RANDY’S ALS MANUAL LIVING WITH ALS (LOU GEHRIG’S DISEASE)

A GUIDEBOOK FOR RECENTLY DIAGNOSED PERSONS WITH ALS, THEIR FAMILIES AND SIGNIFICANT OTHERS

In Memory of Randy Roberts 2007

Compiled by Randy Roberts in association with members of The Living with ALS Group, 11/2006, and dedicated to all courageous persons with ALS: past, present and future

This guidebook is free of charge. It was designed to be read or downloaded into your computer. TO READ ONLY, SCROLL DOWN CLICK HERE TO DOWNLOAD THE CURRENT GUIDEBOOK

In order to download the manual you must have Microsoft Word installed on your computer. To download the manual to your computer right click on the link and select [save target as] and in the box that pops up select where you want to save the manual on your computer. It may take awhile to download on a slow Internet connection. If you don’t have access to the internet, please ask your clinic/program to print a copy for you.

PREFACE

By Randy Roberts

Following the shock of my diagnosis of ALS in 4/04, and lacking any preparation, I felt lost in a nightmare, overwhelmed by negative thoughts. Gradually, by going to numerous websites and other sources, I learned something about coping but it wasn’t until February, 2005, when I joined Living with ALS, a message board group, contributed to by patients and their family members, that it all started to come together. There, I picked up practical and basic information, all with the personal contact missing from other sources, and a philosophy of learning to live as fully as possible with ALS. The supportive and compassionate nature of the posts helped me through some rough times and, through reading the messages and chat transcripts, I came to better understand the meaning of courage.

Eventually, I regained my bearings and had, in the process, accumulated a great deal of information on coping with ALS and related areas. The idea spontaneously arose to use what I knew to compile a Guidebook/Manual for those who had been recently diagnosed so that they would have one resource to turn to that offered integrated information presented in a positive, patient/peer oriented framework, rather than having to go through the time consuming process of sifting through many sources on their own. Thus, this Guidebook was born.

The Guidebook could not have come into existence without the help of the Living with ALS members. Thanks are due toSherry, Susan, Jeff, David, Erin, Wayne, Cookie, Ruth Anne, Lee, Lisa, Alper, Don, Edith, Thom, alsfrombothsidesand the whole group, too numerous to name, from newbie to veteran, who have taught me so much. A special thanks goes to those who contributed personal stories and articles to the Guidebook.Danny Dandignac reformatted the Manual and made it look more professional. Jerry Bergen created my webpage so that the Guidebook could be read on line and/or downloaded and comments left.

I want to thank my two children, Brett Roberts, M.D. and Heather Roberts for sharing their knowledge of Microsoft Word. Without their help, I would still be copying and pasting. Finally, I wish to thank my wife Pam for her loving care and steadfastness throughout my ordeal with ALS.

Randy Roberts Ellicott City, Maryland January, 2006

TABLE OF CONTENTS

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When you want to move a body part, say your arm, an impulse is generated in your brain and the signal to move is then sent via motor neurons to the spinal cord and finally out to the muscle. And then the arm moves. In ALS, however, the neurons begin to die off and the command to move does not reach the muscle as well.

Muscles that receive diminished signals from the neurons will weaken and shrivel up. Muscles cannot stay strong without continual input from neurons. Wherever the neuron death begins, it gradually spreads to other motor neurons in the body, causing further weakness. The rate of this spread is called “progression” and can vary quite a bit between PALS, but tends to stay steady for individual PALS.

The disease affects only motor neurons in the body and only those neurons that serve muscles over which we have voluntary control, such as limbs, swallowing, eating, etc. The heart muscle, for example, over which we don’t have voluntary control, is not affected by ALS. The illness does not affect sensory neurons.

There are 30,000 to 40,000 people in the US with ALS. It can strike as low as the early 20’s and as late as the 70’s, but onset tends to peak at late middle age. ALS is not as rare as people think. It is diagnosed with nearly the same frequency as MS (Multiple Sclerosis), a much more recognized disease. ALS is much more lethal than MS so our numbers (prevalence) is much lower.

There is yet no effective treatment for ALS (Rilutek, the only medicine for ALS,whichincreases life span by only a modest amount), nor is there any scientific understanding of what causes the disease. However, more research is underway now than ever before.
 
 
 
 
 
 
 
 

• If you cannot swallow - get a gastrostomy.
• If you cannot breath - get a tracheostomy and a ventilator.
• If you get contractures (stiff joints and muscles) - get range of motion exercise.
• If you cannot talk - get a computerized machine that talks for you.

Right now you are probably thinking: "How can I possibly handle all of those physical problems?" First, while ALS is a progressive disease, it also reaches plateaus where it stays without new developments and this allows time to adjust and then prepare for future progressions. Second, I am living proof that it can be done. As of this year (1999), I have survived 26 years with ALS and am looking forward to 26 more. I am in no pain, I am involved in many projects, and I truly enjoy my life. My outlook is; I am NOT SICK! I am a very healthy person who has some physical handicaps. I am sure that Stephen Hawking, one of the greatest scientific minds of the twentieth century who has had ALS now for over 35 years and continues to work, would agree with me that ALS is not fatal.

ALS AND PERSONAL GROWTH

By Randy Roberts

It may seem strange or even repulsive to link this disease, which so cruelly ravages our body, with anything even remotely positive. But as ALS pushes us to our very limits, we have the motivation and opportunity to see ourselves and our lives in very different ways. For me, it has been a spiritual awakening, in the broad sense of that word. The psychological walls that I have constructed to protect myself have been crumbling and my heart is open as never before.I have become more sensitive to thesuffering felt by those who have lost their health and more attuned to the pain experienced by so many in this world of ours.I have come to feel a new appreciation of life and all the gifts given to us, gifts that are taken for granted when healthy. I know now that it all isn’t about me (surprise!) and that there exists a vast and indefinable presence that connects all of us. And I have felt brief moments of peace that I have not experienced before.I have also seen the power of living in the present moment or, more precisely, of being the moment. When we are able to still all the internal chatter of reliving the past and thinking of the future, all of which are just dreams and imaginings, what is left is moment by moment living. I have also obtained a clearer distinction between image and reality in ALS. Image is a projection of all of our negative ideas about what it is to be disabled, learned from conventional societal attitudes. Reality is what people actually experience when living with ALS, which can be much more positive than our images, as personal stories in this Guidebook illustrate.Many sources have helped contribute to my new found awareness, including my spiritual training which began in earnest about a year ago, but reading the daily posts on the Living with ALS forum has been an important factor. One’s heart cannot but melt when reading posts of deep distress, helpfulness, courage, compassion, caring, optimism, determination, good humor and encouragement.While ALS takes away options that were once present, it can also act as a stimulus to develop new ways of perceiving oneself and life. I feel that I have barely scratched the surface and look forward to going more deeply into the spiritual and personal development realms as time goes on.

ALS -- NOT CURABLE, BUT TREATABLE

By Edward Oppenheimer, M.D.

Dr. Oppenheimer was on the faculty of UCLA Medical School Neurology Department. He was a strong advocate for PALS and was a member of Living with ALSA, where he often answered questions and wrote valuable, informative articles, such as the one below.Many diseases can, of course, cause death if not treated. High spinal cord injury, a serious infection, an appendicitis, etc., can kill when neglected. When treated, these are still serious conditions, but usually life can continue and death can be prevented. It's true that the average length of life of people with ALS today is about four years. However, this reflects the fact that people with ALS often don't have optimal treatment and resources. If ALS is to be fully treated, people with the disease should have;

• Adequate information about the treatments available
• Access to health professionals who are interested in and experienced
• With all the available options in the care of ALS
• The encouragement to go forward
• The needed resources to go forward
• The desire to use available care

When the patient lacks the desire to use available care, after having been fully informed and provided with a positive approach from professionals such as doctors, nurses, physical and occupational therapists, social workers and others, not proceeding is acceptable. However, when any of the needs cited are insufficiently filled, not treating ALS to the fullest is regrettable. Ideally, this situation shouldn't exist. What is "fully treated" ALS? The answer is my "wish list" for all people with ALS; 1. A positive and experienced team approach to assist patients and their caregivers in solving any of the problems that typically occur. This means a patient should have access to a team of adept and enthusiastic health professionals who are available even when the patient isn't taking part in a research protocol. The team should include professionals who can make home visits and coordinate care as needed. 2. Very good nutrition to maintain appropriate weight. At some point this may include using a gastrostomy tube (feeding tube) if swallowing problems occur. 3. Regular social interaction with friends and the community. It's vital that the person with ALS get out of the house by using mobility assistance, stay engaged in living and maintain ways to communicate even if speech becomes difficult. Good alternative and augmentative communication devices are essential. 4. Personal assistance to cover each 24 hours as needed, without placing an undue burden on family members. 5. Vigorous treatment for infections, particularly respiratory infections. This can often be provided at home with appropriate antibiotics when needed. Medication can be given by mouth, via a feeding tube or even intravenously, depending on the person's condition. 6. Monitoring of breathing capacity so noninvasive assisted ventilation can be available when capacity is decreased and related symptoms are present. An ALS patient can receive mask-delivered or other noninvasive ventilation as long as this works, and then shift to tracheostomy (invasive ventilation, via a tube into the trachea) if that becomes necessary. Equally important are good attention to effective coughing and clearing of secretions, and prevention of aspiration (inhaling particles, such as food, into the lungs). The decision to stop using a ventilator at any time, or to use only noninvasive ventilation and not go on to tracheostomy ventilation, should be made by the patient. 7. Access to ALS research centers and protocols when desired, and access to excellent ALS care even when there is no desire to participate in research. 8. Very good supportive care. 9. Good symptom-relieving care when major aspects of treatment are no longer desired or appropriate. When most of these aspects of care are available to people with ALS, they can often continue living in a productive way and avoid life-threatening complications related to ALS most of the time. ALS motor neuron impairment may become severe, but life and spirit and social ties can continue. Ideally, people with ALS should have choices. They should know that there are options that will allow living to continue even if the resources needed are considerable.

SIGNATURE EXPRESSIONS Gathered from different sites, these expressions, placed at the end of posts, illustrate coping mechanisms in highly condensed form. They are little gems.

Don't forget to live!

Your friend in the fight

Laughing is good exercise: it’s like jogging on the inside

Living & loving everyday

There is no such thing in anyone's life as an unimportant day.

When you find yourself in a hole . . . first stop digging!

It's not in our abilities that we show who we truly are, it's in our choices

I know God will not give me anything I can't handle. I just wish that He didn'ttrust me so much. (Mother Teresa)

Life's a dance, you learn as you go

PSYCHOLOGICAL ISSUES OF NEW PALS AND COPING IDEAS

By Randy Roberts

I am afraid of dying: will itbe painful, will I suffer, will it happen soon.	You are getting way ahead of yourself; ALS is a gradually progressing illness.Morbid thinking creates lowered mood, let such thoughts go. Focus on living now. Find interesting activities to preoccupy your mind.Nobody, sick or well, knows when they will die, only that all of us will pass away at some time.Become involved in spiritual work.There are many ways to extend your life span. Be proactive, stay ahead of the disease.Remember, ALS does not have to be fatal, you have a choice.
My life will never be the same; how can I give up all my dreams, hopes and plans.	Grief is a normal, painful stage that all PALS have to work through. It doesn’t last forever, but may return after each new loss.You may continue with your regular life for awhile and follow thru on some of your plans, just sooner, like special vacations, etc.As you adjust to your limitations, new plans and goals will be set.Reread the personal stories in this section. Pay special attention to PALS’ descriptions of their meaningful, rewarding and happy lives even with ALS.
I feel depressed andanxious all the time.	Depression and anxiety, like grief, are expected reactions.Identify what you are thinking when you feel bad. Is it a negative?Observe how it worsens your mood and then try to let the thoughts go.Stay aware of such negative thoughts and let them go each time.Seek spiritual guidance.Work at focusing on positive goals, projects, things that interest you but you didn’t have time for in the past.Focus on what you still have. Appreciate that which is taken for granted when healthy are truly special gifts.If bad feelings persist, discuss antidepressant/anti anxiety meds with your doctor or increase what you are now taking.Join Living with ALS, ask for suggestions, establish an on line peer support network.Let yourself be inspired and encouraged by what you read.Consider counseling or therapy.Communicate your feelings to others. Don’t let yourself get cut off from family and old friends. Build a new support network.A little humor can work wonders. Find somebody or something that makes you laugh every day.
I am fearful and embarrassed about becoming dependent on others.	Again return to the present. Let negative thoughts dissipate. You are getting ahead of yourself.When the time comes, you will be surprised that you will welcome the assistance, as it becomes exhausting and risky to try to do something independently when capacities diminish. You can now use your energy more productively on other things that really matter.Aren’t all people dependent on others, anyway?Your embarrassment or shame will wither away as you realize that it is no big deal and your caregiver views it as no big deal.
I keep thinking of what liesdown theroad	Go back to the present. Worrying about future events leads to worsened mood. Let the thoughts go as they are preventing you from living well now.Nobody knows what lies in the future, e.g. an effective drug against ALS could be discovered soon. Become involved in interesting and productive activities instead.
I am so angry all the time	Anger is a normal reaction to what has happened and is a stage that must be worked through. It becomes a problem when it persists and you become bitter and closed off. Then you are not living with ALS, but are being eaten up inside.Chronic anger and irritability may reflect underlying depression. Speak to your health care professional about it. Try a visualization technique. Imagine ALS to be an object of some sort. I visualize a hideous looking scarecrow, and then proceed, in my imagination, to inflict all kinds of punishment to him: kicking, chopping, punching, bludgeoning, till he is reduced to pulp. I may do this for 5 minutes and I always obtain a sense of satisfaction and release. Just give back to him what he has done to you, no holds barred.Join Living with ALS. As you read the posts your heart may open up.Bitterness and chronic anger prevents you from living your life to its fullest; they close off options and keeps you imprisoned in their grasp.
How is my illness going to affect my marriage or relationship with my significant other.	It’s very stressful on caregivers, but many handle it well. Some CALS, however, are not able to manage.Discuss issues directly with your CALS; don’t assume anything.Help make arrangements to reduce stress, or encourage your spouse or other family caregiver to do so, when things become too difficult for him/her, e.g. paid care providers, help from family and friends, etcExpress your appreciation. Thank yous, and expressions of affection are helpful.Don’t take out your resentment on your spouse and make an effort to apologize if you should slip.Working together can bring you closer.If conflict or dissatisfaction become intense, and can’t seem to be resolved, consider counseling or begin to think of alternative living arrangements. The earlier you can detect problems in your CALS’ commitment and/or major shortcomings the better.
Why did this happen to me?	Every PALS and their CALS ask this painful question. It seems to demand an answer but of course there is no answer. Coming to grips with it is part of the acceptance process. It lessens in intensity and frequency over time.

COPING WITH ALS

By Loris Buccola

This piece integrates many of the ideas presented in this chapter

I was diagnosed with limb onset ALS in November 1999 at age 58. I'm pretty much completely quadriplegic, retaining head and neck movement. I still have my voice with volume somewhat diminished, swallowing and breathing also diminished (forced vital capacity below 30% at my last measurement a year ago) but intact. I had a feeding tube placed December 2004 and use it for supplemental nourishment once a day. I use a BiPAP at night and portable ventilator (LTV 900) as needed during the day. I have a Permobil power wheelchair with four-way seat adjustment, Switchit head controls and a Roho cushion. I sleep on a Pegasus Airwave mattress and an adjustable bed. I use Dragon Naturally Speaking, Smartnav head mouse, dwell click software and Skeleton Key (an on-screen keyboard) to navigate the computer. I have a tablet computer for my wheelchair with which I can control electronic devices. I have a Ford Windstar with a VMI wheelchair access conversion. Although I'm retired from a 40 year teaching career, I still continue to do professional counseling, writing and e-mail correspondence with friends and family. I still "read" using the Talking Books Program (through the Oregon State library) extensive library of books on tape. I'm also a movie buff and subscribe to the Internet DVD service, Netflix. Here are some techniques and attitudes I have discovered which have helped me to continue adjusting to this disease, avoid depression and continue to appreciate life.

1.Attitude is everything.

ALS is a challenge to adapt, rather than a battle to be won, an opportunity to learn the art of acceptance. Learn how to pick your "battles" by

learning new skills instead of struggling to deny the reality of what is happening to your body. People will think you are really wise and spiritual and

will want to be around you. It's magic.

Although it will be difficult at first, resolve immediately to learn how to ask for and even enjoy getting help from other people. If this disease can

teach us anything, it is that we cannot survive alone. It will bring you closer to them and them to you. Some people may not be able to handle whatis happening to you except by staying away. That is their problem, not yours.

