=============================================================== == == == ----------- ALS INTEREST GROUP ----------- == == ALS Digest (#77, 04 January 1994) == == == == ----- amyotrophic lateral sclerosis (ALS) == == ----- motor neurone disease (MND) == == ----- Lou Gehrig's disease == == ----- == == 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. Currently there are == == 190+ subscribers. == == == == To subscribe, to unsubscribe, to contribute notes, == == etc. to ALS Digest, please send e-mail to: == == bro@huey.met.fsu.edu (Bob Broedel) == == == == All interested people may "broadcast" messages to == == ALS Digest (not a peer reviewed journal, and not edited == == by an MD) subscribers by sending to: == == als@huey.met.fsu.edu == == == == Bob Broedel; P.O. Box 20049; Tallahassee, FL 32316 USA == =============================================================== == Back issues are available via e-mail from: == == LISTSERV@mailer.fsu.edu == == send an e-mail message that says INDEX ALS == == Also available via anonymous FTP at mailer.fsu.edu == == directory ftp/pub/als == == Hardcopy versions (with extensive indexing) available == == from Grant Nicholas (gnicholas@pcgate.lanl.gov) == =============================================================== A Brief technical note from the Editor. I think I made a mistake while reading my e-mail. I lost approximately 200+ messages that were sent to bro@huey.met.fsu.edu. Please re-send messages that were intended for ALS Digest, but that are not in this issue. Sorry. rgds,bro (1) ===== re: feeding tubes ========== Date : 04 Jan 94 17:00:52 EST >From: Charles M Ward <72567.400@CompuServe.COM> Judi, Welcome to the group. I have some thoughts on feeding tubes you might consider. I think a feeding tube is very important when you get to where you need one. In ALS a constant complaint of people is they lost too much weight. You must keep your nutrition up so you feel good and can do things. Also you don't want to lose weight because you need the padding for comfort. Particularly on your bottom so you can continue to sit in a chair. Don't wait to have a feeding tube put in. Like in all things with ALS you must be prepared to start early with adaptive aids, it does no good to wait until the last moment. I have had a feeding tube for three years and have had zero problems with it. There are only advantages with feeding tubes. I have a feeding tube in my neck that goes directly into the esophigus. The local MDA doctor reccomends neck tubes over the more common stomach tubes because he says the stomach tube interferes with the diaphram and this leads to earlier breathing problems. Also the stomach tubes can leak and smell bad. It takes some looking to find an ENT who can do the surgery. It is done under a local anethetic and takes about an hour. You can still talk and eat after the operation. I had the surgery as soon as I started having trouble keeping my weight up. From then on I would eat what I could and then have the rest blended and put down the tube. Very easy! I find the neck tube to be great on the go. When I need water, medication or to eat I just stop and my aide pumps it in. People stare because it is unusual but it is perfectly normal to just syringe it in. I put a piece of silk tape around the tube when it is in position and pin the tape to my shirt. I leave the tube in permanently and change it every six months. It is very simple to change. Another advantage I have found came when I went on the respirator. To keep my lung secretions thin for easy suctioning I found that taking two cups of water every four hours did the trick. Again with the neck tube so easy to use this presents no problem. I strongly urge you to get a feeding tube earlier rather than later. There are no disadvantages. Send me a note if you have any questions. Yours, Mike Ward (2) ===== re: amino acids, speech, etc. ========== Date : Thu, 30 Dec 93 08:36:24 EST >From : Michelle Jensen Subject: Re: ALSD#75 ALS-ON-LINE Dear Bob, I wish to share responses to three inquiries. My responses come both as a care giver who husband Paul, has had ALS for 2+ years, and as a speech pathologist who works with swallowing and communication problems. 