“Hard Choice for a Comfortable Death: Sedation” (NYT)

The New York Times (nytimes.org) seems to have a new series called “Months to Live,” where it address end-of-life issues.  An article for tomorrow’s paper is the first I’ve seen on the topic of sedating someone near death in the hopes that they die while sleeping.  We had one support group member who instructed both hospice and her husband to provide extensive sedation to her, and even spelled out what medication she wanted to be given (phenobarbitol and morphine).

I looked into this topic a couple of years ago as we were preparing for my father to be removed from a ventilator and feeding tube.  (Very fortunately for our family, Dad gave us the ultimate gift and died of a heart attack before we removed life support.)

Besides these two group member stories, I know little on this topic so was very interested in today’s article.  You can find the article here:


Months to Live
Hard Choice for a Comfortable Death: Sedation
New York Times
By Anemona Hartocollis
December 27, 2009

This is a tough subject for many.

Hospice RN Bill Carroll’s great article on when hospice should be contacted

When CurePSP was the Society, it published at least twice hospice nurse Bill Carroll’s article on hospice in the PSP Advocate (now called the CurePSP Magazine). Here’s the text of his great article.  (On the PSP Forum, Bill goes by 104fm.)


William Carroll, RN, CHPN
HealthCare Dimensions Hospice, The Dana Farber Cancer Institute, Boston, MA
The PSP Advocate, 2006 (1st issue)

As a participant in the online discussion forum available at forum.psp.org, I have often been asked my opinion as to when is the apropriate time to contact hospice. I receive this question fairly often because of my background and current employment as a registered nurse working in Hospice for Healthcare Dimensions, a subsidiary of the Dana Farber Cancer Institute. I also have a family member who has been diagnosed with PSP.

There are basically two separate Medicare benefit programs that may be available for people with PSP and their families. These include the Medicare Home Health Benefit and the Medicare Hospice Benefit. Many private insurances have guidelines for qualifying for their own programs, but quite often, they are virtually identical to those offered through Medicare. It is usually worthwhile to review the publications available from the insurer and then speak with the benefit administrator to see what is available.

Each of the two plans has separate criteria which need to be met in order to qualify for the program. For the Medicare Home Health Benefit there must be a need for skilled care (custodial care alone, such as would be provided by a nurse’s aide, generally would not qualify), and the patient must be home bound. In the case of the Medicare Hospice Benefit, both the admitting physician and the Hospice Medical Director must certify that they believe if the disease runs its normal course, the patient has a prognosis of six months or less.

With many diseases that have an unpredictable rate of progression, and PSP is definitely no exception, determining a six-month prognosis with any true accuracy is extremely difficult. In consideration of this, the Medicare Hospice Benefit provides for unlimited renewals. Basically, this means that provided the admission criteria is still met, a person could potentially be eligible to receive all the care and benefits that Hospice provides for well beyond the original six-month prognosis.

Another question I am often asked is, “When is it the appropriate time to contact hospice?” People are sometimes taken aback by my most common response, which is often, simply, “today.” The reason I feel this is the most accurate answer is that by contacting hospice today, you have absolutely nothing to lose, but a priceless amount of information, support and services to gain. When contacted, many hospices will give you the option of having a nurse come to the home (or nursing home if that is where the patient resides) and explain the benefit. The nurse can often tell you on the spot whether the hospice benefit may be available as an option now, or, if not, what criteria would need to be met in order to qualify.

Upon accessing the Hospice benefits, a registered nurse will be assigned whose focus will be on controlling the symptoms of the disease and helping to promote the best quality of life possible. The nurse will come to the home (usually from one to seven times per week, depending on need) for ongoing symptom management. There is also a registered nurse available 24 hours a day by phone for the hours that the assigned nurse is not available. A social worker will also be assigned who can assist in obtaining any available community resources, as well as helping both the person with PSP and the family deal with the emotional aspects of the losses this disease can bring. A non-denominational pastor can also be assigned who can work alone or in conjunction with community clergy to help cope with the spiritual aspects of dealing with the disease.

In addition, nurse’s aides can be included to assist with personal care, such as bathing and dressing. Nurse’s aides generally visit from two- seven days a week, depending on need, and stay from 1-1 1/2 hours per visit. Trained volunteers can also become involved. They can help by making friendly visits to sit and read to the patient, running errands, assisting with rides to appointments or helping in any other way possible. Other services, such as speech or physical therapy, can also be included as part of the hospice plan of care. By invoking the benefit, you gain access to a team of well-trained professionals whose focus will be on providing the person with the absolute best quality of life possible. In addition to the professionals involved in the care, hospice also covers related medications as well as home medical equipment, such as walkers, wheelchairs, commodes, hospital beds and other equipment.

