What follows is the late Dr. D. Eric Livingston's patient's guide to ALS. It is written from the unique perspective of a physician, and one who is afflicted with ALS.

The Guide is written in five parts:

• What is ALS?
• Practical Tips
• Organizations & Resources
• Drugs
• Treatment Centers


ALS: A Guide for Patients

by D. Eric Livingston, M.D.

I am a 38 year old Physician. During a ski trip to Colorado in February 1995, I noticed some mild weakness. In March I was diagnosed with ALS. I had been practicing medicine for about 7 years. My understanding of ALS was limited because it's fairly uncommon and I'd never encountered a patient with the disease. I have since read and learned more about it. Unfortunately, I found that helpful information wasn't always easy to find. As a result I put together a single source of information and resources on ALS, primarily written for newly diagnosed patients and their families. Through sharing information we can make living with ALS as comfortable as possible.

I've tried to create a practical source of information under the following five topics: "What is ALS?," "Practical Tips," "Organizations & Resources," "Drugs" & "Treatment Centers." It is important that you realize I am not an expert on ALS. I am a patient with a medical background which offers some advantages to interpreting available information. I wish to avoid giving any specific medical advice. That is something best left between you and your doctor. I do hope to pass along some information that will help you get the most out of all the medical resources available. Please first discuss any changes that you may wish to make as a result of reading this with your doctor. I'm sure many PALS (Person(s) with ALS) and their families have a tremendous body of knowledge and experience to share. I encourage you to look into the online ALS Bulletin Boards and services, as an excellent format to share and exchange information.



What Is Amyotrophic Lateral Sclerosis?

Amyotrophic Lateral Sclerosis, (also known as ALS, Lou Gehrig's Disease, Motor Neuron Disease, Charcot's Disease), is a neurological disease affecting the nerves that supply all voluntary muscles. ALS was first identified in 1874 by a French doctor named Charcot. It is one of the most devastating diagnoses to receive. There is no known cure and, until this year, no medical treatment that could alter the course of this disease. Fortunately, we now have one drug shown to extend the survival of some ALS patients, Riluzole (Rilutek), as well as several other drugs being investigated.
ALS affects the muscles of arms, legs, posture, face, tongue, speech, swallowing, and eventually, breathing. It may also affect emotion, resulting in periods of inappropriate or exaggerated emotional responses. What it does not affect is thinking or intelligence, sensation/sense of touch, taste, smell, hearing, sight, eye movement, involuntary muscles of the heart, bowel, bladder, or sexual function.

The two currently identified forms of ALS are "Sporadic" and "Familial." The most common type is "Sporadic ALS," accounting for over 90% of the cases of ALS. Here the cause is unknown and may affect anyone at random. "Familial ALS" accounts for the remaining cases (less than 10%) and is caused by inheriting a dominant gene from one parent. In this case, there would be a family history of ALS among blood relatives and also a 50/50 chance of the affected person passing it onto their children. If neither parent or other blood relatives have ALS, you'd expect to have the Sporadic form and, therefore, not expect to pass it onto your children. Presently there is no blood or genetic screening test to detect "the ALS gene" for all familial forms of ALS, but, there is a test for the chromosome 21 defect. However, only approximately 20% of the people who have familial ALS have this defect. These studies are designed for research, & aren't yet available for general testing. It takes months to get results & there can be problems with the tests, (ie. unreadable.) For information call ALSA at (800)-782-4747 or the Les Turner Center (708)-679-3311.

ALS is a very uncommon disease, affecting about 1 per 100,000 each year. It more often affects people over age 40, men slightly more than women. The usual cause of death is through respiratory complications. Survival time, as well as the course of the disease (the order in which symptoms develop) varies widely. Historically, from textbooks, ALS has an average survival time of 3 to 5 years, with about 20% of those affected living past 5 years. The classic "Progressive Bulbar Palsy" variant has a survival time of 1 to 3 years. Let me state clearly that there are a lot of reasons not to get caught up with numbers on survival time. First, those numbers are averages based on thousands of people and that doesn't tell us, as individuals, how we'll do. Every individual's course is different. Second, there are documented cases of spontaneous remission and long term ALS survivors. Third, with the availability of gastrostomy feeding tubes, home ventilators, etc, not only length of survival, but quality of life can be extended. Finally, it's far too early to tell what impact these new research drugs, (and others to follow), will have with early and long term treatment. Also, I firmly believe that state of mind has a significant influence over survival time. I'll comment more on that under "My Personal Philosophy" at the end of "Practical Tips."

