Date: Thu, 3 Aug 95 22:43:40 -0400 From: Bob Broedel To: als@huey.met.fsu.edu Subject: ALSD205 ALS-ON-LINE =============================================================== == == == ----------- ALS Interest Group ----------- == == ALS Digest (#205, 03 August 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 == == 870+ 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 == =============================================================== CONTENTS OF THIS ISSUE: 1 .. re: ALS => bulbar? 2 .. re: fenestrated tracheostomy - ALS 3 .. re: Appel scale 4 .. re: Intelligence and ALS 5 .. Help with diagnosis 6 .. moderator's advisory (from the Neuromuscular Research list) 7 .. cancer and ALS 8 .. Riluzole / Diagnostic Confirmation Centers (1) ===== re: ALS => bulbar? ========== Date : Sat, 29 Jul 1995 15:55:12 -0700 To : rapappas@sherwin.com >From : eaopp@ucla.edu (Edward Anthony Oppenheimer, M.D.) Subject: ALS => bulbar ? Cc : bro@huey.met.fsu.edu Dear Roseann Pappas, You inquired: "If ALS symptoms begin with weakness in the limbs, will it eventually progress to the bulbar area." About 20% of people with ALS do not develop significant bulbar involvement. The absence of bulbar impairment allows speech swallowing, and if a mechanical ventilation is ever needed and desired - then a noninvasive approach without tracheostomy can be tried at home. A good manual regarding ALS was published in 1994: Amyotrophic Lateral Sclerosis, A Comprehensive Guide to Management; edited by Hiroshi Mitsumoto MD; Demos Publications (386 Park Avenue South, Suite 201; New York, New York 10016, TEL 212-683-0072), softcover $40. With best wishes, Edward Anthony Oppenheimer, M.D. Edward Anthony Oppenheimer, M.D Pulmonary & Critical Care Medicine 4950 Sunset Boulevard Los Angeles, CA 90027-5822 (213) 667-7106 office; FAX: (213) 667-5725 Internet: eaopp@ucla.edu or eoppenheim@kpscal.org (2) ===== re: fenestrated tracheostomy - ALS ========== Date : Sat, 29 Jul 1995 15:55:19 -0700 To : ecksteinn@alpha.montclair.edu >From : eaopp@ucla.edu (Edward Anthony Oppenheimer, M.D.) Subject: fenestrated tracheostomy - ALS Cc : bro@huey.met.fsu.edu To: Craig and Norman Eckstein Your letter published in ALS Digest #204 noted: " ... Our father's determination to live resulted in his decision to opt for a feeding tube inserted in his stomach and mechanical ventilation ... we have two ventilators, two suction machines a feeding pump, and a back-up oxygen tank ... we have encouraged our father to speak with his natural voice by connecting a Passy-muir valve to his disposable cuffed fenestrated tracheotomy tube. ... Our father is a champion for overcoming these adversaries. ..." Your father exemplifies a group of about 10-20% of people with ALS who use long-term mechanical ventilation. Some are in nursing homes, but those who can live at home and get out into the community are usually the happiest and most satisfied with their life. As ALS progresses a person can do less and less self care; and, if on mechanical ventilation 24 hours per day, then someone needs to be there to help 24 hours/day. It is usually best if family members can find enough caring helpers to assist, so that no one has to devote all their time to caregiving, so that everyone can get enough rest and have some personal activities too. This may be possible to arrange with a good group of family and friends. Often it may be worthwhile to select and train a reliable personal attendant to assist for 8-16 hours/day so that the burden on other family members is less (if resources permit). The use of a Passy-Muir valve is very helpful to assist with speech, and can help with swallowing too. If bulbar impairment progresses glottal and vocal cord function may result in impaired speech and swallowing problems. Then other methods to assist communication and swallowing need to be considered. There are very good computer-based communication devices available, and often an experienced speech pathologist can help with AAC, if needed. The choice of tracheostomy, if one is needed for mechanical ventilation, depends on specific individual issues. Fenestrated tracheostomy tubes have been used in the past, but more and more we avoid these because they seem to result in more tracheal irritation and "granulations" which block the airway and bleed and make trach changes more difficult. If a person can talk, then usually a cuffless tracheostomy tube (non-fenestrated) works well (with a Passy-Muir valve). If speech is not possible (due to bulbar and vocal cord impairment) a cuffed tracheostomy tube may be best if aspiration is a major problem (however an uncuffed tube is the easiest to change and has many advantages, so we use these if at all possible). Your parents sound like very loving and courageous people. And your father's choice to continue living, even though disabled, appears