===============================================================
==
==
== ----------- ALS Interest Group
----------- ==
== ALS
Digest #980 (15 January 2002)
==
==
==
== ------ Amyotrophic Lateral Sclerosis (ALS)
==
== ------ Motor Neurone Disease (MND)
==
== ------ Lou
Gehrig's disease
==
==
------ maladie de Charcot
==
==
==
== This e-mail list has been set up to serve the world-wide ==
== ALS community. That is, ALS patients, ALS researchers, ==
== ALS support/discussion groups, ALS clinics, etc. Others ==
== are welcome (and invited) to join. The ALS Digest is
==
== published (approximately) weekly. Currently there are
==
== 5100+ subscribers in 70+ countries. Please be advised, ==
== the editor is not a medical doctor and the Digest is
==
== not peer reviewed. This newsletter is not intended to
==
== provide medical advice on individual health matters.
==
== Any such advice should be obtained personally from a
==
== physician.
==
== To subscribe, to unsubscribe, to contribute notes,
==
== etc. to ALS Digest, please send e-mail to:
==
== bro@met.fsu.edu (Bob Broedel)
==
==
==
== Bob Broedel; P.O. Box 20049; Tallahassee, FL 32316 USA ==
===============================================================
== Back issues of the ALS Digest are available on-line at: ==
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==
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==
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== http://health.oldeman.net
==
===============================================================
CONTENTS OF THIS ISSUE:
1 .. Call to Action - Rare Diseases Act, S. 1379
2 .. Umbilical Cord Blood - Human Clinical Trials - ALS
3 .. re: anyone else diagnosed with a stroke? (ALSD979)
4 .. News From ALSA: Follow-up on Gulf War Study
5 .. ALS Drug Treatment Trial Announcement: Insulin Growth Factor 1
6 .. Should we be tested?
7 .. Power Chairs
8 .. Water in Maine
9 .. A potential role for the p75 low-affinity neurotrophin receptor
10 . Laryngeal dysfunction in ALS: a review and case report
11 . ventilator question
12 . re: PEG tube pain
13 . re: ALS and other diseases (ALSD976)
(1)
=====
Call to Action - Rare Diseases Act, S. 1379
==========
>From: ABE11@aol.com
Date : Mon, 14 Jan 2002
>From : "Diane Dorman" <ddorman@rarediseases.org>
Subject: Call to Action - Rare Diseases Act, S. 1379
Date : Mon, 14 Jan 2002 13:59:13 -0500
Name : Organization: National Organization for Rare Disorders, Inc
Subject: Urgent Update - Rare Diseases Act of 2001, S. 1379
On December 28 S. 1379 was placed on the Senate Legislative Calendar
under General Orders - Calendar No. 298, and it is Senator Kennedy's
intention to put the Rare Diseases Act, S. 1379, up for a vote on the
Senate floor when Congress returns from recess. It is critically
important that you call BOTH of your senators as soon as possible
asking that they cosponsor the bill.
1. Call the U.S. Capitol Switchboard today at (202) 224-3121 and ask
for your senator's office.
2. When connected with the proper office, request to speak to the
Health Legislative Assistant (LA).
3. When speaking with the Health LA, give them a brief background of
the bill (outline attached) and tell them why passage of the bill
is important to you and your family.
4. Let them know that
a) you want your senator to cosponsor the bill; and,
b) you want him/her to VOTE YES when the bill comes up for a vote.
5. Ask them to call you back as soon as possible with the status of
the Senator's position.
It would be helpful to me if you would let me know where your senators
stand - will they cosponsor and will they vote yes.
Remember, your organization or group does not vote, but you do. The
ultimate authority of the U.S. Congress to act resides in you and you
alone.
To read the Senate Health, Education, Labor and Pensions Committee
Report 107-129 go to:
http://thomas.loc.gov/cgi-bin/cpquery/R?cp107:FLD010:@1(sr129).
You can also access the bill language at http://thomas.loc.gov.
Please contact me at my e-mail address or at my office (202) 496-1296
if you have any questions.
