“A Life Worth Living” – Radio Interview and Book

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Sadly, the loved ones of five local support group members died in March; one support group member (caregiver) died too. At least one group member was involved in end-of-life treatment decisions, and relied on her husband’s written wishes when he was no longer able to communicate.

Along these lines, I heard a book author interviewed today (Thursday 4/2/09) on NPR’s “Fresh Air” program, and wanted to pass the info along to all of you. The book is “A Life Worth Living,” and the author is Robert Martensen, MD. Dr. Martensen was an ER and ICU MD for many years; he’s also a bioethicist.

In his book he talks about his father’s hospital death, and “argues that safeguarding the quality of a patient’s life sometimes trumps the urge to sustain life at all cost.” He also addresses his father’s death in the “Fresh Air” interview as well as his mother’s impending death. His mother lives in Santa Cruz. In the story about his mother, he describes one of his mother’s first MDs in these final weeks who wanted to place a pacemaker. Dr. Martensen worries that it’s only because he is an MD that he was able to know the right questions to ask the treating physician. Ultimately, he’s very fortunate that both of his parents had given such clear advance directives about what sort of treatment they wanted, given certain circumstances. He contrasts his mother with another elderly woman also suffering from dementia.

I would recommend listening to the 40-minute “Fresh Air” interview. You can find it online here:

http://www.npr.org/templates/story/story.php?storyId=102638208
The interview is titled “The Ethical Way To Heal American Health Care,” which doesn’t really capture the interview.

“Treating an Illness Is One Thing. What About a Patient With Many?”

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This is a good article in a recent New York Times (nytimes.com) about patients with many illnesses, not just one.  A key problem is that these patients end up on many medications.  Here’s an excerpt:

“Doctors know that it’s not right for someone to be on 15, 18, 20 medications,” said Dr. Tinetti, the Yale geriatrician. “But they’re being told that that’s what’s necessary in order to treat each of the diseases that the patients in front of them have.”

Here’s a link to the full article:

http://www.nytimes.com/2009/03/31/health/31sick.html

Treating an Illness Is One Thing. What About a Patient With Many?
Brendan Smialowski
The New York Times
March 30, 2009

Thanks to online friend Joe Blanc for making me aware of this article.

Robin

 

 

Progression of dysarthria + dysphagia in PSP, CBD, etc

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I’ve posted some of the info in this article previously and the PubMed ID# but have never posted these excerpts.

This 2001 study is of dysarthria and dysphagia in autopsy-confirmed cases of DLB, CBD, MSA, PSP, and PD. According to the article, all atypical parkinsonism patients develop either dysarthria or dysphagia within one year of disease onset. (I thought dysarthria meant slurred speech. But, according to this article, it can also mean hypophonic speech or monotonic speech.)

Here’s the citation and abstract:

Archives of Neurology. 2001 Feb;58(2):259-64.

Progression of dysarthria and dysphagia in postmortem-confirmed parkinsonian disorders.

Muller J, Wenning GK, Verny M, McKee A, Chaudhuri KR, Jellinger K, Poewe W, Litvan I.
Cognitive Neuropharmacology Unit, Bethesda, MD

BACKGROUND: Dysarthria and dysphagia are known to occur in parkinsonian syndromes such as Parkinson disease (PD), dementia with Lewy bodies (DLB), corticobasal degeneration (CBD), multiple system atrophy (MSA), and progressive supranuclear palsy (PSP). Differences in the evolution of these symptoms have not been studied systematically in postmortem-confirmed cases.

OBJECTIVE: To study differences in the evolution of dysarthria and dysphagia in postmortem-confirmed parkinsonian disorders.

PATIENTS AND METHODS: Eighty-three pathologically confirmed cases (PD, n = 17; MSA, n = 15; DLB, n = 14; PSP, n = 24; and CBD, n = 13) formed the basis for a multicenter clinicopathological study organized by the National Institute of Neurological Disorders and Stroke, Bethesda, Md. Cases with enough clinicopathological documentation for the purpose of the study were selected from research and neuropathological files of 7 medical centers in 4 countries (Austria, France, England, and the United States).

