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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, nursing leader and mother of a teenage son.

Q: I am having more difficulty swallowing. I think I will soon need a feeding tube. But I am nervous and feel like I am losing my fight against ALS.  Do you have any advice?

A: Sandra Lesher Stuban: A feeding tube, also known as a PEG (percutaneous endoscopic gastrostomy) or g-tube (gastric tube), is actually your way to win this one battle against ALS. ALS isn't defeating you, rather you are defying its paralyzing effects by using a simple comeback: the feeding tube. You can also look at a feeding tube as a pair of glasses. You could do without it but why make yourself miserable. Here are some general tips for living well with a feeding tube.

I am an advocate of keeping things simple. I know some people insist on pureeing the same food the family eats. However the same family mealtime dynamics can occur whatever the food choice. Therefore I recommend a liquid total nutrition meal such as Isocal, Nutren, Osmolite, and others. Plus, it's much easier for your loved one.

Caring for a feeding tube is very easy. Simply mix a few drops of liquid antibacterial soap with warm water then use a Q-tip to
clean the skin immediately beside the tube. Using tap water and Q-tips, remove the soapy fluid. And finally, dry the area with
 dry Q-tips. Do this every day. You can use apply gauze around the tube, but I suggest not using tape. The gauze will stay in
place if the split is on the bottom side of the tube.

If you have a length of tube lying on your abdomen, I find it convenient to loop the tube in a loose knot when not being used.
This keeps it from inadvertently being pulled during transfers.
     
As a precaution, it's best to take an acid reducer.  It's very easy for stomach acid to enter the tube tract. A feeding tube should be completely comfortable. If you feel burning or pain, this may be the cause.

Ask the Doc: Q & A with Edward Kasarskis, MD, PhD 

Dr. Kasarskis

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: How can I slow the progression of ALS? I hear there is one drug that can make some difference now and then others might be on the horizon?

Dr. Kasarskis:  

This is a question every person with ALS understandably asks. Today there is just one drug approved by the FDA to extend life for people with ALS, and that is riluzole (Rilutek®).  Here we are in the 21st century and it seems we have drugs for just about everything, so it is especially frustrating for patients and families to learn that we don’t have more pharmaceutical options when it comes to slowing the progression of ALS and extending survival.  There are many studies being conducted now and new drug approaches are being tested.

Riluzole was approved based on two survival studies from the 90’s which demonstrated that riluzole extended survival compared to placebo. Some people read or hear about the results described in riluzole’s package insert and get discouraged, asking "is that all there is?" After the initial results were submitted to the FDA for the approval of riluzole, further research showed a consistent survival benefit from taking riluzole.

In the future, we will probably be treating ALS with combination therapy in much the same way oncologists treat cancer, not relying on a single drug to cure the entire disease.  It is also important to realize that during the initial studies of riluzole, ALS physicians did not employ multi-disciplinary care as aggressively as we do now. Life can also be enhanced and even extended by getting multi-disciplinary care, which addresses the full range of issues facing a person with ALS. 

Many clinical drug trials are in the development phases or are underway.  For example, ceftriaxone, an antibiotic already approved by the FDA, is under study to determine if it (alone or in combination with riluzole) will slow the progression of the disease. (Ceftriaxone has other effects, in addition to being an antibiotic, but ALS is not infectious). This is a multicenter study at 60 sites in North America coordinated by Massachusetts General Hospital and supported by the National Institute of Neurological Disorders and Stroke (NINDS). For more information, visit http://clinicaltrials.gov/ct2/show/NCT00349622.

You should discuss participating in this or other clinical trials in your area.  As patients, we all stand on the shoulders of other patients before us who volunteered their time and their bodies to participate in a clinical drug study to prove that our blood pressure medication, or our antidepressant, or our drug for ALS is safe and effective. Clinical trials typically require regular testing and evaluation. They usually provide great psychological benefit, just from participating in something that can further our understanding of ALS and could directly help you and other ALS patients in the future.

Ask the Doc: Q & A with Edward Kasarskis, MD, PhD 

Dr. Kasarskis

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: My husband has difficulty accepting the need to adapt to ALS as it progresses. When the doctor or team members suggest he consider getting some additional help like a cane, or a wheelchair he bristles, and puts off the decision. What can I do?

Dr. Kasarskis: This is a common frustration. And it’s actually tough, since we all want to respect the person’s autonomy and their desire to make their own decisions. Typically, the family tries to let the person with ALS call the shots, but then finds they are getting increasingly anxious as they worry their loved one will fall, or choke on food, or have some other kind of difficulty.

