Date: Wed, 11 Oct 95 00:12:37 -0400 From: Bob Broedel To: als@huey.met.fsu.edu Subject: ALSD219 ALS-ON-LINE =============================================================== == == == ----------- ALS Interest Group ----------- == == ALS Digest (#219, 10 October 1995) == == == == ------ 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 == == 1080+ subscribers. == == == == To subscribe, to unsubscribe, to contribute notes, == == etc. to ALS Digest, please send e-mail to: == == bro@huey.met.fsu.edu (Bob Broedel) == == Sorry, but this is *not* a LISTSERV setup. == == == == Bob Broedel; P.O. Box 20049; Tallahassee, FL 32316 USA == =============================================================== == Back issues of the ALS Digest are available on-line at: == == http://http1.brunel.ac.uk:8080/~hssrsdn/alsig/alsig.htm == =============================================================== CONTENTS OF THIS ISSUE: 1 .. ALS Bulletin Board on America On Line 2 .. Neurology Patient Listserver 3 .. re: recent posting 4 .. Is ALS invariably fatal? 5 .. re: Hereditary aspects of ALS 6 .. ALS 7 .. Suggestions needed 8 .. PROMED: ALS 9 .. radiotherapy for drooling in ALS patients 10 . Call for patients BDNF study 11 . DECtalk and Voice Output Communication Aids (1) ===== ALS Bulletin Board on America On Line ========== Date : Sun, 8 Oct 1995 19:28:51 -0400 >From : APALS@aol.com To : bro@huey.met.fsu.edu Subject: The ALS (Lou Gehrig's Disease) bulletin board.. Attention: America On Line Members! For anyone wishing to give and/or receive information and support via the AOL ALS bulletin board, please use the following directions to join us! The ALS (Lou Gehrig's Disease) bulletin board! We hope you'll join us there! Keyword: disABILITIES Double Click-on: disABILITIES Message Center Double Click-on: General Discussions Click-on icon: List topics Click-on: ALS (Lou Gehrig's Disease) This bulletin board system offers support, encouragement and guidance to those of us affected with ALS. There's a wealth of information to be shared and we encourage active participation of ALS patients, families and friends. God Bless Us Everyone! A PALS and A PALS Wife ( A Person with Amyotrophic Lateral Sclerosis). (2) ===== Neurology Patient Listserver ========== Date : Tue, 10 Oct 95 10:37:00 -0400 >From : "Michael H. Rivner, M.D." Subject: Neurology patient listserver I am initiating an INTERNET listserver for patients with neurological diseases. To subscribe: send an EMAIL message to: n-pat@emgmhs.mcg.edu that contains subscribe n-pat This will be run by the Department of Neurology at the Medical College of Georgia. I hope that this will be a valuable resource. Michael Rivner (3) ===== re: recent posting ========== Date : Mon, 9 Oct 1995 11:53:04 -0500 >From : rosen@wadsworth.org (Daniel Rosen) Subject: recent posting Thanks for continuing to operate "The ALS Digest". I just wanted to add an addendum to posting 4 of the recent issue. The poating was entitled "Ripken streak brings a look at illness that fell Gehrig". There were two statements that I thought needed clarification. First, familial ALS is currently believed to represent 5-10% of all ALS cases, not 5-50% as stared in the posting. It is possible that the number could be slightly higher, perhaps as much as 15%, but currently, the 5-10% number seemes to be the best estimate. Second, the one gene that was found that causes some cases of familial ALS, SOD-1, does not cause all cases of familial ALS, as the posting implies. SOD-1 causes perhaps a quarter of familial ALS cases, no more. It is crystal clear that there is a second gene, and several labs, including mine, are intensively searching for this gene. This second gene has the potential of causing all or most of the remaining cases of familial ALS, and could provide greater understanding of the molecular mechanism by which ALS, both familial and sporadic, is caused. Thanks for your attention. Regards, and keep up the fine work, Daniel R. Rosen, Ph.D. Wadsworth Center for Laboratories and Research New York State Department of Health (4) ===== Is ALS invariably fatal? ========== Date : Sun, 8 Oct 1995 21:32:24 -0700 >From : eaopp@ucla.edu (Edward Anthony Oppenheimer, M.D.) Subject: Is ALS invariably fatal? I would be interested in comments from others who follow ALS and motor neuron disease issues. It is frequently said that ALS is a fatal disease. Is this true? In the last ALS Digest (ALSD218 ALS-ON-LINE) item #4 is a typical example": > >(4) >===== >Ripken streak brings a look at illness that fell Gehrig >========== >TITLE : Ripken streak brings a look at illness that felled Gehrig >BYLINE: Dr. Simeon Margolis >DATE : 09/26/95 >SOURCE: The Baltimore