2.Plan realistically for the future.

The name of the game with this disease is adaptation. Try to avoid wasting valuable time which you need to plan for the coming changes in your life.

If you wait for a crisis, it will probably be already too late.

Begin now learning to conserve your energy by not trying to do more than you are realistically capable of. Deciding what is realistic and what is thebest use of limited energy is a major step in adaptation.

Anticipate the future just enough to ask yourself how you will adapt as your physical strength declines. For example, I began learning how to usespeech software before I really needed it. By the time I did, I was proficient at it. It took six months to get wheelchair I needed. Learning to usehead controls, getting used to using breathing assistance, feeding tube, etc. all takes more time than we anticipated.

If you have not already done so, consider attending an ALS support group. It will be difficult at first to see people in all stages of this disease. I hadto resolve to go back for three group meetings. By that time I was already getting used to this look into the future, learning how other people cope. with what is happening in.

We do not easily recover from setbacks from illness or physical injury as we did prior to the onset of this disease. Time wasted is time lost foradaptation. So, for example, don't push yourself to continue walking until you fall and break a bone.

In spite of what you may hear, there is no cure for this disease. Pursuing claims of cures will only cost you money and put you in a time deficit. Once you have been diagnosed, avoid falling into the trap of believing that it is something other than ALS (Lyme disease, mercury fillings, etc.). When aneffective treatment is discovered we will all know about it and someone will no doubt get the Nobel Peace Prize. My own rule on this: put me in touchwith three people who are clearly diagnosed who have at least six months on this "treatment" and let me talk to them personally about how it hasworked.

3.Dealing with Depression

ALS is a lousy disease and quite unfair. But try to avoid taking it personally. It did not happen because of anything we did or did not do. Giveyourself the luxury of feeling sorry for you only on a limited basis, for example an hour a day or, even better, an hour a week.

Avoid dwelling on or worrying about all the awful stuff you may have to go through in the future, or ruminating on all the things you did not do in thepast. Of course, some of us with ALS have other things to be depressed about besides the disease and this can make avoiding depression much moredifficult.

4.Do something every day that gives you pleasure.

I love my morning coffee, having my head scratched, getting a warm shower, watching a good movie, listening to a great book, listening to jazz andblues, following the sports scene.

Be with people whom you love and who love you. You have a right not to be with people who sap your energy. People will like being with you if you

can help them get used to what is happening to you. Be nice.

Plan for things in the future that you can look forward to, like visits from children and grandchildren, friends etc.

Cultivate a sense of humor about this disease. Some of it is so ridiculous that it is actually funny. For example, I have unbitten fingernails for thefirst time in my life.

Seriously consider antidepressant medication. It is not addictive and it is not a weakness to need medication to help out with the symptoms ofdepression.

If you have not already, begin to cultivate a spiritual approach to life. There is more to this life than meets the eye. You will have plenty of timeto think about these things, the ultimate meaning of life, the nature and existence of something or someone beyond us and upon which we depend. Youdon't have to have any formal religious training or upbringing. The process of discovering meaning and purpose beyond our own little worlds is thebeginning of spirituality. Spirituality does not have to have any complicated theology. In fact, the simpler the better.

Trust your instincts. In spite of a lifetime of religious faith, I have come to believe that God did not cause me to have ALS, and in fact is assaddened by the bad things that happen to people as we are. I also am confident that God is with us in all of these things just as he/she/it hasbeen with millions of others over the centuries.

This disease can help us develop a sense of gratitude and wonder about the mystery of life and death. It has taught me to embrace the difficultexperiences of life as opportunities instead of as obstacles to a meaningful life. I enjoyed "Learning to Fall" by Philip Simmons, a teacher and writerwho had ALS.

5.Taking care of your caregivers.

ALS is a social disease; it not only affects us who actually have the physical symptoms, it also profoundly affects and changes the lives of thosearound us who love us and care for us. We have a responsibility, especially if we want to learn how to adapt and prosper, to make sure we don'tovertax our caregivers physically and emotionally. We will naturally tend to become demanding, frustrated and impatient at times. But the more wedo this, the less people will want to be around us.

Make sure your network of care is wide enough to give people some time away to recover and recuperate. Avoid the temptation to just pick out oneor two favorites upon which to rely completely. No one, no matter how much they love us can handle the load by themselves.

You will learn gradually that you can tolerate not getting immediate help that you don't really need: tolerating an itch you can't reach.

TIPS ON DEALING WITH ALS BY alsfrombothsides http://www.living-with-als.org/alsfrombothsides/

As someone quite amazed to find herself in her 17th year of ALS and still busily, happily, and contentedly engaged in living, I have reached the point where I find myself saying "If I'd known I was going to live this long, I would have taken better care of myself!" For that reason, the main focus of this site is on dealing with some of the medical complications we face as a result of ALS. All too often these things are inadequately addressed because the expectation is that we will not be around long enough to worry about "long term" problems. These are problems that can and must be minimized in order to assure a good quality of life even if that life is short. In addition, I believe several factors are going to extend the life of ALS patients in years to come: The development of medications to slow progression. Continued improvements in supportive care (nutrition, respiratory support etc.) Computer aided communication and environmental control equipment will greatly improve the quality of life for ALS patients who opt for ventilation. As a result more of us will take that option and live for many more years. There are also sections with practical tips for dealing with some of the basic problems presented by immobility such as traveling, and more sections to be added. Safe Harbor Rediscovering life on a ventilator. Dress for success: Toileting using a lift. ALS Inservice for Nursing Staff: Read it or download it and pass it on. Swelling of feet and legs: Why it happens and how to minimize it. Constipation: Prevention and treatment tips. Osteoporosis and Calcium in ALS: Why osteoporosis is "different" in patients with neuromuscular disease. BiPAP: A non-life support breathing assistance device. Muscle Spasms -- Cramping and Spasticity Which is it and how can it be treated? Travel Tips: Some things I have learned while traveling. The Attic: No practical tips here, just my response to a friend's question about what it is like to livewith ALS.

Another aspect of coping is knowing about assistive devices that can help compensate for weakened capacities. At http://living-with-als.org/

You can find reviews of such devices, as can be viewed on the homepage:

Bathroom Bedroom Call Systems Clothing Computer Eating/Drinking Entertainment Mobility Respiratory Speech &Communication Travel Miscellaneous	Many of the questions on the living-with-als group concern the equipment needed to deal with disability. This section of our website was set up to help people with ALS in their search for that equipment. Unlike other sites that provide a list of links to retailers and manufacturers, these pages are reviews of specific brands written by people who have purchased or tried them. The members of the living-with-als group hope this will help others find the best possible equipment to keep them living-well-with-als.
Computer Desk	Have you designed, built, or adapted something that makes living with ALS easier? Share your creative genius in the Do It Yourself (DIY) section.
PEG Tubes Buying a Van Buying a Wheelchair Hiring a Caregiver	ALS repeatedly presents challenges that leave us stranded in unfamiliar territory. Dealing with doctors, bureaucratic red tape, purchasing expensive equipment without knowing what options are really needed, picking our way through a financial minefield, as well as adjusting to disability is all new and frustrating. These Tip Sheets are intended to be a source of practical advice from the real experts -- PALS and CALS who have been there, done that, and are wearing the T- shirt that says "I wish I had known..."
Bathroom Design #1 Bathroom Design #2 AccessibleHalf-Bath	Few homes have been built with wheelchair accessibility in mind, so ALS often requires some degree of remodeling. Share your remodeling project with others here! Whether you want to share details such as floor plans, before and after, tips and tricks, advice on getting the job done, or just show off some pictures of the finished product, we'd love to have your input.

Chapter 3A

ESPECIALLY FOR CALS

CALS go through the same psychological stages as their PALS and, in addition, have the added responsibilities of caring for the needs of their loved one, taking on financial burdens and extra chores around the home. It can be overwhelming and CALS are always vulnerable to exhaustion and burn out. The following articles convey the strains felt by loving CALS, how to cope and how to take care of one’s own needs.

My Story

By Trish

My name is Trish Wilson and in my lifetime, have had many titles to my name, some being: sister, friend, girlfriend, wife, mom and now my newest title, CALS or for you not familiar with this new title, Caregiver of a person with ALS. Yep, Lou Gehrig’s disease. Never in my wildest dreams did I ever imagine that this would be a title I would have in my resume. When my wonderful husband Mike and I began searching for a diagnosis to a problem he was having with his speech, we had no idea what this search would lead to. We began as most of you will, with a simple visit to his family doctor. From there we were sent without diagnosis to a neurologist due to his tongue, speech and throat problems all along believing he had some growth in this throat that was tying up his nerves in his mouth and neck.. Diagnosis' were flying from friends and family only to make me a nervous wreck but having no affect on my husband still believing it was some kind of growth that we could have removed and move along with our lives. As we were put through an array of testing, none of which we were told why they were being done, I became more and more nervous every time they told me, "all of his tests are fine". I knew in my gut he wasn't fine and wanted them to find SOMETHING wrong. I know, it sounds insane, please find something wrong with my husband. I just wanted something to be found so we could "fix it" and get back to normal.Needless to say, after our months of testing, two months to be exact which I now find is a very fast time for diagnosis, the verdict was, "I think we are dealing with Lou Gehrig’s Disease" were the words from our neurologists mouth. I, of course, thought the man was a quack and after the initial shock, thought, I'll take this into my own hands. If this doctor thinks Mike has ALS, I'll show him. I'll go to an ALS specialist and prove he has no idea what he is talking about. So, we did. We found the best specialist in Arizona only to discover once they heard Mike was having swallowing problems, had lost over 35 pounds, felt paralysis in his tongue, they rushed us in to see the doctor. Well, my feeling of "that doctor was a quack" was becoming a nightmare. After seeing the ALS specialist the very next day, it was confirmed. Mike has ALS. He was now a PALS and I was now a CALS.Mike and I both have been through an array of emotions from extreme sadness, to denial, which comes often, to acceptance. We have not been through the anger simply because we do have an incredibly strong faith and believe God has chosen our path and this is a path we have to live out with dignity. It is my husband that shows me how to deal with this and it is my husband who is my hero for taking this on in such a brave and unique way. Don't get me wrong, we both dream of the days where "this can't be real" but for the most part, we are LIVING with ALS and plan to continue doing this and embracing life for as long as God will give us. ALS is not a death sentence, just a new way to appreciate your life.

CALS TO CALS

By Erin

Speaking CALS to CALS I want you to know you have every right to feel the way you do. You wouldn't be human if you didn't. Newly married or long time honeymooner's we all feel the loss, the frustration, the anger, the loneliness.You will go in and out of all the above emotions. It is natural; it is just the way it is.I have many years with my precious husband. But his progression was so fast we didn't have one, two, or more years to come to grips with ALS. If he could only do an nth of a fraction that some of our long time PALS here do, my husband and I would have the world.So CALS, cry if you want to. Be mad, frustrated. Let those feelings happen but then go look into your loved ones face, take their face into your hands and tell them "I Love You". Everyday do that. After a while more love and new memories will happen. You will always have the gamut of emotions, sometimes worse than other times. But you will have what time is given to you on this earth with each other.One thing for sure, you will learn the depths of love and compassion. The emotional roller coaster can't be described. The emotions and stress, the loss of living our day to day lives as we both knew it, as our children knew it, is over.Some PALS with a slow progression have lived a good life for years. Each CALS goes through a very isolated gamut of feelings and feels alone at times. We could each write our own book and many of us have website's and blogs to release our feelings, our progression, our journey. These sites and this site, "Living with ALS" are the greatest help there is. Take it from me. My husband’s progression was so fast we didn't have time to realize what was happening. It wasn't until he finally reached a plateau that we could learn about this thing called ALS.I never met anyone in an ALS group meeting that was at the stage of my husband. No help there. It was early on and I needed answers, I needed help. I would write to the ALS Digest and never get any answers. Very disappointing! It wasn't until I discovered the "Living with ALS" site that I began to get answers and discovered the most wonderful and compassionate people in the same boat as us. What a base of humanity this site is to find this place with PALS and CALS that know, that understand, that can cry with you, make you laugh with joy, allow you to express the love, the sorrow, the frustrations, the unknown future. We all do this together.I love you all.Erin CALS to Jeff

FROM ALSA’S INTERVIEW WITH SANDY

You and Bob continue to enjoy vacations. What kind of preparation is needed before, during and after travel with your husband?Travel requires a lot of planning to be successful. At each stage of Bob's illness, we have traveled, and the challenges increase as the illness progresses. However, the rewards are worth it! Initially we just needed a wheelchair to get us through airports and a roomier more comfortable car to rent at our destination. Now, we need a wheelchair accessible room and bathroom with guaranteed access to all public areas (make phone calls, and double/triple check!) We need to be sure that the restaurants in the area have food Bob can eat comfortably or we need a refrigerator so we can bring our own. We don't travel by air anymore - too many hassles with his power wheelchair being damaged or mishandled, but we do have a van that we are comfortable traveling in. We had an easy-lock device installed to make tie downs fast and easy, I pack a cooler with pureed foods for him, lots of water and syringes. Bob's computer goes on his chair up front with me, a good map and a guidebook of accessible trails and we are off. I have to remember to pack the charger for his chair, toilet necessities, pillows, extra clothes (he gets cold),a blender, all his medications/vitamins, emergency communication device(alphabet board) ,etc, and a smile. We have been very lucky and have found that getting away even for 2 days gives us both a change of scenery and renews our strength. We have seen some gorgeous mountain trails that are wheelchair friendly! Once we get back home it is important to give Bob a lot of down time in a comfortable chair, because one of the drawbacks of car travel is he is confined to his wheelchair all the time. How has Bob's diagnosis of ALS changed your life? In every way. It has narrowed the focus of life to its essentials - love and respect, and has deepened our love and commitment to each other. It also has me always alert (sleeping is a challenge), and trying to care for the details of someone else's life as well as my own is tough sometimes. With each change in Bob's condition, using the wheelchair, having to feed him etc., we have grieved the loss and struggled to handle it. I am, however, truly grateful for this time we have together, and am amazed at the grace with which he makes each adaptation. No matter how it has changed my life - his has changed a million times more! What does the word "Caregiver" mean to you? For me, it is a lot of responsibility with a lot of rewards. It is a chance to outwardly express my respect and love for my husband. When hiring outside help I ask for kindness, attentiveness, gentleness and respect for Bob and his changing needs. Knowing that Bob's mind is functioning at top speed, even though his body doesn't, is a challenge for some people. I need people who have the heart and inclination to get to know this wonderful man. As a primary caregiver, it is often a challenge to maintain facets of your own personal life. How do you maintain a sense of balance in your life?

Well, I didn't, initially. But now, we have hired outside help (no help from the insurance unfortunately) and I have re-established my friendships and work associations. Not knowing how long we have together has made me reluctant to miss a moment, but having help 5 days a week, 8 hours a day, makes the 16 hours a day I am Bob's caregiver now have more balance. I also realized early on that I would need guidance and I continue to see a counselor to help me deal with the incredible loss and grief we experience daily. I try to get a massage at least twice a month too!

LETTER FROM LINDA ALLEN

Linda is a former CALS and current Program Manager for Extra Hands for ALS. In this letter she describes the challenges and rewards of long distance travel with her husband, which personifies the idea of living fully with ALS, and also discusses the innovative Extra Hands program, which links young people with PALS and their families. Linda has given her personal email address and is willing to share her knowledge about travel.Also check out the Extra Hands web site for a moving description of the Program and its origins.

http://www.extrahands.org/linallen@swbell.net(email)and Linda@extrahands.org My husband Marshall was dx. Oct 31, 1996 at the age of 54 and earned his wings on Oct.27, 2000.You are right when you say, “do all the things that you have planned on doing as soon after dx”.We decided early on that we were going to continue to live our lives despite ALS.We traveled by air, ship, bus, train, van, with the Permobile wheelchair.(Europe AFOs), US and the Caribbean with wheelchair), we went to restaurants, the theatre, sporting events, to visit grandchildren etc.We learned to expect there would be road blocks a long the way but we believed that we could navigate around them.We had some funny stories surrounding our adventures, others that brought us to tears; times when we educated management/housekeeping staff/ about things they could do to make their establishments more handicap accessible, and coming up with innovative ideas to solve a problem when we could not find a resource/solution for.Was it easy “no it was not” was it worth it…you bet!I have so many wonderful memories because we decided to continue to live life and so do our children, his two sisters/brother-in-laws, my family and friends. I promised Marshall that after he was gone that I would continue in the fight against ALS until there was a cure. I am keeping that promise by working with Extra Hands for ALS.I hope we can reach all our PALS/CALS in every area in every state with this program.What better way of spreading the word about ALS than through young people who will be our doctors, researchers, lawyers, congressmen/women, etc. ALS families have the opportunity to teach these young people about ALS and learn about the disease first hand. The “extra hands” do household chores, yard work, playing with the children, providing company to the PALS, whatever the ALS family needs done that will take some of the burden off the shoulders of the caregiver.Instead of doing what needs to be done the caregiver can read, catch up on phone calls, work in their shop, work on a hobby, spend quality time with the family while the students are working etc.These students learn so many life lessons that they would never learn if they had not been involved with an ALS family.Some PALS/CALS have had such an influence in the lives of the students that some have decided to take up the same career as their PALS or CAL, or by learning about ALS have decided to become a doctor, physical therapist, speech pathologist etc.Also these kids let their families/friends know what they are doing and teach them about ALS.The family members tell other family members, colleagues, neighbors about what their kids are doing….more Awareness of ALS.It is my hope that we can find teams of individual volunteers that would like to get involved and start this program in their area. It takes a group of interested people, ALS families that will use the service and a source in the community that will help to fund the program.

