Fighting Bedsores With a Team Approach

By AMANDA SCHAFFER
Copyright 2008 New York Times

To understand why some doctors and nurses take bedsores so seriously, it helps to call them by their clinical name: pressure ulcers.

An area of skin breakdown that occurs when sustained pressure cuts off blood circulation — usually in patients confined to their beds in hospitals and nursing homes — a bedsore can result in a wound so deep and painful that some patients require narcotics. If a bedsore becomes infected, the complications can be fatal.

“They are not just little sores,” said Susan D. Horn, senior scientist at the Institute for Clinical Outcomes Research in Salt Lake City. “If you’ve ever seen a very bad one, frankly, it would make you sick. You see a very reddened outer area, then you see, depending on how deep it is, just this hole in the skin, and it goes right down to the bone.”

Experts estimate that two million Americans suffer from pressure ulcers each year, usually through some combination of immobility, poor nutrition, dehydration and incontinence. The Centers for Disease Control and Prevention does not keep statistics on fatalities, but one prominent victim was the actor Christopher Reeve, who died of a bedsore infection in 2004 in the middle of a heroic battle against paralysis.

New research is suggesting that the battle against bedsores requires a team approach, enlisting everyone from nurses and nursing assistants to laundry workers, nutritionists, maintenance workers and even in-house beauticians.

In a study of a collaborative program involving 52 nursing homes around the country, The Journal of the American Geriatrics Society reported last August that team efforts had reduced the number of severe pressure ulcers acquired in-house by 69 percent.

“Preventing pressure ulcers is a 24/7/365 kind of job,” said Jeff West, a clinical reviewer at Qualis Health in Seattle, who helped to set up the collaborative in 2003. “It’s not as if one person can get it all done. And if it fails just a little bit, just during the weekends, for instance, you’re not going to get the results. It takes tremendous consistency.”

At the Lutheran Home in Fort Wayne, Ind., for instance, “the laundry workers helped us see that some clothes weren’t fitting the residents properly and were restricting their skin,” said Jeanie Langschied, a registered nurse there.

The kitchen staff began putting protein powders in cookies to boost nutrition. They added buffet dining, so residents would not remain in one position for so long, compressing fragile skin.

Even the beauty shop “realized that wait times needed to decrease,” Ms. Langschied said, and residents should be repositioned while getting their hair done. “It was all departments looking at everything, and it was just amazing the information that flowed through.”

Lutheran Home was one of the 52 facilities that took part in the collaborative, sponsored by the Centers for Medicare and Medicaid Services. Dr. Joanne Lynn, who helped begin the project when she was a senior natural scientist with the RAND Corporation (she has since joined the Medicare centers), said the goal was to educate nursing home workers in bedsore prevention and to encourage them to come up with creative, low-tech solutions of their own. “It was a combination of education, cheerleading and something like systems engineering,” Dr. Lynn recalled.

The number of superficial bedsores did not decrease to a statistically significant degree, for reasons that are unclear.

At David Place, a nursing home in David City, Neb., staff members say they focused on assessing each resident’s risk for bedsores, and noted this risk on the assignment sheets used by nursing assistants.

“The residents at highest risk,” said Dan Smith, director of nursing, “would be the last up for meals and the first down after meals so they would not be in their wheelchairs for long periods of time putting pressure on their bottoms.” Residents at risk from weight loss were given yellow plates, so that staff members would remember to encourage them to eat more.

David Place also bought new mattresses made of high-density foam to reduce pressure in key areas. Staff members say they redoubled efforts to keep feet elevated with pillows so that bedsores would not develop on the heels. And they began to use new moisture barrier creams with residents who were incontinent, since lingering moisture can speed the development of sores.

Staff members at Palatka Health Care Center in Palatka, Fla., initiated a similar blend of measures. They created a “skin-watch action team,” or SWAT, to identify vulnerable residents and to make sure that their heels were floated, that they were given pressure-reducing cushions and that they were repositioned frequently, said Carol Jones, a risk manager at the center.

“We got the grass-roots level, the certified nursing assistants, much more involved, and they were held accountable,” Ms. Jones said. If a bedsore began to develop, she said, “we’d ask them, how did this happen?”

Initially, as the collaborative collected data from participating facilities, the incidence of pressure ulcers did not appear to change, Dr. Lynn said. It was only when researchers focused on data for the most severe bedsores that they saw an improvement.

Clinicians document four stages of pressure ulcers, in which Stages 1 and 2 are superficial sores and Stages 3 and 4 are deep wounds that result from death of the skin and underlying tissues.

“In good care, almost all new stage 3 or 4 pressure ulcers show up fully formed,” Dr. Lynn said, meaning that they do not begin as superficial bruises that then go deeper. The injury, she said, “appears to be in the deep tissues from the start, though it can take a few days for the extent of dead tissue to become apparent.”

The deeper sores may have different underlying causes than the superficial ones, she said. But it is unclear why the less severe ones did not respond as well to the practices instituted by the collaborative.

