Date: Sun, 3 Sep 95 15:56:38 -0400 From: Bob Broedel To: als@huey.met.fsu.edu Subject: ALSD213 ALS-ON-LINE =============================================================== == == == ----------- ALS Interest Group ----------- == == ALS Digest (#213, 03 September 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 == == 960+ 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 .. Trach vs other options for PALS 2 .. Claims of "healer" leave long trail of skepticism 3 .. Cal Ripken, the Baltimore Orioles, and ALS fundraising (1) ===== Trach vs other options for PALS ========== Date : Sat, 2 Sep 1995 17:30:36 -0700 To : dick.tauber@turner.com >From : eaopp@ucla.edu (Edward Anthony Oppenheimer, M.D.) Subject: Trach vs other options for PALS Cc : bro@huey.met.fsu.edu The question you ask requires careful consideration, with the advice of your physicians, and other physicians (if needed) who have experience with home mechanical ventilation. The opportunity to use noninvasive mechanical ventilation often gives hands-on experience that allows a person to decide whether to: [A] stop mechanical ventilation and use palliative care; [B] stick with noninvasive ventilation as long as possible but not go on to tracheostomy if bulbar problems become severe; or... [C] use noninvasive ventilation but accept tracheostomy when necessary. It is often very worthwhile to speak to other people with ALS or other neuromuscular diseases who use home mechanical ventilation - to get their viewpoint and advice, and to talk to members of their family to find out about the caregiving aspects. There are support groups that you can contact, such as the local ALS Assoc. chapter, a post-polio support group, or the International Ventilator Users Network (314-361-0475) may be able to link you up with someone near you. The book (1994) edited by Drs. Hiroshi Mitsumoto and Forbes Norris: "Amyotrophic Lateral Sclerosis - A comprehensive guide"; Demos Publications, New York ($40) is a very useful guide to review. Demos Publications; 386 Park Avenue South, Suite 201; New York, New York 10016. In the last 5+ years it has become clear that many people with ALS (PALS) can do well for long periods of time with nasal mechanical ventilation, using a home ventilator such as those made by Aequitron, Lifecare, Puritan-Bennett, and Respironics (BiPAP S/T). As long as it works well and the problems with bulbar impairment (such as aspiration) do not become severe, nasal mechanical ventilation can continue to work up to 24 hours/day for many people. Only your wife can judge the quality of her life, and whether it would be acceptable to her if she continued living with progressive loss of limb function and possibly bulbar impairment. She could (like Professor Stephen Hawkings) be up and around (even with a small portable home mechanical ventilator), and if necessary use other devices for communication and nutrition. If she is highly motivated and engaged in life, she may wish to do this. Many people with ALS, other neuromuscular diseases and COPD have used tracheostomy home mechanical ventilation over the last 30+ years. They usually report that they are satisfied with the quality of their life and would make the same choice again. If someone requires complete care 24-hours/day then the family members or others (such as paid attendants) must be available and must be able to work out a reasonable life too since this care may last many years when done properly. This requires resources, and good experienced advice. Tracheostomy like other surgery has some post-operative discomfort over a few days but then heals and is well tolerated. There are care problems that usually work out well for most people that need to be reviewed with the physicians (and ENT specialist). Speech and swallowing, if otherwise possible, should be possible with a tracheostomy too. A speech-language pathologist (SLP) who is experienced with PALS using tracheostomy and home mechanical ventilation, may be helpful with this. Your said that "I've heard that a feeding tube can only be in for a limited period of time. Is this the case?" No, a gastrostomy tube can be used for years. It can be placed with a minor medical procedure by a GI specialist, usually only requiring 3-8 hours at the hospital during the day. It is often referred to as a "PEG" (percutaneous endocopic gastrostomy). Liquid nutrients and medications and fluids are given through the gastrostomy tube three to four times a day. It is out of site when not being used. Some people take some food and liquid by mouth, if