TEN TIPS FOR FAMILY CAREGIVERS

1. Choose to take charge of your life, and don't let your loved one's illness or disability always take center stage.

2.Remember to be good to yourself.Love, honor and value yourself. You're doing a very hard job and you deserve some quality time, just for you.

3. Watch out for signs of depression, and don't delay in getting professional help when you need it.

4. When people offer to help, accept the offer and suggest specific things that they can do.

5. Educate yourself about your loved one's condition. Information is empowering.

6. There's a difference between caring and doing. Be open to technologies and ideas that promote your loved one's independence.

7. Trust your instincts, most of the time they'll lead you in the right direction.

8. Grieve for your losses, and then allow yourself to dream new dreams.

9. Stand up for your rights as a caregiver and a citizen, especially when dealing with public agencies.

10. Seek support from other caregivers. There is great strength in knowing you are not alone.

CHAPTER 4

MAJOR ORGANIZATIONS AND WEBSITES

The first website to look at is the Living with ALS group and the link to join is: http://health.groups.yahoo.com/group/living-with-als/messages

This forum has members who are exclusively PALS and CALS and who range from newbies to 20 year veterans.Posts cover a wide array of topics relevant to ALS, e.g. coping strategies, personal issues/problems, symptom control, Medicare coverage, equipment, just letting off steam, etc. Really, anything at all that involves ALS. Practical and helpful information is offered, often more useful than what professionals can provide. Members are enormously supportive, caring, wise and knowledgeable. Many friendships are formed between members. Examples of a page of posts are given below:

Subject Author Date 32205 Re: burning feetAre you taking any kind of blood thinner? Have you had a doppler study done? I... Danny Oct10,2005 10:32 am 32206 Re: psychological impactI am sorry Fern that more people are not coming to see you. When I was told I ... COOKIEDD Oct10,2005 10:33 am 32207 Re: Moms in the hospital again.she has no gall bladder, no appendix, no uterus, etc. they have had to give her... Mr. and Mrs. Travis S... Oct10,2005 10:34 am 32208 Re: Nursing home Thom, Fern and others Here is the starting page on starting a search and how... Sherry Oct10,2005 12:41 pm 32209 Re: new pulse ox neededLinda I paid $199 for mine. I wouldn't pay any less. http://www.savelives.com/... Sherry Oct10,2005 12:41 pm 32210 Re: Heart Race problemDanny You state the oxygen sat rate isn't affected by the BiPAP. Here is a... Sherry Oct10,2005 12:43 pm 32211 Re: One Person Home CareJim, Our stories are amazingly similar including the size differential and the ... Jeff Lester Oct10,2005 3:06 pm 32212 Re: BiPAP Mark & LarryDear Lisa, I don't know if anyone else has mentioned this, but is it possible... Edith Oct10,2005 3:30 pm 32213 Re: One Person Home CareJim, I like your innovative approach and determination. Can you explain what... Randy Roberts Oct10,2005 4:35 pm 32214 Re: Crazy Week ... of a ... say ... chair ... I know Susan, i wanted to hide LOL! Things are... Oct10,2005 6:57 pm 32215 Re: speech Oct10,2005 7:00 pm 32216 (No subject) I was just wondering if anybody has a problem with waking up and the covers ... murrza Oct10,2005 7:01 pm 32217 Re: Nursing homeA good place to find your local resources for possibly living at home is your... Oct10,2005 7:40 pm 32218 Re: One Person Home CareHi Randy, I was referring to a system with a overhead hanging container... Jim Oct10,2005 9:26 pm 32219 peg tube stopped thanksThanks to all who answered my email. My hubby is calling the gastro doc ... Debbie Oct10,2005 9:54 pm 32220 Re: One Person Home CareAllison, Jim and Jeff, THANK you for sharing your stories with us on this... Susan Oct10,2005 9:57 pm 32221 Re: Nursing Home vs. In-home CareHi all, I have read a few of the postings about the dilemma about care at... STOPALS Oct11,2005 2:18 pm 32222 comfort curveI need to hear more feedback on the Comfort Curve mask. Hospice won't pay for... Sherry Oct11,2005 2:22 pm 32223 Flu Shots for People with ALS This is to remind PALS (people with ALS) it's time for your annual flu shot.... Oct11,2005 5:15 pm 32224 Clinic TrialI went to my ALS clinic on Monday. They gave me my FVC breathing test and they... COOKIEDD Oct11,2005 6:31 pm 32225 Re: comfort curveSherry, When I first got the Comfort Curve, I wore it only in bed with my... Randy Roberts Oct11,2005 6:33 pm 32226 Re: re [living-with-als] visionOn Thu, 8 Sep 2005 14:22:19 -0700 (PDT) Erin Smith <erinzinn5@...> ... ... almanna Oct11,2005 8:17 pm 32227 Re: Friends ~ Where Are They?Dear Sue, I can relate to that. Hang in there. God will send new angels. Andi... almanna Oct11,2005 8:17 pm 32228 Re: I'm new herewelcome Connie! Andi ... On Wed, 07 Sep 2005 06:57:09 -0000 "Connie ODonnell"... almanna Oct11,2005 8:17 pm 32229 Re: comfort curve Randy Thanks so much. I only have my bed on a partial turn. I couldn't handle... Sherry Oct11,2005 8:18 pm 32230 ALS Registry Act Introduced in U.S. House of Representatives2005 Advocacy Update October 11, 2005 ALS Registry Act Introduced in U.S. House... lou_gehrig_054 Oct11,2005 8:19 pm 32231 Beds that turn youMy name is Bill and this is my first post. I have been listening in for about... netravelingman Oct12,2005 9:04 am 32232 Re: Nursing Home vs. In-home Care Does your ride for life support your state only? In Calif, I only get 1500 ... rovall Oct12,2005 9:05 am 32233 Re: Friends ~ Where Are They? Dear Sue, Sorry to hear that John's Mom and sister haven't contacted you. It's... COOKIEDD Oct12,2005 10:00 am

The reader just clicks on the first few lines of the post to get the complete post. You can use a search feature to get back posts on a subject of special interest. This site provides a window into the world of ALS and can be inspiring, touching, informative, wise and loving. There is also a chat room led by member of the forum and can be accessed at: http://www.mdausa.org/chat/calendar.html#lalsLocate the Living with ALS chat and sign in as a visitor. Chats are held 2-5 pm on Sundays, 3-6 pm and 9-11 pm on Mondays and 7-10 pm Wednesdays. All times listed are Central time. Transcripts of past chats are on file. Another ALS peer forum, Brain Talk Communities, is also quite good. Its strength lies in its many posts on current research in the field and its section on personal experiences with different treatments. The link is:http://brain.hastypastry.net/forums/forumdisplay.php?f=82The ALS Association (ALSA)is the only national not-for-profit health organization dedicated solely to the fight against ALS. ALSA covers all the bases - research, patient and community services, public education, and advocacy - in providing help and hope to those facing the disease. The mission of The ALS Association (ALSA) is to find a cure for and improve living with amyotrophic lateral sclerosis.

Its website is chock full of information in every area related to ALS. The link is:

http://www.alsa.org/default.cfm?CFID=897541&CFTOKEN=9724212

The home page looks like this (below is a photo you can’t click on it)

As you click on the blue headings, subtopics pop up on every aspect of ALS. For comprehensive information on any or all areas related to ALS, this should be your first stop. The amount of information is awesome and can be overwhelming at first, but will become easier with practice.

“The Muscular Dystrophy Association is a voluntary health agency- a dedicated partnership between scientists and concerned citizens aimed at conquering neuromuscular diseases….MDA combats neuromuscular diseases through programs of worldwide research, comprehensive medical and community services, andfar-reaching professional and public health education.”MDA has an ALS division whose website is very informative. It provides research updates, a newsletter, interesting articles and very useful information. As organizations, there is much overlap between the two. Both offer funds for research and patient services, such as advocacy and support groups.MDA offers funds to help pay for some or all of the cost of certain medical equipment, such as wheelchairs and seat cushions, computers with speech synthesizers, etc. Local offices of both ALSA and MDA have loan closets from which you can borrow durable medical equipment of all sorts, including some your insurance will not cover. Contact your local offices of both ALSA and MDA, which you can locate on their websites, for more information. MDA has more dollars for research and services/DME, while ALSA focuses more on advocacy atthe national level.

It can be reached at: http://als.mdausa.org/

MDA’s ALS home page looks like this: (also a photo only, not a live web page):

The ALS Therapy Development Foundation is a nonprofit biotechnology company discovering treatments for patients alive today.Their laboratory, the leading drug discovery program for ALS, bridges a critical research gap.In-house expertise translates research into potential drug candidates by screening drugs in the SOD1 mouse model of ALS. Their scientific collaborations are designed to bring the most promising leads closer to patient use.ALS-TDF shares emerging knowledge on the disease with patients, physicians, and researchers as quickly and comprehensively as possible. Their unique approach accelerates drug development for ALS. In addition, ALS-TDF offers a forum for discussion of research issues. The link is:http://www.als.net/forum/Ride for Life is an organization directed by a PALS that raises money for research and patient services from various events, including their “Ride for Life”, a yearly event. Their website is very worthwhile to view. It includes a number of personal stories, research findings, information on grants and other material of interest to PALS/CALS. The link is:Ride For Life Helping People Living With ALS - Lou Gehrig's DiseaseThe above are a few of my favorites. There are hundreds of interesting and worthwhile websites that you can choose.

Below is a list of websites that are comprehensive and far reaching:

	ALS and Associated Web Sites Compiled By: Steve Weekes ( )
	ALS INFORMATION WEB SITES ALS SOCIETIES, SUPPORT GROUPS & PUBLICATIONSALS Association Chapter WebsitesALS Related Societies ALS SOCIETIES AROUND THE WORLD(Over 40 Countries Represented) ALS CHAT ROOMS ALS FORUMS and DISCUSSION GROUPS CAREGIVER WEBSITESTravel AssistanceCaregiver Chat Rooms, Forums and Discussion Groups PALS / CALS - PERSONAL WEBSITESInternational PALS / CALS Personal Web Sites MEDICAL SEARCHES, INFORMATION & JOURNALSLyme and ALS Associated DiseasesAdvanced Therapies, Treatments and ResearchGeneral Health Care and Information ASSISTIVE AIDS, DEVICES, and SOFTWAREGeneral Assistance SitesCommunication Assistance and SupportComputer Adaptive Devices & EquipmentWheel Chairs, Scooters & VehiclesSpecific Assistance SitesMind and Spirit Resources NATURAL & SUPPLEMENTARY THERAPIESDiagnostic LaboratoriesNon-Conventional Doctors, Clinics, Treatments, Therapies & Theories PRODUCTS,SUPPLIERS, ETC...

CHAPTER 5

BENEFITS AND ENTITLEMENTS

As PALS and CALS, it is vitally important that you learn about your government benefits: disability payments and Medicare/Medicare. It is also vital that you learn to deal effectively with your insurance companies. What follows are some articles to help you get started.

SOCIAL SECURITY BENEFITS

By Danny Dandignac

For the full version click here

To download the full version in Microsoft Word right click here choose save target as

How do we decide if you are disabled?

SSDI & SSI The process we use to decide if you are disabled involves five steps. They are: 1.Are you working? If you are working and your average monthly earnings, after considering the effect of work incentives, are at the Substantial Gainful Activity (SGA) level, we generally cannot consider you disabled. If your monthly earnings average less than the SGA level, (in 2006 that level was $860 per month) we look at your medical condition using steps 2 through 5. 2.Is your medical condition "severe"? For us to consider you disabled, your impairment(s) must significantly limit your ability to do basic work activities, for example walking, sitting, seeing, and remembering. If it does not, we cannot consider you disabled. If it does, we go to the next step. 3.Is your medical condition in the list of disabling impairments? We maintain a Listing of Impairments for each of the major body systems that are so severe we automatically consider you disabled. If your medical condition(s) is/are not on the list, we have to decide if it is of equal severity to an impairment on the list. If it is, we approve your claim. If it is not, we go to the next step. 4.Can you do the work you did previously? If your medical condition is severe, but not at the same or equal severity as an impairment on the list, then we must decide if you can do your past relevant work. If you can, we will deny your claim. If you cannot, we go to the next step. 5.Can you do any other type of work? If you cannot do your past relevant work, we then see if you are able to do any other type of work. We consider your age, education, past work experience, and transferable skills. If you cannot do any other kind of work, we will approve your claim. If you can, we will deny your claim.

FURTHER INFORMATION ON SOCIAL SECURITY BENEFITS

(TAKEN FROM THE REGULATIONS)

By Randy Roberts

Find out if you're eligible for Social Security Benefits

Benefit Eligibility Screening Tool (BEST) Use our screening tool to help identify all the different Social Security programs for which you may be eligible.

Overview We pay disability benefits under two programs: the Social Security disability insuranceprogram and the Supplemental Security Income (SSI) program. If you qualify, apply for Social Security disability benefits online. No matter what kind of disability benefits you are applying for, you must give us information about your medical, work, and education history to help us decide if you are disabled.

• Note: If you're an Advocate, Attorney or Third Party Representative, we need additional information from you on the application.

Detailed information about each of these programs is available at the following websites:

• Social Security Disability Program
• Supplemental Security Income Program

You don't have to be disabled or blind to collect Supplemental Security Income (SSI) if you are 65 or older.For most people, the medical requirements for disability payments are the same under both programs, and your disability is determined by the same process. Use our Disability Planner to find out medical and earnings requirements, what happens once you’re approved, and more.Our calculatorscan give you an estimate of disability benefit amounts based on your record if you should become disabled. If you get Social Security (not SSI) disability benefits you possibly could be eligible for Medicare. SSI is a program run by Social Security that pays monthly checks to the elderly, the blind and people with disabilities who don't own much or who don't have much income. If you get SSI, you usually get food stamps and Medicaid, too. Medicaid helps pay doctor and hospital bills.While eligibility for Social Security disability is based on prior work under Social Security, SSI disability payments are made on the basis of financial need.

Who Can Get Social Security Disability Benefits

Children may qualify for disability benefitsunder either the Social Security program or the SSI program.You can get Social Security disability benefits until age 65. When you reach age 65, your disability benefits automatically convert to retirement benefits, but the amount remains the same. Certain members of your family may qualify for benefits on your record. They include:

• Your spouse who is age 62 or older, or any age if he or she is caring for a child of yours who is under age 16 or disabled and also receiving checks.
• Your disabled widow or widower age 50 or older.
• Your unmarried son or daughter, including an adopted child, or, in some cases, a stepchild or grandchild. The child must be under age 18 or under age 19 if in high school full time.
• Your unmarried son or daughter, age 18 or older, if he or she has a disability that started before age 22.

If you become the parent of a child (including an adopted child) after you begin receiving Social Security benefits, be sure to notify us so that we can determine if the child qualifies for benefits. For more information about disability benefits for children, ask Social Security for the booklet, Benefits for Children With Disabilities (Publication No. 05-10026).Note: The SSI program also pays benefits to needy disabled children under age 18.

How to Apply for Disability Benefits

Apply as soon as you become disabled. You can file:

• Online using our Internet Social Security Benefit Application
• By phone, mail or in person at any Social Security office. Call for an appointment.

Note: You may receive back benefits from the date you became disabled, but they're limited to one year before the date you filed for benefits.

How to Speed Up Your Disability Claim

It generally takes from 3 to 5 months to process claims for disability benefits. You can help shorten the process by bringing certain documents with you when you apply and helping us to get any other medical evidence you need to show you are disabled. These include:

• Your Social Security number;
• Your birth or baptismal certificate;
• Names, addresses and phone numbers of the doctors, caseworkers, hospitals and clinics that took care of you and dates of your visits;
• Names and dosage of all the medicine you take;
• Medical records from your doctors, therapists, caseworkers, hospitals, and clinics that you already have in your possession;
• Laboratory and test results;
• A summary of where you worked and the kind of work you did; and
• A copy of your most recent W-2 Form (Wage and Tax Statement) or, if you are self-employed, your federal tax return for the past year.

