News story about Pennsylvania family coping with PSP

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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.

www.pennlive.com/midstate/index.ssf/2011/04/as_disease_takes_over_dad_fami.html

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

Robin

 

Short Section of MSA in Perlman Chapter

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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.

Robin
—————————

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

Abstract
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 pubmed.gov for this abstract only)

New Alzheimer’s Diagnostic Guidelines

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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:
http://www.alz.org/research/diagnostic_ … 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

http://www.nytimes.com/2011/04/19/healt … eimer.html

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

FDA Safety Communication – PPIs

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

http://www.fda.gov/Safety/MedWatch/Safe … 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: www.fda.gov/MedWatch/report.htm

* 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.

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

http://www.fda.gov/Safety/Recalls/ucm248552.htm

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

Contact:
Pfizer Inc.
1-800-438-1985

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.