Sun > > "... Amyotrophic lateral sclerosis, or ALS, the medical term for Lou > Gehrig's disease, is a relentlessly progressive disorder caused by > deterioration of the motor nerves (nerves that control the action of > muscles) in both the brain and spinal cord. ... Death often results from > respiratory failure. ALS is invariably fatal; the average life span after > the onset of symptoms is two to five years." > It may be useful to start off by comparing the usual care for high spinal cord injury care with the usual care for people with Amyotrophic Lateral Sclerosis. Spinal cord injury (SCI), particularly C-1 and C-2 high cord injury causes quadriplegia with both motor and sensory nerve loss, and almost always requires life support mechanical ventilation. This is in some ways more severe than ALS but SCI does not usually prevent speech nor go on to a locked-in state (the head is spared). SCI is not referred to as a fatal disease because in the USA it is customary to treat any complications and sustain the person with SCI paralysis. Many people with high SCI initially, during the first six months, request to stop treatment and ventilator support; they wish to die. In the USA this is usually not honored during the first six months because most people learn to accommodate, to live with their disability if they receive good care and rehabilitation. The average age of people with SCI is much younger than those with ALS. When a person with ALS (PALS) has the benefit of medical care, and does not refuse any aspect of available care, ALS itself will not cause death. Infection, malnutrition, respiratory failure, rehabilitation and psychosocial needs can all be treated with reasonably good success. However many professionals and organizations that serve PALS say or suggest that "ALS is invariably fatal". This of course has an overwhelming impact on the person with ALS and their family: a feeling of hopelessness. They are not encouraged to live with ALS, like the person with SCI. And they often are not offered a choice nor a balanced presentation of the options. When infection, malnutrition, aspiration, or respiratory failure are not specifically treated, then these treatable complications can be fatal. I am sure that there might be fund raising or other reasons for speaking about ALS in 1995 as "invariably fatal". Or, the media may have difficulty obtaining the facts, and thus perpetuates the discouraging myth that "ALS is invariably fatal". There is often a major impact from presenting information with a particular "spin" or bias: "ALS is invariably fatal".... Is this honest? Is it beneficial to the patient or family? Is it harmful? The alternative is to present as honestly as possible the available information and treatment options, the pros and cons, and your advice as a physician... And then assist the patient (and family) to make the best possible decisions in view of their values and circumstances. Comments on this important ethical and clinical issue are requested. Regards, Edward Anthony Oppenheimer, M.D. <><><><><><><><><><><><><><><><><><><><><><><><><><><><><><><> (5) ===== re: Hereditary aspects of ALS ========== >From : jacobo@jr1.satlink.net Date : Wed, 4 Oct 1995 21:15:28 Subject: Re: Hereditary aspects of ALS I'm a neurologist, very new in the list, and very interested in keeping in touch with other specialists, ALS patiens/relatives. I live in Argentina and we do not have ALS statistics yet, although is not an uncommon disease in our population. For those interested in a Spanish explanation to possible ALS development mechanism involved, (transcripted from Dr.Sica Roberto E.- SEMCOSIN meeting -march 94 -). Send e-mail request to my address below. Yours JACOBO RUDMINSKY M.D. VOICE/FAX: 54-1-982-7270 E-MAIL: RUMI@SATLINK.COM ADRESS: BOGOTA st. # 21 - Fl. 1 - APT. #7 BUENOS AIRES - 1405 ARGENTINA--------------------------------- * E-mail: Rumi@satlink.com * * Voice/fax: 54-1-982-7270 * * Buenos Aires - Argentina * --------------------------------- (6) ===== ALS ========== >From : pat.younger.20@aspenpub.com (Pat Younger) Subject: als Date : Fri, 6 Oct 1995 20:41:10 GMT I am a hospice volunteer who visits a patient with ALS. Her name is Robin Brown. She is 39 years old and has had ALS for 15 years. I have never heard of anyone having ALS for that long. Is it unusual? Robin is a poet. She would like for me to post one of her poems on ALS if anyone is interested. She would also like to hear from other people out there. Thanks! (7) ===== Suggestions needed. ========== >From : S Panicker Subject: Re: ALSD217 ALS-ON-LINE Date : Fri, 06 Oct 95 17:58:44 GMT My father-in-law, who lives in India, has been diagnosed as