RESULTS: Median dysarthria latencies were short in PSP and MSA (24 months each), intermediate in CBD and DLB (40 and 42 months), and long in PD (84 months). Median dysphagia latencies were intermediate in PSP (42 months), DLB (43 months), CBD (64 months), and MSA (67 months), and long in PD (130 months). Dysarthria or dysphagia within 1 year of disease onset was a distinguishing feature for atypical parkinsonian disorders (APDs) (specificity, 100%) but failed to further distinguish among the APDs. Survival time after onset of a complaint of dysphagia was similar in PD, MSA, and PSP (15 to 24 months, P =.7) and latency to a complaint of dysphagia was highly correlated with total survival time (rho = 0.88; P<.001) in all disorders.

CONCLUSIONS: Latency to onset of dysarthria and dysphagia clearly differentiated PD from the APDs, but did not help distinguish different APDs. Survival after onset of dysphagia was similarly poor among all parkinsonian disorders. Evaluation and adequate treatment of patients with PD who complain of dysphagia might prevent or delay complications such as aspiration pneumonia, which in turn may improve quality of life and increase survival time.

PubMed ID#: 11176964

These results were the most interesting (and depressing):

“Dysarthria or dysphagia within 1 year of disease onset was a distinguishing feature for atypical parkinsonian disorders (APDs).”

“Median dysarthria latencies were short in PSP and MSA (24 months each), intermediate in CBD and DLB (40 and 42 months), and long in PD (84 months).”

“Median dysphagia latencies were intermediate in PSP (42 months), DLB (43 months), CBD (64 months), and MSA (67 months), and long in PD (130 months).”

“Survival time after onset of a complaint of dysphagia was similar in PD, MSA, and PSP (15 to 24 months).”

On this last point, here are the CBD and PSP data from the article:

Survival Time After Onset of Dysphagia, months
CBD 49 (25-89…..including a single patient with dysphagia but without dysarthria)
PSP 18 (6-96)

Here are some excerpts from the article’s Comment section:

“(Early) dysarthria and perceived swallowing dysfunction are not features of PD.”

“Dysarthria) as a presenting symptom has been described in clinical series of CBD, MSA, and PSP. In PD and DLB, hypphonic/monotonous speech represented the most frequent type of dysarthria, whereas imprecise or slurred articulation predominated in CBD, MSA, and PSP.”

“In a clinical study of CBD, Rinne et al described dysarthria as one of the initial symptoms in 11% of the patients, which is close to our findings. At follow-up, on average 5.2 years, dysarthria was diagnosed in 70% of the patients… According to our findings, dysarthria occurred in almost every patient with CBD.”

“In both PSP and MSA, progressive dysarthria is believed to represent a manifestation of brainstem and cerebellar involvement. In fact, PET studies revealed marked hypometabolism in the cerebellum and brainstem of patients with MSA, which correlated with dysarthria.”

“In our study, dysphagia was associated with concomitant dysarthria in all parkinsonian patients except one. This sequence of dysphagia following dysarthria has also been reported in clinical studies of PD, MSA, and PSP.”

“…Golbe et al reported dysphagia after a median of 1 year after the onset of dysarthria in PSP.”

In all these disorders, “bronchopneumonia has been reported as a leading cause of death, which may be subsequent to silent aspiration resulting from dysphagia.”

“Most of our patients with MSA and PSP complained of a swallowing dysfunction, in contrast to patients with PD, CBD, and DLB… Impaired lingual proprioception is hypothesized to contribute to the unawareness of swallowing difficulties in PD and might in part explain significantly longer latencies to dysphagia in our PD cases. In contrast, patients with PSP were reported to be keenly aware of swallowing problems, including those with cognitive impairment.”

“(The) similarly short remaining survival time in PD and PSP after the onset of perceived dysphagia suggests that this symptom represents a reliable marker for the onset of functionally relevant swallowing abnormalities in both disorders.”