Regarding the disease process, there are 2 types of motor nerves that supply voluntary muscles; both are affected in ALS. One type are called Upper Motor Neurons (UMN's). These begin in the brain and extend into the spinal cord. When these nerves are affected, the result is spasticity, weakness and over-reactive reflexes. The second type are called Lower Motor Neurons (LMN's). These begin where UMN's end (in the Anterior Horn Cell region of the spinal cord), and each travels to a specific muscle. When these are affected, the result is marked weakness leading to paralysis, muscle wasting and diminished to lost reflexes. As these nerves become dysfunctional you may see muscle fasciculations. When symptoms begin in the arms or legs it may be referred to as "Limb Onset ALS."

To make matters a little more complicated, there is a special subset of nerves that come directly from the brainstem and control specialized functions like speech and swallowing. When symptoms arise from these nerves they may be referred to as "Bulbar" symptoms. Bulbar is a traditional term for brainstem.

A diagnosis of ALS is made by "Clinical Diagnosis." This means that no single test can prove the diagnosis. Instead, it requires a thorough exam and many tests, sometimes over a period of weeks, all of which need to be interpreted by an experienced neurologist in order to confirm or reject a diagnosis.



Practical Tips

Funds

For services and equipment that you're unable to afford may be available through a variety of organizations, see "Organizations & Resources."

Eating

Eating is extremely important. Missing meals because of difficulties with eating can have a dramatic effect. Poor nutrition may cause weakness, falling, irritability, listlessness, fragility of bones, and even weaken your immune system. In short, accelerate all the bad things. The sensation of choking is a terrifying experience; however, it is rarely the cause of death in ALS patients. Still, you must be extremely careful and know when it's time to talk about a feeding tube.

Concentrate- Avoid distraction, talking, laughing, etc. This may go a long way to keep you enjoying food.
Head Position- Generally speaking, the mechanics of swallowing favor your chin lowered, (looking down at your plate). You may find it easier turning your head either slightly left or right.
Food- Nutritional needs may change, some weight loss due to muscle atrophy is expected. Discuss a dietary plan with your doctor or dietician, especially if eating becomes difficult.
Thin liquids- Thin liquids may be hard to control. Try thicker liquids or use thickeners, eg "Thick-it."
Meat- I like to use a sauce, gravy, mashed potatoes, etc., to keep it moist. Try smaller bites. Let someone else cut it up rather than fatiguing yourself.
Consistency- Choose easy to eat foods or modify those you like in a blender.
Utensils- Try built up handles or specially made angled spoon, fork or "spork."
Feeding- Consider smaller more frequent meals- 5 or 6 times a day. Accept being fed early, nothing worse than a slow, frustrating, cold meal you don't finish.
Pills- Try taking with applesauce or thickened liquids if you have trouble swallowing them.
Independent Movement

Independent Movement

Independent Movement may become impaired but still possible for some time. I believe the phrase, "If you don't use it, you lose it" is particularly relevant to those of us with ALS, of course when done it is important to take appropriate precautions to avoid set-backs. The only thing worse than losing your ambulatory independence is falling. Try to maintain movement around and out of the house with the right level of support.

Footwear

Socks alone on a smooth surface may be slippery. Slippers or good fitting, comfortable shoes with rubber soles are generally better. (High Tops)

Surface

Not only are slick tiled floors potentially hazardous, but thick cushioned carpets, rugs or uneven surfaces may become considerable obstacles.

Rails

Take advantage of railings whenever possible, especially over difficult surfaces, stairs or on "bad days." My wife & I have a code word, "rail," which means for her to put an arm out like a rail for me to use.

Walker

I fell several times before I accepted a walker. When I got it, I realized how much safer and easier it made my life. A tray or bag to carry things (phone, remote control!, book) is easily attached.

Wheelchair

Again, don't be an "Ironman" or woman. Order early, it may take a while, especially if you're going through an insurance company. Wheelchairs are fairly sophisticated. You should have a "Seating," where the chair is custom fitted to your body including a cushion and to meet your specific needs.

