’s a sport in which its fast-walking participants can look odd, even comical, as if they have an urgent need for a bathroom. But truth be told, it requires tremendous athleticism.

Racewalkers stay low to the ground, arms pumping close to their hips, shoulders barely rising. Their exaggerated hip movement is in fact a full rotation of the pelvis. Their only goal is forward propulsion.

World-class racewalkers can clock seven-minute miles. They do it without their feet ever losing contact with the ground.
The equivalent of one full foot must remain on the ground at all times. A “loss of contact” can result in time penalties or even disqualification.

Racewalkers accomplish this by keeping the knuckle and toes of their back foot and the mid-sole and heel of their front foot on the pavement at all times.

This exactitude reduces a racewalker’s stride and forces him to step up his cadence. The resulting stride rates are comparable to Olympic athletes running 400-meter races. Yet, racewalkers can maintain the pace for hours at a time.

Larry Freeman was a master of the sport.

Even a heart attack six years ago couldn’t slow him down. He worked himself back into shape, and he swiveled his hips and shuffled his feet in the annual Perry County Turkey Trot.

That was November 2007.

It would be his last race.

Quietly, inexplicably, he just stopped.

His family figured it was his heart troubles finally slowing him down.

It wasn’t.

A couple years later, Larry gave up his second love — gardening. Hours could pass as Larry tended his planting patch, while oldies tunes blared on his radio.

Now he was ceasing this, too.

Yet, it wasn’t until he began tripping and falling that family members began to worry.

One afternoon, Larry plunged face-first into a cactus plant in the front yard. His grand-niece Jasmine witnessed his frightening fall and let out a terrified scream. “Grandpa fell!”

Larry was already picking himself up and doing his best to make a joke of it. But the dumbfounded looks on his family members’ faces said otherwise.

Something was wrong.

Larry’s speech, never a model of diction, was getting increasingly worse. He was badly slurring words now. And he could be seized by coughing fits so violent, he’d get dizzy, even lose his balance.

At first, family doctors suspected allergies, even Lyme disease. He went to specialists for batteries of tests and ended up with a bevy of pills.

None of it worked.

The coughing — the hacking — not only continued, it grew worse.

One spell caused Larry to crash the local Meals on Wheels truck while making his mid-morning rounds.

Another barrage of medical tests led Larry to a neurologist. The doctor noticed something odd about his eyes.
Larry could not look up. His eyes wouldn’t move in an upward direction. It was a tell-tale sign of a very rare and devastating disease.

A couple of trips to Johns Hopkins in Baltimore confirmed the worst.

The experts finally put a name to what was wrong — progressive supranuclear palsy. PSP for short.

When the same disease first gripped its most famous sufferer, Dudley Moore, people mistook the late actor’s increasingly clumsy falls as a real-life incarnation of Moore’s hard-drinking character from the hit movie, “Arthur.”

Only this was no laughing matter. The disease’s initial symptoms often include loss of balance, lunging forward, knocking into objects and falling. It explained Moore’s problems — and Larry’s.

But while the experts had finally diagnosed what was wrong with Larry, they could do nothing to help him. There’s no cure for this venomous combination of Alzheimer’s, Parkinson’s and Lou Gehrig’s.

And to see his dad now — little more than a year after that fateful diagnosis — is to cause Chuck to wonder where the man he once knew has gone.

“Let’s just say it’s been like giving Kryptonite to Superman,” Chuck says of the disease’s effects. “You know how Superman is with Kryptonite? He’s helpless, and you don’t know what to do. The planet’s in trouble, and he can’t do anything about it. That’s how I feel.”

Day by day, a son watches his hero fade.

Savoring the sun
The Freemans’ double-wide tucked off a rural road west of Newport is a sick bay, a rooming house and, when the stress gets to be too much, a place to party and let go.

Larry is front and center for it all.

Instead of being shunted aside in a bedroom, his medical bed is parked in the living room, right in front of the picture window.

He could be in a warmer spot, especially because he constantly complains of being cold. It’s yet another symptom of the disease. Larry’s wasted muscles can no longer produce those rapid-fire contractions that we take for granted as shivering.
Nevertheless, the lover of all things outdoors insists upon being by the window. Larry keeps his face tilted toward the light, even if the sun hurts his eyes.

The muscles of his eyes have slackened. His pupils no longer properly expand and contract.

His family places a damp wash rag over his eyes, instead. This keeps them moist and protects Larry from the skull-searing brightness that his eyes can no longer regulate.

At least he can feel the warm shafts of sunlight on his face.

While Larry and the demands of his disease are a constant presence in the Freeman house, family life goes on beyond his care.

Working through a temp agency and assigned to a Middlesex Twp. warehouse, Chuck would love a job that’s more permanent and conveniently located.

After feeding his father, he checks the help-wanted section of the newspaper, then trolls the Internet.

Between her morning and afternoon school van runs, Sue is on call for her second employer, the Carson Long Military Academy in New Bloomfield. She’s constantly shuttling cadets between the school, the Army bases in Carlisle and the train station in Harrisburg.

Fresh off her morning school run, a call comes in.

Sue isn’t sure if she should accept the dispatch.