Robin

MRI and MRS in PSP, MSA-P, PD (Brazilian study)

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This small Brazilian study looked at 11 patients with PSP, 7 patients with MSA-P, 12 patients with PD, and 10 controls. Everyone was studied with magnetic resonance imaging (MRI) and spectroscopy by MRI (MRS).

The authors concluded:
“(1) Patients with PSP and MSA-P presented increased motor and cognitive impairment in the scales used, correlating with decrease in NAA/Cr in lentiform nucleus and NAA/Cho in midbrain in the PSP group;
(2) Cerebral and cerebellar atrophy were more prevalent and severe in PSP and MSA-P groups;
(3) Linear hypersignal in the lateral portion of the putamen, hypersignal in midbrain and in pons, all suggest the diagnosis of PSP or MSA-P;
(4) Midbrain or pons atrophy suggests atypical parkinsonism, the former PSP, and the latter MSA-P;
(5) Comparing the two methods, MRI and MRS, the former had better applicability.”

The abstract and lots of excerpts follow. The English-language article is available for free online here:
http://www.scielo.br/pdf/anp/v67n1/a02v67n1.pdf –> PDF form
http://www.scielo.br/scielo.php?script= … so&tlng=en –> HTML form

For me, the most interesting parts of this article were the three figures with captions, indicating what percentage of the patient groups had particular MRI or MRS findings, including the hot cross bun sign in MSA. (You’ll have to go online to see the figures.) And the Discussion section was worthwhile reading.

Robin

Arqivos de Neuropsiquiatria. 2009 Mar;67(1):1-6.

Neuroimaging in Parkinsonism: a study with magnetic resonance and spectroscopy as tools in the differential diagnosis.

Vasconcellos LF, Novis SA, Moreira DM, Rosso AL, Leite AC.
Hospital dos Servidores do Estado, Rio de Janeiro, RJ, Brazil.

The differential diagnosis of Parkinsonism based on clinical features, sometimes may be difficult. Diagnostic tests in these cases might be useful, especially magnetic resonance imaging, a noninvasive exam, not as expensive as positron emission tomography, and provides a good basis for anatomical analysis. The magnetic resonance spectroscopy analyzes cerebral metabolism, yielding inconsistent results in parkinsonian disorders.

We selected 40 individuals for magnetic resonance imaging and spectroscopy analysis, 12 with Parkinson’s disease, 11 with progressive supranuclear palsy, 7 with multiple system atrophy (parkinsonian type), and 10 individuals without any psychiatric or neurological disorders (controls). Clinical scales included Hoenh and Yahr, unified Parkinson’s disease rating scale and mini mental status examination.

The results showed that patients with Parkinson’s disease and controls presented the same aspects on neuroimaging, with few or absence of abnormalities, and supranuclear progressive palsy and multiple system atrophy showed abnormalities, some of which statistically significant. Thus, magnetic resonance imaging and spectroscopy could be useful as a tool in differential diagnosis of Parkinsonism.

PubMed ID#: 19330200

Here are excerpts from the full article:

“Magnetic resonance imaging (MRI) and spectroscopy by MRI (MRS) are noninvasive tools helping the physician to establish a more accurate diagnosis. MRI offers an adequate analysis of abnormalities in the basal nuclei, midbrain, pons, medulla, and cerebellum, which are impaired in atypical” parkinsonism.

“Method
We designed a prospective, case-control, double-blind, 24 months study. The MRI was performed in a GE machine, 1.5 Tesla Sigma Horizon model, the sequences analyzed were T 1, T 2, flair, diffusion, axial-oblique in T 2 in Fast Spin-Echo (FSE) and Proton Density (PD) and T 2 in Spin-Echo (SE). In addition to 5 mm slices, we included 3 mm slices in the lentiform nucleus. The MRS was single voxel (8 cc), PRESS technique (TR/TE=1500/50) bilaterally in lentiform nucleus, midbrain, white matter of frontal lobe and hippocampus.” …

“Forty individuals were included in this study (age range: 50 to 85 years), 30 with Parkinsonian syndrome and 10 without any neurological or psychiatric disorders.”

