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As one year comes to a close and another is about to begin, let's take a moment to pause, reflect and most importantly, count our blessings. We at The ALS Association National Office are most thankful for all of the help and financial support you have given us this year. We are truly grateful to those who have gotten involved with our Chapters, became a Walk Team Captain, or who have created your own special event to benefit our organization. Your determination has truly made a difference and continues to inspire us all!p>By working together we’ve already accomplished so much this year through break-through research discoveries, public policy advances, and fast-track patient care. Let’s continue the momentum through the new year! As we head into 2012, we face tremendous opportunities to maintain our progress and bring us closer to a world without ALS. If you haven’t already, please consider making a year-end tax deductible donation to The Association to help us to continue fighting Lou Gehrig's Disease until viable treatments and a cure are discovered.
This holiday season, let all your family, friends and co-workers know how much you appreciated their help and support throughout the year and remind them where you stand in the fight against Lou Gehrig's Disease. Click the button below to send a holiday eCard to all your loved ones.
Ask the Doc: Q & A with Edward Kasarskis, MD, PhD
Edward Kasarskis, M.D., Ph.D. is Director of the multidisciplinary ALS Center at the University of Kentucky Neuroscience Center in Lexington, Kentucky, professor in the Department of Neurology at the University of Kentucky, and Chief of Neurology at the VA Medical Center in Lexington KY.
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Dr. Kasarskis
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Q: What is your opinion of Rilutek? My husband was diagnosed with ALS three months ago. We’ve talked to two ALS specialists; one doesn’t endorse the drug and one does. My husband just started taking it, but we aren’t sure it will help him.
-- Andrea, New York City
A: Good question. It’s true that, as you have experienced, ALS experts disagree about its relative value. Basically, this is viewing the glass as either half-full or half-empty. Riluzole (Rilutek®) is frustrating because it’s not a perfect drug, and does not stop ALS in its tracks. But, in short, I recommend it. I am in the half-full camp. The evidence-based guidelines for the treatment of ALS developed by the American Academy of Neurology (AAN) conclude that riluzole is safe and effective for slowing disease progression and should be offered to patients.
When we look at the original studies from the 1990’s to get FDA approval for the drug and subsequent studies since then, it appears that the advantages of taking riluzole are consistent. The package insert, which is always conservative, says the drug provides a three month survival advantage -- meaning you are likely to live three more months than if you didn’t take the drug. But several studies done in the last few years indicate that the survival advantage of taking riluzole may actually be greater. It also seems that beginning the drug as soon after diagnosis as possible may offer advantages.
In addition to the modest extension of lifespan riluzole can offer, some people swear it makes them actually feel better. Some say they experience more restful sleep and others feel it controls fasciculations. We estimate that about 50% to 75% of people with ALS are taking riluzole.
There are a few downsides to the drug for some individuals. Some just can’t tolerate it; they feel tired and run down. Of course, it’s hard to know if those symptoms are side effects of riluzole or simply part of what people with ALS often experience. Sometimes people who are older seem more prone to these side effects than are others.
It’s important to monitor liver function enzymes for the first few months (your family doctor can easily do this), but there is usually no permanent liver damage. And I recommend people start with an initial dose of just 50 mg a day, and then after a week take 50 mg twice a day. Studies that have looked at what dose is ideal have found that 100 mg is better than is 50 mg, but upping the dose to 200 mg conferred no additional benefit.
And riluzole is expensive. Without insurance or third-party coverage, the out-of-pocket cost can be about $1,200 a month. With coverage, co-pays tend to range from $20 to $200 a month. Medicare and the VA cover it, and the National Organization for Rare Diseases can help support the cost for the indigent.
No single drug or treatment should be viewed in isolation when dealing with ALS. The best bet is to tackle the disease with a comprehensive approach, including nutrition, respiratory therapy and support, physical therapy, occupational therapy, social support, and, yes, riluzole.
If you would like to submit questions for a future Q & A, please send your questions to Amber Walters at awalters@alsa-national.org. Please understand that we won’t be able to address all questions and we won’t be able to respond to individuals personally.
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Tips for Flying During the Holidays
By Barbara Bronson Gray, RN, MN
 If you don’t have your own sleigh and nine bright reindeer, getting places over the holidays can be challenging. It’s not easy for anyone these days, but for people with mobility problems, assistive devices and lots of gear, flying can be frustrating, exhausting, and demoralizing. But with some careful planning, reliable information and a little luck, you can help ensure happy and successful travel.
Unfortunately, despite federal rules requiring airlines to meet the needs of people with special travel needs, issues sometimes arise. It’s important that you alert the airlines to your needs and ensure they’re ready to support and accommodate you. Keep a notebook of the names and titles of everyone you speak with.