Dr. Horn, of the Institute for Clinical Outcomes Research, praised the collaborative as “the first major national effort driven by Medicare to reduce pressure ulcers.” But she said that better outcomes could be achieved if more nursing homes improved their documentation, so that all of the information on a given resident, including details on eating, urinary and bowel function, appeared on a single sheet, with key reminders to nursing assistants and other staff members about best practices.

Institutional change and work-flow redesign are critical, she added, given the high rates of turnover in nursing home staff across the country.

The changes need to become hard-wired in an organization, said Mr. West, of Qualis. “A lot of places do well when they have a lot of support,” he said. “But it’s hard to keep that momentum going. That’s the real challenge.”

Statewide efforts to reduce pressure ulcers are also under way in California, New Jersey, New York and elsewhere.

Bedsores are “a major quality-of-life issue, and a self-esteem issue,” said Joanie Jones, a nurse at David Place in Nebraska. “No one wants to have sores on their bottom. I don’t care how old you are. You still want your skin intact.”

Happiness and a Trach: They Really Can Go Together

by Jack Orchard, Founder of Extra Hands for ALS
Copyright 2008 Extra Hands for ALS

More than 90% of people with ALS choose not to get a tracheostomy when they lose the ability to get enough oxygen unassisted. Put another way, they'd rather die than use a ventilator. Of course, this choice is a purely personal one which no other person can dictate. But I suspect at least some people opt out of getting a tracheostomy because they think that living with a trach sounds unbearable. While trach management certainly requires some effort and thought, I promise it can be reduced to no big deal. And I know so because I've had a trach for 16 months.

What Is a Tracheostomy?

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A tracheostomy is a surgical procedure in which an incision is made at the base of the neck, just below the larynx, and a tube inserted which can be attached to a ventilator. The tube, which many people call "a trach" (pronounced trake) is about 4 inches long and curved. When inserted, the upper end points forward protruding from the incision, and the lower end points downward inside the trachea, or windpipe. At the lower end around the outside of the trach tube, which is made of hard plastic, it has a small balloon that is inflated or a small spongy cuff. This serves several purposes. First, it holds the trach more or less steady in the trachea. Without it the tube might rub against the trachea which, like anything down there, would set off a coughing fit.

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The balloon or cuff eliminates this possibility because it is nearly impossible to feel. Second, the balloon or cuff closes off access to the trachea from the nose and mouth. This is important because if you tried to inflate the lungs with a ventilator but there was a free passage up to the nose and mouth, the air would escape upward and little or none would make it down where you need it, in the lungs. Third, the balloon or cuff prevents most saliva from dripping down into the lungs. Even people with full control of their throat muscles sometimes aspirate a little saliva or liquids or food particles. For people with ALS whose control of throat muscles is compromised, aspiration can lead to pneumonia very quickly. The trach greatly reduces this risk.

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On the outside of the trach there is a plate about an inch and a half wide that rests comfortably against the skin. It has small holes on either side through which are threaded the ends of a soft collar. The collar aids in keeping the trach still and reducing the risk of pressure sores around the trach incision. When in use, the trach also has a disposable plastic tube inserted in it, called an inner cannula. The inner cannula is replaced every day. This is important because any secretions, such as saliva, mucus, or phlegm, can dry and harden on the inside of the trach. If this builds up the trach can get clogged reducing airflow through it. Also, any secretions become possible nesting places for bacteria. Rather than having to replace the trach itself every day, using a cannula makes trach maintenance and hygiene simple and easy.

Trach Requirements
Mother Nature never planned on having an incision in the lower neck. Like any other cut she tries every minute of every day to heal it by sending blood and lymph to it. These fluids, as well as any saliva that slips past the balloon or cuff needs to be removed from the lungs before they cause pneumonia. This is the part of having a trach that's just no fun whatsoever. To remove fluids from the lungs you have to insert a long thin rubber catheter attached to a suction machine through the trach and down deep into the bronchial tubes and suck it all out. This process is not painful, more like ticklish. The problem is that a tickle deep in the bronchia sets off a coughing reflex. Your body will try to cough until the catheter is removed. Suctioning takes just a few seconds in each bronchial tube, so even if you're fully dependent on a ventilator, the suctioning process won't make you hungry for air.

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Proper suctioning technique takes a little practice, both for your caregiver who needs to learn how to do it quickly and effectively, and for you to identify where in your chest needs suctioning. That may sound strange, that you won't be able to tell where your lungs are congested, but at first it will be hard to place it. Before long you'll become an expert. A suction catheter is about a foot long made of flexible rubber. Its bottom end, the one that goes into the bronchia, has a curved tip to make it easy to guide down into the side you want. The other end has an air port, a little hole so the caregiver can manipulate the catheter's suctioning strength. You should always be on a ventilator when suctioning because your cough reflex may take several seconds to relax away. During those seconds you'll need air. Having the ventilator to help you calm the reflex is a big help.