“All individuals were examined by the same neurologist, and 26 patients met the criteria for probable Parkinson’s disease (PD) [n=10], (Gelb et al.), progressive supranuclear palsy (PSP) [n=10], (Tolosa et al.), and multiple system atrophy-parkinsonian type (MSA-P) [n=6], (Gilman et al.). For clinical assessment, the scales adopted were Hoehn-Yahr stage, unified Parkinson’s disease rating scale (UPDRS) Part III and mini-mental status examination (MMSE). The patients performed the Tilt Table test for evaluation of dysautonomia.”

“Results …
Dysautonomia was documented in 20% of PD and 100% of MSA-P.

In the motor scales (UPDRS and Hoehn and Yahr), the results showed higher scores in PSP and MSA-P than in PD. There was statistical significance in PD versus MSA-P, and a trend to statistical significance in PD and PSP.

Patients with PSP presented lower scores in MMSE, followed by MSA-P and PD, and there was statistical significance in the three groups comparing to controls (Table 1).

Image variables demonstrated cerebral atrophy in all cases of PSP and MSA-P, having statistical significance in PD versus PSP, PD versus MSA-P, controls versus PSP, and controls versus MSA-P. Cerebellar atrophy was more common in MSA-P and PSP, with statistical significance in PD versus MSA-P, controls versus PSP and controls versus MSA-P. We observed a higher prevalence of white matter alterations in atypical [parkinsonism] with no statistical significance. Signal change in the lentiform nucleus was observed more commonly in MSA-P and PSP, but no statistical significance was documented (Figs 1­3).”

Fig 1. Hyposignal in the lentiform nucleus (found in 67% of MSA-P group), and hypersignal in the pons (found in 33% of the MSA-P group) and the midbrain on T2, flair or DP sequences (found in 70% of PSP group).

Fig 2. Posterolateral linear hypersignal in the lentiform nucleus with asymmetric symptoms, T2 sequence (found in 50% in MSA-P group).

Fig 3. Transverse signal (“hot cross bun sign”) in the pons, T2 sequence (found in 33% of MSA-P group).” …

“DISCUSSION
…Parkinsonian signs may be seen in different medical conditions, having variable course, treatment and prognosis so it is important to determine an accurate diagnosis as soon as possible. Based only in clinical data, especially in the early stages of the disease, physicians may not establish a correct diagnosis.

… One study conducted in a movement disorders specialized center, showed that the positive predictive value of PD was 98.6%, and to atypical parkinsonism 71.4%, confirming that the diagnosis of atypical [parkinsonism], even in specialized centers, is sometimes difficult to establish.

… We included the three [parkinsonism syndromes] that most frequently lead to misdiagnosis: PD, MSA-P, and PSP, all compared to control group. …

We used three clinical scales: motor part of UPDRS, Hoehn and Yahr and MMSE. These scales showed increased motor impairment (higher scores in UPDRS and Hoehn-Yahr) in the MSA-P, followed by PSP, and increased cognitive impairment (lower scores of MMSE) in PSP, followed by MSA-P. We did not observe a correlation between the duration of the symptoms with MRS abnormalities, but with the clinical diagnosis of patient.

MRI variables demonstrated that some are helpful to differentiated [parkinsonism] syndromes, as the presence of cerebral and cerebellar atrophy and signal enhancement of some encephalic structures (lentiform nucleus, midbrain and pons), more common in atypical [parkinsonism].

The decreased signal enhancement in the lentiform nucleus may be observed in normal aging, so in our study we only considered it as ‘abnormal’ if the hypointensity was moderate to severe. Our data showed that moderate to severe decrease hypointensity in lentiform nucleus was observed more frequently in MSA and PSP, with no difference between PD and control groups and when it was associated with posterolateral linear hypersignal in putamen, suggested the diagnosis of atypical [parkinsonism] (more frequently in MSA group).