to have been a satisfying choice for him. He can always decide to stop mechanical ventilation if life becomes unacceptable, or if he ever becomes "locked-in" (unable to communicate at all). In fact, most people prefer to make a clear record of their preferences, and most would not want the ventilator continued if they became permanently comatose (not something that happens due to ALS) or "locked-in". You said: "we feel partially guilty for allowing our father to live..." - however this is his choice, even if you might make another choice in his situation (it is hard for "temporarily non-disabled" people to know what choice they will make in this situation). You should not feel guilty, even though you would wish the burdens were lifted. It is much more reasonable to feel a sense of extreme discomfort when a crisis occurs, and a family member has to make a life or death decision for a loved one who has not indicated preferences in advance. When a family member can review the options and make a choice, for example - whether to use mechanical ventilation, with the assistance of experienced MDs and other health care professionals (and hopefully with peer counseling too) - this is the best decision making. Sometimes when bulbar problems are not present, or not severe, people with ALS can try noninvasive mechanical ventilation at home as a trial to assist breathing with a nasal mask or nasal pillows interface to the ventilator, as a trial. You have helped a great deal by your support, your assistance so that he can speak better with the Passy-Muir valve, and can be mobile out of his home. Many people with ALS on a ventilator have not realized that these are possible. I hope that his life continues to be satisfying from his point of view, and that your mother has enough help. With all best wishes, Edward Anthony Oppenheimer, M.D. Edward Anthony Oppenheimer, M.D Pulmonary & Critical Care Medicine 4950 Sunset Boulevard Los Angeles, CA 90027-5822 (213) 667-7106 office; FAX: (213) 667-5725 Internet: eaopp@ucla.edu or eoppenheim@kpscal.org (3) ===== re: Appel scale ========== >From : dendrite@dsp.com Subject: Appel scale Date : Fri, 28 Jul 95 11:54:53 EST Robert Macdonald writes asking how to get a copy of the Appel ALS scale: The reference is: Appel, V., et al (1987) A rating scale for amyotrophic lateral sclerosis: description and preliminary experience. Annals of Neurology 22: 328-333. D. Eric Collins, MD Oakland, CA (4) ===== re: Intelligence and ALS ========== Date : Fri, 28 Jul 1995 13:51:48 +0100 >From : spgtbjg@ucl.ac.uk (Barry Gibb) Subject: Intelligence and ALS Date : Thu, 13 Jul 1995 15:47:00 EST >From : "Dianne Granger (803) 250-8135" Subject: Question I am curious about the relationship between intelligence and the incidence of ALS. My husband was an electrical engineer. In our support group we had a professor, command pilot, designer, and professional engineer. I also know of a minister that had ALS. Thanks! Dianne Granger Morris Dear Ms Morris In my reading of the literature, I have come across no evidence of a link between incidence of ALS and intelligence. It would be of interest to me, however, to know whether higher brain functions, eg reasoning, are in any way affected by ALS. (5) ===== Help with diagnosis ========== Date : Fri, 28 Jul 1995 13:34:39 -0400 (EDT) >From : Andrew Malison Subject: Help with diagnosis Below is a note that a friend, Lou Giacchino, has asked me to post with the hope of coming closer to diagnosing a disease afflicting his son. Lou and his family have unsuccessfully sought for the past four years such a diagnosis through conventional channels including NIH. If you have encountered a similar case or otherwise have any clues or suggestion for Lou or me to pursue, please call him directly in Maryland, United States by phone at (301) 434-9073 or by fax at (301) 434 0787. I will also be passing messages to him sent to me on this matter via e-mail at: amalison@cap.gwu.edu. INTERNET NOTICE - URGENT HELP NEEDED WITH MEDICAL DIAGNOSIS My son, Jon, age 37, lies in a nursing home in Silver Spring, Maryland, immobilized by a disease which we have not been able to diagnose. He has lost all control of motor functions, including speech, cannot feed himself, and is incontinent. Help is needed to give some orientation to research leading to a proper diagnosis of his affliction. The facts of the case, summarized on the occasion of a recent brain biopsy at the Johns Hopkins Hospital in Baltimore, are as follows: Jon is a 37-year old male whose symptons began approximately four years ago. Before that he had been healthy and athletic, achieving a high school education and serving six years in the U. S. Navy. His symptons began with some vague tightness in both lower extremities, progressing to a spastic gait. The family noticed some subtle but progressive