=============================================================== == == == ----------- ALS Interest Group ----------- == == ALS Digest #827 (27 March 2001) == == == == ------ 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 == == 4700+ 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 Please be advised, == == the editor is not a medical doctor and the Digest is == == not peer reviewed.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: == == http://www.glnicholas.com/ == == http://www.alslinks.com == == http://www.alssurvivalguide.com == == http://cc4144-a.ensch1.ov.nl.home.com/~digest == == http://health.oldeman.net == =============================================================== CONTENTS OF THIS ISSUE: 1 .. re: A New and Effective Treatment for ALS (ALSD 826) 2 .. re: A New and Effective Treatment for ALS (ALSD 826) 3 .. Peg Tube 4 .. Question re Sony Playstation 5 .. rilutek, topiramate, progesterone 6 .. Longaberger Basket Fund Raiser 7 .. Wheelchair Van For Sale 8 .. Medicare confusion 9 .. re: vent? 10 . need info on speaking device 11 . re: vent? (ALSD 825) (1) ===== re: A New and Effective Treatment for ALS (ALSD 826) ========== >From : JBull95961@aol.com Date : Mon, 26 Mar 2001 Subject: Re: A New and Effective Treatment for ALS >Dr. Moreira is currently administering the gamma globulin treatment >to 10 patients, of which Jesus and I are two of the ten. I am the >only stateside patient with all the others being Puerto Rico nationals. >Eight of the patients have seen stabilization. Two of the patients >claimed they did not see any benefit and stopped treatment. However, >one of these two, after stopping treatment, has asked to be put back >on the treatment. By my count that makes 8 patients currently receving treatment and one asking to be put back on. >For your information, I am posting the above information in the ALS >Digest, ALS Survival Guide, and MGH Neurology. Why not send to Recognized ALS centers and ask for evaluation/opinion of this treatment? Folks, I suggest you proceed with extreme caution, all along keeping a firm grip on your pocketbook. Jesse (2) ===== re: A New and Effective Treatment for ALS (ALSD 826) ========== >From : Jim Duke Subject: Re: The Gould Pitch Date : Mon, 26 Mar 2001 Let's get some data before trying to sell a treatment which is confessed as expensive, had drop-outs, etc. Most people with or without degrees, afflicted or with family afflicted and thus desperate, will not be able to make reasonable judgements. This smacks of an info-commercial. (3) ===== Peg Tube ========== >From : Sherryeco@aol.com Date : Tue, 27 Mar 2001 Subject: Hello Everyone It is Sherry regarding my Peg Tube. There was some confusion about the name of the surgery. So being a Nurse before ALS interfered with my speech here is the word on Tube Feeding. Gastrostomy is the creation of an opening into the stomach. This procedure is done to provide for the administration of food and liquid when stricture of the esophagus or other conditions make swallowing difficult or impossible. During the surgery a rectangular stomach flap is created into the abdominal stoma (stoma means mouth), used for intermittent tube feeding. The Gastrostomy tube is also know as Peg Tube. When I had my Peg Tube done it is important that the skin around the opening must be protected from irritation by the gastric juices which may leak from the opening and act as a corrosive on the skin. Tube feeding notes: From Sherry 1. Flush the tube before and after each intermittent feeding. 2. Flush the tube with 30 to 60 ml of water. 3. Before and after medication administration (use warm water). 4. If the tube becomes clogged, use 30 ml of water for flushing. 5. Whenever possible use liquid medications instead of crushed tablets. 6. Do not mix medications with the feeding product. Crush tablets as finely as possible and dissolve in warm water. 