Sisters Kandy and Paula are out running errands. And with Chuck unable to leave Larry’s side, there’d be no one to pick up Larry’s lunch from the senior center in Newport.

According to the menu calendar posted on the refrigerator door, today’s lunch is liver, Larry’s favorite.

“Better not take a chance,” Sue tells the dispatcher, sounding reluctant to pass on the assignment.

Chuck grabs his cell phone and quickly calls his wife. The women can swing by Newport for Larry’s meal on their way back from Camp Hill.

Chuck signals this to his mother, and Sue reverses her initial decision.

“Okay, I’ll take it,” she tells the dispatcher.

Before heading for a shower, Sue tunes the TV to “Regis and Kelly,” one of Larry’s favorite shows. The program flashes on the screen just as applause ebbs and the two hosts begin their good-natured banter.

For the rest of the day, the TV furnishes the soundtrack and provides the background noise at the Freeman house.
As the hours roll by, Sue cycles through channels, tuning in Larry’s favorites: “Hogan’s Heroes,” “McHale’s Navy,” “The Cosby Show.”

But with his eyes mostly closed or covered by a wash cloth, it’s hard to tell if Larry is watching or even listening to the laughtrack-laced levity.

‘Work it loose!’
Though mute and often motionless, Larry can make his presence known at any time.

Some of his ways are playful.

Lately, he has a habit of knocking a stuffed animal out of his bed when he wants attention. This forces someone to fetch the toy.

Hospice nurses have warned that Larry will sometimes act like a spoiled two-year-old. And after repeatedly fetching the stuffed frog, Kandy comes up with a back-saving solution.

She tethers the toy to the rails of Larry’s hospital bed. This way he can still toss it, but it won’t hit the floor, merely dangle at the side of his bed.

Fixing Larry’s covers is another constant chore.

His pile of blankets has a way of sliding off his shoulders. He announces his discomfort with a low moan.

“He could have on a million blankets and still be cold,” Chuck says, shaking his head before answering another of his father’s calls.

To cut down on these complaints, Chuck positions folded afghans at either side of Larry’s head. This weighs down the rest of the covers, keeping them from sliding off.

Another way Larry demands attention is scary, even life-threatening.

He chokes.

One moment, Larry is downing his thrice-daily dose of more than a dozen pills. It’s the usual routine following a meal.
What happens next is anything but.

At first there’s no sound at all. No breathing. No coughing. Nothing.

Larry’s eyes expand into saucers. His mouth constricts into an oval. They’re the only expressions on his otherwise blank face.

Finally, he musters his version of a cough. It’s really more of a rasp, a wet terrible sound.

It’s not nearly enough to clear his throat and restore his breathing.

Larry isn’t choking in the traditional way, either. That is to say, a piece of food isn’t lodged in his windpipe.

Rather, the food he’s been eating has halted its descent down his esophagus. The rhythmic muscle movements that usually usher things along do not work inside Larry’s throat. And when the food backs up too far, it plunges Larry into respiratory distress.

At the first sign of this, Chuck and Sue swing into action.

They pull Larry forward, Sue from the front, Chuck from behind.

Sue locks eyes with her husband and shouts directly into his face. She is desperate to establish a connection that will prevent Larry from losing consciousness.

“Are you okay?” she shouts. “Work it loose! Work it loose!”

Chuck massages Larry’s bare back, as if trying to jump-start his father’s throat muscles and get the offending backlog of food moving again.

Instead, Larry’s fear-widened eyes start rolling back in his head.

Sue shouts again.

“Don’t go under on us! Work it loose!”

Larry is still with her — for the moment.

His raspy, desperate half-coughs go on for what seems like minutes. But the whole episode is no longer than 90 seconds.

These scary choking spells are more common as Larry’s swallowing continues to deteriorate. The Freemans must be prepared for such emergencies at all times, even when he’s not eating. Larry’s own saliva can cause the same distress.

And if Larry’s condition ever reaches the point where he can no longer swallow or cannot recover from one of his choking spells — what then?

Sue returns a blank expression, then shrugs. She cannot give voice to the inevitable outcome:

There will be no feeding tube; no heroic measures to resuscitate her husband. Larry has signed a living will banning all artificial means of life support. His disease eventually will take its course.

This time, Larry’s half-hearted hacking finally stops.

The logjam of food has cleared. He is back to breathing normally.

Larry’s mask of a face grows slack once again, as if the violent episode never happened.

Chuck and Sue ease him back on his pillow, but they keep the bed raised for a long while, until he fully digests his food.
Sue reaches for Larry’s Ensure. He eagerly takes the straw into his mouth.

“It clogs right there in his throat and he chokes,” explains Sue, her own fright just beginning to dissipate.

It takes her far longer to catch her breath.

Chaos and clutter
Larry is having a bad day.

Sue’s is worse.

It begins with what seems the simplest of tasks. Returning from her morning school van run, Sue has it in mind to pay the family’s monthly auto loan.

She breezes into the house, checks on Larry, then sets to writing the check.

Only she can’t find the loan payment bill and its return envelope. The combined clutter of three families is consuming her once-orderly, but now-cramped, home. And the loan bill is lost in the ruin.