deterioration in this attention, concentration, and speech content. Workup evaluation began at NIH in August 1992, at which time his spastic gait was documented and his condition categorized as Multiple Sclerosis. Several studies including serologic studies, HIV, HTLV, RPR, B12, Lyme titers, and MRI scans were negative. Cortical function testing in 1993 showed grossly abnormal effects with borderline mental retardation in all parameters. Lumbar puncture in late 1994 was unremarkable and significant for negative VDRL and negative IgG index. Evaluation for metabolic disease as a child was also negative. Repeat MRI scan in February 1995 did reveal abnormal signal intensity in the subcortical white matter areas in the frontotemporal area. At that time evoke potential results were also abnormal. A brain biopsy specimen was taken on 5/31/95. The pathologist reported the cores of tissue as slightly hypercellular, but noted that changes were minimal and did not permit a diagnosis of glioma. No cycling cells were seen with the staining for Ki-67/MIB - 1. As a result of this biopsy, the neurologist in charge stated that there was no basis on which to classify Jon's condition as multiple sclerosis, a tumor, or "any other neurologic condition." He went on to state that since Jon's condition remains undiagnosed, he could not recommend any treatment The final blow in this report lies in the last sentence: "I think that you have exhausted the avenues for finding out what has caused his neurological decline and am sorry that we have not been able to offer more help." Needless to say, we are not comfortable with this assertion. We want to continue to try and get the root of Jon's problem and, if possible, do something about it. My name is Louis Giacchino. I can be reached at home at 301-434-9073. End of message. Andrew F. Malison amalison@cap.gwu.edu (6) ===== moderator's advisory (from the Neuromuscular Research list) ========== Date : Mon, 31 Jul 1995 20:46:39 -0500 Sender : SJU Neuromuscular Research and Information List : >From : "A. Messaros" Subject : moderator's advisory NeuroMus subscribers, Again, in light of recent postings requesting medical advise/diagnosis for personal health problems, we're reminding you that: 1) such postings are inconsistent with the mission of NeuroMus 2) individuals engaging in advise/diagnosis put themselves at risk for malpractice suits or other legal action. Legal counsel at the University of Iowa has advised that this be made explicitly clear to subscribers. Andy Messaros Roger Enoka NeuroMus list moderators Andy Messaros (andrew_messaros@uiowa.edu) (319-335-9795) Physical Therapy; The University of Iowa - USA (7) ===== cancer and ALS ========== Date : 31 Jul 95 12:00:01 EDT >From : Anne Butterfield <73740.1543@compuserve.com> Subject: ALSD204 ALS-ON-LINE My husband, David, joined the Digest as soon as he was diagnosed with ALS and we learned of the Digest. That was in early 1993. His symptoms began to reveal themselves in the fall of 1992, a few months after his 50th birthday. They began with speech that was slightly slurred when he was fatigued or had had a glass or two of wine. Clumsiness manifested itself particularly during his regular weekly volley ball games -- a sport he had always played well. Fortunately, we live in medical Mecca and were able to consult with Munsat, Sheffner and people at Mass General. David was accepted into Sheffner's CNTF trial in the late spring of 1993. A routine X-ray showed an egg-sized tumor in his right lung. It proved to be lung cancer and David was bounced from the trial and we began an aggressive multi-modal experimental cancer treatment. It was not successful, and in June of 1994 David died, essentially of the cancer. There were several odd things about David's case. One was that the oncologists and thoracic cancer team were continually puzzled by the fact that David had had absolutely no symptoms normally associated with a cancer of that size and progression. Another was that, basically, David's decline from ALS appeared to stop immediately after the chemo treatments began. He lost not one bit of additional physical strength for essentially a whole year. It is a bit more difficult to tell about his speech as his vocal chords were nicked in the lung removal surgery, and while repairs were made, his voice never recovered to much above a whisper. Oat cell cancer sometimes mimics the symptoms of ALS. David's cancer was squamous cell cancer which we were told never mimics ALS. However, I am now wondering if David had cancer all along and never had ALS at all. Not only does the coincidence of having these two terrible diseases at one time seem stretching it, but the lack of cancer symptoms and the seemingly halting of ALS progression makes me wonder. Does anyone know if there has been research about these possible links or