1. Amino Acid studies. The question was raised regarding BCAA (chained branch amino acids). We were adivsed in Digest #75 by Dr. Rosen that studies were stopped because of increased morbidity. Paul was on BCAA for 3 to 4 months. We followed the recommended schedule: 45 min. before eating. It was a gruelling schedule to follow; Paul lost weight and we found no benefirt. We quit and would not recommend it. 2. Synthesized speech. Be sure to consider the DynaVox. It's a light weight, self contained unit with 256K, that is easily wheelchair mounted. It can be accessed with a variety of switches including eye-blinks. 1-800-344-1778. Ask for a home demonstration. @$4500. 3. Swallowing problems. There are a variety of texture, taste, temperature and posture adjustments that on should follow first. Difficulty with thin liquids may be masked by avoiding them which will lead to chronic dehydration. The literature says that it's time for a G-tube when aspiration events, food spillage, prolonged meal times and respiratory fatigue lead to an aversion of eating. G tube placement (which is a simple procedure with local anesthetic) relieves those burdens. Malnutrition and dehydration are definitely to be avoided by ALS patients. Thanks for the developing this avenue of communication. Give our Best to Carmen. Paul and Michelle Jensen (3) ===== re: feeding tubes (more) ========== CompuServe Mail Date : 30-Dec-93 22:16 EST >From: MARTIN I. WEINBERGER [72724,324] Subj : Copy of: Feeding Tubes Hi, Judi, I'm principal caregiver for my wife, now aged 68, who has had ALS for 2-1/2 years. This summer she made a decision to go for a stomach feeding tube since her swallowing had become so difficult. Mealtimes were lasting 2-3 hours, even with pureed foods; she was not getting sufficient nutrition and particularly not getting enough liquids. She had a problem with excessive mucous and saliva which were difficult to manage. The physicians who recommended the procedure described it in terms which made it seem no more than a tooth extraction. Then we learned it required an overnight stay in the hospital which my wife dreaded. She is unable to communicate; cannot move her limbs; requires frequent turning during the night. I arranged with the hospital for a private room with a cot for me and they were very accommodating. What started out as an overnight stay turned into six nights in the hospital because of complications. The physicians never mentioned that there was a possibility of stomach distention and trapped air in the intestines. We opted for "bolus" feeding rather than "continuous drip" , not wanting to be tied down to a, IV bag all day. However, with feeding every 4 hours for nutrition, preceded by a digestive aid a half-hour before, and a laxative/fluid feeding in between, we are still ruled by the clock. Feedings are supposed to start at 6am (with medication at 5:30) but more often than not she refuses that one preferring to sleep. Since the tube was inserted she has taken nothing by mouth. Tried some apple sauce the first few days but gave it up. However, the mucuous problem ceased and the saliva build-up has been manageable. Tube's entry point needs careful cleaning each day - peroxide and change dressing - nothing complicated. Caregivers have had no trouble mastering the technique. The gastroenteroligist prescribed Osmoilite and she has been on that exclusively. If there is anything else that you think I can provide, don't hesitate. All the best to you and yours in the coming year. Marty (Compuserve 72724.324@CompuServe.com or America On Line MartyW3818@aol.com) (4) ===== re: speech ========== >From : madbadger@aol.com X-Mailer: America Online Mailer Sender : "madbadger" Date : Tue, 04 Jan 94 10:55:47 EST Subject : Assistive Devices/Speech Output Bob, I wrote this in reply to a gentleman and am now unable to find his E-mail address. I have seen recently a number of people asking for assistance in speech output devices. Anyway, I'm sure you are already familiar with the following but others may not be. Unfortunatly, I'm pretty new to E-mail and all and am unsure of how to E-mail the group but I will find out. In the mean time, I wanted to let everyone know of our solution. My grandfather is in the advanced stages of ALS and has been unable to communicate verbally for a number of years. He wrote notes for a while but for the last year or so his handwriting has been illegible. He only has limited movement in his right arm so this was our solution. I work on a Macintosh computer and knew