that is safe, and take the rest via the gastrostomy tube (as long as some oral intake is possible and desired). On the other hand, most people with ALS do not decide to use long-term mechanical ventilation. They accept that life will end at some point, and prefer to let this occur before they become very impaired and possibly a burden to others. They decide to use medication instead when they become short of breath, knowing that they will forgoe extending their life; desiring symptom relief and accepting that life will end with respiratory failure. Morphine and similar medication are very helpful in the correct dose to relieve distress. Today, in the USA, a competent adult, and anyone who has completed a DPAHC (durable power of attorney for health care) can stop mechanical ventilation when/if they decide they no longer wish to use it. And their appointed health care agent can act for them if needed, and if this is their desire under certain circumstances (such as complete loss of ability to communicate). A word of caution... [1] There are many professionals who have had little experience with these issues, and care alternatives. Sometimes professionals have a very fixed viewpoint because they have had no experience with the modern options of home mechanical ventilation for PALS. And, often it is hard to arrange the needed resources. [2] Without necessary resources it can be a very burdensome life for family caregivers. Some people are able to hire attendants to help with the 24 hour (7 day/week) caregiving; others have been able to organize a network of volunteers to help from their friends, church groups, and others. It is very important to work these issues out before making a final decision. A medical social worker is sometimes very helpful. <><><><><><><> TEXT OF PART OF YOUR MESSAGE BELOW <><><><><><><> Date : Thur., 31 Aug 1995 >From : dick.tauber@turner.com Subject: Should my wife have a tracheostomy? My wife, Marjorie Hirschberg is now bedridden with ALS. She lost the use of her legs last January, and after being stable for several months, began loosing use of her right arm along with stressful breathing at the end of May. Today she has totally lost the use of her right arm and hand and has just minimal use of her left arm. She cannot write or feed herself. On the 4th of July we went out to the Mayo Clinic where ALS was confirmed. ...Since returning to Atlanta in the middle of July, her condition has worsened. She has been on a B-Stat P forced air machine since 7-21-95. At first she only used it during the night, and on occassion during the day. She can no longer breath without the machine. She can still talk while wearing the nose mask (she switches off with a full nose and mouth mask) to get her air, and can be understood 85% of the time. A pulmonologist has offered the possibility of a tracheostomy that would make her lungs work, and keep them working no matter how advanced her condition becomes. He has also suggested that a feeding tube be inserted at the same time to insure proper nourishment. .... ....Here is were we need some help and as soon as possible: 1. Is there anyone familiar with supporting a patient with a trach who is also as far along as Marjorie? What can we expect things to be like in the future? == INFORMATION IS AVAILABLE. SUPPORT GROUPS AND PEOPLE USING A TRACH MAY == BE HELPFUL SOURCES, IN ADDITION TO YOUR PHYSICIANS. 2. I've heard that there is much discomfort with a trach. Is this so? Or does it become so? Or does it truly better the life of the patient. == THE DISCOMFORT HEALS QUICKLY. PEOPLE VARY AS TO THEIR ASSESSMENT OF == THEIR QUALITY OF LIFE, WITH OR WITHOUT TYRACHEOSTOMY. 3. I've heard that a feeding tube can only be in for a limited period of time. Is this the case? == NO 4. While some patients can effect talking by controling the venting of the air flow, Marjorie will not be able to do this. Is there any other chance of talking, or at least communicating while the trach is in place? == YES 5. Since ALS effects the voluntary muscles, are there any muscles or muscle groups that are spared, such as neck or facial muscles. How about the eyes or eyebrows? Are there other ways of indicating "yes" or "no"? == SOME PEOPLE NEVER GET BULBAR INVOLVEMENT, MOST PALS CAN ALWAYS SIGNAL == BASIC RESPONSES. COMPUTER BASED COMMUNICATION IS USEFUL WHEN SPEECH IS NOT == POSSIBLE. 10% to 15% OF PALS USING LONG-TERM VENTILATION LOSE ALL ABILITY == TO COMMUNICATE (BECOME LOCKED-IN); MANY WOULD WANT TO STOP THE VENTILATOR == IF THEY EVER BECOME PERMANENTLY LOCKED-IN OR COMATOSE. 