When inserting the catheter the caregiver should leave the port open so the other end going into the bronchia doesn't stick to anything on the way in. You want the catheter to go as deep as possible. When it's in all the way, a point the caregiver can feel easily, it's suctioning time. The caregiver covers the air port with a thumb and pulls the catheter out slowly while twirling the catheter with the other hand. This makes the bottom end deep in the bronchia sweep around and pull out all the secretions. I usually suction my right and left sides at least twice to make sure we've gotten everything. This makes a big difference for my unassisted breathing. When I'm congested I struggle to get enough air, but when we clean out my lungs I can still breathe comfortably on my own.

Another very important aspect of suctioning is hygiene. While it's not necessary to maintain absolute sterility of the suction catheter, it's nonetheless important to keep it clean. Any foreign material introduced into the lungs can lead to an infection. Caregivers should wear protective surgical gloves at all times during suctioning. The catheter should never touch anything. Once you remove it from its sterile packaging you should not set it down on anything. Keep it held closely in a gloved hand. For me, suctioning usually starts my salivary glands going, so we swap out the catheter for a long, hard plastic tube called a yankeur that we sweep around my mouth. This takes a little practice, not clearing saliva from my mouth, but manipulating the yankeur while keeping the catheter from touching anything.

Another aspect of trach hygiene is keeping the trach itself clean. You should clean the incision and every surface you can reach every day. The skin around the incision will toughen with scar tissue, but at first it will be tender. Your caregiver should wet a cotton swab such as a Q-tip with wound cleanser, and then scrub all around the incision as well as inside the trach. Because you'll be replacing the inner cannula every day anyway, remove it during trach cleaning and swab inside the exposed trach. If you are ventilator dependent, you can skip this last step but it's important to keep your trach as clean as you can get it.

Deep vs. Shallow Suctioning
The method described above is for removing secretions deep in the bronchial tubes. However, sometimes the secretions you need to get are just below or even in the bottom of the trach. These tend to come from fluids trapped above the balloon or cuff. Neither a balloon nor a cuff will form a perfect seal in the trachea separating the lungs from the nose and mouth. Whatever secretions are resting on top of the seal will inevitably seep down. Those fluids tend to be quite thick so when they drip past the seal the in-and-out rush of air from your breathing will push some of them into the bottom of the trach. When this happens you'll feel a light vibration in your chest just like when your lungs need deep suctioning. But the difference will be that you'll feel that vibration high in the chest in the middle rather than deep down one side or the other. This situation calls for a shallow suction.

Shallow suctioning differs from deep suctioning in three respects. First, the caregiver is only going in the trach a few inches. Second, the caregiver will hold the catheter air port closed going in as well as pulling out. And third, the caregiver will twirl the catheter going in the trach. Both you and your caregiver will hear when the catheter tip catches the secretions. When you hear that, quickly twirl the catheter at exactly that spot to clear it from all around the inside of the trach and then pull the catheter out. In most cases requiring shallow suctioning, the secretions are inside the trach. As a result, the catheter doesn't need to touch the trachea or bronchial tubes, and you won't feel a thing, no tickle, nothing!

Balloon Inflation and Deflation
Because saliva, mucus, and phlegm can accumulate on top of the balloon, occasionally you'll feel like you need to swallow or clear your throat when it reaches the point where your trachea and esophagus separate. This can be annoying. No matter how much you try swallowing or clearing your throat it won't go away. My solution for this problem is one my pulmonologist cautioned me not to do, but the solution works well when done properly.

When I feel the accumulation of secretions on the balloon I have my caregiver deflate the balloon completely. The secretions ooze past the balloon, then we quickly reinflate and deep suction. My doctor advises against this because he says it's never a good idea to let anything get in the lungs. Strictly speaking he's absolutely right. But when you and your caregiver become experts at suctioning you can reduce the risk of pneumonia to zero in this situation. And you'll feel much more comfortable having removed the secretions trapped above the balloon.

When you deflate the balloon you'll feel a few things. First, the bottom end of the trach may touch the lining of the trachea. It will tickle which may set off your coughing reflex. Second, because you have removed the seal cutting off access to your nose and mouth, the air getting pushed into you from the ventilator will escape upwards. This means your lungs will get less than they're accustomed to getting, and as a result you may feel some shortness of breath. If you feel bad just reinflate the balloon, suction whatever oozed past it, and repeat until you feel you've cleared out the accumulation of secretions.

How Often Should I Suction?
As often as you need to. Having congested lungs feels uncomfortable, so get rid of it! In the days after I had a tracheostomy I asked one of the nurses in the pulmonary intensive care unit where I was staying about this. She said I would need suctioning every 15 minutes. WHAT?!?! I was shocked and saddened by that. Fortunately she was way off. I suction in the morning when I wake up, I take a pill (through my feeding tube) called robinol which dries up my salivary glands, and then I'm DONE FOR THE WHOLE DAY!!! That's right. All day. I spend my days in an office chair in my study, most of the time breathing on my own but sometimes using a ventilator, and most days I don't need suctioning until I get back in bed at night. The idea that I'd need suctioning every 15 minutes is not just wrong, it's ridiculous. You can get there too. It takes effective and thorough suctioning in the morning to clear out the lungs as completely as possible, and taking a pill to reduce my body's production of secretions.