Sue is determined to find it.

She enlists Kandy and Paula it what soon becomes an infuriating and ultimately futile search.

Finally giving up on locating the actual bill, Sue attempts to make the truck payment by phone.

She calls two local Ford dealers before obtaining the number for Ford credit. She dials, figuring a representative can look up her account and credit her payment.

Instead, she descends into a catch-22 of automated answering hell.

A computer-generated voice demands the account number that she doesn’t have. Determinedly, Sue pushes the number for a representative. But before connecting her, the emotionless, computer-generated voice is back asking for her account number.

Ninety minutes of futility finally boils over.

Sue disconnects the call, tosses aside the phone and lets loose a frustrated torrent.

“They kept saying, ‘I need the account number,’¤” Sue shouts. “How in the world can I give you the account number if I don’t know it!”

A hospice social worker walks in on the turmoil. She asks what’s wrong.

“Where would you like me to start?” Sue shoots back.

For the next half-hour, they hammer out an action plan designed to siphon away some of Sue’s mounting stress.

“I hate seeing my house like this,” Sue says. “I feel like the walls are closing in.”

Paula and Kandy are stone quiet as the social worker lays out a solution calling for the two of them to do more housework.
The plan is memorialized in a handwritten to-do list that’s posted on the fridge. Among the chores, a spare bedroom that has become a repository for three families’ junk must be cleaned, and the claustrophobic kitchen that is swallowing up bills must be uncluttered.

“I’ve always said, I want this place cleaned up,” Sue exhales. “I’m not used to this.”

Paula and Kandy agree, but they make themselves scarce for the rest of the day.

“When families combine, there’s a lot of stuff,” Paula explains. “Organization is mom’s thing. And right now it’s chaos

Yet, it’s only the beginning of Sue’s trials this day.

Hospice sent large-sized adult diapers that don’t fit Larry’s slender athlete’s body. They keep sliding off.

Worse, Larry isn’t eating well. He had a restless night. And because Sue sleeps on a roll-out couch in the same living room, so did she.

“It’s one of those days,” she says. “One of those stressful days. I’d give anything to crawl in a hole, cover it up and have nobody find me.”

Instead, Sue plans an ambitious dinner — beef pot pie with homemade noodles. She’ll spend most of the afternoon simmering meat, dicing onions, slicing potatoes and mixing and rolling dough.

By mid-afternoon, an appetizing aroma permeates the house. Perhaps, this will coax Larry to eat.

‘It gets overwhelming’
Caregiver Nicole Bouder sees it as soon as she enters the house.

Her patient is in trouble.

Only it’s not Larry; it’s Sue.

The family matriarch looks dead on her feet, but she has dinner to finish.

“Are you OK, Sue? Talk to me, Sue,” says Nicole, a private-duty caregiver the Freemans hire for 15 to 20 hours a week. It’s the only major expense not covered by Larry’s Medicare and retiree health benefits.

Nicole is not a nurse, but she changes Larry’s adult diapers, washes him down and freshens him up.

Lately, she’s found herself looking after Sue, as well.

“Sometimes, it gets overwhelming for her with everything that’s going on,” Nicole says. “A lot of it is still on her. Even though there’s help, she’s still doing a lot of it.”

Nicole’s prescription: heavy doses of humor.

“I can usually get her to smile,” Nicole says.

Sure enough, Sue’s drawn mouth manages a grin. Unfortunately, it’s short-lived.

Nicole’s two-hour shift is finished, and Sue’s break is over.

The two women hug goodbye.

“She’s a good one,” Sue says as Nicole departs. Then, she pushes herself up from the kitchen table to finish dinner.

Sue grabs for the coffee maker to perk herself up. But when she pours water into the machine, it overflows onto her cluttered countertops.

Someone already filled it. It’s yet another sign that Sue’s house is no longer her own.

“It’s just everything lately,” Sue mutters as she dots a kitchen rag at the spill. “It’s just a little bit of everything.”

The brief reprieves
Moments of grace come like quiet bolts of lightening.

It’s the way 7-year-old Jasmine can still coax a smile from her sick grand-uncle. It’s how Kandy’s shy, stow-away rat terrier named Digger can snuggle up to Larry in his hospital bed.

Paula and Kandy are boarding their large collection of cats and dogs at Kandy and Bob’s house outside Duncannon. The two make regular runs to check on the animals and collect Kandy’s mail.

Digger is the only pet to get a pass. Larry is allergic to cats but loves dogs. He’s made a fast friend in Digger.

When the family comes together, it can be better than any medicine.

On New Year’s Eve, Paula and Kandy move the party to Larry’s living room.

Why should he be left out?

Having one of those rare days where his mind is quick and his tongue loose, Larry calls out answers as the family plays Trivial Pursuit on the Wii. He even has a beer or two. Other family members favor the harder stuff.

“Life is too short,” Paula insists. “We have to have fun.”

The two younger couples have their own bowling team, appropriately named “Family Affair.” They compete every Sunday night. Their record is nothing to speak of, but the night out is a welcomed release.