Some people with ALS view recommendations to get a new assistive device, a PEG tube for feeding, or BiPap for breathing as a sign that they’re losing the battle with ALS. So they fight on without these aids. Their approach to the disease may reflect their decision-making style before being diagnosed with ALS. Sometimes someone’s coping style was extremely effective when they didn’t have the disease, but it doesn’t work well now.

It’s important to make sure your husband isn’t depressed, and your physician may want to prescribe an anti-depressive medication. It may also be valuable to have a formal battery of neuro-psychological tests done which may help you (and him) better understand any issues your husband may be dealing with. It is also possible that he may have developed a cognitive inability to make these kinds of decisions or to deal with his emotions effectively, a situation that can occur in people with ALS.

Too, you may both benefit from meeting with a counselor who can help you discuss the challenges you face together and assist you in effectively expressing your concerns about your husband’s tendency to delay getting the assistance he needs to effectively cope with his diminishing neuromuscular strength.

We do know that people with ALS must be good at solving problems because they face a variety of significant serial challenges. As Gilda Radner was fond of saying, “It’s always something.” The trick is to deal with issues as they arise by actively making decisions. Ignoring problems will not make them go away.

Ask the Doc: Q & A with Edward Kasarskis, MD, PhD 

Dr. Kasarskis

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: I hear about so many claims on the internet for megavitamins, herbal remedies and supplements to slow ALS. They all look so promising. Do they help?

Dr. Kasarskis:  It’s not ridiculous to think that plants and natural products might have benefit in ALS. After all, many drugs used in medicine were originally derived from plants; For example, colchicine for gout comes from the autumn crocus (Colchicum autumnale, growing in my back yard), and digoxin comes from the foxglove plant (Digitalis lanata). But when we’re talking about using herbal remedies and supplements to slow the progression of ALS, we have to be careful. Virtually none of these products has been tested in persons with ALS.

Here is what we do know:

• Creatine has been tested in North American and Europe and no benefit was found in slowing the progression of ALS, although one study suggested it might increase muscle strength temporarily.
   
• Vitamin E, in very high doses, was studied in Europe, and showed a modest, but insignificant, benefit in slowing the progression of ALS. There may be, however, negative effects from such doses. An analysis of a wide range of Vitamin E treatment trials for a variety of conditions showed an increase in the risk of bleeding and stroke, raising safety questions.
   
• Co-enzyme Q10, at 2700 mg a day, was studied to see if it would slow ALS by 20% (using the ALS Functioning Rating Scale, revised, or ALSFRSr). That high degree of impact was not found, but a smaller benefit may have gone undetected since the study was designed only to determine if there was a very large benefit. Importantly, no adverse consequences were discovered. Although there may be some value in taking Co-enzyme Q10, it is expensive.

I know all of this is frustrating for ALS patients, their families, and their well-intentioned friends who want to be pro-active in managing the disease. This is very understandable. I just did a Google search on “ALS” which returned 6.8 million hits! The only way we will make real progress in this disease is through well-designed, controlled clinical trials. I strongly encourage your participation at an ALS Center near you —for yourself and for the future.

One of the challenges of ensuring mobility and access to what you most need is that the modern American home is often a bit cluttered. Tables, lamps, piles of books and magazines, and even decorations can all make it more difficult for people with impaired movement and mobility to have what they need at hand.

Here are some tips from organizing and design experts to help you simplify your home environment:

Remember, simplification means saving time and energy for  everyone.

• Create a place you love to be. It might be a sunny room with your bird in a cage, or perhaps a spot with a lovely view of the yard, a restful, cozy corner, or the best seat in the house, right in front of the big screen TV. Make it convenient, have the things you like to use right there next to you, and enjoy it!

• Make an organized effort to declutter. Ensure there’s room to get around your furniture and try to convert your home’s obstacle course into more of an open road.

• Put the things you need for certain activities all together, in a plastic carryall or a basket. That will make it easier for you and will also assist those who are helping you to have what you want when you want it. For example, you could have a plastic carryall with everything you need for a shave, or all your post-shower accessories, or even the items you need to send a bill or a note (right down to the pen and stamps).
 

For more ideas, refer to the following resources.
• Simplify Your Space by Marchia Ramsland
• Organizing Your Home by Emily Wilska
 

Ask the Doc: Q & A with Edward Kasarskis, MD, PhD 

Dr. Kasarskis

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: I have bulbar ALS and am troubled by an enormous amount of thick saliva in my mouth. I am using Robinul, but it doesn’t do much, and the TransDerm Scop patch didn’t help either. Mouthwashes are difficult for me to use because I can’t swish them around or spit the fluid out, since my lip muscles are weak. What else can I try?