An additional positive aspect of the hospice benefit is that it can be provided not only in the home setting, but also in nursing facilities and hospitals. Often, people have other insurance in addition to Medicare, such as Medicaid or long-term care insurance. If this is the case, the additional insurance can sometimes be used to cover the cost of being in a nursing facility, while Medicare is used for the hospice services. Some patients choose to use hospice houses, which are facilities that deal exclusively with hospice patients and often strive to create a more homelike environment as opposed to a medical one.

Of all the families I have had the pleasure and privilege of being involved with, the ones who have gained the most from the program all had one basic thing in common. They accepted all of the services and benefits hospice had to offer. Although there is no obligation to accept the involvement of all of the different team members, I strongly encourage doing so. Each member has something different to offer that often can compliment what the others provide.

Hospice is a benefit that is available much sooner than most people realize. Referrals for hospice evaluations can be made by patients, friends or family members, and can be called in directly to any hospice in your area. The service does not need to be initiated by a physician’s office, but it is often helpful to find out which hospices your doctor recommends.

William Carroll, RN, CHPN is a registered nurse who is nationally certified in Hospice and Palliative Care who is currently employed by HealthCare Dimensions Hospice, a subsidiary of The Dana Farber Cancer Institute.

[later on 12/22/09: removed a link Bill thought no longer appropriate.]

Using eye saccade velocity to distinguish PSP-P and PD

This interesting research out of Germany looked at 12 cases of the RS (Richardson’s Syndrome) form of PSP, 5 cases of the PSP-P (PSP-Parkinsonism) form, 27 cases of Parkinson’s Disease, and 23 healthy controls.  (It was just published on PubMed though the journal is dated Dec 2008 and the epub is dated 1/14/09.)

Cases of PSP-parkinsonism are characterized by asymmetric onset, tremor, and a moderate initial response to levodopa.  Obviously, PSP-P cases are frequently confused with Parkinson’s Disease.

Because PSP-P and PD are so similar in symptoms, clinicians need a way to differentiate the two disorders.  The German researchers conclude that video-oculography (VOG) can be used because of the “clear-cut separation between PSP-P and [PD] obtained by measuring saccade velocity.”  Typically, a neuro-ophthalmologist has the equipment to conduct a VOG.

It should be noted that none of the patients had pathologically-confirmed diagnoses, so the results may change based upon that.


Journal of Neurology. 2008 Dec;255(12):1916-1925. Epub 2009 Jan 14.

Differential diagnostic value of eye movement recording in PSP-parkinsonism, Richardson’s syndrome, and idiopathic Parkinson’s disease.

Pinkhardt EH, Jürgens R, Becker W, Valdarno F, Ludolph AC, Kassubek J.
Dept. of Neurology, University of Ulm, Ulm, Germany.

Vertical gaze palsy is a highly relevant clinical sign in parkinsonian syndromes. As the eponymous sign of progressive supranuclear palsy (PSP), it is one of the core features in the diagnosis of this disease.

Recent studies have suggested a further differentiation of PSP in Richardson’s syndrome (RS) and PSP-parkinsonism (PSPP).

The aim of this study was to search for oculomotor abnormalities in the PSP-P subset of a sample of PSP patients and to compare these findings with those of (i) RS patients, (ii) patients with idiopathic Parkinson’s disease (IPD), and (iii) a control group. Twelve cases of RS, 5 cases of PSP-P, and 27 cases of IPD were examined by use of video-oculography (VOG) and compared to 23 healthy normal controls.

Both groups of PSP patients (RS, PSP-P) had significantly slower saccades than either IPD patients or controls, whereas no differences in saccadic eye peak velocity were found between the two PSP groups or in the comparison of IPD with controls.

RS and PSP-P were also similar to each other with regard to smooth pursuit eye movements (SPEM), with both groups having significantly lower gain than controls (except for downward pursuit); however, SPEM gain exhibited no consistent difference between PSP and IPD.

A correlation between eye movement data and clinical data (Hoehn & Yahr scale or disease duration) could not be observed.

As PSP-P patients were still in an early stage of the disease when a differentiation from IPD is difficult on clinical grounds, the clear-cut separation between PSP-P and IPD obtained by measuring saccade velocity suggests that VOG could contribute to the early differentiation between these patient groups.