Sue’s passion is Bingo. Her nights out are Thursdays. She goes right from her afternoon van run to a friend’s house. From there, they head to Selinsgrove for the weekly game.

Sue covers a dozen or more Bingo boards at once. When the numbers are coming fast and her eyes are scanning multiple sheets for matches, Sue can forget just about everything.

But the reprieve is all too brief.

“Sometimes, I feel like I’m getting the short end of the stick,” she says.

Mostly, she puts on a brave front.

“I say to people, ‘I guess I’m really living up to my marriage vows now.’”

A day’s vindication
Larry could go on like this for quite a while — years, perhaps even a decade.

His life as a racewalker has left his body in remarkable shape. Immobile as he might be, Larry’s muscles still have tone. His weight is good.

Besides, those with PSP do not die of the disease. By itself, it’s not considered terminal. Its symptoms are the real killers — falls, choking, pneumonia, infection and lack of nourishment.

Some sufferers literally starve to death.

That’s not Larry. Not now, at least.

Sue’s home cooking does the trick.

After turning up his nose at breakfast and lunch, Larry is ravenous for Sue’s beef pot pie. An afternoon of smelling the homemade meal simmering on the stove has more than whet his appetite.

Sue’s day of hard work is vindicated. “My word, you really were waiting on this,” she says, smiling.

Larry cleans the plate.

Sue returns his dish to the sink, before finally enjoying a plate of her own.

Following her meal, Sue settles into the easy chair.

It’s 7 p.m., and she puts her feet up for the first time this day.

“I think I’m gonna take a little break,” she announces.

Having made some headway on the cleaning to-do list, Kandy hands Sue a wine cooler. The flavor is appropriate — “Jamaican Me Happy.”

Sue sips the drink and seems to sink deeper into the red-cushioned recliner.

She turns on a re-run of the 1980s TV sitcom “Family Ties.” Michael J. Fox, a Parkinson’s survivor himself, looks impossibly young.

Soon, Sue is smiling and laughing with the Keaton family. A few feet away, Larry sleeps. He’s quiet and content, if only for the moment.

“These were the best shows,” Sue says, still grinning at the TV. “The old-time family shows.”

Progressive Supranuclear Palsy, or PSP
The Cause: It involves damage to many cells of the brain. Affected areas include parts of the brainstem that control eye movement; areas of the brain controlling steadiness when walking; frontal lobes of the brain leading to personality changes.
The Symptoms: Changes in facial expressions; difficulty controlling eye movements, swallowing, coordination and speech; personality changes, including forgetfulness, apathy and dementia; repeated falls. All symptoms grow worse over time.
The Cure: None.

Can hospice have a conversation about not eating/drinking?

This is a question for Bill but I thought others would be interested in it.

A local support group member contacted me recently. Her mother has had PSP for several years. The mother would like to hasten her own death by not eating or drinking any further. She has been able to communicate this to her daughter. She asked that the daughter make arrangements to spend time with the mother. Basically, the mother wants to plan her own death.

The daughter talked to the hospice agency they’ve had on board for about 8 months about this. The hospice agency RN basically said “we can’t have this conversation with you.” The daughter deduces that the hospice agency can’t be involved in the planning of this or the weighing of this decision but they are willing to be notified once the decision has been made.

Would you say that all or most hospice agencies have the same policy?

The daughter is worried that the hospice agency, upon notification of the mother’s decision, will try to talk the mother out of it. I encouraged the daughter to speak with the highest authority possible at the agency, and communicate that this was the mother’s decision and hospice should not attempt to talk her out of it. Your comments?


News story about Pennsylvania family coping with PSP

This long article on a family dealing with PSP was published last week in a Central Pennsylvania newspaper, The Patriot-News. It’s a very realistic portrait of 69-year-old Larry Freeman in the final stages of PSP. He is cared for by wife Sue, son Chuck, and other family members. Larry is on hospice at home. He is mute and moves very little. He was diagnosed with PSP by Johns Hopkins in Baltimore.

It’s also a realistic portrait of Larry’s wife Sue and the overwhelming stress she feels. Part of the stress comes from so many family members living in her home.

Online, there are a dozen photos of the Freeman family as well as a one-minute video of Sue feeding Larry.

There are two points made by the author that I don’t agree with. Families who place their loved ones in care facilities are described as keeping a buffer between “themselves and their loved one’s impending demise.” The only difference in my mind is that the care facility is doing some of the “dirty work.” They certainly weren’t a buffer to my father’s impending demise.

Also, patients who stop taking in nutrition are described as starving to death. There’s a lot of data showing that people whose bodies are shutting down do not experience starvation.

As disease takes over dad, Perry County family crowds together to provide care
By John Luciew
The Patriot-News
Published: Monday, April 11, 2011, 11:50 AM



Short Section of MSA in Perlman Chapter

A chapter on spinocerebellar degenerations, written by Dr. Susan Perlman of UCLA, was recently published.  It’s part of a “Handbook of Clinical Neurology.”  Obviously this is written for neurologists so it is entirely medical terminology.

There is a small section on multiple system atrophy (MSA) in the chapter.  MSA is described as “a sporadic ataxia which is felt to have a genetic substrate.”