You also should be ready to answer other questions we must ask. Don't delay filing for benefits just because you don't have all the information and documents you need. The people at the Social Security office will be glad to help you.

Who Can Get SSI Benefits

To get SSI benefits, you must be elderly or blind or have a disability.

• "Elderly" means you are 65 or older.
• "Blind" means you are either totally blind or have very poor eyesight. Children, as well as adults, can get benefits because of blindness.
• A disability means you have a physical or mental problem that is expected to last at least a year or result in death. Children, as well as adults, can get benefits because of disability.

You must live in the U.S. or Northern Mariana Islands and be a U.S. citizen or national. (Certain non-citizens also may be eligible for SSI. A Social Security representative can tell you if you qualify.) Also, the things you own and your income must be below certain amounts. See our publication on Supplemental Security Income (SSI) (Publication No. 05-11000) for details about the income limits.

How Much You Can Get from SSI

The amount of your benefit depends on where you live. You could get more if you live in a state that adds to the SSI check. Or you could get less if you or your family has other money coming in each month. Your living arrangements also make a difference in whether you can get SSI and the amount you can get.

MEDICARE

By Andy Etherington

Andy was diagnosed in 2002 and is now trached and vented. He is 45, married and has two young children. Formerly, he worked in the telecommunications industry, writing software and working with customers.I will try to help as much as I can. Note that I am not an expert or a lawyer, just an affected person with a vested interest in this topic, and have been studying these issues for the last six months. So everything below here is my interpretation of the issues, and may not be correct.We will start with some definitions. Medicare - coveragefromthefederalgovernment that you are entitled to. There are 3 PartstoMedicare. Do not confuse the parts of Medicare with the supplement plans described below, they are vastly different. Part A coversmosthospitalizationcosts,exceptfora deductible of $952foreachhospital stay of less than 61 days. If you stay for more than 60 days, there is a co-insurance of $238/day for the next 30 days. After 90 days it goes up to $476/day through day 150. As near as I can tell, no one knows what happens after day 150. Days 91-150 are referred to as lifetime reserve days, and can only be used once. Note also that if you are discharged from the hospital after less than 60 days, and are re-admitted again less than 60 days after your initial admission, it counts as the same hospital stay. You are not charged the deductible again, and counting of days does not start over. Part A costs you nothing extra, it is funded through a 1.45% payroll tax on all workers. Part A is not optional, but also costs nothing. Part B coversmostoutofthehospitalexpenses,usuallyatthe 80%/20%rate.Thisincludesdurablemedicalequipment (DME)suchasventilators,cough assistmachines,etc.thisispaidforthrough adeductionof $88.50permonthfromyourSSDIcheck. This part is optional, but be careful. If you don't sign up for it when you become eligible, you must wait until an open enrollment period (usually January 1-February 28). For every year you are eligible for part B, but decline coverage, you are subject to a 10% penalty for each year that you declined coverage. This penalty is cumulative, so if you had declined for 2 years, and then enrolled this year, instead of paying $88.50 per month, you would pay $106.20 per month. Furthermore, if the Medicare rate goes up by $10 next year, yours would go up by $12, and would continue to rise 20% higher than the standard each year. There is an exception to this, however. If you are covered by an employer sponsored group health plan, either through your employer or your spouse's, and the person whose coverage you are under is considered to be an active employee, even if you're not being paid, you have what is called a special enrollment period.You may enroll in Part B without penalty any time while you're still covered by the group health plan, or during the 8 months following the loss of that coverage. Note that COBRA coverage is provided to people whose employment has terminated, and does not provide or extend a special enrollment period. Part D– the infamous prescription drug program. This is a program that is provided through private insurance companies to help with the cost of medicine. This part is also optional, but again, be careful! If you don't sign up for one by May 15, you are again subject to penalties. This time it is 1% per month for each month you are eligible, but choose not to participate. There is, once again, an exception to the penalty. If you have been covered by a prescription plan from your company or union, or their retirement program (I am interpreting this to include COBRA), and it provided equivalent or better coverage than Part D, you may join without penalty. Part D plans cost anywhere from $0-$100 per month, depending on the company. Each plan has it's own formulary, or list of covered drugs. If you take prescriptions that are not on the formulary, not only do you pay full price for it, but the amount you pay does not count toward your Part D deductible or out of pocket maximum. So your best bet is to find a plan that covers your high cost medications, and pay for the less expensive medicines yourself. I also dropped rilutek, because it not only expensive, but even on plans with it on the formulary, because limits are based on the full price of the drugs, it was going to increase my out of pocket cost by over $1000/year. Since I'm already on a ventilator, none of my doctors could (or would) give me a recommendation. If you have trouble understanding the Part D limits, I have another document that describes it in more detail.

You are eligible for these,butnotrequiredto accept them(see above) as of the date you became eligible for social security disability.Mostpeopleinthesocialsecurityofficewilltellyouthatyou'renoteligibleuntil 24monthslater.These people are wrong! The law was changed three years ago; the word just hasn't trickled down to the local offices yet. You also cannot be denied Medicare because of your ALS diagnosis! That is what it's there for. Find more information at www.medicare.gov Medicare supplements - standardized plans, labeled A-J, often called Medigap plans, are offered by private insurance companies to cover the perceived "gaps" in Medicare coverage. Each company offering a Medicare supplement is allowed to charge whatever the market will bear, but unlike the prescription program, supplement policies are required to provide standard coverage. Thus company 1 can provide a plan A supplement (not the same as Part A above) for $59/ month; while company 2 has a plan A supplement for $150/month. By law, the coverage provided by both companies must be exactly the same, despite the wide range of costs. In Texas, I am only guaranteed to be able to obtain a supplement plan A. when I look at the Medicare web site, I can find at least 20 companies willing to sell a plan A to me, but only 2 say they would sell me a plan D policy, and they are such small players that they don't even have web sites! Looking in Indiana, I only see one company offering a plan A supplement for under 65 (Anthem), and only one willing to sell a plan B (Bankers Fidelity). There were none selling any other supplement policies to people under 65. That would imply to me that, with no competition for either company, prices will be high. It may be worth it though. Here is my take on what they provide: Plan A supplement – covers the Part A co-insurance (not the deductible, see above Part A discussion), and the Part B co-insurance. Part A deductible is $952 for each hospital stay, but doesn't reset until you've been out for 60 days, so it is physically impossible to incur more than 6 of these per year. Plan A supplement also adds 365 additional lifetime reserve days. The Part B co-insurance is the 20% that Medicare doesn't cover for outpatient things like doctors' visits, outpatient surgery, and DME. However, there is a comment, in the home health services section, that says you pay 20% for DME, so I am not sure whether ventilator, cough assist, etc. would fall in this category or not. Plan B supplement – the only thing the Plan B supplement adds is coverage for the Part A deductible (that $952 payment for each hospital stay). Everything else under Plan A supplement is the same under Plan B.I know that during your initial enrollment period, they are not allowed to "rate" or reject you based on pre-existing conditions (your ALS, for example). I don't know if they are allowed to use underwriting (underwriting: using current or past medical conditions to charge higher rates or reject applicants) if you're outside your initial enrollment period. I may be finding out soon, I'll let you know. Medicare Select – really a misnomer, because it applies only to the supplement policies above, it provides the same coverage as the supplement plan it refers to (i.e. Medicare Plan A Supplement Select), but only if you use their doctors and facilities. Kind of like a Medicare HMO. A moot point, because the Medicare site doesn't list any that will sell a select policy if you're under 65. Medicare Advantage – you didn't mention these, but I'm sure you've heard of them. Around here we see ads for these 40 times a day, mostly during business hours. These plans completely replace Medicare and supplement plans. If you choose to use a Medicare Advantage plan, you will be unenrolled in Medicare! Every Medicare Advantage plan has different terms and conditions. It's just like shopping for any other health insurance. I don't know if any underwriting is involved, but it was never mentioned during my investigation of the subject. If you decide to go this way (I decided not to), there is one more thing to know: there are two kinds of Medicare Advantage programs. Those that cover prescriptions and those that don't. Consistent with the idea that you are out of the Medicare system if you enroll in a Medicare Advantage plan, if you choose a Medicare Advantage plan that does not cover prescriptions, you will not be allowed to join a prescription only drug plan, and you will not be able to get prescription coverage!

Medicaid – is a state-run program, primarily intended for low-income patients. There are generally both income and asset limitations for qualifying. States set their own limits, so they are different everywhere. I have found anecdotal evidence that Indiana has their limits set to $1273/ month in income, and $2000 in total assets (excluding certain items like house, 1 car, etc. for a still living spouse). My SSDI payment is more than that, so I have not pursued it. There are ways to get within these limits, but I am not familiar with them. I would recommend that you consult an attorney that specializes in elder care law for additional information if you're thinking of going this route.

Risk pool – I don't know if Indiana has this concept, but in Texas we have a Risk pool for individuals that are unable to get health insurance otherwise. An ALS diagnosis automatically qualifies me for the pool. There is no underwriting, but they are required to charge twice the going rate for the opportunity to be in the risk pool. This policy (again, this is my interpretation of how it works in Texas, Indiana may be different, or may not even have a Risk pool) pays as secondary to Medicare.See the discussion below for primary vs. secondary. Another thing to think about is respiratory therapy. Medicare does not cover respiratory therapy visits. The Risk pool does. That hasn't been enough, in my case, to justify going this route yet. One more hitch, I cannot join the Risk pool as long as I am eligible for COBRA, even if I don't take it. Primary vs. secondary – this is an issue when a patient is covered by 2 different insurance policies, and is called coordination of benefits, or COB. This can be caused by numerous things, like the Risk pool example above, or group coverage from a spouse's employer. The way COB works is that one policy gets the claim first, and forwards it to the secondary after paying their portion. The secondary then processes the remainder of the claim according to their policy. There are 2 ways I have heard this works. The more favorable way, which is what I've experienced, the primary pays 80%, then the secondary pays the rest, up to 80%. It's strange to think about, so here is an example:My respiratory company charges $590/ month for my cough assist machine. Medicare gets the claim, and allows $400 (all these figures are hypothetical). My provider accepts assignment, so they are happy with $400. Medicare then pays 80% of it, or $320.Now they pass the claim to my secondary. My provider is out of network for them, so they would normally only pay 70%, or $280. But Medicare already paid $320, so the secondary pays $80, and counts themselves lucky, because they paid $200 less than what they are contractually obligated to pay.Obviously this is advantageous to me, as I end up paying nothing. The other way that I have heard, but not experienced, works like this:Same scenario as before, company bills $590, Medicare allows $400, and pays $320. But now the secondary gets it. They look at it and say "we would have paid $280 for this service. Medicare has paid $320, so we don't have to pay anything."I'm not nearly as happy, because I'm stuck with the $80 remainder, and am paying premiums for a policy that never seems to pay out. Again, I have not experienced this happening, but others on this board have reported it. How do companies decide which is primary? In my case, Medicare became primary because I wasn't being paid, thus no FICA taxes were being paid. If my coverage had been through my wife's employer, and she was still having FICA withheld, Medicare would be secondary. Others have said it has more to do with the number of employees being greater or less than 100, but I'm more inclined to believe mine, since I heard it from the COB desks at both plans. Medicare capped item - One more thing that you may want to know about, especially if you're thinking about buying your equipment supplier out, is the idea of Medicare capped items. Many of the things we use are actually rented by Medicare. After 10 months of renting the equipment you are given a choice to buy it or keep renting it. If you choose to buy it, Medicare will pay for 2 more months (total of 12 months). Title to the equipment goes to you, and no more payments are due, but you are responsible for upkeep. If you choose to continue to rent, Medicare will continue to pay for 5 more months (total of 15 months). Title to the equipment remains with the equipment supplier, no more payments are due, and the supplier is responsible for upkeep.Now to the answer that you didn't want, but I'm sure you knew was coming. No one can make the decision for you. It is highly dependent on your financial position and your risk tolerance. I would strongly recommend that you talk to a specialist in elder care law before making a final decision.I will tell you what my decision was. The gentleman that was here specifically to sell me a Medicare Advantage plan, told me to stay away from them because they weren't very good with DME extensive conditions, of which ALS is certainly one. So I have decided that, when they kick me off COBRA, I am going to immediately apply for a Medicare Part D plan and a Plan A supplement. Good luck with your research. If you have any other questions, feel free to ask. Just be forewarned: you may get another tome like this one!

MORE ON MEDICARE PART DPRESCRIPTION COVERAGE

By Andy

Let me try to summarize it for you. Each state has several companies that offer Medicare Part D coverage. each of these companies offers several plans, with different premiums, different deductibles, and different coverage. All this adds up to an overwhelming amount of information. In Texas I had 48 different plans that I could choose from. Every prescription drug plan has a formulary, which lists the drugs they will cover, and the (up to 4) different prices that they will charge for each drug. There are 4 different levels of support included in each plan: Deductible

The first $250 dollars of drug expenses are paid for out of pocket at full price. Some plans have no deductible, and start coverage at the next level. SupportAfter the deductible is met, drugs can be obtained for either 25% or a fixed co-payment. Each formulary uses a different base price for each drug, though formularies within the same plan family tend to use the same price structure (Humana's prescription drug only plans uses the same prices as the Humana Medicare Advantage plans with drug coverage). Once you reach $2250 in total drug costs (what you pay plus what the plan pays), you move to the next level Donut hole. This is the part that is most confusing. Between the time that you reach $2250 in total drug costs and when you reach $3600 in out of pocket expenses, you pay 100% of the drug prices. Did you get that? That means that, between $2250 in total drug costs and $5100 in total drug costs you pay 100% of your prescription costs. Still with me? Ok, one more thing.After you've paid $750 for your prescriptions ($250 deductible plus 25% of the next $2000), and before you've paid $3600 in out of pocket expenses, you pay full price for all your medication. That's all the ways I can think of to explain it. Catastrophic coverageOnce you exceed the threshold of the donut hole, you get catastrophic coverage, under which you pay the greater of $5 ($2 for generics) or 5% of the full price. For complete information on all aspects of Medicare benefits, always go to

http://www.medicare.gov/

CHAPTER 6

PARTICIPATINGIN CLINICAL TRIALS

By Randy Roberts

Clinical trials are formal tests of safety and efficacy of new drugs or procedures. There are three phases in trials:

1) Safety- to assess if the new compound can be tolerated without major side effects. 2) Safety and efficacy-involving more subjects, including a placebo group (no medication) or so called “control” group; 3) Efficacy- The final phase-to determine efficacy using a much larger sample size and held at multiple sites around the country, always uses a placebo group.

All drugs used in the trials are free of charge. It is important to consider participating in a trial at an early point in your illness because the studies require subjects to meet certain criteria, e.g., FVC > 60 or 70 or the ability to walk unaided, and one never knows how fast one will progress.PALS have to weigh potential benefits against inconvenience, as the trial location may be many miles from home. And PALS who volunteer are helping in the quest for a cure. Discuss the open trials with a neurologist, to help guide your decision. Below is a current list of trials that are recruiting patients, as of 11/06. Check back at the website below monthly to get updates.The ALSA and MDA sites also keep updated lists of trials, so check these, too.

Click the Logo above to

see the ClinicalTrials.gov

web site.