suffering from ALS. Before I go any further, some background. It all started in 1991 when one of his feet developed "numbness" resulting in foot dragging. This gradually progressed up his leg and presently uses one leg for mere support and, with the help of another person, uses the other for walking. After various tests and after seeing a handful of neurologists all that he was prescribed was physiotherapy, muscle relaxant drugs and painkilling drugs (Baflon, Valium 5, Neurobion) . Importantly, however, only two members of the family, the two daughters, understand his condition and its consequences. The daughters, with the full support of the neurologists, have taken a decision that it would be in the best interests of the patient to not divulge the true character of the disease. Presently the effects are restricted to the legs, but we have recently noticed slight slurring of speech and deterioration in handwriting. I write this letter in the hope that somebody out there will have answers which will bring a little comfort into the life of a man whose pain is slowly but surely increasing. The most useful would be those concerning pain relief and the arresting of muscle wasting. I know that a couple of companies in the US have brought out drugs which are currently on trial and available to US citizens. Is it possible for a person resident in India to join one of these trials (under medical supervision, of course)? Can anybody recommend neurologists in India with a specific and active interest in ALS sufferers? In general I would like anyone to share any experience with me which they feel would enhance the quality of life of the patient. Presently we are looking for a fulltime person to assist him in all aspects of his day to day ilfe. Can anyone suggest anything on the sort of qualities and qualifications we should look for in that person which would make him/her the most appropriate? Name S Panicker My address af64@dial.pipex.com My phone London (171) 283 9494 work London (181) 341 4690 home My fax London (171) 283 0323 (8) ===== PROMED: ALS ========== >From : HOAR@hcs.gwumc.edu (Sandra Hoar) Subject: PROMED: Amyotrophic lateral sclerosis (ALS): RFI Date : 29 Sep 95 20:08:32 EDT ------- Forwarded Message Follows ------- Send reply to: owner-promed@usa.healthnet.org Subject: PROMED: Amyotrophic lateral sclerosis (ALS): RFI >From : "Dr. Hukowich" Date : Wed, 27 Sep 1995 14:49:33 +0000 ETIOLOGY OF AMYOTROPHIC LATERAL SCLEROSIS (ALS): RFI ==================================================== Does recent information about etiology of amyotrophic lateral sclerosis suggest any potential for a transmissible agent or some common exposure? -- Dr. Hukowich Organization: Port Hope E-mail: hukowich@eagle.ca .......................................................................... (9) ===== radiotherapy for drooling in ALS patients ========== Date : Tue, 10 Oct 1995 22:18:38 +0200 >From : Aad Nienhuis Subject: radiotherapy for drooling in ALS patients Cc : deyej@aol.com Some time ago I forwarded a message from my brother Hans Krotje to this list concerning his radiotherapy treatment for drooling. Hans died last saturday. He left me a letter from his doctor about the exact dose he got during his treatment (several people asked for this data). I hope this will help other people, like it did to him. Bye, Aad ----------------------------------------------------------------------------- To whom it may concern, Amyotrophic lateral sclerosis (ALS) is characterized by a progressive bulbar paralysis. One of the symptoms of this loss of nerve function is a decrease in the ability to swallow. Together with a normal (physiologic) saliva production of about 1 liter per day, which could be increased by artifical feeding via a gastrostomy (feeding catheter), this results in a constant drooling. This complaint is treated by decreasing the saliva production. Unfortunately medical inhibition of the saliva production can either result in negative side effects or has insufficient effect of the saliva production. It is a well known fact that radiation therapy reduces the saliva production which can be a severe side effect in patients treated with high doses on the oral cavity. Since the salivary glands are radiosensitive organs a rather low dose on the parotic gland (which is the main contributor of the saliva production) can significantly decrease the saliva production. The treatment protocol for ALS patients in the Royal Marsden Hospital in England consisted of 10 fractions of 2 Gy (total dose 20 gy) to both parotic glands. Since most of our patients are coming out-patients, and are in a poor neurologic condition, we use a shorter radiation scheme