Bathing

Bathing can be made safer and easier with a few simple modifications:

• Hand Held Devices- consider built up grips, elec razor & battery operated toothbrush.
• Toilet- Handrails and toilet seat extension.
• Shower- Hand held sprayer a must, some type of seat (bench type or shower chair), hand rails and a non-slip surface.

Dressing

Dressing can also be made easier as you lose use of your hands by making some simple modifications:

• Larger- looser fitting Tee shirts, underwear, socks.
• Elastic- waistbanded shorts or sweat pants.
• Velcro- in place of buttons or zippers on pants and shirts.
• Elastic- shoe laces, long handled shoe horn.

Exercise

Exercise is an issue that remains unclear as to whether there is an ideal strategy. What most can agree on is that:

• Exercising- to strengthen muscles won't prevent progression of the disease.
• Exercising- doesn't seem to worsen the progression of the disease, although you may have a prolonged refractory time, or recovery period, and feel weak for a couple days.
• Moderation- of exercise or activity level is probably the best way to maintain optimal muscle tone and range of motion. Do what you can, safely. Preservation is the goal.

Appliances

Appliances may make a dramatic difference in your independence, safety, and quality of life. Consider what difficulties you have and try to anticipate those you may yet encounter. Find a catalog with such devices, ie VNA (Visiting Nurses), Sears, etc. Then discuss them with your spouse, caregiver and your therapist or doctor - very important. I'll discuss more about this later under "Insurance." Check with your insurance company or "Case Manager" before ordering anything. They can be very fussy about how and from whom to order items. Some examples follow:

A.F.O.- Ankle Foot Orthosis, custom made. Helpful to battle against footdrop.
Adjustable Tray Table
Flexible Straws
Speaker Phone
"Bunny Boots"- or "Deluxe MultiBoot" (800-225-2610) also helpful for footdrop.
Easy Writer- appliance to hold pencil.
Hoyer Lift- Transfer from bed to wheelchair and vice versa.
Lift Chair- Elevates you to a near standing position and reclines by elec control.
Light Touch- a device that turns on a light by touching any metal part of a lamp.
Gait/Ranger Belt- to help caretaker lift and transfer.
Safety Frame- for toilet.
Toilet Seat Extension- Elevates seat several inches, allowing easier "departure."
Trapeze- Triangular bar suspended above the bed to help you move yourself in bed.
Walker- Aluminum, lightweight. A Walker Tray - or pouch to carry items.
Wide Mouth Cups- or cups with a cut out for nose to allow drinking w/ minimal raising of chin.
Hospital bed- An electronically operated bed capable of various positions.

Physical Therapy

Physical Therapy (PT) makes an important contribution to the care of ALS patients. The role of the physical therapist is to evaluate the patient's physical abilities, design a PT plan, provide therapy, including active (you do the work) and passive (someone else does the work) activities as well as teaching this to patient and family or caretakers. The therapy is usually designed to maximize your functional ability. The difficult part is making sure the therapist is familiar with this disease process and the goals for ALS patients. Generally speaking these goals are different from the majority of their patients, i.e. hip and knee replacements, strokes, etc. In those cases the loss of function is fixed or expected to improve and the therapist gives them a plan to strengthen available muscles to compensate for what is lost. It is very difficult to explain to the unfamiliar (therapists, insurance companies, etc.) that deterioration, not progress, is expected with ALS and our goals are to preserve as much strength and range of motion for as long as possible. The generic criteria insurance companies may use to approve PT is often "continuing improvement." You may remind them that foot drop and other problems may develop as a result of inadequate PT, which presents a safety issue. This may provide enough justification. The progression of our symptoms will vary and our needs may be vastly different after only a few weeks. If you aren't getting ongoing PT or periodic re-evaluations your needs may not be met. An effective therapist will discuss any changes in your status and adjust your PT plan and orthotics to meet your changing needs. They may advise on the need for various appliances and aides, i.e. walkers, AFO's. Occupational Therapy may also advise on appliances and orthotics, some overlap is normal.

Occupational Therapy

Occupational Therapy (OT) focuses on assisting you to perform necessary everyday activities, often with an emphasis on use of hands. They are usually very helpful in identifying and solving problems having to do with eating, dressing, bathing, etc. They may be able to get you a catalog of appliances and aides; go back and look periodically. Again, because this disease is not very familiar to most people, it is also a good idea to discuss the changing needs and ask about periodic contact to make necessary adjustments.