Planning is critical. Whenever possible, book flights well in advance and be sure to tell your travel agent or airline representative if you have assistive devices, mobility equipment (manual or powered), dietary requirements or need for help opening packages on a meal tray. Tell them whether someone will be with you. Call the airline directly to help ensure any and all of your disability-related needs will be met. Select the best seat on the flights you reserve with as much room as possible. Some airlines are reserving front row and other seats with more legroom for people with special needs.
If you’re planning to fly internationally, know that while some airlines have great reputations for being helpful to people with special needs, others do not. Some foreign airlines require a medical certificate for people with disabilities or may even require a personal assistant or travel partner to accompany you. If you do not need to travel with another person, U.S. and Canadian law protects passengers with disabilities from being required to bring an assistant, including on foreign flights to and from the United States.
If it’s difficult or impossible for you to use the airplane bathroom, consider booking shorter connecting flights (which can also be less expensive). But be sure to allow at least 90 minutes between connecting flights to help ensure adequate time to transfer between gates (and get to the restroom).
While you’re planning, don’t forget to make arrangements for travel to and from the airport. Again, be sure the taxi or shuttle companies understand any mobility or assistive needs or other support that you’ll require.
 Airport security can feel like a huge hurdle. It helps to know a few important things. All disability-related equipment, aids and devices are allowed through security checkpoints, although they are screened. If you use oxygen, tell the TSA agent if it can’t be safely disconnected and ask for an alternative inspection process. Tell the security officer your level of ability – whether you can walk, have limited arm movement, have difficulty talking, and be sure to let them know if you have any special equipment or devices. Your wheelchair or scooter will be inspected; it’s OK to stay in the chair for the inspection if you tell the agents you cannot easily get out. If your disability isn’t evident, tell the security officers and ask them for assistance or alert them to the fact that you may have to move through the process more slowly than will others. Medications no longer need to be labeled; they will be x-rayed, or you can request a visual inspection. BiPAP machines and respirators are allowed but also need to be screened. You will be asked to take the machine out of its carrying case and put it in a bin for x-ray screening. (Face masks and tubing can stay in the case). To keep the equipment clean, bring a plastic bag and put the equipment in the bag before putting it in the bin.
Once you’re snuggled into your seat, you may want to have a few items handy to make your flight more pleasant. Consider bringing eyeshades and earplugs to block out the light and the noise. Look for eyeshades that don’t press on your eyes and have an adjustable strap (bucky.com) and be sure your earplugs are at least NRR33 rated (available at most drugstores). If you like music or books on tape, bring an iPod or MP3 device with earphones or headphones. Consider having a small inflatable travel pillow for neck support and comfort. Antiseptic wipes are great to have and you can use them to wipe off the tray table to ensure a more hygienic environment.
 There are consumer protection rules under the U.S. Department of Transportation that protect people with disabilities: http://airconsumer.dot.gov/publications/flyrights.htm. You are not required to give advance notice of having a disability but you do need to give up to 48 hours notice for certain accommodations that could require preparation time, such as respirator hook-ups, transportation of an electric wheelchair on a plane with fewer than 60 seats, etc. The number of people with disabilities on a flight cannot be limited. And no matter what you need to bring with you, items stored in overhead bins must conform to FAA rules for carry-on baggage.
All this makes air travel sound utterly daunting, but it is do-able and can be very rewarding, fun and well worth the effort. If you know the rules and do all you can to make sure the airlines are ready for you, you’re likely to have a good travel experience.
Here are some resources you may find useful:
- www.travelinwheels.com: encouraging disability travel around the globe by providing ideas about accessible places in the U.S. and abroad
- www.disabledtravelers.com: provides resources such as travel agents, tours, accessible van and equipment rental, access guides, and ideas
- To help manage flight problems from your iPhone, iPad or Android, use Flightboard, an app that shows arrivals and departures for any airport in the world, viewed in real time. The app costs $3.99; visit the Apple or Android app store.
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Advice from the Trenches
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Sandra Stuban
with her husband, Steve
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Sandra Lesher Stuban is an RN who was a 38-year old Lieutenant Colonel in the Army when she was diagnosed with ALS. She’s the author of The Butcher’s Daughter: The Story of an Army Nurse with ALS, in which she describes her journey openly and honestly. Although she is completely paralyzed and uses a ventilator, she lives an active life as a writer and nursing leader.
Q: Every holiday seems more and more precious to me. Do you have any ideas about how I can give gifts to family and friends that will always remind them of me, and perhaps help them remember my philosophy and thinking about life, too?
A: Personalizing holiday gifts is a great way to remind family and friends of your contributions, convictions and principles related to work, family values and living in general. These kinds of gifts can be very satisfying to give and meaningful for the recipient.
Here are three gift ideas that are expressive, practical and affordable:
- A favorite book or movie can convey part of your personality. This gift can be further personalized by including a note that explains why it’s so special to you.
- A customized photo calendar. Several websites offer easy-to-design calendars using your own photos for each month. One possible theme could be a chronicle of your life in pictures including photos as a baby, toddler, school years, graduation, and your adult life. Another option could be family photos on summer vacations, special events, and holiday gatherings. The possibilities are endless. Here are two websites that offer this service: http://www.lulu.com/publish/calendars/ and http://www.zazzle.com/custom/calendars.