A few things were interesting to me as I’d never heard them before:

* “Upper motor neuron signs were seen in 50% (spasticity; brisk tendon reflexes, pseudobulbar speech, and swallowing difficulties).”

* “Dementia, ophthalmoplegia, and chorea are not seen.”  (I never heard that chorea is not seen in MSA before.)

*  “Later in the course, stridor due to laryngeal abductor paralysis, progressive signs of obstructive sleep apnea, and neck muscle weakness heralded the terminal stages.”  (I never heard that neck muscle weakness marks the end stages.)

* “Of the SCAs (1­5% of which can present with no family history), SCA3 is most likely to mimic MSA.”

Copied below is the abstract to the chapter.


Handbook of Clinical Neurology. 2011;100:113-40.
Spinocerebellar degenerations.
Perlman SL.

The spinocerebellar ataxias (SCA) are a large group of inherited disorders affecting the cerebellum and its afferent and efferent pathways. Their hallmark symptom is slowly progressive, symmetrical, midline, and appendicular ataxia. Some may also have associated hyperkinetic movements (chorea, dystonia, myoclonus, postural/action tremor, restless legs, rubral tremor, tics), which may aid in differential diagnosis and provide treatable targets to improve performance and quality of life in these progressive, incurable conditions.  The typical dominant ataxias with associated hyperkinetic movements are SCA1-3, 6-8, 12, 14, 15, 17, 19-21, and 27. The common recessive ataxias with associated hyperkinetic movements are ataxia telangiectasia and Friedreich’s ataxia.

Fragile X tremor-ataxia syndrome (FXTAS) and multiple-system atrophy (a sporadic ataxia which is felt to have a genetic substrate) also have hyperkinetic features. A careful work-up should be done in all apparently sporadic cases, to rule out acquired causes of ataxia, some of which can cause hyperkinetic movements in addition to ataxia.

Some testing should be done even in individuals with a confirmed genetic cause, as the presence of a secondary factor (nutritional deficiency, thyroid dysfunction) can contribute to the phenotype.

Copyright © 2011 Elsevier B.V. All rights reserved.

PubMed ID#: 21496573  (see for this abstract only)

New Alzheimer’s Diagnostic Guidelines

A New York Times article today is about new Alzheimer’s diagnostic guidelines, which have been in the works for awhile. One new component is that “the guidelines specify that Alzheimer’s biomarkers — including abnormal levels of the proteins amyloid and tau, and shrinkage of certain brain areas — should not yet be put into widespread use, but used only with patients enrolled in clinical trials.”

“The guidelines also clarify diagnosis criteria for people with dementia symptoms, distinguishing Alzheimer’s from other dementias, including vascular, fronto-temporal and Lewy body. And they note that the earliest symptom of Alzheimer’s dementia is not always memory loss, but could be mood changes or problems with language, spatial perception or reasoning.”

I haven’t had a chance yet to look through the new guidelines to learn about these clarifications. You can find a link to the new guidelines and an overview of them on the Alzheimer’s Association website here: … erview.asp

The article also mentions that there is legislation in Congress that would “create specific Medicare cost codes for Alzheimer’s diagnosis, including steps involving discussions between the patient’s doctor and caregivers, a recognition that keeping family members well-informed can result in better planning and care.”

Of course it would be nice to have those discussions paid for as part of a medical appointment about any disorder, not just AD.

Robin … eimer.html

Guidelines Allow Earlier Definition of Alzheimer’s
By Pam Belluck
New York Times
Published: April 19, 2011

FDA Safety Communication – PPIs

PPIs are used to treat GERD and other GI tract problems. … 245275.htm

Proton Pump Inhibitor drugs (PPIs): Drug Safety Communication – Low Magnesium Levels Can Be Associated With Long-Term Use

Prescription PPIs include Nexium (esomeprazole magnesium), Dexilant (dexlansoprazole), Prilosec (omeprazole), Zegerid (omeprazole and sodium bicarbonate), Prevacid (lansoprazole), Protonix (pantoprazole sodium), AcipHex (rabeprazole sodium), and Vimovo (a prescription combination drug product that contains a PPI (esomeprazole magnesium and naproxen).

Over-the-counter (OTC) PPIs include Prilosec OTC (omeprazole), Zegerid OTC (omeprazole and sodium bicarbonate), and Prevacid 24HR (lansoprazole).

[Posted 03/02/2011]

AUDIENCE: Consumer, Gastroenterology, Family Practice

ISSUE: FDA notified healthcare professionals and the public that prescription proton pump inhibitor (PPI) drugs may cause low serum magnesium levels (hypomagnesemia) if taken for prolonged periods of time (in most cases, longer than one year). Low serum magnesium levels can result in serious adverse events including muscle spasm (tetany), irregular heartbeat (arrhythmias), and convulsions (seizures); however, patients do not always have these symptoms. Treatment of hypomagnesemia generally requires magnesium supplements. In approximately one-quarter of the cases reviewed, magnesium supplementation alone did not improve low serum magnesium levels and the PPI had to be discontinued.