that Apple computer has a Disability resource group so I started there. We set my grandfather up with a Powerbook 145 and are running software called "Speaking Dynamically" by Mayer Johnson. This software is incredibly easy to "program". It consists of a virtually unlimited number of screens. We programmed it to have only 3 basic screens with a number of subset screens. The three basic are "Health/Emergency", "Conversation", and "I want ...". The way it works is as follows: Using a mouse he selects a box (by simply moving the cursor within the box) containing the Icon of the phrase he wants to say. At the top of the screen the phrase is spelled out so he can verify that it is indeed the correct phrase. He then simply clicks the mouse once and in this case the computer plays a recording of MY voice speaking the phrase (the software allows several types of speech output including synthesis). The program also allows the user to type in words or phrases at any time (spelling need not be accurate) and the computer will sythesize it into speech output. We also use a "keyguard" so that he can rest his hand on the keyboard and push the key thru individual holes in the keyguard. If you are interested in more information please let me know, and if you purchase the software I'd be more than happy to send you what work I've done as far as recordings and icons to help speed the programming process. It has given my Grandfather much joy to be able to communicate and carry on the semblance of a conversation again. Here are some addresses you may find useful. Apple Computer Inc. Worldwide Disability Solutions Group 20525 Mariani Ave., MS 2-SE Cupertino, CA 95014 408-974-7910 AppleLink: AppleDSG America Online: AppleDSG@aol.com Mayer-Johnson Co. POB 1579 Solana Beach, CA 92075 619-481-2489 Let me know if I can be of more assistance Lafe Bailey - Mad Badger (5) ===== samples of new references to the literature on ALS ========== ================================================ Title : Evidence for a dopaminergic deficit in sporadic : amyotrophic lateral sclerosison positron emission : scanning Author : Takahashi H.; Snow B.J.; Bhatt M.H.; Peppard R.; : Eisen A.; Calne D.B.; Source : 1993 342/8878 (1016-1018) LANCA Lancet ================================================ Title : Conjugal amyotrophic lateral sclerosis: Report of : a young married couple Author : Cornblath D.R.; Kurland L.T.; Boylan K.B.; : Morrison L.; Radhakrishnan K.; Montgomery M. Source : 1993 43/11 (2378-2380) NEURA Neurology ================================================ Title : Novel lacto-ganglio type gangliosides with : G(M2)-epitope in bovine brain which react with IgM : from a patient of the amyotrophic lateral sclerosis- : like disorder Author : Nakao T.; Kon K.; Ando S.; Miyatake T.; Yuki N.; : Li Y.-T.; Furuya S.; Hirabayashi Y. Source : 1993 268/28 (21028-21034) JBCHA Journal of : Biological Chemistry ================================================ Title : Abnormal acidic amino acids and N-acetylaspartylglutamate : in hereditary canine motoneuron disease Author : Tsai G.; Cork L.C.; Slusher B.S.; Price D.; Coyle J.T.; Source : 1993 629/2 (305-309) BRREA Brain Research ================================================ Title : Amyotrophic lateral sclerosis and structural defects : in Cu,Zn superoxide dismutase Author : Deng H.-X.; Hentati A.; Tainer J.A.; Iqbal Z.; : Cayabyab A.; Hung W.-Y.; Getzoff E.D.; Hu P.; : Herzfeldt B.; Roos R.P.; Warner C.; Deng G.; Soriano E.; : Smyth C.; Parge H.E.; Ahmed A.; Roses A.D.; : Hallewell R.A.; Pericak-Vance M.A.; Siddique T. Source : 1993 261/5124 (1047-1051) SCIEA Science ================================================ Title : Decreased cereborspinal-fluid superoxide dismutase : in amyotrophic lateral sclerosis Author : Iwasaki Y.; Ikeda K.; Kinoshita M.; Source : 1993 342/8879 (1118) LANCA Lancet ================================================= Title : Role of gene defect in hereditary ALS clarified Author : Marx J.; Source : 1993 261/5124 (986) SCIEA Science ================================================= Title : Cu/Zn superoxide dismutase (SOD1) mutations and : sporadic amyotrophic lateral sclerosis [7] Author : Jones C.T.; Brock D.J.H.; Chancellor A.M.; : Warlow C.P.; Swingler R.J.; Source : 1993 342/8878 (1050-1051) LANCA Lancet ================================================= Title : Diaphragmatic