Dr. Kasarskis: Dealing with excess saliva can be a very frustrating problem and is quite common.  It can be challenging indeed. People with ALS produce the same amount of saliva as do people without the disease. But people who have trouble swallowing eventually have difficulty swallowing their own saliva. Those of us who swallow normally are regularly swallowing our saliva without even being aware of it.  When the ability to swallow is compromised by muscle weakness, saliva builds up and causes problems like drooling or even choking. This is only made worse when the facial and lip muscles are weak, as you note.

There are several available approaches to the problem:

• Medications: So-called anti-cholinergic medications (such as amitriptyline) can reduce the amount of saliva produced. But they often have side effects: drowsiness, difficulty focusing the eyes, and excessive dryness of the mouth. Some people, especially men, also can find the drugs make it harder to urinate. And sometimes the secretions decrease in quantity but they become thicker and more tenacious, which makes them harder to cough up. It’s frequently a balancing act between reducing the extra secretions and making the mouth and mucous too dry, thereby causing other problems. Some people take an anti-cholinergic drug to dry secretions, along with another drug, such as guaifenesin, that is designed to thin secretions. But even with that combination, some people find that this is not totally satisfactory and additional measures are needed.

• Parotid gland radiation: This is reserved for patients who cannot tolerate anti-cholinergic medications.  The procedure involves focusing low-dose radiation to the parotid gland on one side. The radiation reduces the gland’s ability to produce saliva. Even one session of radiation tends to produce good results. Some people will be able to  lower their dose of anti-cholinergic medications after the procedure. Typically it takes about three to four months to see what the maximum benefit will be from the radiation.

• Oral suction devices: A low-tech solution, these devices, typically used in the hospital setting, help you remove the excess saliva as it builds up (and can also be used for oral hygiene).  A further refinement for oral hygiene is the Plak-Vac toothbrush (click here to learn more). The Plak-Vac is used primarily when brushing the teeth to remove excess secretions during the process.

• Adequate fluid intake: When you lose strength in the muscles that control your mouth, it can be harder to close your mouth tightly, and you tend to lose moisture by mouth breathing.  Thick secretions can be a sign that you are dehydrated and need additional fluid intake, probably via a PEG tube.  If you do not yet have a PEG, you should consider discussing the option with your physician.  If you already have one, you may need to increase your fluid intake through the tube.  Talk with your physician or dietician about the best options for you.

• Moisten the mouth.  Whether a side effect of medications or mouth breathing, you can moisten the inside of your mouth and tongue by swabbing with lemon glycerin swabs.  This will provide some relief for excessive dryness.  These are available from many on-line sources.

Ask the Doc: Q & A with Edward Kasarskis, MD, PhD 

Dr. Kasarskis

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: Can fatigue make ALS worse?

Dr. Kasarskis: That’s actually a more complicated question than it may seem, depending on what you mean by the word “fatigue.” Do you mean the inability to sustain a muscular contraction the feeling that you can’t physically do what you used to be able to do? Or perhaps you’re referring to malaise, that “blah” feeling?

People with ALS have weakened muscles without any reserve capacity. That means the muscles are less effective, which is recognized as fatigue. You may find you hit a wall a lot sooner than you have experienced before. Typically, when you generate force in a muscle, you increase its strength by stimulating the nerve cells in the spinal cord (the spinal motor neurons) to fire more rapidly or by stimulating more nerve cells to fire. This natural process is hindered in ALS because you have fewer neurons, and therefore you will have trouble generating the force you want or need. Muscles will get physically tired sooner. Basically, we all have a physical limit and with ALS you reach your limit sooner than before you developed the disease.

You need to pace yourself and acknowledge your limits. Save your energy for what you most need or want to do. And don' hesitate to use tools to extend your abilities. Roofers use power air hammers, not hand hammers, to maximize their ability to do a job. Like them, you can use tools that will help you be more successful in performing tasks. Talk with an Occupational Therapist or a Physical Therapist about what options are available to minimize our fatigue and extend your capabilities.

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See and read the entire brochure at: http://catfishchapter.org/sites/default/files/catfishfiles/center_brochure.pdf

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: I heard that the American Academy of Neurology (AAN) has just come out with new guidelines for the care of people with ALS. Will those guidelines change the care I'm getting now or what my doctor is doing for me?