BACKGROUND: PPIs work by reducing the amount of acid in the stomach and are used to treat conditions such as gastroesophageal reflux disease (GERD), stomach and small intestine ulcers, and inflammation of the esophagus.

RECOMMENDATION: Healthcare professionals should consider obtaining serum magnesium levels prior to initiation of prescription PPI treatment in patients expected to be on these drugs for long periods of time, as well as patients who take PPIs with medications such as digoxin, diuretics or drugs that may cause hypomagnesemia. For patients taking digoxin, a heart medicine, this is especially important because low magnesium can increase the likelihood of serious side effects. Healthcare professionals should consider obtaining magnesium levels periodically in these patients. For additional information, refer to the Data Summary section of the FDA Drug Safety Communication.

Healthcare professionals and patients are encouraged to report adverse events, side effects, or product quality problems related to the use of these products to the FDA’s MedWatch Safety Information and Adverse Event Reporting Program:

* Complete and submit the report Online:

* Download form or call 1-800-332-1088 to request a reporting form, then complete and return to the address on the pre-addressed form, or submit by fax to 1-800-FDA-0178

[03/02/2011 – Drug Safety Communication – FDA]

Recall of Citalopram, etc.

This Pfizer press release was posted to earlier this month. It may be of interest to some of you.

Recall — Firm Press Release
FDA posts press releases and other notices of recalls and market withdrawals from the firms involved as a service to consumers, the media, and other interested parties. FDA does not endorse either the product or the company.
Page Last Updated: 04/01/2011

Greenstone Announces Voluntary Nationwide Recall
Of Citalopram And Finasteride Due to Possible Mislabeling

Pfizer Inc.

FOR IMMEDIATE RELEASE – March 26, 2011 – Greenstone LLC announced today that it is voluntarily conducting a recall, to the patient level, of medicines with lot number FI0510058-A on the label. This includes Citalopram 10mg Tablets (100-count bottle) and Finasteride 5mg Tablets (90-count bottle), both distributed in the U.S. market. The recall is due to the possibility that incorrect labels have been placed on the bottles by a third-party manufacturer. This is the only lot number being recalled and no other lots or markets are believed to be impacted.

Importantly, bottles labeled as Citalopram Lot # FI0510058-A may contain Finasteride. Patients who believe they may have ingested the wrong medication should contact their physician as soon as possible. Women who are, or may become pregnant, should not take or handle Finasteride due to the possible risk of side effects which may cause abnormalities to the external genitalia of a developing male fetus. Citalopram is contraindicated in patients taking monoamine oxidase inhibitors (MAOIs) or pimozide, It is also contraindicated in patients with a hypersensitivity to Citalopram or any of the inactive ingredients in the tablet. Patients who discontinue Citalopram abruptly by inadvertently taking the mislabeled product may experience discontinuation symptoms and/or worsening of depression.

Bottles of either Citalopram (used to treat depression) or Finasteride (for the treatment of benign prostatic hyperplasia) with lot number FI0510058-A should be returned to the pharmacist.

Consumers should contact their physician or healthcare provider if they have experienced any problems that may be related to taking or using this product. Also, any adverse events that may be related to the use of these products should be reported to Pfizer Inc. at-1-800-438-1985 (24 hours a day) or to FDA’s Med Watch Program either online, by regular mail or by fax.

Greenstone LLC is a wholly owned subsidiary of Pfizer Inc.

Review of CBD (Mayo)

Mayo researchers just published an article on CBD. The abstract is a mix of clinical information and pathological information. If you are like me, you’ll want to skip past the pathological terminology!

The clinical information in the abstract leads me to believe that the article’s content is very similar to Dr. Irene Litvan’s webinar several months ago on CBD. You can find my notes on that webinar here:

The abstract mentions the term “Richardson syndrome.” This refers to the most common form of PSP (progressive supranuclear palsy); one of the symptoms of this form is cognitive impairment.


Nature Reviews. Neurology. 2011 Apr 12. [Epub ahead of print]

Corticobasal degeneration: a pathologically distinct 4R tauopathy.

Kouri N, Whitwell JL, Josephs KA, Rademakers R, Dickson DW.
Department of Neuroscience Mayo Clinic, Jacksonville, FL.

Corticobasal degeneration (CBD) is a rare, progressive neurodegenerative disorder with onset in the 5(th) to 7(th) decade of life. It is associated with heterogeneous motor, sensory, behavioral and cognitive symptoms, which make its diagnosis difficult in a living patient.

The etiology of CBD is unknown; however, neuropathological and genetic evidence supports a pathogenetic role for microtubule-associated protein tau.

CBD pathology is characterized by circumscribed cortical atrophy with spongiosis and ballooned neurons; the distribution of these changes dictates the patient’s clinical presentation. Neuronal and glial tau pathology is extensive in gray and white matter of the cortex, basal ganglia, diencephalon and rostral brainstem. Abnormal tau accumulation within astrocytes forms pathognomonic astrocytic plaques.

The classic clinical presentation, termed corticobasal syndrome (CBS), comprises asymmetric progressive rigidity and apraxia with limb dystonia and myoclonus. CBS also occurs in conjunction with other diseases, including Alzheimer disease and progressive supranuclear palsy.