Speech Therapy

Speech Therapy can evaluate both speech and swallowing as problems arise. They can explain why you may have trouble making certain sounds and offer suggestions to get the most out of what you've got. Similarly, they'll evaluate your swallowing problems, break down the process and show you methods of more effectively handling liquids and solids.

Insurance

Insurance can be a blessing and a curse. First, learn everything you can about your policy. For example, number of Home Health Visits per year, i.e. PT, OT, Treatments, etc. Some policies may say 60 days, meaning any 60 individual days throughout the year. Another may say 60 days, meaning 60 consecutive days, i.e. June 1st through July 30. Know spending limits, deductibles and the anniversary date, when you begin a new spending year. Know your policy! It will help adjust your expectations and better equip you to deal with disputes. Second, check with your insurance company or Case Manager before purchasing any medical items. If they cover specific items, they'll probably have restrictions. They may only buy from a certain company or rent some items rather than buy. Remember, try to be practical in your requests. If you want every little item, you may reach your spending limit with unnecessary items and not be able to afford important items later. Try to avoid frivolous requests. Insurance companies and therapists have a pretty good idea what is reasonable, but if they don't agree with your doctor's prescribed care, let him, or her, know.

Your Physician

Your physician beyond the obvious, plays a key role in setting many processes into motion. Before each doctor appointment, know your health status and be prepared with questions, requests and any new problems. Having been on the other side of that fence, I know most patients I've seen have had questions at one time or another until they walked into the office. For some reason its easy to get distracted, right at the wrong time. Sometimes I'd be able to guess or say something that reminded them, sometimes not. Either way, the things forgotten are often the most important. So, make a list and discuss the issues with your spouse or caretaker ahead of time. My wife comes up with all the important questions. If you're prepared and concise, your doctor will appreciate it and you'll get much more out of the visit. He or she will be the one determining the scope of your care. This may include PT, OT, a Wheelchair, Letters of Disability, Hospice as well as prescribing everything from drugs to remodelling a bathroom to accommodate your health needs, which may then qualify as a tax deduction. So the better you can communicate your situation to your doctor, the better he or she can help provide for your needs. Then if your insurance company doesn't seem to agree with or "approve" what you and your doctor have planned, let your doctor know. Either wait until your next appointment or, and this is important, call the "shaker and mover" in the office, usually the Nurse or Office Manager. She, or he, is the one who most frequently deals directly with the insurance companies for the doctor. Find out who they are and make friends. Having them prepared to respond to an issue will help immensely. If it is a covered service, it can often be worked out. Physicians and their staffs, unfortunately, are very used to these type of dealings.

Your Spouse

Your spouse and immediate family will experience the shock and devastation of this illness to an extreme degree comparable to your own. Too often the support from outside family and friends is focused solely on the patient, while the spouse is overlooked. Our spouses meanwhile, are forced to take on more responsibilities at home, continue working and then care for us. We need to remind ourselves, our spouses and our families of this. We have to make sure that our spouses' needs are met. For example, visitors need to help out, not add to host responsibilities, our spouses need to be able to get away and maintain other relationships, and they need to have access to local support services.

Home Health Aides

Home Health Aides may be necessary at some point. Early planning may help locate the type of person you'll want in your house during the hours you need them there, more affordably and allow more effective training while you're still able to move and communicate.

Specific Symptoms

Specific symptoms such as spasticity, cramps, emotional instability, excessive salivation, bowel trouble, etc., may be treated in a variety of ways. What works for one person may not for another, or may even be harmful. One size does not fit all. For example, excessive salivation may be controlled with one simple "antichlolinergic" drug with one person, while another may not respond to multiple drugs and require irradiation of their salivary glands. I encourage you to work closely with your doctor. Many symptoms may be "managed" rather than completely eradicated. It's important that you accurately describe the symptoms you experience. Then discuss with your doctor the goals of the treatment and follow the instructions you're given. If the problem isn't adequately controlled, hasn't met your goals, you've done your part and the ball is back in your doctor's court. Don't be passive, ask about other options, be persistent, offer ideas you may have heard about, but don't "live with it" unnecessarily.