1.	Recruiting	Pilot-Study of Thalidomide in Amyotrophic Lateral Sclerosis (ALS)Condition: Amyotrophic Lateral Sclerosis (ALS)
2.	Recruiting	Clinical Trial Ceftriaxone in Subjects With ALSConditions: Amyotrophic Lateral Sclerosis; ALS
3.	Recruiting	Amyotrophic Lateral Sclerosis Web Based Patient Care Database: ALSConnection.OrgCondition: Amyotrophic Lateral Sclerosis
4.	Recruiting	Study of Myobloc in the Treatment of Sialorrhea (Drooling) in Patients With Amyotrophic Lateral Sclerosis (ALS)Conditions: Amyotrophic Lateral Sclerosis; Sialorrhea
5.	Recruiting	Genetic Epidemiology of Familial Amyotrophic Lateral Sclerosis (ALS)Condition: Amyotrophic Lateral Sclerosis
6.	Recruiting	Amyotrophic Lateral Sclerosis (ALS) Veterans RegistryCondition: ALS
7.	Recruiting	Efficacy and Safety Study of MCI-186 for Treatment of Amyotrophic Lateral Sclerosis (ALS)Condition: Amyotrophic Lateral Sclerosis (ALS)
8.	Not yet recruiting	Clinical Trial of Glatiramer Acetate in Amyotrophic Lateral Sclerosis (ALS)Condition: Amyotrophic Lateral Sclerosis
9.	Recruiting	Phase II Study Using Thalidomide for the Treatment of ALSConditions: Amyotrophic Lateral Sclerosis; ALS
10.	Recruiting	Early Treatment of Amyotrophic Lateral Sclerosis (ALS) With Nutrition and Non-Invasive Positive Pressure Ventilation (NIPPV)Condition: Amyotrophic Lateral Sclerosis
11.	Recruiting	Noninvasive Ventilation in ALS Patients With Mild Respiratory InvolvementCondition: Amyotrophic Lateral Sclerosis
12.	Not yet recruiting	Clinical Trial of Vitamin E to Treat Muscular Cramps in Patients With ALSCondition: Amyotrophic Lateral Sclerosis
13.	Recruiting	Trial of Sodium Valproate in Amyotrophic Lateral SclerosisCondition: Amyotrophic Lateral Sclerosis
14.	Recruiting	Mitochondrial Functions and Oxidative Stress in ALS PatientsCondition: Amyotrophic Lateral Sclerosis
15.	Recruiting	Frontotemporal Dementia and Amyotrophic Lateral SclerosisConditions: Frontotemporal Dementia; Amyotrophic Lateral Sclerosis; Dementia
16.	Recruiting	The Effect of Intensive Controlled Exercise in the Early Stages of Amyotrophic Lateral SclerosisCondition: Amyotrophic Lateral Sclerosis
17.	Recruiting	Combination Therapy Selection Trial in Amyotrophic Lateral SclerosisCondition: Amyotrophic Lateral Sclerosis
18.	Recruiting	R(+) Pramipexole in Early Amyotrophic Lateral SclerosisCondition: Amyotrophic Lateral Sclerosis
19.	Recruiting	Familial Amyotrophic Lateral Sclerosis: A Preliminary Study to Identify Subjects Who Might Be Willing to Participate in a TrialCondition: Amyotrophic Lateral Sclerosis
20.	Recruiting	Memantine for Disability in Amyotrophic Lateral Sclerosis (MEDALS)Condition: Amyotrophic Lateral Sclerosis
21.	Recruiting	Confirmation of Self-Reported Amyotrophic Lateral Sclerosis Cases in the AARP-Diet and Health StudyCondition: Amyotrophic Lateral Sclerosis
22.	Recruiting	Amyotrophic Lateral Sclerosis and Frontotemporal DementiaConditions: Frontotemporal Dementia; Lateral Amyotrophic Sclerosis
23.	Recruiting	Brain Function in Primary Lateral Sclerosis and Amyotrophic Lateral SclerosisCondition: Cerebral Cortex Dysfunction
24.	Recruiting	Levetiracetam for Cramps, Spasticity and Neuroprotection in Motor Neuron DiseaseConditions: Motor Neuron Disease; Amyotrophic Lateral Sclerosis; Primary Lateral Sclerosis; Progressive Muscular Atrophy
25.	Recruiting	Collection of Blood Samples for DNA in Motor Neuron DiseaseCondition: Motor Neuron Diseases
26.	Recruiting	A Study of the Expression of Nogo and Reticulon Genes in Skeletal Muscle of Patients With Amyotrophic Lateral Sclerosis.Condition: Amyotropic Lateral Sclerosis
27.	Recruiting	Influence of G-CSF and EPO on Associative Learning and Motor SkillsConditions: Chronic Stroke; Amyotrophic Lateral Sclerosis
28.	Recruiting	Preterm Fetal Growth Restriction and Developmental CareCondition: Preterm Birth
29.	Recruiting	Adolescents Born PretermCondition: Preterm Birth
30.	Recruiting	Riluzole to Treat Depression in Bipolar DisorderCondition: Bipolar Disorder
31.	Recruiting	Treatment of Refractory Schizophrenia With RiluzoleConditions: Schizophrenia; Schizoaffective
32.	Recruiting	Safety Study of AVP-923 in the Treatment of IEED (Involuntary Emotional Expression Disorder) Also Known as Pseudobulbar Affect (Episodes of Uncontrolled Crying and/or Laughter)Conditions: Alzheimer's Disease; Stroke; Parkinson's Disease; Traumatic Brain Injury
33.	Recruiting	Vaccine Therapy and Interleukin-2 After Combination Chemotherapy in Treating Patients With Relapsed or De Novo Stage II, Stage III, or Stage IV Mantle Cell LymphomaConditions: Recurrent Mantle Cell Lymphoma; Contiguous Stage II Mantle Cell Lymphoma; Noncontiguous Stage II Mantle Cell Lymphoma; Stage III Mantle Cell Lymphoma; ...

Chapter 7

ADVOCACY

Over the course of the illness, many PALS become interested in advocating. Some become so engrossed that it becomes a meaningful and satisfying focus of their lives. The following articles provide you with comprehensive information on advocacy and the tools to get involved.

Advocacy!

By Catherine Wolf

Catherine Wolf was diagnosed with ALS in September, 1997 at age 50. Cathy had a tracheostomy in 2001 and is now completely ventilator dependent. She faces ALS with optimism, a fiercely independent nature, and an occasional tear. Before ALS, she worked for IBM, and was an avid runner and dancer. She has two adult daughters and is the enthusiastic grandmother of one grandson. Cathy lives with her husband Joel in Katonah, NY.

Introduction What is advocacy? The dictionary defines advocacy as “active support for a cause.“ Most people feel better when they are doing something. We feel that our lives have purpose. Although there is not yet a cure for ALS, PALS typically feel good when they are involved in advocacy. As a newly diagnosed PALS, you have the physical capability to do more than your less recently diagnosed fellow PALS. So get involved! This chapter describes some of the ways you can become involved in advocacy. What are your skills and interests? When contemplating how to get involved, assess your skills. Were you a bookkeeper? Perhaps you can volunteer to keep the books for an ALS fund raiser, or help a fellow PALS organize his/her finances. Were you a nurse? Now is the time to educate yourself about ALS so you can explain the often mystifying jargon doctors sometimes use about ALS. In my case, I had spent many years as a usability expert in software design. I made sure when I was still working that the software I worked on was usable by people with motor disabilities. And when I encountered programs made by my company that were difficult for me to use, I suggested ways to improve them. Perhaps most important, I made suggestions for improving the assistive technology I used. Thus, I used my work skills to advocate for all PALS. Altogether your body will lose physical function, there is no limit to what your mind can do, especially when armed with a computer. Kyle Hahn, a musician, started the ALS March of Faces, an awareness and advocacy organization. Will Hubben compiled and distributed ALS research. David Jayne, stricken with ALS at 27, started living-with-ALS and started a group to agitate for change in the Medicare laws regarding homebound people. You may be thinking, “I don’t have the personality for a major undertaking.“ Advocacy takes many forms, large and small. For example, I shared what I learned from my successful insurance battle with the MDA ALS News magazine.

Sometimes ALS can wake up talents you didn’t know you had. In my case, it was poetry. I started writing poetry about my reaction to ALS, as well as political poetry and sending it to friends. One friend with connections finagled an article about me, ALS, and my political poetry in my county’s section of the New York Times. That article described ALS, and I’m sure helped raise awareness of ALS. Another article in Neurology Now included some of my ALS poetry. The doctor who wrote it said my poems gave her a new appreciation of the patient’s perspective. While I can’t speak for all PALS, I know that there are many who share my optimistic perspective. Since the magazine is distributed to every neurology office, I hope my upbeat poems will be helpful to people afflicted with neurological diseases. I use these examples from my life, not to toot my own horn, but because I know them and to show one little person can make a difference. Advocating for yourself Sooner or later, you will find yourself in need of services your insurance company denies. Don’t take no for an answer! Most insurance companies know that many people will not appeal a denied claim. Have your doctor write a letter of medical necessity that explains why you need the service. Notes from other health professionals can also help. Demand that your insurance company gives you a letter of explanation for the decision, not a cryptic code. Read your policy. Consult the social worker at your ALS center, or if the stakes are high, hire a lawyer. MDA, ALSA, or the local bar association can recommend one. Put everything in writing and send it return receipt requested so you have a paper trail. If you speak to anyone from your insurance company, get their names and make notes and date the conversation. You’re not allowed to record the conversation without permission from the other party. The insurance company is usually your enemy, but sometimes you can sweet talk someone into giving you more information. I don’t know much about Medicare or Medicaid.I assume that the same principles apply to these. There are, of course, other reasons than insurance for which you might need to advocate for yourself. Sometimes well-meaning caregivers or family members want to make decisions for you. Your mind still works, and you should have as much control over decisions affecting your care as you desire. Advocating for a fellow PALS You can put your skills, experience and knowledge to work to help a fellow PALS. Over the years, I have helped several PALS, mostly by email. I have also written about what I have learned in my ALS center’s newsletter. Every time you post something in living-with-ALS you are making a fellow PALS’ life easier. As a newly diagnosed PALS, you may have the energy and voice to help a more advanced PALS. For example, you might organize a Share the Care group for someone in your local support group. Cause advocacy Advocating for a specific cause can be as easy as forwarding email you receive from a group supporting that cause to everyone you know. For example, before the Senate vote on the stem cell research bill in July 2006, I forwarded the United Spinal Association’s email urging me to contact my senators to vote yes to family and friends. And I emailed my senators. Sometimes you have to write your own cause letter. The trick is to make it easy for your friends to take action. This may include providing talking points, phone numbers or email addresses. In June 2005 after the House of Representatives passed the stem cell research bill, I emailed friends to put pressure on those representatives who voted against it to change their minds. Here is an excerpt from my email.

Thank you for your help in passing the Stem Cell Research Enhancement Act. The House of Representatives passed the bill 238-194, with 14 Democrats and 180 Republicans voting against it. The Senate is expected to pass a similar bill soon. The sponsor of the Senate bill, Republican Arlen Specter, says he has enough votes to override a presidential veto. Bush said he will veto it when the bill reaches his desk. He is obviously out of tune with most Americans, since a recent poll indicated only 28% of the people oppose embryonic stem cell research for therapeutic purposes. Bush has little to lose by vetoing this bill because he is a second term president. But the Representatives will have to defend their votes on this issue in the 2006 elections. It takes a two-thirds majority to override a presidential veto. Therefore, we should focus our efforts on the representatives who voted against this bill or who did not vote. To see how your representative and others voted go to http://www.nytimes.com/2005/05/25/politics/25stem_rollcall.html

For convenience, I am including the text of this article at the end of this email. . if you know people in states with a large percentage of no votes, please consider forwarding this to them.

To find out who your representative (or any representative) is and how to contact him/her go to http://www.house.gov/writerep/

[roll call vote by state included]

Whether or not you agree with me on stem cell research, the principles of cause advocacy are the same.

• Be specific about what you want your audience to do.

• Provide information on the topic, perhaps including talking points.

• Provide contact information for the people you want them to contact, or an easy way of getting the information.

Organization advocacy

There are many groups that raise money for ALS research or services, or raise awareness of ALS. These include, but are not limited to, Muscular Dystrophy Association (MDA), ALS Association (ALSA), ALS Therapy Development Foundation (ALS-TDF), Ride for Life, ALS March of Faces, United Spinal Association, and your local ALS center. The first two organizations have many local chapters which may hold fund raising events such as walks, bike rides, dinners and golf outings. Some other organizations, such as the Stamford, CT Fire Department, raise money for one of the organizations in the first list. If you want to get involved, there are many organizations and activities. If you’re not on the mailing list or email list for these organizations, they will welcome your participation.

Your involvement can be as simple as wearing a blue and white pin striped ribbon, to participating in an event, being on the organizing committee for an event, or organizing your own event. Fund raising is a good way to involve family, friends, colleagues and your employer who often want to do something for you, but don’t know what. Time for a personal story. I have always been reluctant to ask friends for contributions. My cousin has been doing the ALSA bike ride in Boston along with her husband and several co-workers who don’t even know me for several years. This year they had only raised $350 towards their goal of $750. She asked me to contact my friends for help. I figured if she could ask people who don’t know me to ride and contribute, I could certainly ask my friends. The response was phenomenal. We raised over $2000!

Working on the organizing committee of a major organization, such as MDA, for a fund raising or an awareness event has the benefit that the organization provides most of the structure. When I worked on the organizing committee of a local MDA fund raiser, they provided the solicitation letter, which I personalized.

If you want to organize your own event, it is wise to recruit family and friends to help. Such events always take more effort than anticipated. Alternatively, if you know someone who wants to organize an event, you can let her/him be in the driver’s seat and help as you can. Several years ago, one of my daughters organized a folk music concert to benefit ALS-TDF and raised about $5000. ALS-TDF has family funds which are a good way to honor a PALS and track your fund raising for the organization. Try to attend the event, if possible. It helps to put a face on ALSWhatever form your organizational advocacy takes, remember it is much easier to get businesses to donate goods and services than money. It is not only a donation. It’s free advertising. Your life as a model for advocacy Finally, your advocacy activities can inspire people with and without ALS to become advocates. Thus, your own advocacy has a multiplier effect. Conclusion This chapter has described some of the ways you can put your skills and experience to work to become an advocate for PALS, including yourself. Advocacy can take as much or little of your time and effort as you desire. The types of advocacy described here are:

• Advocating for yourself
• Advocating for a fellow PALS
• Cause advocacy
• Organization advocacy
• Your life as a model for advocacy