and thus more convenient for the patient; 2 fractions of 6 Gy are given to both parotic glands with either 250 KV photons or 10-13 MeV electrons. So far this scheme has resulted in a good palliative effect in a number of patients, although not all. We did not see any negative side effects. We are aware of the fact that this is rather a low dose which could result in either a too small effect on the saliva production or a relapse. In both cases we can give a second course with the same radiation scheme. Maybe we will increase our standard dose in the future, when we have treated a sufficient number of patients with adequate follow-up data. M.C.C.M. Hulshof M.D., radiation oncologist --------------------------------------------------------------------------- Dr. Hulshof is working in the "Academisch Ziekenhuis" connected with the University of Amsterdam. The fax number for the radiotherapy department is: +31 20 6091278 (10) ===== Call for patients BDNF study ========== Date : Sun, 8 Oct 1995 21:38:25 -0400 (EDT) >From : "Daniel M. Wetzel" Subject: Call for patients BDNF study I am posting for friends please (please) do not respond to this email address. The Department of Neurology, ALS Center and the Clinical Research Center at the University of Vermont are undertaking a trial with recombinant methionyl human brain-derived neurotrophic factor (r-metHuBDNF) in ALS patients. This trial is being sponsored by AMGEN, Inc (Thousands Oaks, CA). ALS patients that meet the inclsuion criteria will be asked to particiapte in a 9 month double blind, placebo controlled, trial with r-metHuBDNF. Patients will be required to come to the CRC for screening and baseline visits and return to the CRC once amonth for nine months. All charges related to this study will be covered by the sponsor.. Please contact Patcicia Krusinski 802-656-4177 or Mary Fitzpatrick 802-656-7717, Research Coordinators, University of Vermont Neurology, Given Building, Burlington VT 05401. FAX 802-656-2461 (11) ===== DECtalk and Voice Output Communication Aids ========== >From : vitale@dectlk.ENET.dec.com Date : Tue, 10 Oct 95 13:29:34 EDT Subject: DECtalk & VOCAs Due to the barrage of calls and e-mail I've gotten in response to DECtalk(tm) and Voice Output Communication Aids (VOCAs), I've prepared the following information for people who subscribe to this discussion group. Many people have a friend or loved one who has difficulty trying to communicate. The following list will hopefully be helpful. Voice Output Communication Aids DECtalk(tm) speech synthesis technology is licensed and integrated to third party products intended for use by people with disabilities. This licensing program facilitates the widespread availability and use of Digital's DECtalk speech synthesis technology in a manner that complements the DECtalk based assistive products developed internally. For example, it is licensed for integration in third party Voice Output Communication Aids, commonly known as `VOCAs'. These devices are extremely useful tools for communicating with DECtalk speech output and a variety of forms of input, on behalf of non-verbal individuals or those with other forms of disability which affects communicate via speech. VOCAs and Augmentative Communication Software The following two tables provide information about VOCAs that incorporate the DECtalk text-to-speech synthesis technology and VOCA software products that support DECTalk text-to-speech synthesizers like the DECtalk PC and DECtalk Express. For support from Digital Equipment Corporation, call Ray Theil at 603-884-9317. VOCA's available with licensed DECtalk Speech Synthesis: Product Company Address LightWRITER SL35 ZYGO Industries Inc. P.O. Box 1008 Portland, OR 97207 800-234-6006 Multi-Voice Institute on Applied Children'sHospital Technology 300 Longwood Ave Boston, MA 02115 617-355-6998 Touch Talker Prentke Romich Co. 1022 Heyl Road Wooster, OH 44691 800-262-1984 DynaVox Sentient Systems 2100 Wharton Street Pittsburg, PA 15203 800-344-1778 Vois 160 Phonic Ear Inc. 250 Camino Alto Mill Valley, CA 94941 800-227-0735 Say-It-All Innocomp 26219 Emery Rd, Ste 302 Say-It-Simply Warrenville Hghts, OH 44128 Scan-It-All 800-382-8622 Augmentative Communication Software which supports DECtalk text-to-speech synthesizers. Product Company Address POPCOMM & Pointer Systems Inc. One Mill St. ONEKEY Burlinton, VT 05401 802-658-3260 E Z Keys Words +, inc. 40015 N. Bernadino Ave. Palmdale, CA 93550 800-869-8521 Handichat Microsystems Software 600 Worcester Rd. Inc. Framingham, MA 01701 800-828-2600 WriteAway Institute on Applied Children's Hospital Technology 300 Longwood Ave. Boston, MA 02115 617-355-6998 === end of als 219 ===