Moreover, the pathology of CBD is associated with clinical presentations other than CBS, including Richardson syndrome, behavioral variant frontotemporal dementia, primary progressive aphasia and posterior cortical syndrome.

Progress in biomarker development to differentiate CBD from other disorders has been slow, but is essential in improving diagnosis and in development of disease-modifying therapies.

PubMed ID#: 21487420 (see for this abstract only)

Adjusting to the diagnosis – 4/6/11 webinar notes

If anyone else attended this webinar today, I’d be interested in receiving your comments and any notes you took (especially in places where I missed things). I thought the middle part of the webinar was especially good. If you missed it, here are my notes. And CurePSP staff indicated that the recording of this webinar should be available online ( by 4/8/11.

Early Stages of Diagnosis: A Family Guide for PSP, CBD & MSA — the process of adjusting to the diagnosis, and important steps for patients and families to take during the early stages of the diseases

Webinar Speaker: Trish Caruana, MSW, VP of Programs and Education at CurePSP

4/6/11 (rescheduled to this day from 3/30)

* Provide a brief overview of PSP, CBD, and MSA
* Discuss the process of being diagnosed
* Review the initial adjustment to the diagnosis
* Outline the steps to take to ensure the best overall care
* Develop a framework to maintain health

What is PSP?
* Rare neurodegenerative brain disease (5-6 per 100K – “incidence.” Robin’s note: she means “prevalence”)
* Problems with stiffness, balance and vision, speech and swallowing
* Changes in personality
* Cognitive changes

What is CBD?
* Similar to PSP (balance, vision, speech and swallowing)
* Rare: estimated 2K-3K in the US
* Difficulty generating and articulating speech
* Stiffness, jerkiness, slowness, and clumsiness either in the upper or lower extremities
* Asymmetric onset of symptoms
* Memory or behavior problems
* Many of these symptoms may not be appearing now, if you are in the early diagnosis phase

What is MSA:
* Also an atypical parkinsonian disease
* Involuntary functions are affected such as blood pressure, heart rate, actions of the intestines and bladder and breathing activity
* OH
* Urinary incontinence and constipation
* Impaired speech and swallowing
* Inability to sweat
* Sleep apnea and sleep disturbance
* Waving hand movements and staggering gait

Someone initially given this info is going to be taking in a lot of info and trying to figure out how their lives will change.

The process of being diagnosed
Buddhist saying: “If you are facing the right direction, all you need to do is keep walking.”
Getting a diagnosis affects everyone psychologically

Misconceptions about diagnosis
Our simplistic view: Symptoms + Evaluation = Diagnosis

Finding out what’s really going wrong
Medical response: Evaluation; treating symptoms; referrals to other specialists; new and worsening symtoms; no diagnosis given; some diagnosis given; correct diagnosis
Patient and family reactions: Hope for answers; poor response to treatments; frustration; fear; confusion; the power of “naming”; disbelief; panic (what can we do? what info is there? assumption is often that there is one thing that can be done)

Initial adjustments to the diagnosis on the part of the patient and family:
* intellectual (making sense of info; paring down the info)
* physical (probably the patient has already made physical adjustments to the falls, for example)
* psychological (our sense of who we are)
* emotional (people feel that the illness defines them)
* social (people may drop out of activities they enjoy due to fatigue or depression)
* financial (healthcare costs, job, career, retirement)
* life plans (what were my future plans? time for reassessment of these plans)
* spiritual/religious

It’s natural for patients to think about many of these things on their own. We hope they have a good support system.

It’s natural for people to question the diagnosis.

After diagnosis: denial, anger, bargaining, depression, acceptance
These are the five stages of grief (and dealing with loss) by Elizabeth Kubler-Ross

The most important thing about acceptance is that it can lead to taking action. Taking action has four components:
1- Education: disease; current treatment; research; organizations; experts. (“Can’t take action until you know these things.”)
2- Medical: neurologist; movement disorder specialists (“usually this is a neurologist but sometimes it can be a psychologist or nurse practitioner.” Robin’s note – a psychologist cannot be a movement disorder specialist!); primary care physician (who can “synthesize info”); rehab specialists; study sites
3- Resources (“information in the community that may be helpful”): organizations (including Parkinson’s Disease support groups); adaptive equipment; home health; transportation
4- Social Supports: family; extended family; friends; colleagues; church/synagogue; support groups

Creating a framework for maintaining the best health possible for yourself and your family

Continuing to move forward if you have the disease:
* Ask for help from medical providers. Come prepared to appointments with your questions.
* Acknowledge and accept your own thoughts and feelings
* Share your concerns and emotions with someone close to you
* Consider professional counseling/group support
* Ask for help from your family and friends
* Stay in touch with people you love and help them understand the disease
* Remember what helped you cope with other difficult situations in your life and draw from those experiences
* Maintain daily routines as much as possible
* Enjoy the simple things
* Focus on what you can control
* Find courage and direction in your faith or belief system

If your loved one has the disease:
* Recognize it’s normal to feel a variety of emotions
* Maintain and/or start new routines that promote your physical and mental health. Don’t get out of balance by spending 7×24 with your family member with the disease.
* Create a support system
* Continue to learn as much as you can — connect with others going through the challenges of the disease and share ideas
* Accept that you are human

CurePSP can help
* Visit our website
* Request or download our educational materials
* Read our newsletters (Robin’s note: I have not seen a CurePSP newsletter in perhaps a year. Even the old issues are no longer posted on the CurePSP website.)
* Listen to our ongoing webinars
* Attend our family conferences
* Contact us

Question & Answer Period: (all answers are given by Trish)

Robin’s Comment: The #s are prevalence numbers, not incidence #s.