Thank you. Diane Dorman
Diane E. Dorman, Vice President for Public Policy
National Organization For Rare Disorders - Washington Office
1050 17th Street, NW, Suite 600
Washington, DC 20036
Phone/202.496.1296 x 3014; Fax/ 202.463.6245
Cell/202.258.6457
ddorman@rarediseases.org
(2)
=====
Umbilical Cord Blood - Human Clinical Trials - ALS
==========
>From: RSiblo@aol.com
Date : Thu, 10 Jan 2002
Seeking research and data:
Patient and physician information on human clinical trials with
umbilical cord blood and the potential benefit for ALS patients
(3)
=====
re: anyone else diagnosed with a stroke? (ALSD979)
==========
>From: Medico71@aol.com
Date : Tue, 15 Jan 2002
To bossjo @ juno.com
ALS is a diagnosis of exclusion, which means the physician must rule out
all other conditions which may present with the same or similar symptoms
and have an EMG that shows nerve deregulation IN at least 3 limbs before
the diagnosis can be made. Would it have made any difference if he was
diagnosed one week earlier? One month? Six months?
ER's are set up to handle trauma, to suture wounds and set fractures and
to separate medical patients who need to be admitted so that the correct
specialist should see them. AGAIN and again I have preached the value
of a family physician who knows you, your history, and would have known
how long that murmur was present. He will also guide you through the
minefields that exist when a cardiac surgeon is speaking to a
neurologist.
Your excitement about lawsuits disgusts me. It is not the doctors fault
that your husband has ALS, and suiing him will not make him better. Or
were you going to donate any award to the ALS foundation?
Medico71@aol.com Dx12/99.
(4)
=====
News From ALSA: Follow-up on Gulf War Study
==========
Date : Tue, 15 Jan 2002
>From: webmaster@alsa.org
FOLLOW-UP ON GULF WAR STUDY
In mid-December 2001, the Department of Veterans Affairs reported that
preliminary evidence from the Study of ALS among Gulf War Veterans
indicated that veterans who served in Desert Shield-Desert Storm were
twice as likely as their non-deployed counterparts to develop ALS. The
data from the study is subject to peer review and has not yet been
published.
Since that time, ALSAs Patient Services Department has just received the
following information from the Veterans Administration Research and
Development Department.
CASE ASCERTAINMENT CONTINUES
The Study of ALS among Gulf War Veterans will continue to screen and
"count" new patients - both Gulf deployed and non-Gulf deployed.
Although these "new" cases cannot be added to the study data that have
already been analyzed, they will be counted and reflected in a revised
rate of incidence (as appropriate). Veterans with ALS or families of
these veterans who served in the military between August 2, 1990 and
July 31, 1991 are encouraged to consider self-referral to this study.
Note that veterans need not have been deployed to the Gulf War region
to self refer to the study.
The toll-free number is still in effect and is the best way for veterans
and their families to self refer. To be included in a revised count,
veterans or family members should call 1-877-342-5257. This telephone
number should not be used for benefits questions.
NEXT PHASE OF GULF WAR STUDY
The next phase of the study is funded and is in its early stages. This
part of the study evaluates environmental and other exposures that the
veterans with ALS experienced to determine if a statistical association
between any exposure(s) and ALS is observed. The analysis will include
both the deployed and non-deployed groups. We do not yet have a timetable
for when the results of this study will be concluded and reported.
VETERANS BENEFITS UPDATE
The study staff has begun contacting the 40 Gulf deployed ALS patients
and families, asking their consent to have the Veteran's Benefits
Administration (VBA) contact them to discuss benefits. Thirty-five of
the 40 patients/families have been contacted as of January 9th. The VBA
has already begun reaching out to the thirty-five patients/families to
discuss benefit eligibility. At this time, ALSA does not know what
benefits are being offered.
CONTACT:
Mary Lyon
Ph: (818) 880-9007, Ext. 217
e-mail: mary@alsa-national.org
For more information about ALS and the work of The ALS Association,
please visit www.alsa.org.
(5)
=====
ALS Drug Treatment Trial Announcement: Insulin Growth Factor 1
==========
>From: "Les Turner ALS Foundation" news@lesturnerals.org
Date : Tue, 15 Jan 2002
The following information has been provided by the Great Lakes ALS
(GLALS) Study Group and is not being held at Northwestern University
Medical School.
Drug Treatment Trial Announcement: Insulin Growth Factor 1
Overview: A 24-month treatment trial on the above medication will
commence in approximately April 2002. This trial will be under the
auspices of the Great Lakes ALS (GLALS) Study Group. The closest site
to northern Illinois will be at the Medical College of Wisconsin in
Milwaukee. This trial is supported by the NIH and the ALS Association.