7. The Foods to be given through the tube should be cooked until soft and then pureed in an electric blender. They can be diluted with the water in which they have been cooked, so that no vitamins are lost. I have had my Peg Tube for three weeks. I had it done while healthy and even although the first two weeks of discomfort were uncomfortable I do not regret having this procedure done. I had been in the hospital a month ago or dehydration and now I will never have that problem again thanks to my Peg tube. It takes some time to adjust placing in your under garments after a bathroom break but ironically it cannot be seen under clothes. For you out there thinking about having this procedure done I hope my email helped you and I will be glad to answer any questions. God Bless All PALS AND CALS AND OUR NEUROLOGIST AND ALS STAFF, HUSBANDS, WIVES, FRIENDS AND FAMILY. (4) ===== Question re Sony Platstation ========== Date : Mon, 26 Mar 2001 >From : "RoseMarie Berkau" Subject: Question My husband was diagnosed in March 1999 with ALS. It started in his hands and arms and to date he has lost most of the use of both, however, he can still do minimal amount of movement with mainly the left hand. My question is this: he loves to play video games and I just recently purchased a Sony Playstation I for him but it is still difficult for him to work the controls; is there any kind of equipment that would make it easier for him to work the buttons on the hand held control? I would appreciate any information anyone can give me. He does get very bored and I believe this would help him stay alert and focused. Thank you in advance. rosemarie.berkau@em.doe.gov (5) ===== rilutek, topiramate, progesterone ========== Date : Fri, 23 Mar 2001 >From : Wayne Phillips Subject: rilutek, topiramate, progesterone Rilutek: I was on Rilutek for a few days and it made me feel like a zombie. I wasn't capable of much more than staring at the tv. That wasn't ok, since I was still working and supporting my family. I guess my reaction wasn't surprising since glutamate is the most common neurotransmitter in the CNS and is used by most of the CNS, and Rilutek supposedly works by reducing glutamate production and/or release. I didn't consider it any benefit to live a few more months if I had to live like that. And I'd already found out that progesterone protects neurons from glutamate excitotoxicity (see below) and that seemed to me a better way to go. Topiramate (Topamax) supposedly protects neurons: "enhances the activity of the inhibitory neurotransmitter gamma-aminobutyric acid (GABA), and blocks the action of the excitatory neurotransmitter glutamate"(1). But progesterone does the same thing(2), is natural, and a lot cheaper. Why isn't anyone pursuing progesterone? Maybe because there's no money to be made on an existing, cheap med? I have been taking progesterone orally for several years. It has noticeably reduced my cramping and at first eliminated my insomnia, though my insomnia has gotten a little worse over the last couple of years. It probably has slowed the progress of my ALS. It seems to progress more quickly when I'm off progesterone. I think that is part of the reason I've survived ALS for more than 12 years. Larger doses might even halt ALS for some people. I'm on 10mg/day at night now. I only recently became aware that people can handle much higher doses, but haven't pursued that because I'm in the middle of my doxycyclene and tinidazole treatment. While under my endocrinologist's supervision I took doses of 5 and 10 mg. at bedtime and 20 mg. 3 times daily (60 per day total). (He said 20 to 40 at 3 times daily is typical for treating sleep apnea, so 5 is pretty small.) I noticed no difference between doses initially. On the fourth day after decreasing my dose I would have a couple of days of intense cramping which would then subside. After stopping completely, my cramping and insomnia would return to pre-treatment levels. insomnia. pre-treatment: go to bed, lay awake for 1 to 2 hours, sleep lightly 2 hours, lay awake 1 to 4 hours, sleep heavily 2 to 3 hours progesterone: initially, go to bed, usually asleep within 30 minutes, sleep heavily for up to 9 hours, except when waking to turn over; after a couple of months on 5 mg. my insomnia returned slightly, but going to 10 fixed it for years. My insomnia has gotten a little worse over the last couple of years. cramping. pre-treatment: tongue, neck during daytime; abdominals, intercostals especially after a sneeze or cough; calves, thighs at night (though less intense than earlier in my illness) progesterone: reduced intensity in tongue, neck; little or no cramping in abdominals, intercostals, calves, thighs I am a 110 lb. male with sporadic, limb onset, early onset (age 29) ALS. I'm just reporting, not recommending treatment (disclaimer!). You need to see your doctor if you want to try it since progesterone requires a prescription (in the U.S.). My illness may have a different cause than yours, so it may effect you differently. It may confuse or halt the menstrual cycles of pre-menopausal women. It's inexpensive and readily available. I've noticed no difference between Provera brand and generic. A