The most useful measurement of encephalic diameter in our study was the midbrain, as it had been shown by Warmutth et al. Values below 15 mm in the midbrain suggested PSP or MSA-P, with lower values seen in PSP.

Some values of MRS had statistical significance, the most useful were from the lentiform nucleus, hippocampus, and midbrain, depending on the diagnosis, indicating a severe neuronal impairment (neuronal death). There are few studies in which the brainstem is evaluated by MRS, due to technical difficulties (bone proximity). In our study we demonstrated that it is feasible, but we had to repeat the exam, in some cases several times, to achieve a consistent chart. The study done by Watanabe et al. demonstrated the usefulness of MRS of the pons in MSA patients. As the midbrain is the most affected area in PSP, we analyzed it by MRS. We have found NAA/Cho decrease in midbrain of PSP group with statistical significance, indicating neuronal loss.

Based on our data we concluded that:
(1) Patients with PSP and MSA-P presented increased motor and cognitive impairment in the scales used, correlating with decrease in NAA/Cr in lentiform nucleus and NAA/Cho in midbrain in the PSP group;
(2) Cerebral and cerebellar atrophy were more prevalent and severe in PSP and MSA-P groups;
(3) Linear hypersignal in the lateral portion of the putamen, hypersignal in midbrain and in pons, all suggest the diagnosis of PSP or MSA-P;
(4) Midbrain or pons atrophy suggests atypical parkinsonism, the former PSP, and the latter MSA-P;
(5) Comparing the two methods, MRI and MRS, the former had better applicability.

Our study showed that anatomical analysis through MRI and MRS of some areas could be useful in the differential diagnosis of PD and atypical [parkinsonism], helping physicians to establish a more accurate diagnosis of [parkinsonian syndromes].”

Book recommendation: “AfterShock”

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This book recommendation came around again to me recently and I thought I’d pass along the title here. The recommended book is:
“AfterShock: What To Do When The Doctor Gives You – Or Someone You Love – a Devastating Diagnosis”
by Jessie Gruman, PhD
$12 new or $1 used on amazon.com
www.aftershockbook.com

On Friday, someone on an online MSA support group noted that Dr. Gruman was on the Martha Stewart show on Friday 3/27. I dug around on marthastewart.com but couldn’t find any segments from that Friday show available online. (Maybe the segment with Dr. Gruman will be posted later.) The Martha Stewart website did have some additional info on “Dealing with a Devastating Medical Diagnosis”:

“What would you do if you were diagnosed with a life-threatening illness? How would you handle this news? While shock, fear, and even hysteria might be normal reactions, it’s helpful to have a guide for what’s often a very tumultuous road ahead.

When you’re given the news that you have cancer, HIV, or another serious diagnosis, it may feel as if your world has shattered and all of your plans for the future have vanished in a flash. You feel fear, despair, anger, sadness — often all at once. It’s understandable; a serious diagnosis is a crisis, and you should treat it as one. Don’t force yourself to go to work or make big decisions while you’re really upset. Give yourself time to pull it together: Spend time with loved ones; don’t forget to eat; nap if you can; cry if you feel like it. There are no rewards for being tough. It’s a tribute to human resilience that as you learn more and adjust to the shock, you’ll find you regain some focus and are able to take the important next steps.

Finding a good doctor is really important — begin by looking for a specialist who has extensive experience treating the exact disease you have. Finding that person can be a puzzle. There are many referral sources, and none of them will tell you everything you need to know. The tried and true way is to ask a physician you know and like to refer you to another physician that he or she has worked with before. …

Often, friends, family members, and co-workers don’t know how to respond. They should begin by acknowledging the difficult situation. People say they’re uncomfortable raising the topic with someone who is sick and that they don’t want to remind the sick person of it or make them cry, but saying nothing is far more damaging. Say this: “I hear you’ve had some bad news. I’m so very sorry. I hope everything goes OK.” It means so much.