The medication is injected subcutaneously twice daily, similar to
insulin. The Milwaukee site hopes to enroll at least 25 patients.
Inclusion criteria:
- history of chronic weakness of less than 24 months duration
- fulfill El Escorial criteria for probable or definite ALS
- be able to comply with protocol requirements & provide written
informed consent
- have mild to moderate limb weakness
- forced vital capacity should be greater than 60% at time of entry
into study
Exclusion criteria:
- active neoplasm, renal disease, or active systemic disease
- clearly significant abnormality on laboratory and general health
screen
- type I or type II diabetes
- use of investigational drug within 30 days of baseline visit
- tracheostomy
- depression as measured on the Beck Depression scale
- females: pregnancy or unable to maintain strict birth control
measures during the study
Individuals who are interested in the study are requested to contact
the Milwaukee site Principal Investigator, Paul E. Barkhaus, M.D.
(phone: 414-805-5547 / address: Department of Neurology, Froedtert
Memorial Lutheran Hospital, 9200 W. Wisconsin Ave, Milwaukee, WI, 53226
Les Turner ALS Foundation
8142 N. Lawndale Avenue
Skokie, IL 60076
(847) 679-3311
www.lesturnerals.org
(6)
=====
Should we be tested?
==========
>From: APRILA1113@aol.com
Date : Fri, 11 Jan 2002
This has been a difficult thing to find out for my family. Over the
years, 3 people in my family have died from ALS, and just recently we
have found that a couple more people have been diagnosed with having
the gene. The doctors around here don't really take it seriously
and don't feel there is a need for any of us to be tested for the gene.
Should we be tested? Please contact me by mail.
My address is 1000 Monticello Rd., Apt. 103, Charlottesville, VA 22902.
Sincerely, April Applegate
(7)
=====
Power Chairs
==========
>From: TJCOUGH@aol.com
Date : Fri, 11 Jan 2002
Permobil power chair (Chairman model) w/elevating seat,used one year.
Also, a Jazzy 1115 power chair. North eastern Penna. area. For more
details call 570-672-2008 or --- tjcough@AOL.com
Thank You, Tom Coughlin
(8)
=====
Water in Maine
==========
>From: "Donna" dela@midmaine.com
Date : Wed, 9 Jan 2002
A while ago I wrote a desperate letter asking for help. Our Well had
been dry for 3 months and with my husband, Joe, not being able to take
a Bath. I am now writing to Thank everyone so much for their help. An
article in our local newspaper produced the full estimate amount and
we received mail and checks from many. As soon as I knew we would
have enough for a well, I called for the driller. As of December 27th
2001, we have Water! 500 feet down and a gallon a minute. We had the
whirlpool tub that we had purchased second hand back in July, hooked
up finally and Joe was able to take a Whirlpool Bath for the first
time. He loved it and said it was, " Heaven". Thanks to all the
Generous PALS and CALS that donated and made this possible!
Joe and Donna Delano
(9)
=====
A potential role for the p75 low-affinity neurotrophin receptor
==========
>From: whubben@als-tdf.org
Date : Mon, 07 Jan 2002
A potential role for the p75 low-affinity neurotrophin receptor in
spinal motor neuron degeneration in murine and human amyotrophic
lateral sclerosis.
Source : Amyotroph Lateral Scler Other Motor Neuron Disord 2001
: Sep;2(3):127-34
Author(s): Lowry KS, Murray SS, McLean CA, Talman P, Mathers S,
: Lopes EC, Cheema SS.
Institute: Department of Anatomy and Cell Biology, Monash University,
: Clayton, Victoria, Australia.
Published: 09/01/2001
Abstract:
INTRODUCTION: The p75 neurotrophin receptor has been recognized as a
death-signalling molecule under certain circumstances. Its role in
motor neuron degeneration in amyotrophic lateral sclerosis (ALS) was
analysed in SOD1-G93A transgenic mice and in spinal cords from human
amyotrophic lateral sclerosis. METHOD: The precise loss of motor
neurons in SOD1-G93A transgenic mice from birth to adulthood was
established using the unbiased fractionator/optical dissector neuronal
counting technique. RESULTS: This study showed an early trend in the
loss of lumbar motor neurons in SOD1-G93A mice, beginning at birth and
progressing to a massive 80% reduction by 4 months of age, when the
disease is severe. This study also found that the p75 neurotrophin
receptor was expressed in lumbar motor neurons in symptomatic
SOD1-G93A mice and in motor neurons in the cervical spinal cords of
patients with ALS. CONCLUSIONS: The murine and human ALS data suggest
that the p75 neurotrophin receptor may play a death-signalling role
in the pathogenesis of motor neuron degeneration. The precise
mechanism by which this receptor drives the apoptotic process, both
in murine SOD1-G93A motor neuron degeneration and in human amyotrophic
lateral sclerosis, remains to be determined.