month's supply at 5 mg./day costs about $6 U.S. THE THEORY: While searching Medline for "steroid? and neuron?" I saw some papers on "neurosteroids". Neurosteroids (pregnenalone, progesterone, dehydroepiandrosterone) are created in glial cells in the CNS. They and their metabolites have neuroprotective qualities. This includes protection against glutamate toxicity (2,3,4), which has been implicated in ALS. Progesterone inhibits the stimulatory effects of the neurotransmitter glutamate, and modulates the responsiveness of GABA receptors(2). Any or all of these may explain the results. Insomnia is common in ALS, but in what I've read it's usually attributed to inactivity. But if neurosteroids play a part in sleep cycles, there may be a connection. Steroids have long been known for their anesthetic effects. Insomnia is a logical, though unproven, result of a CNS bathed in excess excitatory amino acids. take care, Wayne 1. Topiramate Approved for Epilepsy Reprinted from the January/February 1997 issue of Medical Sciences Bulletin [Med Sci Bull. 1997; 20(1)] The FDA has approved a novel antiepileptic agent - topiramate (Topamax/ Ortho-McNeil)-for the adjunctive treatment of adults with partial-onset seizures. Topiramate was identified by scientists at the National Institutes of Health during random screening of promising drug candidates, and was developed by the R.W. Johnson Pharmaceutical Research Institute. The drug blocks voltage-sensitive sodium channels, enhances the activity of the inhibitory neurotransmitter gamma-aminobutyric acid (GABA), and blocks the action of the excitatory neurotransmitter glutamate. It also inhibits carbonic anhydrase, although this may not contribute to anticonvulsant activity. 2. Progesterone administration attenuates excitatory amino acid responses of cerebellar Purkinje cells. Smith SS, Department of Anatomy, Hahnemann University, Philadelphia, PA 19102. Neuroscience 1991, 42 (2) p309-20 We have previously shown that the sex steroid progesterone plays a modulatory role in amino acid physiology by suppressing excitatory responses of cerebellar Purkinje cells to glutamate and augmenting inhibitory responses of these neurons to GABA. In the present study using the rat, progesterone effects on neuronal responses to the specific excitatory amino acid agonists quisqualate, kainate and N-methyl-D- aspartate were tested using iontophoretic, extracellular single unit recording techniques. In addition, the effect of systemic administration of progesterone on quisqualate-evoked excitation was evaluated in the presence of the GABAA blocker bicuculline. Progesterone consistently attenuated excitatory neuronal responses to local application of all three excitatory amino acids by 40-51%, but exerted variable effects on combined administration of quisqualate and N-methyl-D-aspartate which were dependent on temporal and dose-related factors. Progesterone- induced attenuation of the quisqualate response was found to be mediated primarily by a non-N-methyl-D-aspartate receptor. In addition, bicuculline application did not block progesterone effects on quisqualate excitation, suggesting that the observed steroidal modulation of excitatory amino acid function is not secondary to progesterone-induced potentiation of GABA inhibition. 3. Steroid hormones protect spinal cord neurons from glutamate toxicity. Ogata T; Nakamura Y; Tsuji K; Shibata T; Kataoka K Department of Physiology, Ehime University, School of Medicine, Japan. Neuroscience (ENGLAND) Jul 1993, 55 (2) p445-9 The effects of steroid hormones on glutamate neurotoxicity were examined in cultured spinal cord neurons. The extent of neuronal damage, produced by glutamate exposure for 15 min, was estimated based on the activity of lactate dehydrogenase released from degenerated neurons to the media during 24 h of post-exposure incubation. This damage was dependent on the glutamate concentrations used. The addition of dexamethasone, a synthetic steroid, in post-exposure media remarkably reduced the extent of damage in a dose-dependent manner. The half effective concentration for the steroid was approximately 0.7 microM, which was in the range of pharmacological concentration. Dexamethasone was effective even when it was added 2 h after glutamate exposure. Some endogenous steroid hormones --aldosterone, progesterone and testosterone --also showed similar neuroprotective effects. However, cholesterol, a precursor of these steroid hormones, had no effect on glutamate neurotoxicity. This direct protective effect on neurons against glutamate neurotoxicity may explain, at least partly, the mechanisms of beneficial effects of steroid hormones on in vivo spinal cord injury. 