paralysis preceding amyotrophic : lateral sclerosis Author : Annane D.; Korach J.M.; Templier F.; Durand M.C.; : Dinet-Busso N.; Le Corre A.; De Latte J.; Gajdos P.; Source : 1993 342/8877 (990-991) LANCA Lancet ================================================= Title : Effects of multiple subcutaneous doses of rhIGF-1 : on total and free IGF-1 levels and blood glucose in : humans Author : Stong D.B.; Raskin R.; Source : 1993 692/- (317-320) ANYAA Annals of the New York : Academy of Sciences ================================================= Title : Neural degeneration and the transport of neurotransmitters Author : Edwards R.H. Source : 1993 34/5 (638-645) ANNED Annals of Neurology ================================================= Title : Detection of tau proteins in normal and Alzheimer's : disease cerebrospinal fluid with a sensitive sandwich : enzyme-linked immunosorbent assay Author : Vandermeeren M.; Mercken M.; Vanmechelen E.; Six J.; : Van de Voorde A.; Martin J.-J.; Cras P.; Source : 1993 61/5 (1828-1834) JONRA Journal of Neurochemistry ================================================= Title : The potential of insulin-like growth factor-I as a : therapeutic for the treatment of neuromuscular disorders Author : Lewis M.E.; Vaught J.L.; Neff N.T.; Grebow P.E.; : Callison K.V.; Yu E.; Contreras P.C.; Baldino F. Jr.; Source : 1993 692/- (201-208) ANYAA Annals of the New York : Academy of Sciences ================================================= Title : Neuronal damage induced by beta-N-oxalylamino- : L-alanine, in the rat hippocampus, can be prevented : by a non-NMDA antagonist, 2,3-dihydroxy-6-nitro-7- : sulfamoyl-benzo(F) quinoxaline Author : Willis C.L.; Meldrum B.S.; Nunn P.B.; Anderton B.H.; : Leigh P.N.; Source : 1993 627/1 (55-62) BRREA Brain Research ================================================== (6) ===== of possible interest #1 ===== Topic 678 Alzheimer,s disease and spirocheteali bionet.neuroscience 4:49 pm Dec 27, 1993(at biobase.aau.dk)(From News system) Recent observation suggesting that soirochetes may be one of the causes of Alzheimer,s disease ( miklossy.J 1993 neuroreport july ; 4 (7) : 841-8 ) was wery interesting to me becouse i,m working on molecular biology of Borrelia burgdorferi ( lyme disease agent )this spirochete couse neurological disorders in som cases . if anyone can offer any other new references concerning the Alzheimer disease and spirochete specially Borrelia . Thank you very much Ali Karami Department of Medical Microbiology and immunology. Copenhagen university Denmark ali@biobase.aau.dk (7) ===== of possible interest #2 ========== Topic 688 Charcot-Marie-Tooth Disease? bionet.neuroscience 4:06 am Jan 3, 1994(at nyx10.cs.du.edu)(From News system) Please forgive me if I've posted to the wrong newsgroup, but I haven't found any others that seem more appropriate. I am looking for information on a nueromuscular disease called Charcot-Marie-Tooth (CMT). I understand the basics, but need more detail. Information available on the NET would be preferred, but I'll take anything. Also, if anyone out there has CMT (there are only about 150,000 in the U.S.) I would love to hear from you. Thanks for your help. Mark Choules mchoules@nyx10.cs.du.edu Topic 688 Charcot-Marie-Tooth Disease? Response 1 of 1 bionet.neuroscience 11:28 pm Jan 3, 1994(at oscar.gdb.org)(From News system) > >I am looking for information on a nueromuscular disease called >Charcot-Marie-Tooth (CMT). I understand the basics, but need more >detail. Information available on the NET would be preferred, but I'll >take anything. > Mark, I have already e-mailed the appropriate entries from OMIM, the Online Mendelian Inheritence in Man database of human genetic disorders. OMIM is part of the GDB Human Genome Data Base, containing human gene mapping and genetic disease information. For more information on GDB or OMIM, or to obtain a user account free of charge, please contact GDB User Services at: help@gdb.org (e-mail) (410) 955-7058 (voice) (410) 614-0434 (fax) bionet.molbio.gdb (USENET news) GDB and OMIM are available directly on the Internet using Gopher, WAIS, World Wide Web, and Sybase client software. I hope this is of some use to you. Ken Fasman, Ph.D. Informatics Director Genome Data Base Johns Hopkins University School of Medicine 2024 E. Monument St. Baltimore MD 21205 ken@gdb.org == end of als 77 ==