My Personal Philosophy

My Personal Philosophy is one of optimism, trying to be positive and productive in the face of this cowardly disease that seems to do everything it can to dismantle our humanity. I firmly believe that state of mind plays an important role in the course of this disease. The opinions that follow are conclusions I have drawn based on a combination of facts and speculation.

First, and most apparent, is basic physiology. If you stop using a normal healthy muscle or muscles, they will become weak and wither away. This is termed "disuse atrophy." If you've ever had a cast on an arm or leg for weeks to months, you probably know what I'm describing. The limb is weak and takes a while to get back to normal, through using and rebuilding muscles. With ALS, I've found that the symptoms can vary from day to day, sometimes hour to hour. You may lose hand strength temporarily and later notice it has returned. Therefore, try to remain persistent in attempting to use these weakened muscles, short of fatigue or frustration. If we get depressed we're generally less active. If we're less active and don't re-challenge these weakened muscles, over time we risk some degree of disuse atrophy. This adds to the unavoidable weakness directly due to the ALS. This makes us weaker and, in turn, we may experience more depression and inactivity, more weakness, more depression... Resulting in acceleration of the disease. On the other hand, if you can somehow stay as positive and as physically active as possible in spite of this disease, drawing on the support of family & friends, I believe you will simply retain more functional strength, thus improving both the quality and length of your life. I apply this concept to movement as well as speech and swallowing. Of course, always with prudence and safety in mind. I fully admit that it's easier said than done. Some of us have the added burden of emotional swings on top of all the expected stresses. We sometimes hurt those we love and need the most. It's important not to let these outbursts create depression or cause withdrawal. I try to explain to those around me that it is sometimes difficult for me to control my emotions. Then, if I say something I regret, I apologize as soon as I get over it and move on.

Second is a concept I've pondered that is admittedly more imaginative speculation than science. I've read about long term ALS survivors, (20+ years), who claim their survival is the result of a life change, which invariably involves a change in their behavior. They describe events such as "accepting the disease and being at peace," encountering an inspirational book or person who "changes" them, or accepting a new or renewed set of religious beliefs. Although there remains a lot that is unknown about ALS, investigators suspect it arises through a cascade of events in the Central Nervous System involving chemical mediators, possibly Glutamate, Superoxide Dismutase, Free Radicals or abnormal neurofilaments. We don't know where this cascade of chemical events begins or ends. Can behavior itself affect ALS? Different behaviors involve different neurons or neurotransmitters. Can different behavior patterns, like anger or contentment, produce different mediators in the brain that influence ALS? Many intelligent men of science and medicine may explain these survivors as a small number of inevitable survivors, reason unknown, who attribute their remarkable survival to something that fits their belief system. I readily admit that this may be true. But what if behavior can influence ALS? We once thought the world was flat, blood letting would cure most disease and that surgeons didn't need to wear gloves or wash their hands before performing surgery.

Unfortunately, this just raises more questions and provides no answers. I doubt that we'll know the answer any time soon. Nonetheless, we still have some long term survivors with very compelling stories. We have little to go on, but perhaps one common thread throughout most of these accounts - they all seem to reflect a positive, peaceful attitude. It is very difficult to do research involving subjective human feelings or emotions. "Emotions?" Hmmm, that sounds strangely familiar when talking about ALS. So, I say there's nothing to lose and everything to gain by keeping a positive attitude. At the very worst, it will enrich our lives and make us more pleasant to be around. And it may...?

Loose Ends

1. Last Will and Testament
2. Insurance Beneficiaries
3. Names on Deeds and Titles
4. Funeral/Burial Arrangements
5. Living Will

Consider reviewing and making decisions on these issues at some point early enough when you're able to communicate and not distracted by physical limitations. They aren't very pleasant topics, but so very important in order to see that things are done, and your loved ones treated, the way you wish. You may not feel ready to make "final" decisions or are concerned about changing your mind later. Its much easier to modify than to create, especially during periods of critical health. For example in writing a Living Will, you indicate your wishes regarding things like being on a ventilator in the event your muscles of repiration fail and the degree of resuscitation in the event your heart or blood pressure begin to fail. Let's say today you choose to not be put on a ventilator and opt for no "Heroic Measures" (elec shock, powerful drugs to maintain heart beat & blood pressure), instead request only drugs to keep you comfortable. Later you may change your mind and choose to accept a ventilator. All you need to do is indicate this and its changed, regardless of what has been previously written, signed & witnessed. Whether you're at home with Hospice or in a hospital, you'll be asked and offered many things and have ample opportunity to make your wishes known.