PubMed ID#: 19224319   (see pubmed.gov for this same abstract)

“Living with PSP: Norma’s Story”

I stumbled across this 6-minute video today:

“Living with PSP: Norma’s Story”
July 17, 2009

It features Norma (with PSP), her husband Joe, and her daughter Susan.

The daughter’s advice:

  • communicate with the neurologist
  • find a local support group
  • lay the groundwork now for help and community resources

Norma’s advice:

  • be active

The husband’s advice:

  • take life as it comes, but with humor

Perhaps those dealing with a recent diagnosis of PSP would get something out of this video, or perhaps any PSP patient would value seeing that they are not alone.

I don’t know why but this video is titled “Physical therapy: Turn and sit” and is supposed to feature physical therapist Heather Cianci.  (It’s listed this way on the CurePSP website and on YouTube.)  “Norma’s Story” has nothing to do with PT.  I believe this video was posted to YouTube by CurePSP.



The “Other” Dementias (featuring Lewy Body Dementia story)

There’s a wonderful article in the November/December 2009 issue of Neurology Now magazine.  It features Jerome and Renata Rafferty; Jerome had Lewy Body Dementia and Renata was his caregiver.

Renata is a fixture on the LBDA Forum (“raffcons”).  (She and Jerome used to live near Palm Springs; they moved to Indiana this spring.)  Renata emailed me on Sunday about the article:

“The writer interviewed me just a  few days before Jerome died, and it was a fitting cap to bring at least some usefulness to what Jerome went through. I think the article is pretty good and would like to see it disseminated as widely as possible.”

Here’s the full text of the article, and a link to it online.  In the online version, you can see a nice photo of Jerome and, of course, the formatting is prettier.  You can also download the PDF of the article.



journals.lww.com/neurologynow/Fulltext/2009/05060/The__Other__Dementias.14.aspx –> HTML version

The “Other” Dementias
Alzheimer’s disease is not the only cause of dementia. Knowing the others may help you or your loved one get the right diagnosis and treatment.
by Tom Valeo
Neurology Now:
November/December 2009 – Volume 5 – Issue 6 – p 26-27, 31-34

Renata Rafferty first suspected trouble when her late husband Jerome, a bright, curious, and articulate man, couldn’t tell her about an article he had just read.

“He would start to tell me and then say, ‘I’d better go find the article and give it to you’,” she recalls.

Jerome was nearly 70 at the time (he died on Oct. 20, 2009, at the age of 76), so Rafferty attributed such lapses to typical age-related forgetfulness. But that wasn’t the first disturbing change she had noticed in him. For more than two years he had been having terrifying dreams several nights a week.

“They were scary, violent dreams, and he would act them out,” Rafferty remembers. “He would kick, push, and speak angrily, and if I tried to wake him he’d lash out at me. I learned to get out of bed, shake his foot, and call to him so I wouldn’t be close enough to get hurt.” Their doctor attributed the dreams to the pain medication Jerome had been taking for a ruptured disc in his back. But he prescribed the Alzheimer’s drug donepezil when Jerome could no longer follow the plot of Law and Order, one of his favorite TV shows. A neurologist added another Alzheimer’s medication, memantine.

After Jerome went through eight hours of neuropsychiatric testing over two days, the doctor who administered the tests concluded he did not have Alzheimer’s disease (AD) but rather vascular dementia, which is caused by the damage that accumulates from several small strokes.

Since strokes can occur in any part of the brain, the symptoms of vascular dementia can vary greatly. But they often cause memory problems, mood disorders, and difficulty with walking and other movements-symptoms found in some Alzheimer’s patients as well.

If the strokes accumulate in the front of the brain, they may produce symptoms of frontotemporal dementia (FTD). This group of disorders affects the prefrontal cortex, which modulates mood, judgment, speech, creativity, and other distinctly human functions.

Still, the neuropsychologist who performed the testing insisted Jerome had vascular dementia, and predicted that unlike patients with AD, who decline steadily, Jerome would decline, remain stable for a while, then have another small stroke and decline again, and so on.

“We went for a year or so thinking it was AD, and then we went for another year or so thinking it was vascular dementia,” Rafferty says.

Then Jerome went to the Mayo Clinic in Scottsdale, AZ, seeking relief from his persistent back pain. After a thorough exam his doctors concluded that Jerome did not have vascular dementia or AD. They had noticed that the toes on one foot were constantly wiggling, a sign of a very rare condition known as painful leg moving toe syndrome.

“Everyone was very excited about that,” Rafferty says. “They wanted to enroll him in a study, videotape his foot and leg.”

But during the exam she heard the senior neurologist on the team mention–in passing–that Jerome did not have vascular dementia, so she followed him into the hall and asked him what he meant.