Abstract Archived at: http://www.als-tdf.org/alstdf/research/hubben/
viewarticle.asp?id_article=260
PubMedID: 11771768
PubMed Link: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=
Retrieve&db=PubMed&list_uids=11771768&dopt=Abstract
Will Hubben whubben@als-tdf.org
(10)
=====
Laryngeal dysfunction in ALS: a review and case report
==========
Date : Wed, 28 Nov 2001
>From: Will Hubben whubben@earthlink.net
Laryngeal dysfunction in Amyotrophic Lateral Sclerosis: a review and
case report.
BMC Ear Nose Throat Disord 2001;1(1):1
Watts CR, Vanryckeghem M.
Department of Speech Pathology & Audiology, University of South
Alabama, Mobile, AL, USA. cwatts@usamail.usouthal.edu
BACKGROUND: Laryngeal dysfunction can be a salient feature in the
clinical symptomatology of speakers diagnosed with Amyotrophic
Lateral Sclerosis (ALS). In addition to dysphonia, swallowing
function is also disrupted. This paper reviews what is known about
laryngeal dysfunction resulting from ALS. RESULTS: Presented is a
case report of a female, diagnosed with ALS, whose initial symptoms
were caused by laryngeal bulbar involvement that was characterized by
dysphonia and dysphagia. CONCLUSIONS: In bulbar forms of ALS, voice
and/or swallowing difficulties are often the initial signs of
disease. Careful examination of the muscles innervated by bulbar
nerves, and tracking the rate of progressive deficit in the affected
muscles, will help to solidify an accurate diagnosis. With therapy,
the ability to swallow safely may still be maintained even when voice
and articulation abilities are such that oral communication is
inefficient.
PMID: 11722802
Will Hubben
whubben@earthlink.net
(11)
=====
ventilator question
==========
>From: "Mary Ellen Brown" mebrown@speakeasy.org
Date : Sun, 18 Nov 2001
I wrote a week or so ago for my father asking about a tracheostomy.
He is still in the process of deciding what he wants to do. He wants
to consider all the options and ramifications of performing the
surgery including the possibility that he might end up on a Ventilator.
Dad has mostly Bulbar ALS. He is fully ambulatory and mobile otherwise.
He can walk, and use his arms and hands. He is not in a wheelchair.
He cannot speak, swallow, eat and his breathing is reduced to critical
levels. He has had a feeding peg for almost a year. He is having
troubles with mucus and saliva and even with suctioning and the use of
a cough machine, is very uncomfortable.
We have a few questions for those of you with experience with
ventilators.
1. Are there small, portable ventilators that he can either pull, put
on a stand or somehow carry?
2. Are there features and quirks that we should be looking for in
choosing both a small portable one as well as one for in home use
during the day and at night?
3. Are they comfortable and will it be difficult to adjust to using
one (quality of life issues)? Is it difficult to keep everything
sterile?
Many thanks for those of you who previously responded to our posted
questions and some follow up questions. Our thoughts are with you all.
Mary Ellen Brown
(12)
=====
re: PEG tube pain
==========
Date : Tue, 15 Jan 2002
>From: Wayne Phillips wsphillips@compuserve.com
Hi Craig,
I haven't seen a reply to your PEG question in Digest 973. My tube
doesn't cause pain, and I see no reason why yours should. You should
definitely talk to the doctor. Maybe a different style or brand would
help. I have a "MIC-KEY", made by Ballard, and highly recommend
them.
See http://www.bmed.com for more info. good
luck, Wayne
(13)
=====
re: ALS and other diseases (ALSD976)
==========
>From: "Deanna Hammond" ljde@mindspring.com
Date : Mon, 14 Jan 2002
As for Dr. Ching-Chee Chan" comments, I had Juvenile Rheumatoid
arthritis in 1949 and had to be carried around. In 1968 I had a
reoccurrence lasting 2 years. Now I have ALS, Dx Jan 2000.
=== end of alsd 980 ===