The Raging Bear

By Jeff Lester

   Those of us affected by ALS are always attentive to dramatic performances depicting life with this disease. Recently on the television program ER James Woods portrayed a medical professor who had ALS.  The episode, dedicated to this character’s journey with ALS, was extremely moving and a terrific tool for helping viewers understand what life with ALS is like. Mr. Wood’s incredible performance, which should garner him an Emmy and thus create more awareness about ALS, was more than a generalized portrayal of ALS. Rather it was a portrayal that captured even small nuances of living with ALS, such as falling down the stairs (something I did twice early in my progression), being resistant to using adaptive devices and feeling shear frustration from losing physical abilities. I also have to mention Frances McDormand’s Oscar-nominated performance in North Country where her character (based on a real life story) developed ALS. Despite her deteriorating condition she demonstrated how someone with ALS could still make a difference. Her performance raised awareness of the ALS cause through an actor’s fantastic portrayal of life with ALS.  There were, however, some inaccuracies in each performance due to the dramatic license needed to create tension or more drama within a scene. While I have no problem with this generally when watching a show or movie, I wanted to share my observations and address these inaccuracies for the benefit of those who are newer to ALS.

     In North Country, Frances McDormand’s character used a voice wand typically used by someone who has throat cancer. Perhaps this was common when the character had ALS, but there are more effective options for augmentative communication now. MDA is an excellent resource when you need this assistance.

     In ER, the two most apparent mistakes I recognized involve medical power of attorney and placement of a trach. In one scene, the doctor argued with the caregiver over whether or not a trach should be placed after James Woods’ character lost consciousness. The power of attorney controversy was light on details so we cannot be sure if it was a realistic scenario or if the writers were just using some dramatic license. There were some extremely valuable lessons to be lessons to be learned from this exchange. The first lesson is to think in advance about what you want to happen when certain critical issues arise in case you are unable to direct your care. Then make your wishes legally binding by going to a lawyer to draw up all the necessary documents to ensure that your wishes will be honored. If you do this, you should be able to avoid any controversy in your care. The second lesson here is to always take the legal documents with you anytime you go to a hospital setting, especially if your assigned person does not have the legal standing of a spouse. Finally, if somehow a trach is placed against your wishes please remember you can always reverse this decision immediately when you regain consciousness. The drama of the trach placement seems to have caused people newer to ALS the most concern. First, it would be rare that a tracheotomy would be performed on a person with ALS in an emergency room. In the case of an emergency, they would intubate to stabilize the person and then perform the tracheotomy in the controlled environment of an operating room--the normal setting for the procedure. The lesson here is very clear; do not wait until your breathing is critical to have a tracheotomy done. With proper preparation this part of your disease process can be fairly easy and peaceful, whether it’s slipping into unconsciousness with the invaluable assistance of a hospice team or continuing your fight, like I did, with a preplanned tracheotomy.

     As you might know, we opened a small on-line store just before Christmas but I have now turned Raging Bear's Closet (http://WWW.ragingbearscloset.com/) into our personal website detailing our journey with ALS and a place to archive my various writings. We now have opened a totally NEW and separate on-line store called "Living with ALS Shop" with over 35,000 discounted items from fragrances to household and much, much more plus now 50% of the profits from each sale will go to ALS organizations including to the St. Louis MDA!!! To see our NEW store, go to http://www.livingwithalsshop.com/ and add it to your favorites for your future shopping needs! I have also opened an ALS website which is going to be a centralized location for a person dealing with ALS in their lives to find information either through website links or the library of documents; share information in the chat room; create a personal picture album; and finally, sharing their inspirational stories through individual personal website listings or creation.  I am extremely excited and proud of this project!  Go to http://livingwithals.net/ to see this website.

The Raging Bear

By Jeff Lester

    Welcome to my new column that will appear in upcoming newsletters!  Some of you might already know me from my MDA chat, my research digest, the “Living with ALS” Yahoo group or my family’s appearance on this year’s St. Louis Labor Day Telethon. I hope to use this column to share some of my 12+ years experience living with ALS and to address any current issues or questions for those who are living with MD, ALS or a related neuromuscular disease. For future columns, please email me at ragingbear@charter.net with any topics you would like discussed. I decided that for this first column I would tell you about my journey with ALS (for those who don’t receive the ALS newsletter) and an abbreviated discussion of hospitalization problems when you have muscular dystrophy or other neuromuscular disease.

     I was 26 when I first noticed the signs of what would become a very different path for my life. I was diagnosed with ALS in October 1993 following my 27^th birthday after months of frustrating misdiagnosis. Luckily I found organizations to help us out like the local chapter of the MDA. They have not only provided the hope of ground breaking research but also a clinic dedicated to ALS, equipment assistance, support groups, counseling and chats on their national website.

     I committed myself to living as normal a life as possible. To this end, I got married to an incredible woman, Lisa, in October 1994 and we had a beautiful daughter, Kelsey, in May 1995. Our family has expanded some in recent years when we had our second daughter, Emily in July ‘02 and in April ’05 with the birth of a third daughter, Jordan. In December 1997, my breathing had declined to the point where I went on a vent. I have learned so much because of ALS, especially what’s really important in life, the love of family and friends. I discovered that there is much more to life than our physical capabilities and I have grown in many ways, mentally, emotionally and spiritually as I lost physically. I have succeeded in living with ALS by having a positive attitude and especially the love and support of my wonderful wife. I am enjoying raising my daughters; writing various things including a completed children’s book based on Mak Shulist’s life and death that I am currently looking for a literary agent and illustrator for, as well as currently working on a novel; the MDA chat; I put together a monthly World Research News Digest for ALS-TDF; and my most recent endeavor is Raging Bear’s Closet (www.ragingbearscloset.com), an on-line discount department store where 50% of the profits will go to support ALS organizations including the St. Louis MDA  ALS Division chapter. I believe that despite the financial and physical strains of ALS, my wife and I have achieved our goal of living as normal a life as possible.

     A recent brief hospitalization for the stomach flu reminded me just how different and possibly dangerous a hospital visit can be for those of us with physical limitations due to a chronic disease, especially if we have impaired verbal communication. I have run into a variety of problems over the years when I had to be admitted to the hospital, especially early on. These problems have ranged from some staff assuming I have cognitive delay because of my slurred speech—to unnecessary procedures despite my strong objections—to negative attitudes and conditions created by those in charge of my daily care. Do not assume that just because you are going to a hospital that all staff will have an understanding of your specific diagnosis! 

     This is why from now on when I go into the hospital we will take certain steps to assure that my stay will be as comfortable, incident free and as SAFE as possible. These steps will include:

¨      A laminated detailed list of my care directions including the following, to name a few: my health care directive; no catheters unless consented to by me; I am able to eat regular food; instructions on my positioning; details regarding my own ventilator since I don’t like to use the hospital ventilators; trach care instructions; room temperature preferences. (I know some of you are thinking “but shouldn’t they already know how to do some of this?” and that’s exactly the kind of thinking that I have learned will get you in trouble)

¨      A second laminated sheet describing ALS from my perspective, NOT a textbook’s (the more rare your condition the more detail I would include)

¨      A simple communication board and my own shaving supplies (hospital disposables are torture devices!)

¨      Finally, if I am going to be left alone without family for an extended period, a plan with my family for them to call

      and speak directly to me to assure that everything is going okay with my care. 

With just some simple planning, you too can have a more pleasant hospital stay.