“I’m so sorry to tell you this, but it’s obviously Lewy body dementia,” he said, and rushed off.

“That was the first time I heard those words,” Renata says.

Now that she knows more about Lewy body dementia (LBD), she can see early symptoms that should have pointed to the diagnosis. Acting out violent dreams, for example, is one poorly understood symptom of the disorder. And when Jerome took olanzapine, one of the newer drugs used to treat psychiatric symptoms, he had a violent reaction that produced high blood pressure and delirium.

“It turns out that this is a sign of LBD too,” Rafferty says. “We found out that people with LBD often have a severe reaction to atypical antipsychotic medications-also, that LBD patients should not be put under general anesthesia because they may proceed rapidly to end-stage disease.”

To complicate the diagnosis further, LBD may overlap with other conditions, including AD, Parkinson’s disease, FTD, and vascular dementia. Although Jerome did not have vascular dementia, he did have a fourth transient ischemic attack-a temporary interruption of blood flow to a part of the brain. A brain scan revealed signs of a previous stroke, which could have produced symptoms of its own.

Despite his memory problems, and occasional hallucinations, and fleeing bouts with anxiety and aggression, Jerome remained acutely aware of his condition in a way that Alzheimer’s patients seldom are. When a hospice nurse recently asked him if he needed anything, he replied with a mordant, “Yeah, a getaway car.”


Everyone knows about AD, which accounts for 65 percent of all dementia in the United States. Alzheimer’s begins with degeneration of the hippocampus, a brain structure essential for the creation of new memories, and spreads to other brain areas, producing problems with speech, mood, judgment, motor skills, and other abilities.

But the hippocampus is not the only region subject to degeneration. Other brain structures can develop problems, and although they may produce similar symptoms, the underlying diseases each have a life of their own.

“There are well over 100 causes of dementia, but the big four that make up 94 to 98 percent are Alzheimer’s disease, Lewy body dementia, frontotemporal dementia, and vascular dementia,” notes James E. Galvin, M.D. M.P.H., assistant professor of neurology, anatomy, and neurobiology at Washington University School of Medicine and director of the Memory Diagnostic Center and the Wolff Neuroscience Laboratory. If you take 100 people with dementia, 65 will have AD, 10 or 12 will have LBD, 10 or 12 will have vascular, and eight will have FTD.

Because Alzheimer’s was identified more than 100 years ago and accounts for the vast majority of dementia, it attracts the largest number of research dollars, and therefore is better understood than the others.

But the other types of dementia tend to strike earlier, consuming many years of productive life.

“These other diseases affect people in their 50s, 60s, and early 70s,” observes Dr. Galvin. “Alzheimer’s affects people in their late 70s and early 80s.”

Although the precise causes of these different dementias (including AD) remain obscure, they all seem to involve the faulty production or management of proteins in the brain. In AD, tau protein accumulates within the body of neurons, while amyloid protein forms clumps in between neurons.

In LBD, a protein known as alpha-synuclein aggregates into clumps named after Frederich Lewy, the German-American physician who described them in 1912. (Dr. Lewy worked in the same lab as Dr. Alois Alzheimer, who identified the protein clumps and tangles characteristic of the disease named after him.)

FTD involves the accumulation of a protein known as TDP-43, which also plays a role in amyotrophic lateral sclerosis (ALS), or Lou Gehrig’s disease. Discovered just three years ago, TDP-43 plays such a decisive role in both diseases that some researchers suspect that FTD and ALS may be different manifestations of the same disease process.

One type of FTD known as Pick’s disease, named after Arnold Pick, a professor of psychiatry at the University of Prague who described the disease in 1892, involves the accumulation of tau protein-one of the two proteins associated with Alzheimer’s disease. In Pick’s disease, however, the protein accumulates in the frontal lobes, where it causes erratic behavior and the loss of normal inhibitions. A normally reserved man with Pick’s might make lewd sexual comments to women or become belligerent. Pick’s disease may also produce speech difficulties that eventually leave the patient mute.

Vascular dementia is an imprecise term that refers to dementia caused by brain cells that have been damaged by lack of oxygen from several small strokes. Some research suggests that the most common form of vascular dementia, known as multi-infarct dementia, merely causes or accelerates AD, producing a decline in memory and cognitive function.

“There can be some overlap in pathology, but in vascular dementia you think the primary dementing component is due to vascular disease,” says Dr. Galvin. “If you have AD and then have a stroke, you don’t then have vascular dementia too; you have AD and a stroke. It’s not clear cut, though.”