Why does this work? It's a simple secret: Wet lungs get wetter, but dry lungs stay dry. If you don't remove all the secretions in the morning your body will attack what it perceives as a threat and send fluids into the lungs to fight off infection. Clearly, this magnifies your congestion. Wet lungs get wetter. However, if your suctioning technique is thorough you can get your lungs dry enough that there's no threat of infection, and consequently you'll stay dry for hours, even all day.

Another Trach Management Issue
Trachs get dirty. It's an inescapable truth. Because you just can't get your trach pristinely clean every day, it needs to be replaced regularly to avoid becoming a nesting ground for bacteria. Trachs with balloon cuffs, made by a company called Shiley, need replacement every month. Trachs with sponge cuffs, made by a company called Bivona, need replacement every 1-2 weeks. There's no question that trach replacement is the least pleasant aspect of living with a trach.

To replace a trach you need to be in bed using a ventilator regardless of your breathing capability. Your caregiver should roll up a towel until the roll is 3-4 inches in diameter. Place the roll under your upper back so your head arches back, not just a little but way back so your neck is fully exposed. This position opens your trachea significantly. Those who have taken classes on CPR will recognize this position as the necessary one to ensure the trachea is as open as possible. Next, your caregiver should remove the trach collar, remove the ventilator, and deflate the balloon completely. Then pull the trach out. I won't lie, this hurts. The incision is smaller than the diameter of the trach with the balloon around it even when deflated. Your skin will stretch more at first than later when it toughens with scar tissue. Pulling out the trach will make the incision bleed, but this is not the time for soaking it up with a gauze pad. The highest priority is getting the new trach in place. Your caregiver should push the new trach into the incision, inflate the balloon, insert a new inner cannula, place the ventilator on, secure the trach with a new collar, and then do a deep suction. Yes, it's a lot to do in a short period of time. The first time you do a trach replacement you should have your pulmonologist or a pulmonary nurse demonstrate and explain it. Like at suctioning, your caregiver will become an expert at trach replacement. It just takes practice.

Your Trach Doesn't Have to Dominate Your Life
Choosing to get a tracheostomy is a decision that takes considerable thought regardless of whether you think the requirements laid out in this article sound like a lot or a little. You'll need 24-hour care, not because you need someone working on you every minute, but because you may get secretions inside the inner cannula or congested lungs without much warning. I often have days when I don't need suctioning, deep or shallow, all day. It's taken me quite a while to build a top-notch team of caregivers and train them properly, and I had to experiment with many techniques to settle on those that work best for me. But I'm now absolutely certain I made the right choice undergoing a tracheostomy. Only you can decide what's right for yourself.

If you have questions, ask me whatever you want and I'll post your question and my response. If you disagree about anything in this article I'll also post your comments for all to see. Let's share our knowledge! Here's the forum editor's email: editor@extrahands.org

Intrathecal Baclofen Therapy Can Provide Improved Quality of Life for ALS Patients with Spasticity-Related Pain

Newswise — An estimated 5,600 people in the United States are diagnosed every year with Amyotrophic Lateral Sclerosis (ALS), also known as Lou Gehrig's disease, and as many as 30,000 Americans have the disease at any given time. Unfortunately, there is no cure for ALS, and it leads to death most typically two to five years after diagnosis. One of the side effects of ALS is spasticity, a condition resulting from excess motor neuron excitation due to lesions in the upper motor neuron pathway that lead to the absence of inhibition of alpha and/or gamma motor neurons. Spasticity can cause uncontrollable and often painful muscle contractions. Although the pain associated with spasticity can usually be controlled with medication and physical therapy, some patients are unresponsive to this treatment.

Researchers at the University of Minnesota Medical School and the Cleveland Clinic explored the efficacy of treating ALS patients with intractable spasm-related pain with an intrathecal baclofen (ITB) pump. Eight patients were implanted with the ITB pump between January 2003 and December 2005 at the Cleveland Clinic.

The results of this study, Intrathecal Baclofen for Spasticity-Related Pain in Amyotrophic Lateral Sclerosis: Efficacy and Factors Associated with Pain Relief, will be presented by Shearwood McClelland III, MD, 3:57 to 4:09 p.m. on Tuesday, April 17, 2007, during the 75th Annual Meeting of the American Association of Neurological Surgeons in Washington, D.C. Co-authors are Francois A. Bethoux MD, Matthew H. Sutliff, PT, Darlene K. Stough RN, Kathleen M. Schwetz, RN, Danuta M. Gogol, RN, Michelle Harrison, PT, Erik P. Pioro, MD, PhD, and Nicholas M. Boulis, MD.

Patients were selected by the operating surgeon and neurologists specializing in the treatment of ALS on the basis of a history of longstanding ALS-associated spasticity, despite optimal medication management. Following successful test injection, patients were referred to the Cleveland Clinic Center for Neurological Restoration for programmable ITB implantation.