Beware of "Snake Oil"

Beware of "snake oil." I believe in keeping an open mind and considering every conceivable form of treatment in the case of terminal illnesses. Everything is fair game; vitamins, inherent mental healing powers, prayer, touch healing, herbal therapy and many others. However, "look before you leap." Jumping in with both feet may be risky, disappointing and costly. Unfortunately, there are many people who deliberately, and probably many more who with honorable intentions, seek desperate, terminally ill people to whom to sell their product(s). Be wary of those who boast confidence or a "sure cure," especially when it can only be had at a considerable cost. Be an informed consumer. Ask for information on how it works, risks, side effects, etc. These people can be very convincing. Look to other unbiased sources for information on the subject and, of course, discuss it with your doctor. Most doctors are very aware of the limitations of medicine, the slow plodding pace of the "scientific method" used in medical research and the small number of diseases that are actually curable. Advances in medicine have often been "discovered" by scientists years, even centuries, after use by the "lay public." In medieval times some cultures would treat their sickly women by driving a nail through an apple, removing it after a couple days and letting the woman eat the apple. Now, of course, we understand Iron-deficiency Anemia and treat it with iron tablets instead of waiting for an iron nail to oxidize in a moist apple. Keep your mind open as well as active and discriminating.

Hospice Care

Hospice is a multidisciplinary team approach to the care of terminally ill patients. Nurses, Social Workers, Pharmacists, specially trained Home Health Aides and "Sitters" work together to provide comfort and relief to terminally ill patients and their families during the last months of life. Their services adapt to almost any envviornment; hopitals, nursing homes, Hospice Centers or at home. They are extremely dedicated and very good at what they do.



Organizations and Resources

Organizations

The ALS Association

21021 Ventura Blvd., Suite 321
Woodland Hills, CA 91364-2206
Tel: (800) 782-4747
Tel: (818) 340-7500
Fax: (818) 340-2060

Forbes Norris MDA/ALS Research Center
at California Pacific Medical Center

(Deborah Gelinas, M.D.-Michelle Murray)
2324 Sacremento Street, Suite 150
San Francisco, CA 94115
Tel: (415) 923-3604
Fax: (415) 673-5184

Muscular Dystrophy Association

(Michelle Girard)
3300 East Sunrise
Tucson, AZ 85718
Tel: (800) 572-1717
Fax: (520) 529-5454

The Les Turner ALS Foundation

3325 Main Street
Skokie, IL 60076
Tel: (708) 679-3311
Fax: (708) 679-9109

The above organizations provide tremendous resources and a variety of services. Some offer funding for care or equipment as well as materials and services at a reduced rate or no charge. If you haven't yet been in contact with each of them, call or write today. The MDA number will give you a local or regional office. Also, just to have several pieces of information in one place, I've included the number to call when you're ready to initiate Social Security. Once you're disabled from work, call the number below and make sure you explain to them the circumstances of your illness, (terminal illness, prognosis), and that they should expedite the paperwork. If eligible, payments should begin in approximately six months.

Office of Social Security

Tel: (800) 772-1213

Online/Internet ALS Services

America Online ALS Bulletin Board

Main Menu -->
"Clubs & Interests" -->
"disABILITIES" -->
"disABILITIES Message Center" -->
"General Discussions" -->
"List Topics" -->
"ALS (Lou Gehrig's Disease)"

CompuServe ALS Bulletin Board

Click on Go, then type mda or mdaforum; read the disclaimer, then click on Proceed - that puts you on the MDA (Muscular Dystrophy Association) forum. From there you can browse the library or message sections - both of which have a separate ALS section. To get to the bulletin board, click on Messages, then on the ALS section.

Prodigy

Jump Medical Support BB; Subject: neurological; Topic: A.L.S.

ALS Digest

The ALS Digest is an e-mail subscription service that announces developments, answers questions and provides information and discussions among Researchers, physicians, patients and other interested parties. To subscribe address e-mail to: Bob Broedel (broedel@gly.fsu.edu)

Brunel MND Research Unit

The Centre for the Study of Health, CSHSD, Brunel University, England Stuart Neilson, PhD. maintains a data base with links to medical and technical terms on ALS available on the World Wide Web at:

http://http2.brunel.ac.uk:8080/~hssrsdn/alsig/review.htm

or access it now by clicking here.