EIGHT RULES TO GET WHAT YOUNEED

By Danny Dandignac

Danny was Dx'd 12/2000. He retired from being a paramedic in 12/2004 after 23 years. Currently he spends his time co-hosting the MDAchats that are held 4 days a week and continuing the Will Hubben ResearchDigest hosted on the ALSTDF website. He is married with 2 children, ages 12 and 15. He has brought our attention to a very useful article.Bob’s friend Jason doesn’t have ALS, but he faced a problem all too familiar to many who do: an insurance company ruled that equipment he needed was “not medically necessary.” Sound familiar?Jason might have started fighting back by talking to his doctor first. His doctor may be as disagreeable and rushed as Jason pictures him. Moreover, his doctor may work for the very HMO that turned him down. But his doctor has a professional stake in preserving Jason’s shoulders—and in seeing that his prescriptions are filled as written.When I picked up the phone, Jason sounded desperate. Jason has cerebral palsy and has always walked by leaning heavily on two canes. As a result of decades of wear and tear, he developed severe arthritis and rotator-cuff tears in both shoulders. Most days it’s hard for him to cross the room because of excruciating pain.His doctor told him the pain and muscle tears were going to get worse—that rotator-cuff and shoulder-replacement surgeries were on the horizon—unless he got a power wheelchair. Although Jason’s doctor had sent the proper paperwork to his insurance company, a Medicare HMO, the wheelchair was denied because it was “not medically necessary.”“Not medically necessary?” Jason screeched into the phone. “When will it be medically necessary? When I can’t walk at all?”Sadly, the answer is yes. But I’m getting ahead of the story. Because Jason’s speech is hard to understand, he asked me to call the insurance company. In the process I discovered that there are Eight Simple Rules for getting anything you need from anyone. Rule #1: Get Name, Rank, and Phone Number I called Jason’s insurance company. After spending a turtle’s lifetime on hold, I was connected to a customer representative. I tried to explain my friend’s circumstance, but she said she couldn’t talk to me because I wasn’t “the insured” and hung up.I was massively ticked! Back on the phone, and after another turtle’s lifetime, I was connected to a different representative. This time I said that I was Jason and wrote down her name, phone number, and title. All she did was repeat what Jason knew—that the wheelchair was denied because it was not medically necessary. With an edge in my voice I asked who made that decision. She told me that she was not allowed to give out that information. I asked who could. She said that I would have to speak to her supervisor, Ms. Lemon, and that she would transfer me. Before she did, I got Ms. Lemon’s phone number—and good thing! I wasn’t transferred; I was disconnected. Rule #2: First Do Your Homework Since it was 5:00 p.m., I decided to do some research first and call Ms. Lemon the next day. On the Internet I found the Medicare regulation for power-wheelchair medical necessity.It turns out that a power chair is considered medically necessary only if you’re “bed or chair confined,” not Jason’s situation—yet. I then got the name and phone number of the president of Jason’s insurance company and went to the Web sites for the Department of Health and the Insurance Commission in Jason’s state to find out what process insurance companies must follow if they deny medical equipment. I also decided I would keep dated notes of my phone conversations and take exact quotes. Rule #3: An Ally Is Better Than an Adversary At 9:01 the next morning I called Ms. Lemon. Instead of screaming as I wanted to, I decided to take a different tack: I started by saying, “Ms. Lemon, you are the only one who can help me.” I told her that two of her representatives had hung up on me (hoping a little guilt would soften her up) and explained the situation. She apologized. I told her I needed to know who had denied the power chair. Pleasant as you please, she volunteered that she had denied the chair. Aha! I had her! Victory was mine. My homework had uncovered that this state’s law permits only a physician to deny medical equipment.When I told her this, she stammered and said she was sure that one of the medical directors, a physician, had ultimately signed her denial. When I asked for the medical director’s name and phone number, she said she was not allowed to give out that information. However, she said she’d transfer me and (you know what’s coming) I was disconnected. Rule #4: Go Right to the Top! It was clear I was getting nowhere talking to the hired help. So my next call was to the president of the insurance company—let’s call him “Robert Bucks”. Of course I didn’t get Mr. Bucks on the phone; I got one of his personal assistants. Starting again with “You are the only one who can help me,” I quickly explained the hang-ups, the denial by a clerical worker, and the refusal to let me talk to the medical director.The assistant sounded both shocked and concerned. He apologized and confirmed that indeed only a medical director can deny a power chair and gave me the name and number of the medical director assigned to the case. Rule #5: Be a Name Dropper I immediately called the medical director’s office and got his secretary. I asked to speak to the doctor and was told that “insureds” were not allowed to speak to medical directors and how had I gotten the number? I said that I had just spoken to “Bob” Bucks, and was told to call the doctor directly. Implying that “my buddy Bob” told me to call got me through to the medical director in a flash.The doctor explained that he had indeed signed the denial based on the Medicare regulation that required someone to have “severe weakness” in the arms, be unable to push a manual chair, and be “bed or chair confined” before a power chair could be approved.Still speaking as Jason, I told him that my arms might not yet be severely weak, but that severe shoulder pain prevented me from pushing a manual chair and that shoulder degeneration would soon make me bed- or chair-confined if I kept on as I was. With the Medicare regulation in front of me, I then quoted another paragraph which said that “a patient who uses a power wheelchair is usually totally non-ambulatory.” I suggested that the word “usually” gave him some leeway, since it means not everyone who gets a power chair is totally unable to walk.To my amazement the doctor listened! He agreed the shoulders would just get worse and that I would eventually be chair or bed confined. “Tell your doctor to write a new prescription and an appeal letter explaining that your shoulders are falling apart, that you can’t push a manual wheelchair and soon won’t be able to walk,” he said. “I can argue that it would be better to give you the power chair sooner rather than later and save your shoulders.” Rule #6: Tell, Don’t Ask Ecstatic, I called Jason. I explained that his doctor only had to send a prescription, write a letter, and the power wheelchair was his. But Jason hemmed and hawed. He said his doctor was very busy and hadn’t liked filling out the insurance forms for the power chair in the first place. I told Jason he needed to advocate for himself. I told him he needed not to ask but to tell his doctor—quietly but firmly—that he “was the only person who could help” and to write that letter. Indeed, Jason’s doctor was not happy, but write a letter he did.Within two weeks the power wheelchair was approved. I was king of the world ... until Jason called me a month later. Rule #7: Call in the Marines! Jason told me that he was greeted one morning by a truck driver with a huge box. Inside was the promised power chair. Jason’s excitement gave way to confusion and anger when he discovered the wheelchair was twice as wide as he was. I asked him how this could have happened when he had been fitted for the chair. Jason told me the delivery was a total surprise because he had never been fitted.I immediately called the medical director to find out what had gone wrong. The doctor coolly explained that the insurance company provides only one type of power wheelchair from only one manufacturer. When I told him the chair had never been fitted and was too wide, the doctor’s response was as simple as it was final: “We had a doctor’s prescription.” Click.The doctor’s answer was not only ridiculous, it smelled way bad. Why would an insurance company provide only one type of power wheelchair that was drop-shipped from another state without being fitted?I called Jason’s state insurance commissioner and was told that insurance companies cannot provide only one brand of wheelchair, that wheelchairs are indeed custom items that must be fitted. I was referred to the state department of health’s office responsible for overseeing HMOs. And I heard the same thing. For good measure I called the state attorney general’s office. What the insurance company was doing smelled bad to everybody. And, since this was a Medicare issue, I called Jason’s congressman too.Turned out that the insurance company and the wheelchair manufacturer were under federal investigation for—guess what?—a kickback scheme involving payoffs by the wheelchair maker to the insurance firm.I once again called my buddy the medical director. I told him about all the offices I had just called and—my voice calm and even—explained that everyone believed that his insurance company was involved in a kickback scheme with the wheelchair manufacturer that broke bushels of state and federal laws. I told him that it wasn’t my idea to testify against him in court or to speak at a television press conference, but that I’d do what the attorney general asked if it would get me a usable power chair. Rule #8: One White Lie May Be Worth a Thousand Truths Of course, no one I talked to had mentioned testifying in court or a TV press conference. But the medical director didn’t know that, and there was no way that he was going to find out. What was he going to do, call the attorney general and ask if he was going to be indicted? Which is why I heard the doctor’s voice raise an octave. “No, no, no! “he said. “You must have misunderstood me.”He quickly explained that the brand of wheelchair that was shipped was the company’s first choice, not the only choice, and that of course every patient’s wheelchair should be individually fitted. He told me that the insurance company would arrange for the chair to be returned, that a local vendor would be in contact, and that any brand of power chair that met my needs would be provided.True to his word, within a week the offending wheelchair had been removed and Jason was fitted for a chair that met his needs. Jason and his shoulders are now happily rolling along.God Bless Alexander Graham BellSince helping Jason, I have told others about “The Eight Simple Rules” and how they have proved helpful in dealing with everything from refusals by employers to provide reasonable accommodations under the ADA to reversing denials for Social Security disability benefits.The phone can be a powerful weapon. Don’t be afraid to call anyone and everyone who could possibly help you. State officials, congresspersons, and senators. Elected officials love to help their constituents (read: voters).Sometimes, however, the pen can be mightier than the phone. You can file official complaints under the ADA, state civil rights and consumer laws with your state’s insurance commissioner and with the attorney general. Ultimately, you may need a lawyer specializing in disability or consumer issues if it’s time to lock and load and take the bums to court.But if you follow “The Eight Simple Rules”, you may get what you need without paperwork or lawsuits by doing your homework, being pleasant but assertive, using your wits, and using Alexander Graham Bell’s marvelous invention.

TIPS AND INFORMATION ON ADVOCACY

By David Abell

David was diagnosed with ALS in June 1996. He was 29 and serving in the US Navy when he was diagnosed. He has adapted to being paralyzed, ventilator dependent, fed through a feeding tube. His attitude is “Iwill never give up.”He rarely feels sad and has discovered numerous, meaningful ways to enrich his life.Advocacy & Awareness are important in getting funding for research andcare for ALS. Some great advocates groups for ALS areThe March of Faces http://march-of-faces.org/ Project ALS http://projectals.org/ Ride For Life http://www.rideforlife.com/ There are many others but these are my favorites. It is up to all of us to raise awareness if possible. When friendsask if there is anything they can do, ask them to write a letter totheir Congressperson & Senators. (see below) Check the www.ALSA.orgwww.MDA.org or NORD http://www.rarediseases.org/ for sample lettersthat you can modify for personal info. If you believe in stem cellresearch www.stemcellaction.org and http://www.curesnow.org/ havepetitions to sign and sample letters. Write to your congressperson. It's hard to represent you properly ifthe congressperson isn't aware of your feelings. Select your state or territory and enter your ZIP code. You'll be givena form that will be sent directly to your representative. If you want to contact your senator, go here: http://www.senate.gov/general/contact_information/senators_cfm.cfm And to contact members of the House, go here: http://www.house.gov/writerep/ This website has tips on How to write your congressperson. http://www.protest.net/activists_handbook/write.html Writing a real letter or fax will not get ignored as easily as Emailbut it takes a long time for a response especially since the anthraxidiot. I do both sometimes.Anyway, there are some ideas. We can make a difference.

Thoughts on Advocacy

By Chuck Hummer

Chuck retiredas a senior executive with the U.S. Army Corps of Engineers in Washington, D.C., in 1989, culminating a thirtyyear career as a government worker.He was diagnosed withALS in October 2004, after a three yearjourney to determine why he was falling and experiencing slurred speech.He and his wife, Sandra, live in Pinellas Park, Florida.Advocacy is defined as the act of pleading or arguing in favor of something, such as a cause, idea, or policy; active support.How does this apply to ALS?In the strictest sense, advocacy is often limited to a type of lobbying, or seeking legislation to provide for some service or rights.In the broadest sense, I take it to mean speaking out and up to assure public awareness of the disease called ALS.Without this basic understanding of what ALS is, how it affects those who have been diagnosed with ALS and the sorry fact that no one knows the cause or causes, there are no real pharmaceutical therapies to treat the disease, and there is no cure.Unless and until we get that message out to the broadest possible audience, including health care workers, politicians and even our own families and friends, it seems futile to think that we can plead or argue for any relief from the disease.In short, education seems to be a first crucial step to advocating for those of us with ALS.At first, this seems pretty elementary and an objective easily achieved.But if my case is typical, then it grossly underestimates the existing ignorance about ALS or Lou Gehrig’s disease.I had heard of it, and in fact an uncle had it and died from it.When I was diagnosed, I suddenly learned a great deal about this disease and the gloomy prognosis for those so afflicted.Once that sunk in, it inspired me and my wife to learn all we could and to tell as many people as would listen that I had an incurable terminal illness that would likely take my life in a short period of time.No cure, no effective medicines, no confirmatory diagnostic test or tests, all of this came as an incredible shock and led us to think, just what we can do to learn to live with this disease and somehow stimulate the search for effective therapies and a cure.That is when advocacy suddenly came into clear and distinct focus for us.We had to help get the word out, the public educated and the research community put on a fast track.It came as another shock to learn that there is no national database of ALS patients.Without this crucial basis on which to seek funds for therapies and a cure we were simply bouncing around in a very dim room without much hope for an early resolution to the seemingly endless ignorance that surrounded the disease that I now had, was dying from, my body shutting down the use oflimbs, speech, breathing.I was encouraged when ALSA made the National ALS Registry one of its key legislative goals.And so it seems that the first order of business is to educate using every avenue available, educate as to the lack of a diagnostic test or tests, no effective therapies and certainly no cure, nor the hope for one on the horizon.Because unless we make major inroads on the ignorance about ALS, it seems any effort to inspire research, or for legislation to fund increased research would be a whisper in the hurricane of all the diseases for which cures and therapies are being sought.Now the sad fact is that those of us with ALS don’t stay around long enough to build an effective voice made up entirely of the afflicted.In the U.S. it is estimated that there are no more than 30,000 people with ALS at any one time.Of those, more than likely only a quarter or less than 10,000 are healthy enough or still have the ability to do much more than try to deal with the rapid progression of the disease.That is not a large constituency by any means, and without any cure on the horizon these numbers are not likely to change much.About as many die from the disease as are diagnosed.Remember what advocacy is, it is arguing or soliciting aid and if there are not sufficient numbers of people with ALS to constitute an effective voice what avenues are open to us.I think that the answer lies in those who care for us, those who are family, friends, and acquaintances, colleagues in the work place or faith community.These are the groups that can be large enough to have sufficient voice and volume to speak on our behalf.If one looks that those involved with the national organizations that have grown to represent us, you will find that many have had their lives touched by a loved one with ALS or a coworker, or friend.Thank God for that, because we PALS are a pretty anemic group if counted on our own.Awareness, education, appreciation, all of these must be our goal and those who we hope will advocate for us.While we are healthy enough to speak on own behalf, we must do so, but we must also enlarge the community around us to achieve a noise level that will be heard when we ask for more research, for better patient care and services, accessibility as our mobility spirals downward.We, the PALS, have to be at the center of this effort because it is a life and death challenge for us.But we must also grow the community around us through education, to become an effective voice for us with enough depth and volume to make our needs heard in all the places where action must be taken on our behalf if we are ever to realize our hope for better therapies, therapies that will bring ALS into the community of chronic but treatable illnesses and finally, one day, a cure.

How do we do this?I think that it requires a multifaceted approach, but that approach needs some healthy but vocal PALS at its center. Awareness events such as the ALSA Walk to Dfeet ALS, the Ride for Life, Faces of Courage, and the many other public initiatives are an essential part of this awareness effort. Seeking celebrity spokespersons, maximum exposure and use of all aspects of the media are vital parts to this campaign.We need to use the national organizations that represent us to be our advocates and wemust be our own advocates to keep the rest of the advocates heading in the right direction with enough passion and vigor to make a difference.I found that participating in the annual ALSA Advocacy Day and Public Policy Conference has all sorts of good results.It allows our advocates to network and in doing so become a harmonized chorus, rather than a bunch of distinct weak voices each singing in isolation.It also brought the face of ALS and our needs to our legislators.Some states have set up similar events to interface with their state legislators. It remains that the true strength comes from those who care for us.Their lives have been touched on a very personal level, and they willround to keep advocating on behalf long after we have gone.If their voices become strong enough, then maybe we will see therapies come along that will extend the lives of PALS significantly and we can break above that 30,000 ceiling that has plagued us for so long.Simply stated, each of us has to be our own advocates arguing on our own behalf, but we need to be active in growing and extending the advocacy communities made up of our family, friends and coworkers, the researchers and service organization volunteers and staffs that work for us.It isn’t going to work if we shrink into our own lonely little space and do not reach out to others and continuously make that group of others larger and larger, better organized and educated, to carry the message of awareness and education that will make advocacy work for better times for all PALS.

A Letter to Advocate for Greater Research for ALS

By Randy Roberts

I wrote this letter some time ago to draw attention to the fact that ALS isunder-funded compared to other diseases. It has been modified from the original.Dear Senator/Congressman,I am writing this letter to bring to your attention to the fact that ALS (Lou Gehrig’s Disease), an illness that receives far too little publicity in view of its devastating impact on the lives of so many Americans, is under-funded by NIH (FY 05) when compared to other diseases and to ask for your help. But first, a few words about this disease. ALS attacks the motor neurons (nerves) that send commands and nourish muscles. As the neurons die, the muscles that they support weaken and atrophy. Although the disease can progress in different ways, a typical scenario is for a limb to weaken first, and then spread to other limbs, rendering the body paralyzed. Over time it progresses to muscles controlling speech, swallowing and finally breathing itself.While ALS ravages the body, it generally leaves the mind intact, so patients are fully aware of what is happening to them. One patient who wrote about his experience with ALS said "With keen mind and open eyes We watch ourselves die “. In a testimony to the human spirit, many ALS patients manage to rise above their suffering and continue to find meaning in their lives up to the end. ALS is not an inherited disease: (95%) of all cases do not have a family history of ALS. And it is not nearly so rare as people think, i.e., it is diagnosed about 2/3 the frequency ofMS (Multiple Sclerosis), a much more recognized illness. It can strike at any age from the 20’s thru the 70’s (Lou Gehrig himself was diagnosed at age 36) but tends to peak in late middle age. There is no effective therapeutic agent against ALS and no understanding of causation. Nobody is safe from this killer.MS seems to offer a reasonable standard of comparison as it has about the roughly similar incidence as ALS (10,000 new cases per year for MS vs. 6000-7000 for ALS). There are approximately 400,000 people in theUS living with MS vs. about 30,000 with ALS. Why the difference in prevalence if the diseases have a somewhat similar incidence rate? The major factor is lethality. MS patients live a normal life span while, as mentioned earlier, while ALS patients have a much shorter life span. If ALS were not such an effective killer, its prevalence rate would obviously be much higher.It seems reasonable that the two diseases should have nearly equal public funding, since they both occur with the similar frequency, but this is not the case.NIH budgets $110 million on MS research but $41 m on ALS.It is immoral to base funding on prevalence in the context of similar incidence rates, thereby penalizing ALS patients for the lethality of their diseaseThe next disease for comparison is Huntington’s disease, another disorder caused by degeneration of brain cells, i.e. neurons, in certain areas of the brain. This degeneration causes uncontrolled movements,loss of intellectual faculties, and emotional disturbance. HD is a fully inherited disease, passed from parent to child through a mutation in a certain gene. Its incidence and prevalence rate are much lower than ALS and patients usually live 10-25 years after diagnosis. Yet, this disease has an NIH budget of $48m vs. $41 m for ALS.For the, 3rd and final comparison, I will move from diseases involving neuron damage to one caused by a viral infection, HIV/AIDS. This illness has a large incidence (85,000) and prevalence (1,100,000) but thanks to advances in treatment, mortality has been significantly reduced. The number of patients who die from this disease each year is only about a bit more than twice the number of ALS deaths, yet HIV/AIDS has a research budget of nearly $3b vs. $41m for ALS.CONCLUSION I: Given itsincidence rates and high lethality, ALS is woefully under-funded compared to other diseasesand this may explain, at least inpart, why so little has been learned about this disease since Lou Gehrig’s death 65 years ago and why there have been no significant advances in treatment. It does not get the recognition it deserves because its high lethality severely limits the number of Americans who are living with the disease at any one point in time. RECOMMENDATION I:The NIH budget for ALS research should be immediately increased to $110m or roughly the same amount as MS.A second issue I wish to address is the fact that the low prevalence rate of ALS, due to its high lethality, does not provide any incentive for major pharmaceutical companies to search for a cure.The only drug for ALS, Rilutek, about 15 years old now, extends life only 2-3 months, is used by many patients at high cost, yet the maker of the drug, Aventis, claims that it loses substantial money on Rilutek. MS, in comparison, with its 400,000 prevalence in the US alone, presents significant incentive for drug companies to invest in research. In fact, there are 5 meds that have proven very beneficial and sales are big. Avonex, for example, had close to $1b in sales in the last year. Another drug, Copaxone, may have the greatest potential to alter the course of MS and sales, once the issue of side effects is resolved, should be huge. If we accept the view that it costs the companies $800 m over 10 years to develop and bring to market a successful drug, then about $4 billionhas been spent to produce thesedisease mitigatingmedications, or $400 m per year, not including current research.The FDA offers an orphan disease program to provide incentives to companies to invest in research of diseases having a prevalence of fewer than 200,000. The program provides tax incentives and other advantages for only small grants; unfortunately the incentives offered are too small to interest large companies with all their research potential. Conclusion II:Because of ALS’s high lethality, prevalence will always be low.As a consequence, big pharma will not have the incentive to invest in ALS research and the high tech power of drug companies won’t be harnessed to find new therapeutic agents in the fight against ALS. Recommendation II:Federal health agencies must provide the incentive to big pharma to invest in ALS research.The FDA Orphan Disease Program must be substantially increased to accomplish this.$80m per year in incentives must be allotted to get major companies interested in working on this research.Funding could include a combination of grants, tax credits and exclusive marketing rights, etc.The grants could be reduced as companies come closer to marketing the new agents. Under-funding of ALS will not be righted without your active intervention. I am askingfor your help on behalf of boththe 30,000+ Americans living with the disease today and the estimated 70,000 who will die every 10 years until a cure is found. ALS has been neglected far too long. Thank you for your interest. We and our families are counting on you. Randy RobertsPerson with ALS, diagnosed 4/04 A FINAL WORD ON ADVOCACY Our numbers are small, our voices are too weak to be above the din, wecannot “act up” like the early AIDS activists and we have no famous person to champion our cause.But we must try to keep public awareness on ALS and help raise funds for research. This chapter has shown you some ways. Use your energy and creativity to help us in this fight.