•The eight patients ranged in age from 33 to 77, with a mean age of 43.8 at surgery.
•The duration of the patients’ ALS symptoms ranged from 14 to 108 months, with an average of 47.4 months at surgery.
•Both pre- and postoperative pain was quantified using a 0-10 scale, with 0 representing no pain and 10 representing maximum pain.
•The mean modified preoperative pain score was 7.69, ranging from 6 to 10.

Following surgery, patients were transferred to an inpatient rehabilitation unit before eventually returning home with continuing outpatient or in-home rehabilitation. Patients were subsequently followed by the ALS and related disorders team at the Cleveland Clinic for ITB pump adjustments and refills. Mean follow-up was 9.8 months.

The following outcomes were noted:

•Following ITB pump placement, the average pain score was 3.56 (range = 0 to 8), a reduction of 54 percent from preoperative scores.
•No patients experienced neurological morbidity or mortality.
•Six patients (75 percent) experienced pain relief, of which three received complete pain relief (postoperative pain score of 0).

“The degree of pain relief following preoperative ITB test injection correlated with the degree of postoperative pain relief following ITB implantation,” stated Dr. McClelland. The two patients who did not experience pain relief from implantation were the same patients who failed to experience pain relief from preoperative ITB test injection. The three patients who experienced complete pain relief also experienced complete pain relief following preoperative ITB.

“These results support ITB as a treatment modality for pain associated with refractory spastic ALS, and underscore the importance of pain documentation following preoperative ITB test injection. In appropriately selected ALS patients with spasticity, ITB implantation can offer a significant quality of life improvement for an incurable and often painful condition,” concluded Dr. McClelland.

Help for the flakies

From Heather Brannon, MD

Have you noticed red or flaking skin around your nose or in your eyebrows? How about in your scalp - especially over your forehead or ears? If you answer yes, you may have something called seborrheic dermatitis or seborrhea.

Cause of Seborrhea
Seborrhea is a chronic inflammatory skin disorder that affects the areas of the head and trunk that have sebaceous glands. A type of yeast that has an affinity for these glands called Pityrosporum ovale may be the cause, but this has not been proven yet. It is believed that the build-up of yeast in these glands irritates the skin causing redness and flaking.

Who Gets Seborrhea?
Seborrhea is more common in men than women and affects 3 percent of the general population. It occurs more commonly in older people who are bedridden or have neurologic conditions such as Parkinson's disease [and ALS]. Seborrhea also affects almost 85 percent of people with AIDS.

Treatment of Seborrhea
Adults who have seborrhea usually experience a waxing and waning course. In other words it can't be "cured". The good news is with proper maintenance, seborrhea can be controlled. Furthermore, most of the treatments can be found over-the-counter.

Proper hygiene plays an important role in treatment. Frequent washing with soap gets rid of the oils in the affected areas and improves symptoms. Sunlight inhibits the growth of the yeast; therefore exposure of affected areas to sun is helpful, although caution should be exercised to avoid sun damage. The main medical treatments are antifungal shampoos and topical steroids.

Seborrhea Shampoos
There are several good antifungal shampoos on the market that can be purchased without a prescription. The main shampoos are selenium sulfide found in Selsun, Pyrithione zinc found in Head & Shoulders and Sebulon, Coal tar found in Sebutone and Tegrin, and finally ketoconazole found in Nizoral.

All of these shampoos have a medicated smell. The way to use them is to shampoo and leave on for at least 10 minutes then rinse off. The shampoos can be used on the face and other parts of the body as a lotion with the same instructions as long as precaution is used around the eyes. Do this daily until the redness and flaking is controlled then use 2-3 times a week as needed to keep symptoms from returning.

Topical Steroids For Seborrhea
Topical steroids reduce the inflammatory response and help control itching. Hydrocortisone cream 1% can be bought over-the-counter and is safe to use on the face. Apply twice a day to the affected area until the redness resolves. Save the hydrocortisone for flare-ups and use the antifungal shampoo for maintenance because long-term steroid use can cause side effects like acne and thinning of the skin.

When to See a Doctor For Seborrhea
You should see your doctor if you're not sure you have seborrhea. Other conditions that can be similar are psoriasis, rosacea, atopic dermatitis, or superficial fungal infections.

You should also consult your doctor if you have tried this over-the-counter treatment and you still have symptoms, especially if you have very thick flakes. Your doctor may recommend a stronger steroid or a combination medicine to help dissolve the dead skin. Finally, it may not be feasible for black persons to shampoo every day. Ask your doctor about a special steroid preparation in oil that can be used on the scalp like a pomade.

Managing Neck Muscle Weakness

© 2000 Pamela A. Cazzolli, RN, Canton, Ohio USA. All Rights Reserved.

by Pamela A. Cazzolli, R.N.

Some people with ALS develop muscle weakness of the neck. This may cause limited mobility of the head. Turning the head from side to side or holding the head upright may be difficult, as the neck supports the head that weighs about 20 pounds. Limited head movement can contribute to a stiff neck, a poor head posture, and severe pain if these problems are not managed. In fact, neck muscle weakness can lead to a vicious cycle of limited head mobility and a stiff neck (resulting in pain and impairing head mobility ever further), causing more pain with emotional distress. Emotional stress can also compound muscle tightness and cause pain.