Also, don’t talk about a friend or family member’s illness without his or her permission, even to other family members. Ask what information can be shared and with whom. And then listen — many people with a serious illness swing between hope and fear.

When it comes to health care, you have to force yourself to act like a consumer. Things have changed a lot in health care in the past decade. Advances in surgery and drugs and diagnostics mean it’s now possible to live long and well with diseases that were a death sentence as recently as 10 years ago. But we will only benefit from these advances if we are involved. We have to decide which doctors to visit, get the tests, take the pills, and seek help when we can’t manage on our own. Patients have a critical role in the success of our health care.”

That’s from:
http://www.marthastewart.com/article/de … ewart-show
There’s a list of online resources available.

This page on Dr. Gruman’s website gives a great description of the book:
http://www.aftershockbook.com/book_inde … troduction

Below is an email I sent out in June ’07 to the local support group in which a social worker recommended “AfterShock” and gave some additional ideas for self management of long-term conditions.

Robin

Date: Mon, 18 Jun 2007 17:34:47 -0700
To: [email protected]
From: Robin Riddle <[email protected]>
Subject: “Self Mgmt of Long-term Conditions” (Meeting Notes)

I attended the Palo Alto Parkinson’s Disease support group meeting last week where I picked up one book suggestion and a few general suggestions that I thought I’d pass along. The speaker was Kate Lorig, Patient Education at Stanford Hospital. The topic was Self Management of Long Term Conditions.

Here’s some general information on Stanford’s workshops and programs to help people live with long-term health problems: http://patienteducation.stanford.edu/programs/
Some programs are meeting-based and others internet-based.

This particular support group is mixed with caregivers and those with Parkinson’s Disease. The speaker asked those with PD “what are the biggest challenges of living with PD?” and, for the caregivers, “what are the biggest challenges of living with someone who has PD?” Then the group voted on the top challenges, which were:

* living with ever-diminishing hopes
* sense of loss
* comparing how things used to be (“the good ole days”) to how things are now
* balance/coordination/muscle weakness

On the psychological issues of ever-diminishing hopes and a sense of loss, the speaker said that once you have a chronic condition, death is a reality. She highly recommends a new book called “After Shock” by Dr. Jessie Gruman. (You can find info on this book at http://www.aftershockbook.com/. You might listen to some interviews with Dr. Gruman before purchasing the book.) None of your family and friends know how to deal with the “new you.”

The speaker recommends planning at least one thing every day that the patient and caregiver can still enjoy — such as having an ice cream cone, watching the news, or travelling. She thinks it would be best to have several small things every day planned.

She emphasized that it’s the patient’s job to tell other people exactly what they can do to help. Again, she thinks that having something small planned is worthwhile, such as telling someone “you can invite me to a movie once a month.”

The speaker believes that doing volunteer work is useful: “helping others will help you.” A meeting attendee recommended the website dosomething.org for volunteer activities of any time duration.

The speaker says that most people with chronic conditions and most people caregiving for those with chronic conditions are depressed — some sub-clinically (ie, not needing therapy) and some clinically. She recommends that everyone have a specific thing to do or think about if they perceive they are having negative thoughts. A specific thing to do might include going to the movies, exercising, or baking. A specific thing to think about might include polar bears or penguins. (These were her examples!)

The speaker addressed the challenge of balance/coordination/muscle weakness by saying that these issues can be addressed through exercise. She recommended that everyone with a chronic health problem get exercise that doesn’t hurt. In particular she likes tai chi, and recommends the tai chi tapes of Paul Lam (see taichiproductions.com) and Jon Kabat-Zinn. JKZ also has mindfulness training tapes.

If you fall, don’t ask someone to lift you as they probably don’t know what they are doing and will hurt themselves or you. Instruct any helper to get others to help, particularly others who are trained in helping get people up off the floor.

She said that everyone using a cane, walker, or wheelchair needed lessons on using these tools properly.

That’s it!
Robin