- A motivational framed poster. There are thousands of these inspirational posters and art prints available that can express your exact sentiments. Many use a single word like achievement, imagination, persistence, perseverance and courage against a stunning nature-based background that exemplifies that particular value. Other choices include the “Live One Day at a Time” print by the Dalai Lama and the “Character” print that says “Character is doing the right thing when nobody is looking.” Many more choices are available at allposters.com, for example.
If you would like to submit questions for a future Q & A, please send your questions to Amber Walters at awalters@alsa-national.org. Please understand that we won’t be able to address all questions and we won’t be able to respond to individuals personally.
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Participate in these Educational Web Calls
 Harnessing Technology to Create Family Keepsakes
December 12 (Monday) at 11:00 a.m. PT (2:00 p.m. ET)
With Alisa Brownlee, ATP, ALS Association, Greater Philadelphia Chapter
Learn how to use phones, cameras and computers to easily create lasting family keepsakes.
Call-in toll-free number (US/Canada): 866-699-3239
Meeting Number: 824 581 864
Meeting Password: TopicCall2011
https://alsa.webex.com/alsa/j.php?ED=158583397&UID=1188059197&PW=NMGI1NWZlNDJk&RT=MiM0
Research Update
What You Should Know About C9orf72
December 13 (Tuesday)
A newly-discovered mutation is the most common known genetic cause of familial ALS and also accounts for some sporadic ALS cases. Researchers now have new clues about ALS and familial frontotemporal dementia (FTD). Hear Rosa Rademakers, PhD, and Bryan Traynor, MD discuss the implications of the new research.
Rosa Rademakers is with the Department of Neuroscience at the Mayo Clinic in Jacksonville, Florida. Her research is centered around the molecular genetic analyses of neurodegenerative diseases with a main focus on frontotemporal dementia (FTD) and ALS.
Bryan Traynor is Chief of the Neuromuscular Diseases Research Group in the Laboratory of Neurogenetics at the National Institutes of Health (NIA) and an adjunct faculty member of the Neurology Department, Johns Hopkins.
For information about how to join this call, please visit http://web.alsa.org/site/Calendar?id=147881&view=Detail.
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Open Enrollment Announced for
Cytokenetics CK-2017357 ALS Drug Trial
Cytokinetics, Incorporated, manufacturer of the FDA-designated orphan drug CK-2017357, recently announced that it has opened enrollment in a third Phase II clinical trial for people with ALS. The primary objective of this phase of the clinical trial is to evaluate the safety and tolerability of CK-2017357. A secondary goal of the trial is to evaluate the ALSFRS-R, other measures of pulmonary function, muscle strength and fatigue, relationships between dose, plasma concentrations and functional effects, and physician and patient global assessments in the participants.
This clinical trial is a double-blind, placebo-controlled, ascending dose study to evaluate the safety, tolerability, and pharmacological effects of multiple ascending doses of CK- 2017357. At this time, an estimated 24 patients with ALS who are also receiving the drug riluzole are scheduled to be enrolled at approximately 10 study centers in the United States. The trial’s participants will be randomized to one of two dosing groups and receive twice daily doses of CK-2017357 or a placebo. Clinical assessments will occur at pre-determined times during the course of the treatment, and participants will also take part in follow-up evaluations one week after receiving their final dosage.
For information about enrollment, go to www.clinicaltrials.gov. Click on "Search for Clinical Trials" and enter "CK-2017357 and ALS." After clicking on the title of the clinical trial and scrolling down to the "Contacts and Locations" section, you will see the names of the sites participating in the trial. If one is convenient for you and is indicated as "Recruiting," you may contact that site directly or email jmasonek@cytokinetics.com for additional information.
In addition, Jeremy M. Shefner, M.D., Ph.D. made a presentation on CK-2017357 at the 22nd International Symposium on ALS/MND in Sydney, Australia. Results showed those in the trial tolerated the daily dosages of CK-2017357 with minimal side effects. Some recipients experienced mild dizziness during the initial phase of the treatment, but the dizziness appeared to cease as dosing continued during the two-week treatment period.
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New Issue of Research ALS Today Available
 The Fall 2011 issue of Research ALS Today is now available. Published twice a year, Spring and Fall, the magazine provides articles and information about current ALS research and activities of The ALS Association. Many of the articles are written by investigators, who are making progress in the search to answer important questions about amyotrophic lateral sclerosis. Research ALS Today is edited by ALS Association Chief Scientist Lucie Bruijn, Ph.D., a former ALS research investigator who remains focused on the cutting edge of ALS research.
This issue highlights resources and journal news and features the following topics:
- TREAT ALS Research Portfolio
- RNA Processing
- Call for Research Abstracts
- Safenowitz Post-Doctoral Fellowships
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