Trish’s Reply: We don’t have good numbers on these diseases. Hopefully our numbers will improve with research.

Robin’s Comment: Perhaps you can address denial. Many caregivers comment that their family members are in denial. Is this a problem?

Trish’s Reply: It’s a defense mechanism. It can serve a good purpose. It can be seen as a stubbornness to stay involved in life. But it can also lead people to think that they don’t need help, and they aren’t open to talking.

Never advisable to take someone’s defense mechanism away unless you have something equally effective to substitute for it.

Question: To what extent are medical students exposed to these diseases?

Answer: When PD is mentioned in the curriculum, it gets short shrift. Therefore, one can imagine that these diseases, as a subset, are not well-covered. It would be hard to find people to interview with the disease.

Comment: PT is helpful.

Trish’s Comment: CurePSP is doing some advocacy to help insure that PT is covered over the course of the disease.

Question: Husband with CBD has depression but isn’t taking anti-depressant.

Answer: Medication can make symptoms worse and can have side effects. Even a positive reaction can be short-lived. Hopefully a decision about taking an anti-depressant is made with a neurologist or psychiatrist. There are many kinds of anti-depressants. Raise this issue with the care provider. Perhaps ask for a psychiatry referral.

Question: What about erratic behaviors?

Answer: People can be impulsive. Example: someone with gait problems but they get up to walk on their own. No one way to help with these impulsive behaviors.

Try to assess whether there are times that make these behaviors worse. Then come up with a plan for the person.

Behavioral changes in PSP correlate with brain atrophy

This abstract is a bit hard to understand because it uses anatomical terminology. The Mayo Rochester authors attempt to determine whether brain atrophy (“gray matter loss”) correlates with severity of behavioral changes in PSP.

Some of the behavioral changes mentioned in the article include apathy (experienced by 83% of the 18 probable PSP patients), logopenia (being less talkative), severe behavioral dyscontrol, asponteneity, and disinhibition. The authors note that “behavioral changes in probable PSP subjects are far less severe than behavioral changes in bvFTD, at least early in the disease course.” [bvFTD = behavioral variant of Frontotemporal Dementia]

This point is interesting: “Although this suggests that behavioral changes in PSP are not reflecting cognitive severity, it is also important to recognize that cognitive impairment in PSP is usually mild and due to processing speech or executive function, neither of which are adequately captured by the MMSE.”


Here’s the abstract:

Movement Disorders. 2011 Feb 15;26(3):493-8. doi: 10.1002/mds.23471. Epub 2011 Jan 12.

Gray matter correlates of behavioral severity in progressive supranuclear palsy.

Josephs KA, Whitwell JL, Eggers SD, Senjem ML, Jack CR Jr.
Department of Neurology, Mayo Clinic, Rochester, Minnesota

Background: Behavioral changes occur in progressive supranuclear palsy. This study aimed to identify the anatomic correlate of behavioral severity in progressive supranuclear palsy.

Methods: We performed standardized tests of behavioral severity (Frontal Behavioral Inventory), cognitive severity (Mini-Mental State Examination), motor severity (Movement Disorder Society-sponsored revision of the Unified Parkinson’s Disease Rating Scale Part III), and a 3.0-T volumetric head magnetic resonance imaging scan in 18 prospectively recruited subjects meeting National Institute of Neurological Diseases and Stroke-Society of Progressive Supranuclear Palsy criteria for probable progressive supranuclear palsy.

Atlas-based parcellation was utilized to obtain regional gray matter volumes of frontal, temporal, and parietal lobe, and caudate and putamen, and voxel-based morphometry was used to assess voxel-level gray matter loss.

We performed correlation analyses between total Frontal Behavioral Inventory score and gray matter volume, as well as assessed gray matter volume across three groups defined according to behavioral severity (mild, moderate, and severe) based on total Frontal Behavioral Inventory score.

Results: Specific behaviors, with the exception of apathy that occurred in 83% of the subjects, were relatively infrequent. There was no association between Frontal Behavioral Inventory and cognitive or motor severity.

Regions of the frontal lobe, particularly, the lateral posterior frontal cortex, significantly correlated with the total Frontal Behavioral Inventory score when using both regional volume and voxel-level analyses.

The groupwise analyses also supported these findings.

The presence of apathy correlated with atrophy of the putamen.

Discussion: Behavioral severity in progressive supranuclear palsy appears to be associated with volume loss of frontostriatal regions, in particular, lateral posterior frontal lobe and putamen.

Copyright © 2010 Movement Disorder Society.

PubMed ID#: 21462261 (see for this abstract only)