CHAPTER 8

MATERIALS AND RESOURCES AVAILABLE AT NO CHARGE

By Catherine Wolf

Introduction Many organizations offer free stuff, grants and/or services to people with ALS and their families. As a newly diagnosed PALS, you’re probably overwhelmed by the challenges ahead. This chapter puts in one place information about free stuff, grants and services that can make your life easier and save you money. The conditions that apply, if any, and how to contact the organization are included here. There may be other organizations offering services to PALS. These are the ones we know about. Muscular Dystrophy Association (MDA)MDA services are administered by the local offices. To find your local office, go to

http://www.mdausa.org/. To receive services, you must register with the local office, but registration is free.

Financial benefits

. These apply after all insurance has been paid.

• Up to $2,000 towards the purchase of wheelchairs or leg braces every five years. This benefit may be used for wheelchair upgrades.

• Up to $500 annually for wheelchair repairs.

• Up to $2,000 towards the purchase of augmentative communication devices. This is a one time benefit.

Other services and free stuff

• One physical, occupational, and speech therapy evaluation per year

• Annual flu shot

• Transportation to MDA clinics

• Loan closet containing items such as wheelchairs, hoyer lifts, hospital beds, commodes, etc

• Research updates, informative publications (MDA ALS News Magazine, Quest), resources & referrals, web site

• Support groups for Pals, Caregivers and Family

ALS Association (ALSA)ALSA is organized differently from MDA. Each local chapter decides what services to offer. To find your local chapter, go to Welcome - The ALS Association.To receive services, you must register with your local chapter, but registration is free. I will use the services of the Greater New York chapter as an example. But please contact your local chapter since services and eligibility are sure to differ.

Loan of augmentative communication devices. To qualify, you must have no functional speech. Also, you must be in the process of applying for coverage of such a device by your insurance, have no insurance coverage for such devices, or have no insurance.Listening library. Loan of books on CD or tape by mail to PALS who can no longer hold a book or turn pages easily. Check your local library for loan of audio books. The Library of Congress offers audio books by mail, - www.loc.gov/nls. The New York chapter lists a variety of nationally available sources of audio books. Go to http://www.als-ny.org/ and click on Patient Services, then Patient and Family Programs, then Listening Library. Loan closet with similar items to MDA loan closet. To qualify, the equipment must be recommended by a health professional and you must be in theprocess of applying for coverage of the equipment by your insurance, have no insurance coverage for the equipment, or have no insurance.Transportation to ALSA clinics and support groups. To be eligible, you must have no other way of getting there.Home visits by nurses, social workers and assistive technology specialists Support groups for PALS, caregivers and family Your local chapter can provide referrals and the national website has educational materials and publications, see Welcome - The ALS Association and click Resources and Patient, Family, Caregivers

Ride for Life Ride for Life is an awareness and fund raising organization whose main event is a ride for PALS in their wheelchairs in the New York area.

Financial benefits

• Respite care grants. Ride for Life periodically offers a limited number of Care for Life grants of up to $1500 for the purpose of giving your caregiver a break. See http://www.rideforlife.com/

• Legal grants. A limited number of grants of up to $750 are periodically offered for legal expenses such as wills, dealing with insurance companies, and applying for disability benefits under the Plan for Life program.

• While not a direct benefit to you, when you enter certain online stores from http://www.rideforlife.com/, Ride for Life gets a percentage of the purchase price.

Other services and free stuff

• Information, educational materials on the web site

United Spinal Association Membership in the United Spinal Association is open to people with spinal cord injuries or diseases that affect the spinal cord, including ALS. See the web site at http://unitedspinal.org/ .Although this organization is not well known in ALS community, it provides valuable services to PALS. To receive services, you must join the organization, but membership is free. United Spinal is headquartered in Jackson Heights, NY with several regional offices.

Financial benefits

• Under the Wheelchair Medic program, you get a 10% discount on the price of wheelchairs and scooters and 15% off on parts. See http://www.wheelchairmedic.com/

• Low income members can get up to $5000 for accessibility modifications to the home. See http://www.unitedspinal.org/publications/action/2006/03/24/affording-accessibility-home-modifications/

• Low income members may qualify for grants for assistive technology and automobile refits. Call : 800-404-2898 or email

Other services and free stuff For a more complete description of services see http://www.unitedspinal.org/how-we-serve/1.ABLE to Travel is full service travel agency that can arrange accessible transportation to and from the airport, book flights, arrange accessible accommodations, give information on access to medical equipment, and answer your questions about wheelchair and disability travel. See http://www.abletotravel.org/ 2.Personalized consultations with experts in the areas of

• Accessibility Rights

• Benefits Counseling

• Social Services

• Adaptive Sports & Recreation

• Self Advocacy

• Legislative Issues

• Healthcare & Service Locating

• Peer Counseling

• Spinal Cord Issues

• Veterans Benefits

• Accessible Travel

• Wheelchairs & Assistive Technology

• Design of accessible home modifications

3.Special services for veterans under the VetsFirst program 4.Educational materials and excellent ACTION Online magazine,

http://www.unitedspinal.org/publications/action/

Conclusion You are not alone in your fight against ALS. These organizations and others are ready to help you in a myriad of ways. This information is accurate to the best of our abilities.

CHAPTER 9

RESEARCH

Some PALS and CALS are interested in learning about developments in ALS research and in looking beyond the surface of the latest headline finding displayed on websites. Because PALS and CALS are not familiar with the technical terms, I (RR)have supplied some simple definitions taken from ALSA’s website and other sources which will make the article that follows easier to read.Amino Acids: They are the building blocks of proteins.Blood Brain Barrier (BBB):A protective barrier formed by the blood vessels and glia of the brain. It prevents some substances in the blood from entering brain tissue.Central Nervous System (CNS): The brain and spinal cord combined.Embryonic Stem Cells:Embryonic stem cells are the "blank slates" of an organism, capable of developing into all types of tissue in the body. Gene:Genes are the basic biological units of heredity. They are composed of DNA. Glutamate:Glutamate is one of the most common amino acids found in nature. It is the main component of many proteins, and is present in most tissues. Glutamate is also produced in the body and plays an essential role in human metabolism. It is a primary excitatory neurotransmitter in the human CNS. L-glutamate is present at a majority of synapses. Over-stimulation of these same receptors is thought to trigger the neuronal damage associated with a wide variety of neurological insults and diseases, including amyotrophic lateral sclerosis (ALS), lathyrisms, and Alzheimer's disease.Glutamate toxicity:Toxicity resulting from excess glutamated synapse. Mutation:A permanent change, a structural alteration, in the DNA or RNA. Mutations can be caused by many factors including environmental insults such as radiation and mutagenic chemicals. Mutations are sometimes attributed to random chance events. Neuron:Neurons are the nerve cells which make up the central nervous system. They consist of a nucleus, a single axon which conveys electrical signals to other neurons and a host of dendrites which deliver incoming signals. Neurotransmitters:Chemical substances that carry impulses from one nerve cell to another; found in the space (synapse) that separates the transmitting neuron's terminal (axon) from the receiving neuron's terminal (dendrite). Protein:Proteins are large molecules required for the structure, function, and regulation of the body's cells, tissues, and organs. Each protein has unique functions. Proteins are essential components of muscles, skin, bones and the body as a whole. Protein is also one of the three types of nutrients used as energy sources by the body. RNA: A long-chain, usually single-stranded. The primary function of RNA is protein synthesis within a cell. However, RNA is involved in various ways in the processes of expression and repression of hereditary information. The three main functionally distinct varieties of RNA molecules are: (1) messenger RNA (mRNA) which is involved in the transmission of DNA information, (2) ribosomal RNA (rRNA) which makes up the physical machinery of the synthetic process, and (3) transfer RNA (tRNA) which also constitutes another functional part of the machinery of protein synthesis. Stem Cells:Cells that can differentiate into many different cell types when subjected to the right biochemical signals. Stem cells are a promising new therapeutic approach to treating CNS disorder. The most versatile stem cells, called pluripotent stem cells, are present in the first days after an egg is fertilized by sperm. Researchers believe they can coax stem cells to become whatever tissues patients need. Stem cells come from embryos, bone marrow and umbilical chords. Superoxide Dismutase:An enzyme that destroys superoxide. One form of the enzyme contains manganese and another contains zinc. Superoxide is a highly reactive form of oxygen. For ALS, 20% of the total patient population has mutations in the gene for copper/zinc superoxide dismutase type SOD1. SOD1 normally breaks down free radicals, but mutant SOD1 is unable to perform this function. Synapse:A tiny gap between the ends of nerve fibers across which nerve impulses pass from one neuron to another; at the synapse, an impulse causes the release of a neurotransmitter, which diffuses across the gap and triggers an electrical impulse in the next neuron. Toxin:A poisonous substance of animal or plant origin. Transgenic:An organism whose sperm or egg contains genetic material originally derived from an organism other than the parents or in addition to the parental genetic material.

RESEARCH OVERVIEW

By Wayne Gimlin

Wayne is 39 years old and is an aerospace engineer.He is married with 3 kids.In Fall 2002, the ALS clinic said that he had motor neuron disease with uppermotor neuron symptoms.They changed the "official" diagnosis to PLS in2003 but won't rule out ALS.Today in 2006, he walks very stiff with awalking stick and trips and falls on a semi-regular basis, speakswith aslur, and has general weakness from head to toe.However, he still worksas an engineer and “enjoys” life.Wayne posts regularly on Brain Talk and is a frequent contributor to ALS-TDF.The Modern Era of ALS Research BeginsThe modern era of research into ALS began in 1991 with the discovery of the cause of a small subset of ALS in its familial form, namely the SOD1 gene. http://www.eurekalert.org/pub_releases/2006-04/nu-dsa042506.phpSoon after, this same gene was modified in mice and a paralyzing disease similar to what human ALS occurred.By over expressing the defective SOD1 gene in mice, scientists can cause mice to show symptoms in their legs at approximately 90 days with death occurring from 110 to 150 days.Although other mice and rat models (e.g. the wobbler mouse) have been used when studying ALS, the SOD1 mouse has been the mostly widely used in the last 12 years.

The First Approved Drug In 1995, the FDA approved the drug Rilutek as atreatmentforALShttp://www.fda.gov/fdac/features/796_als.htmlRilutek was first proposed as an ALS treatment in the late 1980s with Phase I and II clinical trials meant to show safety completed in the early 90s.By 1993, two large Phase III trials in Europe and the US evaluated the efficacy of Rilutek.These trials divided ALS patients into placebo and treated groups and compared the survival rate of both groups over 18 months.In both trials, there was reported a small statistical difference between the two groups and therefore Rilutek was determined to be marginally effective in slowing ALS.The method of action of Rilutek is the reduction of a chemical called glutamate that is used by motor neurons to pass signals.It is theorized that an excessive amount of glutamate is present that damages and eventually kills a motor neuron in a process called excitotoxicity.http://products.sanofi-aventis.us/rilutek/rilutek.htmlDue in part to the apparent success of Rilutek, there was a flurry of specialized drugs and treatments that were introduced to clinical trials as a potential treatment.Examples of these trials included BDNF and GDNF which were injected into the spinal fluid with a pump and the brain with an injection port.Other specialized drugs were developed by Sanofi and other companies that were taken orally or injected subcutaneously or peripherally.Unfortunately, all these drugs failed their Phase III clinical trials that were to show efficacy and have not been approved for use for ALS. Clinical Trials | MDA ResearchBDNFOne possible exception was a drug called Myotrophin that passed a Phase III in the US but failed in Europe in 1997.This drug is actually a form of the brain protein called IGF-1.In the Myotrophin study this drug was injected subcutaneously directly into muscles of the patients. The theory behind IGF-1 is that it acts as a way of protecting the motor neurons from damage caused by an external agitator.Currently, Myotrophin is undergoing a trial to determine a “final” answer to the conflicting results of the two trials from the 90s.

http://www.rideforlife.com/news/als_research/clinical_trial_of_myotrophin.htmlRecent Research into Pre-existing Drugs The ALS research community is mainly comprised of university researchers and a few non-profit and for profit companies.Due to the multiple failures of very expensive developed drugs in the late 90s, the ALS research community focused more on the use of existing drugs to treat ALS. The advantage of using existing drugs is that they do not have near the development cost and if found to be effective, can be cross-prescribed for ALS patients.Some examples of pre-existing drugs taken to trial for ALS research are:

Pre-existing drugs taken to Phase II or III trials.