People with neck muscle weakness are vulnerable to injury. If the head suddenly flops backward, forward, or sideways, this can cause muscles and ligaments in the neck to tear. It can also cause serious injury to the cervical spinal cord or vertebrae in the neck. Such injuries may be a result of "overstretching" due to a sudden excessive force that propels the neck forward, backward, or to one side or another. Hyperextension (backward) or hyperflexion (forward bending) injuries may be referred to as a "whiplash." This can occur, for example, when a person is transferring in or out of a bed or a chair and the neck is jarred. For some people, even a slight jerk of the head can cause a torn muscle in a weak neck. Injuries to the neck can be very painful, especially when turning the head, raising and lowering into bed and rolling over when in a lying position. Besides causing pain and injury to the neck, poor head posture can aggravate breathing, swallowing and communication.

Through effective management of neck muscle weakness, com-plications of pain and injury can be prevented or treated. Finding the best methods for supporting the head in an upright position to protect against injury is essential.

Strategies for improving the head posture and promoting head / neck mobility, comfort and safety include:

1. Achieve and maintain a good body alignment when sitting to promote an upright head position.

Use of a cushion, pillow, or rolled towel behind the lumbar region of the low back not only helps to align a slumped posture, but will help keep the head and shoulders from slouching forward. If sitting in a high back chair, placing a rolled towel behind the neck also improves the head posture and helps prevent or alleviate discomfort in the neck.

Placing a pillow under each arm or on top of each armrest of the chair helps promote an upright head position, and provides maximum comfort when sitting for a long time. Proper alignment of the head, neck, and body allows the lungs to expand for better breathing.

2. Wearing a soft collar or a neck brace, called a cervical collar, is an effective method of holding up the head if this is difficult.

A collar supports the head when the person is walking or being transferred from one sitting position to another. To protect against injury from sudden motion, people with advanced neck weakness should wear a collar when they are moving or being trans-ferred from one seat to another, when walking, or riding in the car. Use of a collar to hold up the head when walking permits a better sight line and may reduce the risk of falling.

3. Identify and use the appropriate cervical collar(s) that will best meet your needs.

Most people with neck weak-ness are unable to tolerate wearing a collar all the time, especially one that can lead to skin breakdown and dis-comfort. A variety of com-fortable collars are available. One of the most common is a soft foam collar. Although non-rigid collars provide comfort, they offer minimal support if neck weakness is advanced. One popular lightweight collar is the Headmaster Collar™ (Symmetric Designs Ltd., Salt Spring Island, BC). It is a low-profile cervical collar with a padded chin rest. The padded tubular design feels cool and comfortable, even in warm weather, and does not con-strict the neck. Another collar to consider is the Aspen Cervical Collar™ (Long Beach, CA). It offers good neck support with a breathable type of foam and is found to be com-fortable by a number of collar wearers. Cervical collars can be obtained at most medical suppliers, and the cost is usually covered by health insurance if ordered by the physician.

4. To promote comfortable use of head support, alternating the use among several collars may be a solution to reducing pressure points on the skin of long-term collar wearers.

Perhaps the Headmaster Collar™ might be used as a supplementary collar to relieve pressure points. In addition, a thin-cushioned skin dressing, such as Duoderm® (ConvaTec, Princeton, NJ), can be applied over pressure areas to protect against skin breakdown. In some countries, Duoderm is referred as Granuflex® or Varihesive®.

5. Laying back in a reclining chair is another method to support the head and to help keep the head from falling forward.

This may include a reclining wheelchair with a high back or one in which a headrest can be attached. Power lift chairs also recline, and with a push of a button they can recline back exactly to the desired angle. People with excessive oral secretions might have difficulty reclining backward. To help prevent choking on oral secretions, the head can be positioned to the side and propped with a pillow.

6. Using a head support system can position the head in an upright position by a band across the forehead that attaches to an adjustable headrest that mounts to a chair.

Some head support systems include the use of an elastic band, such as the Dynamic Forehead Strap System (Whitmyer Biomechanix, Inc., Tallahassee, FL) that moves with the user for a greater degree of move-ment and comfort than is provided by conventional bands. Head support systems usually can be obtained from a medical supplier who specializes in wheelchair accessories. Peo-ple who need maximum support in holding up the head and who cannot tolerate long term use of a cervical collar might benefit from intermittent use of a head support system.

7. When in bed, avoid sleeping on a pillow that is too high.

This will not only strain the neck, but may cause wake-fulness at night. A rolled towel placed underneath the back of the neck with the head resting on a low pillow can provide support and comfort of the neck and head when sleeping. In addition, people who have trouble keeping their head upright might try lying in bed a few times during the day to relieve the neck muscles.

8. Stretching exercises of the neck muscles can help promote mobility of the head, alleviate stiffness of the neck, and help prevent and treat neck pain.