4. Progesterone alters GABA and glutamate responsiveness: a possible mechanism for its anxiolytic action. Smith SS; Waterhouse BD; Chapin JK; Woodward DJ Brain Res Jan 6 1987, 400 (2) p353-9 (6) ===== Longaberger Basket Fund Raiser ========== >From : "Richard Katucki" Subject: Longaberger Basket Fund Raiser Date : Thu, 22 Mar 2001 For those of you who are Longaberger fans or are looking for an unusual gift and at the same time helping fund ALS research, here is an opportunity. My daughter Laura, runs a fund raiser for the Philadelphia ALSA chapter, each March. Her brother Christopher has had this disease for over five years. As a Longaberger consultant, she donates all of her commissions from her March sales to the Philadelphia chapter of the ALS Association. You can order a gift via her web site at www.longaberger.com/laurakatucki . Think mother's day gifts. All proceeds go toward research. Thanks a lot. Keep up the spirit, we will find a cure. The end of March is upon us so if you are interested the time is now. (7) ===== Wheelchair Van For Sale ========== >From : "Bob C." Subject: Wheelchair Van For Sale Date : Mon, 26 Mar 2001 98 Dodge van, lowered floor, power ramp, removable passenger seat with auto wheelchair lockdown. Body excellent and has with 44K. Deceased PALS wanted another PALS to have at bank cost- $21,000 (FIRM). Located on Long Island 631.474.3035 or LINUMOM@aol.com (8) ===== Medicare confusion ========== >From : "Maury Pratt" Subject: Medicare confusion ... Date : Thu, 15 Mar 2001 I am confused about the Medicare ruling that is to "kick in" this summer. I am 63 and thought I would be covered. It seems I won't be covered because I am not on SSDI. I thought it was for all of us with ALS. Could someone explain. Thanks, Dawn Pratt (9) ===== re: vent? ========== Date : Sun, 25 Mar 2001 >From : Wayne Phillips Subject: vent? Hi "hacker" (don't know your name), See my web site for lots of stories from PALS about life on a vent and other ALS issues. Wayne S. Phillips wsphillips@compuserve.com http://www.redrival.com/wsphillips/tpals.htm (10) ===== need info on speaking device ========== >From : "Leonard Brunner" Subject: Info. on Speaking Device Date : Mon, 26 Mar 2001 I am looking for something to help me with speaking.. Seen WORDS+ has MessageMates for talking but not sure how good it is. If anyone can help please e-mail me at: Leob@1st.net Thanks, Leonard Brunner (11) ===== re: Vent? (ALSD 825) ========== Date : Sun, 25 Mar 2001 >From : "Edward Anthony Oppenheimer, MD" Subject: RE: Vent? If your recent FVC is 30% you certainly need to immediately discuss arranging mechanical ventilation with your physician or pulmonary specialist. You can expect to stay mobile with this assistive equipment. Over ten years ago physicians waited to advise considering mechanical ventilation for people with ALS until the FVC decreased to 30% and for arterial blood gas values to be abnormal while awake. We now know that this is very risky and often results in death or emergency hospitalization with pneumonia, loss of consciousness and severe respiratory failure requiring difficult treatment in intensive care. There are enough published studies to support recommending that Mechanical ventilation should be started when the FVC reaches 50% or when there are abnormalities of MIF and MEF, or decreases of oxygen saturation during sleep. This is the official position of the American Academy of Neurology; and the Medicare guidelines for starting respiratory assistive devices for ALS also support this. Many people with ALS are hesitant to make the decision. However if you are able to start with nasal mask noninvasive ventilation you can easily develop experience and make further decisions (to continue or to stop) based on hands-on experience. Many people with ALS can use the ventilator during the night and have many hours of free time off the ventilator during the day (at least in the beginning). These are small portable ventilators designed to allow people to get out of the house, to work, to friends, and also for travel. Thus mobility is part of the goal. The alternative is of course a "911" emergency or not surviving. If you want to continue living you should waste no time, and arrange for assisted ventilation without delay. All best wishes, Edward Anthony Oppenheimer, MD, FCCP Pulmonary Medicine, Los Angeles === end of alsd 827 ===