1. Celebrex. Failed Phase III trial. 2. Creatine Failed two Phase III trials. 3. Minocycline Currently in a Phase III trial. 4. Tamoxifen Completed a Phase II trial showing safety and possible efficacy 5. Hydroxyurea. Currently in a Phase II trial for safety and possible efficacy. 6. Ritonavir. Currently in a Phase II trial for safety and possible efficacy. 7. Indinavir. Failed a Phase III trial. 8. Ceftriaxone. Currently in a Phase III trial. 9. Pentoxifylline. Failed a Phase III trial. 10. Sodium Phenyl Butryate (PBA). Currently in a Phase II trial 11. R+ Pramipexole. Currently in a Phase II trial 12. Thalidomide. Currently in a Phase II trial 13. Aramiclomol. Currently in trial. 14. Others.

http://www.mda.org/research/ctrials.aspxSOD1 Mouse Studies One important point about these drugs is that nearly all were justified to the FDA and the funding agencies as worthy of a human clinical trial, by the successful completion of a SOD1 mouse test.In these tests, the mice are divided between control mice and mice given a treatment.The variables of these tests include the point in their lifetimes that the mouse should begin treatment, the amount of dose given, and the length of time given the dose. Further there is a question of how many mice must be used per test and how closely related the mice must be.Ideally, a researcher should be able to take any two sets of mice, treat them identically and the average lifetimes of both sets should be about the same. However, there have been questions over the results of these reportedly successful mice tests. ALS-TDF is a non-profit ALS research company started by James Heywood in 1999 as a response to the diagnosis of his brother Stephen with ALS in 1998.ALS-TDF first investigated the use of stem cell and gene therapy to treat Stephen’s ALS in the late 90s.However by 2002, ALS-TDF started large scale testing of SOD1 mice in the hope that a pre-existing drug may substantially slow the rate of ALS progression.To their surprise, as of 2006, and after testing several thousand mice, they have not been able to reproduce with repeatability the results reported from other researchers’ mice tests.This includes Celebrex, Creatine, Minocycline, Tamoxifen, PBA and others.They have been able to find small (< 10%) life extensions which prompted them to help start a Phase II trial for Hydroxyura and Ritonavir at the University of California in San Francisco (UCSF) but have not been able to repeat these tests with consistency. The concept of repeatability is a basis of science and experimentation.If a small set of PALS were divided into two groups and their rate of survival tracked, it would be expected that the average length of survival of both sets would not be exactly the same. The two groups’ survival might be different by a few weeks or months. If larger and larger groups were studied it would be expected that the difference in survival between the two sets would diminish.The same is true for mice.If small samples of mice are chosen to be in the control and in the treated group there is some certainty that the two groups will have different life spans by random chance.The way of proving that the treatment and not random sampling is the cause of the treated group outliving the control group is by repeatability, meaning that the test can be repeated by any competent researcher anywhere and has approximately the same results.There is a large question on whether the tests that have been published as “successful” are actually repeatable.Other concerns over the use of the SOD1 mice are the fact that the mutated SOD1 protein is not present in most PALS both those with familial and sporadic forms.This has caused much speculation that a successful SOD1 mouse test would not translate into a successful human clinical trial.Also there is concern over the differences between humans and mice on how they react to different drugs.Differences in how drugs cross the blood brain barrier could easily complicate whether a drug that affects or doesn’t affect a mouse would have the same type of result in a human.To date, what is lacking is what is called a “positive control”.Essentially this is a drug or treatment that has proven success in mice and humans which can be used to gauge how other reactions in mice might affect a human. Since the SOD1 mouse is one of the few animal models that closely mimic ALS, researchers will continue to use them for drug discovery. What should be asked by PALS and closely noted is how many mice were used, how many times was the test repeated using the same parameters, and how many different laboratories also repeated the test.Past research has shown that many if not all of the reportedly successful mouse tests cannot answer these questions adequately. Causes of ALS ALS research is hampered by the fact that how it is caused and how it progresses is mainly unknown.Although approximately 10% of ALS cases are known to be genetic, how the damaged genes cause ALS is still not known.Since SOD1 is a gene that produces a SOD1 protein that acts as an anti-oxidant, some have theorized that oxidation could be a key player in ALS.However, later research has pointed more towards the concept that these proteins could be tangled or misfolded and thereby causes cell death. In either case, one of the current main focuses of ALS research is identifying what genes are defective in familial ALS and if there are similar defective genes in sporadic ALS. Currently there are several trials sponsored by the MDA that concern identification of genes in ALS.

http://www.mda.org/research/ctrials.aspx Researchers are also interested in environmental factors that could cause ALS but so far, none have been proven.Besides the root cause, researchers have focused much of their efforts into identifying what happens in the cells that cause ALS death.This is a large subject but some of the areas of interest are:

1. Protein Misfolding 2. Immune system and Inflammation 3. Oxidation 4. Axonal transport 5. Excess Glutamate 6. Calcium 7. Mitochrondria malfunction

Biomarkers in ALS Other interests of ALS research include finding markers for the disease.For many diseases, identification of biomarkers in the blood or cerebral fluid help to both diagnose a patient with the disease and mark the efficacy of test drugs.To date, Dr. Robert Bowser of the University of Pittsburgh has published a biomarker study that identifies biomarkers in the cerebral fluid but it has not been developed for general use. Gene Therapy Studies The concept of using gene therapy has been investigated for ALS. There have been two basic approaches examined.These are synthesis of growth factors and the use of gene silencing

Synthesis of Growth Factors - In 2003, Dr. Fred Gage of the Robert Packard Center announced that they had significantly extended the life of a SOD1 mouse by the use of IGF-1 gene therapy. In this approach, an AAV-1 virus is injected into various muscles of the mice.The virus then moves up the nerve into the lower motor neuron nucleus located in spinal cord. There it “infects” the motor neuron and changes its DNA structure.This allows the motor neuron to synthesize a chemical called IGF-1 that is thought to be protective.In the 2003 announcement it was stated that trials could begin in one year but as of 2006, no human trial has begun.

http://www.hopkinsmedicine.org/press/2003/AUGUST/030807A.HTM

Another growth factor which has been theorized as having a potential effect is VEGF.

http://jama.ama-assn.org/cgi/content/extract/291/23/2809-c

http://www.scienceblog.com/community/older/2004/10/20049657.shtml

Gene Silencing - The use of a compound called RNAi or sRNAi has been proposed for ALS to silence the effect of a defective gene.This compound is essentially tailored to block the production of specific proteins.In particular, for SOD1 mice, RNAi has been used to block production of the SOD1 protein that is thought to be the cause of a small number of ALS cases.In particular, this type of therapy is thought to have potential to stop ALS progression where the actual defective gene such as SOD1 is known.

CytRx - RNAi Therapeutics

One of the most discussed topics of ALS research is the use of stem cells.Stem cells are the master cells in the body that differentiate into other cells.Embryonic stem cells which are the first cells created after conception are pluripotent meaning that they have the ability to transform into any other cell in the body.Adult stem cells are not pluripotent but do have the ability to turn into specific cells such as neural or blood cells. Stem cells have not yet been used for ALS by any legitimate research group although numerous scam institutions around the world have used the controversy surrounding them to take advantage of ALS and other patients. Despite that, the legitimate research community has envisioned using stem cells in various ways for ALS.The most ambitious may be the use of them to regrow the entire motor neuron network that has already been lost due to the destruction wrought by ALS.The motor neurons die in ALS and they are very large.The lower motor neurons are centered in the spinal cord but their axons or nerves extend all the way to the muscles making them up to 3 feet in length.The upper motor neurons are centered in the brain and their axons extend down the spinal cord to connect to the lower motor neurons.Recent work by Dr. Kerr at John Hopkins University has investigated reconstructing motor neurons in rats.
Less ambitious but perhaps not as complicated use of stem cells would be to use them to protect existing motor neurons from destruction in ALS.It is thought that stem cells could migrate to areas of the spinal cord not easily penetrable by drugs.They could perhaps be pre-programmed to secrete growth factors or other protective agents that could keep motor neurons alive. ALS Research Funding The majority of ALS research is conducted by non-profit institutions or universities.This is unfortunate because if for-profit pharmaceuticals had more of a role in this research, more resources including personnel and equipment could be utilized. As it is, the government through NINDS or the National Institute of Neurological Disorders and Stroke is a major provider of the money for research, approximately $50 million in 2006.Other providers are the MDA (Muscular Dystrophy Association) and the ALSA or ALS Association.In addition to these are smaller organizations developed by the families of PALS such as Project ALS developed by the Estess family and ALS-TDF created by the Heywood family which is also an active development laboratory that has been searching for drugs that may slow or stop the disease.Websites are below:

http://www.als.net/

http://www.projectals.org/

CHAPTER 10

DEFINITIONS OF MEDICAL AND RELATED TERMS AND SUPPLEMENTAL INFORMATION

Age of Onset- ALS onset ranges from the early 20’s through the 70’s, with the greatest frequency in the 50’s and 60’s. Average age of onset is 55. Baclofen: a prescription medication to reduce stiffness that often accompanies ALS. BiPAP – a small piece of medical equipment designed to take pressure off the breathing muscles (diaphragm), usually when sleeping. The device is attached to the nose or nose and mouth by a mask and kicks in right after the start of an in-breath. It finishes the inhalation by blowing a volume of air into your lungs, thereby resting the diaphragm. Can be hard to get comfortable with at first, but becomes easier with practice. Bulbar Onset – When symptoms first manifest in muscles that control swallowing, chewing and speech. Constipation- a common problem for PALS, fortunately numerous remedies are available. Cough Assist Machine (Insufflator-Exsuflattor) – A type of non invasive medical equipment that helps clear the lungs and throat of secretions by producing a cough. For more Information, go to J.H. Emerson - Cough Assist Durable Medical Equipment (DME) - Medical devices designed to be used repeatedly such as bipaps, canes, cough assist machines, wheelchairs, etc. Electro Diagnostic Tests - Studies including electromyography (EMG) and nerve conduction velocity (NCV), that evaluate and diagnose disorders of the muscles and motor neurons. Electrodes are inserted into the muscle, or placed on the skin overlying a muscle or muscle group, and electrical activity and muscle response are recorded. Fasciculations: Small, involuntary, irregular, visible contractions of individual muscle fibers. Often seen in the legs, arms and shoulders of PALS. This is often described by people with ALS as "persistent rolling beneath the skin." Commonly known as muscle twitches. Feeding Tube or PEG - Percutaneous endoscopic gastrostomy tube - a tube placed directly into the stomach through the abdominal wall to provide another way to receive nourishment and liquids. FVC (Forced Vital Capacity) – A formal measure of the amount of volume of air that can be exhaled from the lungs using maximal effort. You will be tested each time you attend clinic. Your performance is compared to what the average person would score. The result is expressed as a percentage. Hyperreflexia: Excessive response of muscle reflexes when a normal stimulus is applied.One of the characteristic findings in ALS. Limb Onset- when first symptoms occur in legs, arms, feet or hands. Lou Gehrig – (1903-1941), American professional baseball player, also known as the Iron Horse because he established a record for the most consecutive games played by a professional baseball player, appearing in 2130 games from 1925 to 1939. From 1923 until 1939 he played first base for the New York Yankees of the American League. Gehrig was twice voted the league's most valuable player (MVP). Stricken with the spinal disease ALS, which later became known as Lou Gehrig's disease. Lower Motor Neuron- Nerve cells starting at the spinal cord or brain stem and ending at the muscle fibers. The loss of lower motor neurons leads to weakness, twitching of muscles (fasciculations), and loss of muscle bulk (atrophy). Miracle Cures –You have to be careful in this area. There are unscrupulous people out there trying to sell useless and expensive products that are bogus. Be sure to use common sense and don’t fall for false claims. Remember, no matter what the advertising states, no matter what is promised, there is no current cure for ALS. A good site to help you identify charlatans and false advertising can be found at: http://members.aol.com/alspinpoint/quack.htmlMotor Neurons-The cells that control voluntary muscle activity such as speaking, walking, breathing, and swallowing.Electrical signals are sent from the brain and spinal cord via neurons which in turn stimulate the muscles. In ALS, these neurons slowly die off, leading to muscle atrophy and the other symptoms of the disease. Motor Neuron Disease- A group of disorders in which motor nerve cells (neurons) in the spinal cord and brain stem deteriorate and die. ALS is the most common motor neuron disease. Progression – ALS advances over time, it is not static. Some PALS progress slower or faster than average. Range of Motion (ROM) - The extent that a joint will move from full extension to full flexion. Many PALS do daily active or passive ROM exercise to maintain flexibility, as recommended by a physical therapist, occupational therapist or other health professional. Rilutek- The only medication so far demonstrated to be effective in ALS, although only modestly.It is quite expensive. Spasticity - An upper motor neuron problem and is present to some degree in ALS. For some patients it is minimal, for others extreme. Spasticity can actually be helpful in maintaining function as the rigidity helps replace normal muscle strength, but it causes jerky, hard to control movements. Spasticity causes a tightening of muscles resulting in a stiffening of that part of the body in an exaggerated reflex. It is actually triggering both the muscles to flex and the muscles to extend that part of the body at the same time. It can occur in any muscles - the arms, legs, back, abdomen, or neck. A simple touch can trigger it and it may last only a moment or persist longer. Sporadic ALS (SALS) – When ALS occurs without afamily history vs. Familial ALS FALS) where ALS occurs within the context of a strong family history. Tracheotomy or tracheostomy is a surgical procedure performed on the neck to open a direct airway through an incision in the trachea(the windpipe). Later in the course of ALS, the muscles that assist in breathing weaken. Bipap is the first line of defense, but when that is no longer adequate to maintain lung function, some PALS opt for a tracheotomy, which is attached to a vent, a mechanical device that replaces spontaneous breathing.Ventilation or Vent- A method to assist or replace spontaneous breathing using a mechanical device.See Tracheotomy. Upper Motor Neurons- Nerve cells (motor neurons)that originate in the brain's motor cortex and run through the spinal cord.In ALS upper motor neuron loss produces stiffness and problems with balance. Vitamins and supplements - The vitamins and supplements and the daily dosage range often used by PALS are vitamins C (1000 to 2000mg ), B complex, E (1000 IU), coenzyme Q 10 (200 to 1800 mg) and NAC (1500mg)Some PALS take many other supplements, too, and use very few. There is a school of alternative medicine that recommends a large variety of controversial supplements and procedures. If interested, you can check this out, but again use your common sense and think critically about claims being made.

CHAPTER 11

READING LIST

As suggested by Jodi Hall and other

Some people enjoy reading personal stories of PALS’ lives from cover to cover. Another strategy is to read only the early parts of the book, and then return later when you feel more ready to process the later parts. There are so many excellent books, below is just a small sampling.

Learning to fall: The Blessings of an Imperfect LifeBy: Philip Simmons Philip Simmons was diagnosed with ALS in 1993.An associate professor of English at Lake Forest College in Illinois, Simmons was just 35. In his quest to help others-and himself-Simmons turned his experience living with ALS into his first book, LEARNING TO FALL: The Blessings of an Imperfect Life. The insights Simmons shares in LEARNING TO FALL are less about self-improvement than celebrating the everyday. Tuesdays with Morrie - An Old Man, A Young Man, And Life's Greatest LessonBy: Mitch Albom Morrie Schwartz was Mitch Albom's college professor and mentor. Knowing he was dying, Morrie visited with Mitch (an ALS patient) in his study every Tuesday. Their rekindled relationship turned into one final "class": lessons in how to live. This book has been on the best seller list over one year. ALSA PAMPHLETS

Maintaining good nutrition with ALS- A guide for patients, families and friends

Caregiving- When a loved one has ALS

Basic home care for ALS patients-The ALS Association guide for patients and families Counting on Kindness: The Dilemmas of DependencyBy: Wendy LustbaderIt deals with disability and aging issues for those of us who rely on the help of others such as guilt and resentment, why "little things" become so important, and the importance of having a regular schedule. For example, disabled people often loose out on the regular activities of life which provide structure, and days and weeks seem to run together. Forgetting which day it is may just be a symptom of lack of schedule rather than developing dementia. Regular visits, trips for meetings, shopping, worship, or coffee can provide time structure, and gives one things to look forward to and some sense of control to the powerless. Closing Comments: ALS A Spiritual Journey Into The Heart Of A Fatal Condition By: Brian Smith I Choose To Live, A Journey Through Life With ALS, By: William Sinton Journey with ALS, By: David Fergenbaum Tales from the Bed, on Living, Dying and Having It All By: Jennifer Estess Waking up By: Terry Wise Charlie's Victory By: Charlie Wedemeyer His Brother's Keeper By: Jonathan Weiner ALS: A Guide for Patients and Their Families By: Jonathan Weiner

Children's books: In my Dreams I Do, By: Linda Saran Lou Gehrig, the Luckiest Man Alive, By: David Adler

CHAPTER 12

CLOSING COMMENTS

What began as an attempt to develop a brief manual for newly diagnosed PALS and their CALS has grown into a large guidebook. Whenever I tried to pare down the length, I realized instead that something important had been left out.And so it grew. Still, there was so much more that I wanted to include, but the manual would have become so lengthy and unwieldy that it would have defeated its purpose. You will have much time to learn more on your own, as soon as you are comfortable with the basics.The goals of the guidebook were several: to ease the pain and suffering that follows the diagnosis and to offer a more positive and hopeful perspective; to provide ideas on how to cope effectively with ALS; to begin to lift the clouds of confusion created by all the unfamiliar material to which you have been exposed; to introduce you to the peer community of PALS and CALS and to provide introductory information that will broaden your knowledge of both ALS and available resources to battle it. And, all contained in one manual, integrating numerous sources of information, and designed to meet the specific needs of those recently diagnosed and their loved ones. I hope some of these aims were met.The emphasis in this guidebook has been on living well with ALS and these pages have pointed you in that direction.It will be the most difficult challenge that you and your CALS have ever faced, but it can be done, as you have seen in the stories in this guidebook. You can do it, too. And there are so many out there to help.I hope you will join me and the many PALS and CALS at the Living with ALS group. You can learn and gain encouragement from our posts and we need to hear your questions and receive the benefits of your insights. We are all one in the fight against ALS. To all new PALS and CALS, welcome to our family.Randy Roberts, PhD (psychologist, retired), age 60PALS, Diagnosed: 4/04

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