Ask the Doc:
Q & A with Edward Kasarskis, MD, PhD
Edward Kasarskis, MD, PhD is Director of the University of Kentucky ALS Multidisciplinary Clinic at Cardinal Hill Rehabilitation Hospital 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.Q: This may sound odd, but my husband, who has ALS, and I were visiting a friend. She became ill and was vomiting, and all I could think of was what would happen to my husband if he became sick to his stomach? What normal things that the body does could become difficult for a person with ALS?
Dr. Kasarskis: This is an excellent question. I can see how this would be a concern. For someone with ALS, the reflex to vomit would most likely be intact. The issue would be that your husband would be more prone to aspirate (inhale) what he might vomit than would be someone without ALS. If your husband feels he is going to vomit, it’s wise to turn him on his side right away to protect his airway and lungs, and to help get the material out of his mouth as soon as possible.
Other normal bodily functions can pose special challenges for people with ALS. Although bladder control remains normal some ALS patients voluntarily reduce their fluid intake to limit the number of trips they have to make to the bathroom. This may make some sense from a mobility perspective, but this approach cannot be endorsed since dehydration creates its own set of serious problems. Constipation is very common and can be a side effect of medications. However, many diets are lower in fiber than they should be, and most people with ALS get less exercise than before they developed ALS. Stool softeners, a bulking agent (like Metamucil) and fluids are helpful.
For some people with ALS, being able to cough productively can be difficult. For someone without ALS, coughing is normally easy. We take a deep breath and cough without even thinking about it. But for people with ALS, taking that deep breath can be difficult. Respiratory therapists teach a technique called “air stacking”: the person takes a series of four to five smaller breaths to inflate their lungs incrementally, and then they have the air volume they need to produce an effective cough. There are also assistive mechanical devices that inflate the lungs with gentle pressure by mask, and then the pressure switches to a vacuum-type function and the machine sucks out secretions.
Mouth secretions can be a challenge, too. If they’re too thick, perhaps from dehydration, they can be difficult to cough up. A thinning agent, such as Mucinex® (which is available over the counter) can be effective. But if secretions are too thin, they may increase a person’s propensity to drool. The best bet is to work closely with a Respiratory Therapist and your physician to deal with these issues when they come up.
There are some functions of the human body that do not change due to ALS. Sex, for example, continues to work as it did before the person developed ALS, although positioning can sometimes pose challenges and fatigue can interfere with desire. Other functions directed primarily by the autonomic nervous system – such as digestion, urination, defecation -- are typically not affected.
Don’t assume that you can’t ask about these basic issues. Whatever questions you have, be sure to ask your physician and other professionals who are supporting you through your journey with ALS.
Each issue we feature a leading neurologist specializing in ALS responding to a question. 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. TOP
Flying? Now Your Respiratory Equipment Must be Labeled by the Manufacturer
If you, a family member or friend uses a ventilator, respirator, CPAP, BiPAP or personal oxygen concentrator, here is some information from the Department of Transportation that you need to know.
There’s good news and bad news. The good news is that the U.S. Department of Transportation (DOT) has issued a new rule, “Nondiscrimination on the Basis of Disability in Air Travel;” and the rule relates to existing Federal Aviation Administration (FAA) regulations. The rule applies to passenger aircraft with 19 seats or more. It’s designed to provide a way to ensure that people using these respiratory devices can still fly.
The bad news is that electronic respiratory assistive devices must now be tested and labeled by the manufacturer as meeting the FAA requirements. Without the label, the airlines can refuse to seat you. But if the label is there, (and other conditions in the fine print are met), the airline must allow you to board. The FAA also requires that those relying on respiratory equipment ensure they have enough battery power to run the equipment for at least 150% of the anticipated maximum flight time. Some airlines also require that those flying with respiratory equipment notify them at least 48 hours in advance of the flight, and insist the passenger appear at the gate an hour before boarding time so staff can make sure the equipment is labeled as required. Some experts suggest that you carry a copy of the rules and requirements with you when you travel.
Visit the DOT site to read the entire rule: http://airconsumer.ost.dot.gov/rules/rules.htm.
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Developing Emotional Endurance
Getting the diagnosis of ALS is just the beginning. After an initial stage of adjustment, you may experience a long period of ups and downs, and will continually experience a roller coaster of good news and bad news, treatments, lab work and health assessments. Some days and weeks are good and others may be more challenging, frustrating and discouraging. Gradually you adjust but you may find yourself feeling like an emotional yo-yo.
Experts say dealing with a disease like ALS naturally creates great emotional challenges. But they say that if you face the situation the way a cyclist tackles a Century (a 100-mile race), you can develop emotional endurance.
That’s what emotional endurance is all about: learning to cope in such a way that your energy is not wasted on stress but is applied to actively and constructively coping. Such mental stamina will help even out the ups and downs and provide you with a sense of stability and greater self-confidence.