6. How have patients with advanced ALS done while on a trach? == REPORTS FROM THOSE USING TRACH + HOME VENTILATION ARE THAT PALS ARE == SATISFIED. THERE ARE A NUMBER OF STUDIES THAT CONFIRM THIS. 7. If all muscles fail, how can an advanced patient let you know they have decided it's time to turn off the machines? == IT IS BEST TO DISCUSS THIS IN ADVANCE AND AGREE ON A STRATEGY, AND OBTAIN == THE ASSISTANCE AND AGREEMENT OF YOUR DOCTOR TOO. IT IS LEGAL AND ETHICAL == TO STOP ANY TREATMENT INCLUDING A VENTILATOR IF THIS IS NO LONGER == ACCEPTABLE (DESIRED). THE LAW SUPPORTS THIS AS A "PATIENT'S RIGHT" THAT == MUST BE RESPECTED. Marjorie is 52 years old. Her birthday is at the end of November. We have two children -- a 17 year son, a senior in high school; an 11 year old daughter in sixth grade. We've been married 22 years. I hope some folks out there can offer some helpful advise to the questions above, and to those questions Marjorie and I may not have thought of yet. Thanks for any help you can give us. dick.tauber@turner.com <><><><><><><> TEXT OF A HANDOUT BELOW <><><><><><><> "Its Your Choice" ~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~ Essential Points for patients and their families: Making an informed decision about whether to use a ventilator requires time, thought, and careful evaluation of the facts. In order to have choice you must have good information. You should know: <> Your condition and prognosis. <> Your options - and the pros and cons of each. <> The resources you will need for each alternative. <> Which resources are available to you: financial and psychosocial. <> People and families who can share their experience with you. <> The impact each alternative will have on both you and your family. Your family need to be partners in decision making. <> Information about decision making, advance directives, proxy decision making, and other ways you can ensure that your decisions will be known, and that your wishes will be carried out. And=8A <> Whether a trial using some equipment at home might be useful. Once you have gathered the information you need, and have evaluated the resources available to you, you should develop a plan, a strategy: <> Include your family, and other people who are important to you, in your decision making process. <> Seek advice from professionals who have experience working with people who use a ventilator at home. <> Consider whether using some equipment at home on a trial basis might be helpful. <> Do a careful evaluation - don't base your decision on initial emotional reactions. <> Avoid denial and procrastination. <> Talk to other people with similar medical problems who are using a ventilator at home or have already tried options you are considering. Such people, and their families, can often provide the best information. Remember, other people like you already have experience with this. Be sure to talk to them because they can often provide the best information. It's your choice: make your decision an informed and thoughtful one. - The one that's best for you. <> The video "Its Your Choice" is helpful to use in conjunction with the manual. It may be obtained from Valona Productions; 5105 East Los Angeles Avenue - Suite E-178; Simi Valley, CA 93063; (805) 527-8557. For a copy, send your request with a check for $20. The video was produced by Ismail Tsieprati, a ventilator user, and his wife Cheryl. <><><><><><><><><><><><><><><><><><><><><><><><><><><><><><> I hope that this is helpful. There are other regular contributors to this OnLine forum who have had considerable experience with home mechanical ventilation for people with ALS, both using nasal mechanical ventilation and also via tracheostomy. Regards, Edward Anthony Oppenheimer, M.D. Pulmonary & Critical Care Medicine Southern California Permanente Medical Group 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) ===== Claims of "healer" leave long trail of skepticism ========== TITLE : Claims of `healer' leave long trail of skepticism BYLINE: Nicole Parton CREDIT: VANCOUVER SUN DATE : 08/23/95 In promotional literature that targets the desperate and gravely ill, a North Vancouver man who calls himself a ``metabolic therapist'' brags about his lack of formal medical training. ``Frank Ludde's greatest asset is that he has no medical background, there- fore, he has not been indoctrinated and is not obliged by the bylaws of any college to maintain the standards of a profit-driven industry,'' the literature says. ``Frank Ludde's only concern is the health of