Understanding dementia, which is a complex and varied dysfunction, requires understanding the complex and varied function of the brain itself.

What we experience as consciousness involves the seamless integration of signals generated by dispersed regions of the brain. To produce accurate perceptions of the environment, appropriate emotions, reliable memories, and good judgment, brain regions must perform efficiently, and the fibers that link those regions must transmit signals smoothly and swiftly.

All this activity depends on the ability of brain cells, known as neurons, to manufacture, transport, and recycle proteins, a process that requires huge amounts of glucose and oxygen. (The brain accounts for two percent of the body’s weight, but consumes 20 percent of the body’s energy.)

This constant and arduous process provides many opportunities for mistakes. A neuron may start to manufacture defective or misfolded proteins, or fail to manufacture enough to provide the chemical signals that enable neurons to communicate with each other. Proteins may not be broken down or recycled efficiently enough, causing debris to build up within and between the neurons, which can result in harmful inflammation.

This variety of brain functions points to one of the great mysteries of dementia: Why do various regions of the brain degenerate so differently?

“It’s what we call selective vulnerability, and no one understands why it exists,” says Bradley Boeve, M.D., professor of neurology at Mayo Clinic College of Medicine in Rochester, MN. “Why does AD affect the hippocampus, while FTD affects the frontal and temporal lobes and LBD affects the brainstem and neurochemical centers? I’ve never heard a good hypothesis as to why. Even in patients with end-stage FTD, their parietal and occipital regions [other major brain regions] look pretty normal. If dementia is a protein dysfunction, why is it so selective for certain parts of the brain?”

The variety of dementia makes treatment extremely difficult. Antipsychotic drugs, for example, quell the voices and hallucinations of schizophrenia and help some people with AD, but often produce delirium in LBD patients.

And the complexity of the brain-from protein synthesis within the neuron to the dense highways that transmit signals-makes effective treatments difficult to devise, leaving physicians with little to offer but relief for some symptoms. Donepezil, for example, developed to stimulate the memory of AD patients, may help people suffering from another form of dementia that causes memory problems. Patients with LBD who develop rigidity of movement may benefit from drugs used to treat Parkinson’s disease. Such drugs do nothing to treat the underlying cause of the dementia but may provide some relief from the consequences.


The investigation of Alzheimer’s disease demonstrates how elusive the cause of dementia can be. For nearly a century scientists believed that AD was caused by the plaques and tangles that Alois Alzheimer spotted through his microscope in the brain tissue of a women who had been severely demented. (The tissue had been taken at autopsy.) Inside of the neurons he found neurofibrillary tangles-strands of tau protein that looked like a length of thread crammed into a ball. Between the neurons he found clumps of amyloid protein, which he dubbed amyloid plaques.

The solution seemed obvious: Get rid of the plaques and tangles. But treatments that clear the brain of these toxic proteins have failed to cure the disease, suggesting that tangles and plaques develop relatively late in the disease process.

The same may be true of other dementias. The toxic proteins they produce probably are not the cause of the problem but the consequence, and an understanding of the cause may be many years away.

In the meantime, is there anything we can do to reduce the risk of dementia?

“Pick your parents well,” says Dr. Galvin, noting that genes seem to predispose some people to dementia. In addition, exercise that promotes cardiovascular health will help deliver a generous supply of blood to the brain, providing neurons with the nutrients they need. Keeping the brain active also helps, according to Dr. Galvin.

“But that’s not absolute,” he adds. “There are astrophysicists who are also vegetarian marathoners who get dementia, and couch potatoes who don’t. But from a population perspective, those behaviors seem to afford some protection.”

Other advice: If someone diagnosed with AD doesn’t appear to have the right symptoms, voice skepticism.

“If you suspect that it’s not AD but one of these other dementias, see a neurologist, preferably a cognitive neurologist well versed in these disorders,” says Dr. Boeve. “A lot of primary care physicians haven’t been as well educated in these less common disorders, so they may not recognize them. I hear this from families all the time: My doctor diagnosed AD, but I read about AD and it doesn’t sound like AD. And they’re usually right.”

That’s why Renata Rafferty spoke to Neurology Now about her husband’s long and arduous illness: to encourage others to be skeptical of a diagnosis of Alzheimer’s disease when the symptoms don’t seem right, and to educate themselves about other dementias that the doctor may not be considering.

“I have made it my personal mission to talk to people about Lewy body dementia, she says. I have my little elevator speech ready in which I describe symptoms that are not typical of Alzheimer’s, and I don’t hesitate to suggest that people with those symptoms be evaluated for one of the other dementias.”