if there are other similar cases? I will stay on the Digest for a little while longer, at least, as we found it to be a very special resource and to give us a sense of community around the thing that had absorbed our lives. If any of you have any insights or information on my questions concerning the cancer and the ALS, I would appreciate hearing from you. Anne Butterfield 73740.1543@CompuServe.com (8) ===== Riluzole / Diagnostic Confirmation Centers ========== Note: The following represents the current list of Diagnostic Confirmation Centers (DCCs) providing patients with confirmation of their ALS diagnoses. Up to 78 centers are expected to participate. To update this list with centers added after the release date, please call 1-800-RX-TRIAL (1-800-798- 7425), Monday through Friday from 9AM to 6PM Eastern Time. DIAGNOSTIC CONFIRMATION CENTERS (DCC's) ALABAMA University of Alabama Birmingham, AL 35294-0007 ARKANSAS University of Arkansas of Med. Sci. 4301 W. Markham/Slot No. 500 Little Rock, AR 72205 ARIZONA Mayo Clinic 13400 East Shea Blvd. Scottsdale, AZ 85259 University of Arizona Med. Ctr. 1501 N. Campbell Avenue Tucson, AZ 85724 CALIFORNIA University of California California Pacific Medical Center 3698 California Street, Room 545 San Francisco, CA 94118 COLORADO University of Colorado Health Science Center 4200 E. Ninth Ave. Denver, CO 80262 CONNECTICUT University of Connecticut Health Center 263 Farmington Ave Farmington, CT 06030 Yale University School of Medicine P.O. Box 20818 New Haven, CT 06520-8018 DISTRICT OF COLUMBIA George Washington University Med. Ctr. 2150 Pennsylvania Avenue, NW Washington, D.C. 20037 FLORIDA University of Miami School of Medicine 1150 NW 14th Street Miami, FL 33136 GEORGIA Emory University 2040 Ridgewood Drive, N.E. Atlanta, GA 30322 ILLINOIS Rush Presbyterian-St. Luke's Medical Center 710 South Paulina - 8N Chicago, IL 60612 University of Chicago 5841 S. Maryland Avenue/BH, Box 425 Chicago, IL 60637 Northwestern Medical Faculty Foundation 645 N. Michigan Avenue Chicago, IL 60611 University of Illinoi Hospital & Clinics 912 South Wood Street Chicago, IL 60612-7330 INDIANA Indiana University Hospitals 545 Barnhill Drive Indianapolis, IN 46202 IOWA University of Iowa Hospitals 200 Hawkins Drive Iowa City, IO 52242 KENTUCKY University of Kentucky School of Medicine 820 S. Limestone Street Lexington, KY 40536-0226 LOUISIANA Louisiana State University Medical Center 1542 Tulane Avenue New Orleans, LA 70112 MARYLAND John Hopkins School of Medicine 600 N. Wolfe Street, Meyer 6-109 Baltimore, MD 21287 MASSACHUSETTS Massachusetts General Hospital 15 Parkman Street Boston, MA 02114 Boston University Medical Center 80 E. Concord St. Boston, MA 02118 New England Medical Center 750 Washington Street NEMC 314 Boston, MA 02111 MICHIGAN University of Michigan Medical Center 1500 E. Medical Center Drive Ann Arbor, MI 48109-0316 Henry Ford Hospital 2799 West Grand Avenue { Detroit, MI 48202 MINNESOTA University of Minnesota, Box 295 420 Delaware Steet, S.E. Minneapolis, MN 55455 MISSOURI Mid Missouri Mental Health Clinic 3 Hospital Drive Columbia, MO 65201 Washington University 216 South Kingshighway St. Louis, MO 63110 NEW YORK Strong Memorial Hospital/Univ. of Rochester 601 Elmwood Avenue Rochester, NY 14642 Mount Sinai Medical Center 100th Street/Fifth Avenue New York, NY 10029 OHIO University of Cincinnati 231 Bethesda Avenue Cincinnati, OH 45267 Ohio State University 1654 Upham Drive Columbus, OH 43210 Cleveland Clinic Foundation 9500 Euclid Avenue Cleveland, OH 44106 PENNSYLVANIA Hospital of University of Pennsylvania 3400 Spruce Street Philadelphia, PA 19104 Thomas Jefferson University Hospital 111 Soth 11th Street Philadelpha, PA 19107 Hahnemann University Hospital 1427 Vine Street Philadelphia, PA 19102 University of Pittsburgh/322 Scaife Hall 3550 Terrace Street Pittsburgh, PA 15261 RHODE ISLAND Rhode Island Hospital 110 Lockwood Avenue Providence, RI 02903 TENNESSEE Baptist Memorial Avenue 1211 Union Avenue Suite 400 Memphis, TN 38104 Vandebilt University Medical Center 2100 Pierce Avenue Nashville, TN 37212 TEXAS University of TX-Southwestern Medical Center 5323 Harry Hines Blvd. Dallas, TX 75235 Baylor University 6501 Fannin St, Suite NB-302 Houston, TX 77030 UTAH University of Utah Medical Center 50 N. Medical Drive, Room 3-R Salt Lake City, UT 84132 VERMONT University of Vermont School of Medicine 1 South Prospect Street Burlington, VT 05401 VIRGINIA University of Virginia Health Science Center P.O. Box 395 Charlottesville, VA 22908 WEST VIRGINIA West Virginia Univ. Hosp. Health Sci. Center Medical Center Drive Morgantown, WV 26506 WISCONSIN University of Wisconsin 337 Waisman Center 1500 Highland Avenue Madison, WI 53705 CONTACT: Bob Pearson, 610-454-3872, or Rob Partridge, 610-454-3890, both of Rhone-Poulenc Rorer === end of als 205 ===