Two exercises can be done safely:

1. Slowly turn your head to the right as far as possible, hold, and return to the center. Do the same to the left.

2. Tilt your head straight over towards the right ear, hold, and return it to the center. Do the same on the left side. To avoid injury, evaluation and training of other exercises by a physical therapist or a visiting nurse may be warranted.

9. People who have unresolved neck pain or difficulty holding up their head should consult with their physician who may prescribe physical therapy for treating their problems.

A physical therapist is specially trained to evaluate the neck, help relieve neck pain, make recommendations for sup-porting the neck and head, and provide education on reducing the risk of neck injuries. Some methods of treatment may include the use of moist heat, massage and exercises that stretch the neck to alleviate stiffness and to enhance mobility and comfort. Life should not have to be a "pain in the neck."

Preventing and Treating Complications of Immobility in People with ALS

© 1999 Pamela A. Cazzolli, R.N., Canton, Ohio USA. All Rights Reserved.

Pamela A. Cazzolli, R.N.

Although no two people with amyotrophic lateral sclerosis (ALS) are affected exactly alike, progressive muscle weakness is a hallmark of the disease, resulting in problems with immobility. Weakness usually begins in a group of muscles and gradually spreads to other muscles, causing gradual loss of muscle movement. While some people continue to maintain use of their arms or legs, others may slowly lose the ability, and therefore, activities become increasingly difficult, or perhaps not possible as muscle weakness progresses. People with limited mobility are a risk for com-plications, as prolonged immobility or inactivity may cause changes in the muscles and joints and affect every system in the body, as well as impact one's psychological well being.

Complications of immobility include:

Disuse atrophy is muscle wasting caused by inactivity or disuse of the "good" muscles, those that are unaffected by motor neuron degeneration. Although the disease causes muscle weakness that may result in trouble walking or performing daily activities, people who do not use their limbs, if they have the ability to move, will lose muscle mass more rapidly. Thus, immobility will compound weakness of the working muscles. Although they say, "if you don't use it, you'll lose it," overexertion should be avoided. People with ALS should also know that if any muscles are unable to contract because of motor neuron damage, then exercise will not restore the strength of those muscles.

Joint stiffness and pain can occur if weak limbs are not stretched and if joints are not given adequate full range of motion. The stiffness is due to tightness of the muscles and tissues surrounding the joints. Limitation of motion of the shoulder joint may also cause inflammatory thickening of the tissues enclosing the joint, resulting in adhesive capsulitis, often referred to as a "frozen shoulder." Unresolved joint stiffness and pain can hamper activities and lead to premature disability if symptoms are not reversed. People who do have use of their arms or legs are a risk for contractures of the joints.

Contractures are shortenings of muscle or connective tissue around the joints that prevent the normal range of movement of joints. The tightening of these muscles and tissues are due to immobility and cause deformities or joints to become bent in a fixed position that is resistant to stretching to a straight position. Contractures can occur in the finger, hand, wrist, elbow, shoulder, ankle, knee, and hip joints, including flexion contractures of the neck. Weak limbs can lead to an unending cycle of decreased range of motion, increased muscle and tissue tightness of the joints, development of contractures, further impaired mobility, resulting in more joint tightness and contractures.

Pressure sores or ulcers can result from pressure to an area of the body from a bed or chair. Any surface that presses against the skin and underlying tissues decreases circulation to the area, as the pressure collapses capillaries, interrupting the tissue's supply of oxygen and nutrients. When a person remains in one position for too long, the pressure obstructs the blood flow, causing tissue to break down. The first sign of this is redness. Unrelieved pressure, however, can lead to ulcer development, beginning with a red mark that won't blanch and then ends with deep tissue destruction. Thus, a red mark that won't go away is the first sign a pressure sore is developing. Many pressure sores go unnoticed for long periods because they begin at the bone or muscle and progress upward. This means underlying tissue can be severely damaged before the skin turns red. Although pressure sores can develop over any bony prominence pressing against the mattress or chair, the areas that are the most prone to tissue breakdown are the bony prominence of the vertebrae in the lower spine, tailbone and hipbones.

Poor circulation can result from lying or sitting in one position for too long. Improper positioning can also hamper blood flow to any body part. Prolonged sitting on an unpadded surface can obstruct the large blood vessels behind the knees, as well as damage the nerves, causing loss of sensation. Impaired blood flow can also lead to blood clot formation. Lack of fluid intake may increase the risk of clotting of the blood. Although serious, this complication is usually preventable. Inactivity can also cause fluid retention and swelling of extremities.

Urinary tract infections are another risk for people who may spend long periods of time on their back. This can promote urinary stasis or stagnation in the flow of urine from the kidneys to the bladder, and thus, lead to infection. Prolonged immobility also causes an increase of minerals and salts to circulate in the blood that can promote the formation of kidney stones.