But it’s not easy. Here are some tips:
- Take a practical approach if you’re feeling overwhelmed. Make a spread sheet list with as many columns as you need, and head the columns with the type of issues you’re facing. Perhaps one column will be “Questions,” another may be “Worries,” “Fears,” and there may be logistical issues, like “Legal Paperwork,” “Finances,” Insurance Problems,” and “Disability Application.” The headlines at the top of the columns will vary between people, but be sure what you identify is what is really overwhelming you. Then write in each column everything you can think of that is bothering you under that category. Next step? Review each column with the person best able to help you sort through the issues and solve them. Do this frequently. It will clear your head and help solve problems. It’s a lot harder to stew about things when you’re addressing the questions head on.
- Try to talk about your fears. For some, it’s the overall uncertainty of things that is most difficult to cope with; for others, it’s just the everyday issues that highlight their ultimate concerns about the course of the disease. While often very difficult to do, experts say that just discussing your fears releases your stress level and allows you to accept some of the questions and issues you’ve shared. You can develop emotional endurance by finding a way to look at your fears honestly and get answers to many of your concerns. Physicians, nurses, physical and occupational therapists, respiratory therapists, nutritionists, lawyers, financial counselors and clergy, among others, may be able find practical and realistic solutions to the problems and fears you’ve identified.
- Refuel. Just as a cyclist eats high-energy food, sips Gatorade® and takes breaks now and then, people with ALS and their families and caregivers need to take care of themselves. Do things you enjoy. See friends. Stay connected. Take a nap. Watch a sunset
- Establish a routine you like. Routines can get boring, for sure, but they provide a sense of stability in the chaos and challenge of dealing with ALS. Develop a schedule that works for you and includes what you need to and want to do.
- Walk the steep inclines. Even the best cyclists have to get off their bikes and walk when a hill is just too tough to tackle. That’s true for people with ALS, too. When the emotional challenges are toughest, give yourself a little breathing room and allow yourself some space to rebalance. Read something you find inspiring. Talk to someone you love and trust. Do something a little wild and crazy. Whatever works, know that endurance is built in the steep inclines and special challenges.
- Identify your best coping strategies and use them. Music, bird-watching, time by the ocean, star gazing, movies, comedy shows: do what works for you. But know that such diversion and engagement is no longer just an option; it’s required for people enduring significant stress and strain. Build it into your day.
Everyone is different, and you may find that you have your own ways of developing emotional endurance. But just identifying your need for that marathon attitude will help you remain cognizant of ways to maintain it. Such resilience will reduce your stress and leave you more energy for what you really want to be doing.
Resources
- The Resilience Factor: 7 Keys to Finding Your Inner Strength and Overcoming Life's Hurdles, by Karen Reivich and Andrew Shatte (Paperback).
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Protective Gene Enables People with ALS to Live Longer, Study Finds
By Richard Robinson A new genetic discovery may help researchers understand factors that improve survival in people who have amyotrophic lateral sclerosis (ALS). The discovery, made as a result of a study funded in part by The ALS Association, also strengthens the theory that changes in cellular transport contribute to the death of motor neurons, the cells that die in ALS.
“This discovery will help us understand more about the ALS disease process,” according to Lucie Bruijn, Ph.D., senior vice president of research and development at The ALS Association. “That knowledge will help us develop better treatments for patients with the disease. This work also highlights the value of genome-wide studies of ALS patients and global partnerships.”
An international consortium of scientists performed the study, which looked for normal variations in the genetic material, or DNA, of almost 3,000 patients with ALS. They found that patients whose DNA contained a specific form of one gene (called the C form) survived longer than patients whose DNA contained a different form (the T form).
Every person carries two copies of the gene in question, called the KIFAP3 gene. The researchers found that patients whose two genes both contained the survival-promoting “C form” lived about 14 months longer than those whose two genes were both of the “T form.”
The study was carried out in patients with “sporadic” ALS, those for whom no genetic cause or family history is known. About 90% of all ALS is sporadic. The researchers showed that the KIFAP3 gene forms did not influence the risk of developing ALS, only survival once the disease began.
The KIFAP3 protein helps transport cargo within nerve cells. The survival-promoting form appears to reduce the amount of KIFAP3 protein that motor neurons make. It is not clear how this increases survival, and this is a question scientists will now address. Altered cell transport function has previously been implicated in the ALS disease process. Further work also will be needed to replicate the genetic finding in larger populations of ALS patients.
“This discovery is important, because it provides an important clue about progression in ALS,” said John Landers, Ph.D., of the University of Massachusetts Medical School, who is lead author for the publication. “As we learn more about this gene, we may be able to develop therapies that mimic the protective function of this newly discovered variant, which may benefit all ALS patients.”
Access to the paper is available at http://www.pnas.org/content/early/2009/05/15/0812937106.abstract
The ALS Association's mission is to lead the fight to cure and treat ALS through global, cutting-edge research, and to empower people with Lou Gehrig’s Disease and their families to live fuller lives by providing them with compassionate care and support. Click here to contact your local chapter, certified center or clinic.
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