mankind.'' But a two-week Vancouver Sun investigation revealed a host of concerns about Ludde's unproven treatments, exorbitant fees and claims of ``cures.'' Ludde ignored The Sun's request to support his written claim that his 14-week program ``has been proven to heal ALS (amyotrophic lateral sclerosis), MS (multiple sclerosis), Parkinson's, muscular dystrophy, Alzheimer's, arthritis, anorexia, manic depression, CFIDS, cancer and leukemia.'' The B.C. Medical Association says it is unaware of any disease known as CFIDS. Ludde also did not respond when The Sun asked to what those initials refer. Among the other concerns revealed by The Sun's investigation: A June 1994 press release issued by Ludde's Global Right to Health Society states the society ``announced today a cure for amyotrophic lateral sclerosis (ALS).'' The release hails the society's ``most recent success,'' Carl (Kip) Peterson, who claims Ludde ``healed'' his ALS. < parts deleted > Following an intensive search, The Sun located Peterson in Boulder, Colo., where he is a resident of the Boulder Manor Progressive Care Center, a nursing home. Speaking with extreme difficulty, Peterson fell silent when The Sun asked if his ALS was indeed cured. Peterson then gave the telephone to an attendant who said his dinner tray had arrived and he would answer questions in the early evening. He did not return The Sun's subsequent telephone messages. Ludde's method of connecting with clients is ingenious. Either Peterson or someone using his name writes testimonial letters to the editors of community newspapers throughout B.C. and the U.S. The letters, which state ``North Vancouver'' under Peterson's name, praise Ludde's work but do not directly solicit clients. Readers who are given Peterson's number by the newspaper in fact reach Ludde's home office. The Vancouver Sun has not published any letters bearing Peterson's or Ludde's names. Peterson's letter states: ``It's scary for me to think about how close I came to dying. If it weren't for my mother's dentist's receptionist's friend's husband whose ALS was healed by this therapy ... It shouldn't have to happen that way. ALS can be cured.'' The letter further states: ``Neither the ALS Association nor the medical community is interested in this therapy because no physician is involved, yet the medical community still claims there's no cause or cure.'' < parts deleted > The Sun also had difficulty with complainants willing to go on the record, in part because Ludde responds to questions by issuing aggressively worded letters. The ALS Society's Eisen is one of those reluctant to speak out: ``I've heard this guy is keen on litigation.'' < parts deleted > Ludde's faxed response to The Sun's faxed questions: ``I don't take orders from anybody.'' < parts deleted > Asked about Peterson's claims to have been cured of ALS, Boulder Manor administrator Gary Walker said federal regulations prohibit him from discussing any patient's condition or diagnosis. Walker said, however, he would cooperate with the RCMP should an investiga- tion ensue. RCMP Sgt. Peter Montague confirmed this week the RCMP suspended its investigation into Ludde's practice several months ago because there were few complaints and they were not substantantive enough to pursue. < parts deleted > Ludde's claim to have an ALS ``cure'' also concerns Linda McKnight of Isaquah, Wash. The founder of a national ALS information and research centre, McKnight says: ``There is nothing available that cures ALS at this point.'' McKnight also says Ludde contacted her last summer to offer her a ``discount'' if she sent him groups of ALS patients. At that time, she says, Ludde quoted his per-patient rate at $50,000 US. ``I understand he's since lowered it,'' she says. < parts deleted > (3) ===== Cal Ripken, the Baltimore Orioles, and ALS fundraising ========== DATE : 08/23/95 SOURCE: The Salt Lake Tribune BALTIMORE -- The battle against the disease that killed Lou Gehrig will not be forgotten on the night Cal Ripken breaks the Hall of Famer's record for consecutive games played. Gehrig died in 1941 of a degenerative disease that slowly destroyed his spine and nerve cells. Now, the Baltimore Orioles, in honor of Ripken's 2,131st consecutive game, plan to raise $1 million by selling seats -- for $5,000 each -- on the edge of the playing field Sept. 6. The money will be used to set up a foundation at Johns Hopkins University for research on neuromuscular diseases, including amyotrophic lateral sclerosis, commonly called Lou Gehrig's disease. === end of als 213 ===