Books & Manuals

"Amyotrophic Lateral Sclerosis: A Comprehensive Guide to Management"

Hiroshi Mitsumoto, M.D. And Forbes H. Norris, Jr., M.D., Editors

Send to:
DEMOS VERMANDE PUBLICATIONS - $39.95 + $4 p&h
386 Park Avenue South, Suite 201
New York, NY 10016
Tel: (800) 532-8663
Fax: (212) 683-0118

Managing ALS Manuals

Cost: $3 each

I - Finding Help
II - Muscular Weakness
III - Breathing Problems
IV - Swallowing Problems
V - Communication Problems

Write to:
The ALS Association
21021 Ventura Blvd., Suite 321
Woodland Hills, CA 91364-2206

"Love, Medicine & Miracles" - Bernie S. Siegel, M.D.

"The Road Less Travelled" - M. Scott Peck, M.D.

"Peace, Love & Healing" - Bernie S. Siegel, M.D.

"When God Doesn't Make Sense" - James Dobson


Videos


"What is ALS?"

Cost: $15

Write to:
The ALS Association
21021 Ventura Blvd., Suite 321
Woodland Hills, CA 91364-2206


"A Brief History of Time"

From Paramount Pictures

A film about a genius/physicist, Stephen Hawking, and his remarkable accomplishments in the field of physics in spite of living with ALS for 30 years. Check your local Video Store.


ALS Patient Support Group Visual Library

Borrow tapes for up to two weeks at a time.

• Management of ALS
• Home Care/Long Term Care
• Legal/Financial Issues
• Research in ALS
• Special Speakers
• Speech and Swallowing
• Independent Living

Write to:

Norris MDA/ALS Research Center
at California Pacific Medical Center
2324 Sacremento Street, Suite 150
San Francisco, CA 94115



Drugs


RILUZOLE (Rilutek)

At present, Riluzole is the only drug approved by the FDA for the treatment of ALS. It is also the only drug shown to extend the survival of ALS patients. In a phase III clinical trial involving nearly one thousand participants, the group treated with Riluzole showed a modest, but statistically significant increase in length of survival. One theory of the cause of ALS is that Glutamate, a neurotransmitter, accumulates to toxic levels around motor neurons causing cell death. Riluzole seems to do two things: slows the release of Glutamate from neurons and blocks the effect of glutamic acid on Glutamate receptors. Riluzole or Rilutek is manufactured by Rhone Poulenc Rorer, (RPR). For information about Riluzole, contact RPR at the below address or phone number. For information on the Rilutek Early Access Program, (EAP), contact the National Organization for Rare Disorders, (NORD), at the below phone number.

Rhone-Poulenc-Rorer
(Rob Partridge)
500 Arcola Road
Collegeville, PA 19426-0107
Tel: (800) RX TRIAL
Tel: (800) 798-7425

Rilutek Early Access Program
National Organization for Rare Disorders
Tel: (800) 459-7599
Fax: (203) 746-8483


NEURONTIN (Gabapentin)

Neurontin is currently available by prescription for the treatment of seizure disorders. Investigators believe that it may have anti-Glutamate properties, but this hasn't yet been proven. Currently, Parke-Davis is investigating any beneficial effects. So far there is no evidence that Neurontin is effective in the treatment of ALS although, patients who have taken it report a sense of well being. For more information, contact:

Dept. Of Neurology Attn: Amy Quinn, RN
California Pacific Medical Center
3698 California Street, Rm 545
San Francisco, CA 94118
Tel: (415) 750-6261
Tel: (415) 750-6040
Fax: (415) 750-5022


BDNF (Brain Derived Neurotrophic Factor)

BDNF is a nerve growth factor. It is a naturally occurring protein produced by the human body in small quantities. The BDNF being used for this study is a synthetic version produced by using recombinant DNA. Some experiments in animals have shown that BDNF helps injured motor neurons survive for longer periods of time. The first human tests on ALS patients indicated slower deterioration of breathing and possibly longer survival. For more information contact:

AMGEN, Professional Services
1840 DeHavilland Dr.
Thousand Oaks, Ca. 91320-1789
Tel: (805) 447-1000
FAX: (818) 865-3707


VITAMINS

Many investigators believe that nerve cells may die as a result of oxidation injury. Although there have been no proven benefits to anti-oxidant therapy, interest has been somewhat renewed since the recent discovery of "the ALS gene," (in Familial ALS), near the gene for an enzyme, Superoxide Dismutase, responsible for protection against oxidation. The MDA Medical Advisory Committee recommends:

Vitamin C: 1,000 mg 3 times a day ( 3 grams daily )

Vitamin E: 800 units 3 times a day ( 2,400 units daily )

Beta-Carotene 10,000 units 3 times a day (30,000 units daily )


MYOTROPHIN or IGF-1 (Insulin-like Growth Factor)

A naturally occurring protein that helps the nervous system recover from injury. It is thought to promote survival of motor neurons and enhance regeneration of damaged motor neurons. For more information contact:

Cephalon, Inc.
Assistant Director Corporate Communications
Tel: (800) 797-0705


BCAA (Branched Chain Amino Acids)

There is some evidence that treatment with BCAA (Leucine, Isoleucine, Valine) is not only ineffective, but may actually be detrimental . For more information contact:

The ALS Association
21021 Ventura Blvd., Suite 321
Woodland Hills, CA 91364-2206


GDNF (Glial-Derived Neurotrophic)

For more information contact:

AMGEN, Professional Services
1840 DeHavilland Dr.
Thousand Oaks, Ca. 91320-1789
Tel: (800) 447-1000
Tel: (800) 772-6436
FAX: (818) 865-3707


CNTF (Ciliary Neurotrophic Factor)

For more information contact:

National Organization for Rare Disorders
Tel: (800) 999-6673
Fax: (203) 746-6481

CytoTherapeutics
Seth A. Rudnick, M.D., ext. 2113
or Elizabeth Razee, ext. 2132
Tel: (401) 272-3310



Treatment Centers

"These clinical care facilities have been certified by The ALS Association (ALSA) as having met a rigorous standard of quality care for ALS patients. Each has a multidisciplinary team approach to provide a continuum of care as well as appropriate diagnostic capabilities."

I don't use one of these facilities and believe I receive excellent care. It's important that you find a Neurologist experienced in the diagnosis and care of ALS patients, with whom you have a good relationship, whether or not they are at one of the below centers. Also, it is very reasonable to have a second opinion with such an important diagnosis.

ALS Clinical Department of Neurology (Rup Tandan, M.D.)
Fletcher Allen Health Care
UHC Campus
Department of Neurology
1 South Prospect Street
Burlington, VT 05401
Tel: (802) 656-4550
Fax: (802) 656-2461

ALS Clinical Services Center of Hahnemann University ( Ted Tatarian, D.O.)
Hahnemann University Hospital
Broad and Vine Streets
Philadelphia, PA 19102
Tel: (215) 643-5434
Fax: (215) 643-9307

ALSA Center, Desk S 90
Cleveland Clinic (Hiroshi Mitsumoto, M.D.)
Cleveland Clinic Foundation
9500 Euclid Avenue
Cleveland, OH 44195
Tel: (216) 444-5418
Fax: (216) 444-9401

Harry J. Hoenselaar ALS Clinic (Daniel S. Newman, M.D.)
Henry Ford Hospital
2799 West Grand Blvd
Detroit, MI 48202
Tel: (313) 876-2594
Fax: (313) 876-3014

Motor Neuron Disease Clinic (James W. Albers, M.D.)
University of Michigan Hospital
Department of Neurology
1324/0322 Taubman Center
1500 E. Medical Center Drive
Ann Arbor, MI 48109-0322
Tel: (313) 936-9010
Fax: (313) 763-6545

Forbes Norris MDA/ALS Research Center
at California Pacific Medical Center
(Deborah Gelinas, M.D.-Michelle Murray)
2324 Sacremento Street, Suite 150
San Francisco, CA 94115
Tel: (415) 923-3604
Fax: (415) 673-5184

ALS Clinic (Janice Weisman, M.D.)
Boston University Medical Center
720 Harrison Avenue, Suite 707
Boston, MA 02118
Tel: (617) 638-8456
Fax: (617) 638-8465