Constipation is a common problem that may result from decreased physical activity. Other factors may aggravate bowel evacuation. These include loss of privacy and embarrassment if toilet assistance is needed; uncomfortable positioning while using the commode; excessive delay in elimination because of the inconvenience in going to the bathroom; and the unavailability of caregiver assistance if help is needed to use the commode. Bowel irregularity may produce abdominal discomfort, as well as cause loss of appetite.

Aggravation of respiratory problems may also result from immobility. Being confined to a chair or bed for long periods in one position may suppress lung expansion and the ability to take a good breath that is needed for an effective cough force to clear the airways. Consequently, secretions in the lungs may accumulate and thicken, causing mucus plugs that may aggravate breathing. Secretions in the lungs also harbor bacteria that may multiply and possibly result in pneumonia.

Depression is a normal response to progressive loss of muscle function and impaired mobility. People who are homebound because of trouble leaving their homes, or who are embarrassed about their disability, may have infrequent contact with people. As a result, they can become victims of social isolation that may compound feelings of depression. Emotional responses to immobility vary widely and may include such feelings as helplessness, despair, irritability, anger, outbursts of rage, constant sadness, frequent crying, listlessness, and social withdrawal.

Key Interventions For Preventing And Treating Complications

1. Continue with daily activities that you are able to perform safely and as tolerated, while avoiding overexertion.

Exercise helps maintain optimal function of the muscles that helps prevent disuse atrophy. The general rule is to "do as much as you can for as long as you can, and rest when you are tired." Overexertion may aggravate breathing. Therefore, shortness of breath is a sign to stop activities and rest. Also, avoid lifting weights since that is too strenuous. Weight lifting will not strengthen muscles weakened by the disease.

2. Do stretching and range-of-motion exercises to each of the joints everyday, and several times a day.

(a) to maintain joint mobility (b) to help prevent joint stiffness and pain (c) to stretch tightened joints and help prevent or reduce joint contractures or deformities. Active exercises are done without assistance, but passive exercises are done with assistance, as this is usually required to achieve a full range-of-motion of each joint. Exercises should first be demon-strated by a physical therapist or a trained registered nurse to assure the proper technique and to avoid injury.

3. Maintain proper body alignment, and change positions at least every two hours.

Repositioning helps prevent continuous pressure on any body part. People who are immobile should be turned or moved regularly and properly supported by pillows or foam to provide maximum comfort and to prevent pressure sores and deformities.

A mattress overlay, such as an alternating pressure pad, protects against pressure sores, but not a two-inch foam overlay that provides only comfort. Variable-pressure mattresses composed of air pockets and spongy material are also available for optimal protection against pressure points, particularly the bony prominences. When sitting, use air, gel or foam seat cushions. Proper limb positioning helps prevent joint stiffness and contractures.

To help prevent ankle contractures or deformities of the feet in a footdrop position, place a footboard at the end of the bed to support the feet at a right angle. Placing pillows at the bottom of the feet or wearing padded footdrop protection boots can support the feet when in bed.

To help prevent flexion contractures of the neck, try to promote an upright head posture to keep the head and shoulders from slouching forward. Good body alignment and regular repositioning also promotes circulation; enhances lung expansion for optimal breathing and coughing; promotes gastrointestinal function; and reduces the risk of urinary tract infections and blood clot formation. Since the calf is a risk site for a clot, call your physician if you experience pain in the calf and notice localized warmth, redness and swelling.

4. Maintain an adequate fluid intake.

To help prevent thick secretions in the airways; to help prevent or relieve constipation; and to protect against urinary tract infections and kidney stones. A sufficient fluid intake also prevents dehydration that can also lead to increased viscosity of the blood that can lead to blood clot formation.

5. Focus on abilities and not disabilities.

Find methods to enhance mobility through the use of assistive devices and making the home accessible. This will help minimize feelings of helplessness, depression and giving up.

6. Maintain regular contact with people and do not allow immobility or embarrassment to be a barrier to interacting with others.

Participate in activities with family, friends or caregivers. This will minimize feelings of loneliness, boredom and the effects of social isolation. If possible, attend sporting events, church or group activities, and take trips or go outdoors. People who have trouble leaving the home can invite family and friends in to visit. By using a computer, anyone can also keep in touch with family and friends through the Internet. In fact, through advanced technology and the development of switches to activate the computer, the whole world is only one "click" away. People with ALS who have social interaction and an effective means of communication can achieve life satisfaction despite immobility.

References:

1. Bach JR: Guide to the Evaluation and Management of Neuromuscular Diseases. Philadelphia, Hanley & Belfus, 1999.

2. Metzler DJ and Harr J: Positioning your patient properly. AJN Vol. 96, No. 3: 33-37, 1996.

3. Maklebust J: Pressure ulcer update. RN. Vol. 54, No. 12: 56-63, 1991.

Supportive Treatments

Physical therapy and occupational therapy

Assistive devices

Speech and communication

Nutritional support and saliva control

Sleep disturbances

Respiratory support

Psychological support

Physical therapy and occupational therapy

Physical therapy and occupational therapy have four possible roles in the treatment of patients with ALS:

1. Assessment of what the patients can do and their need for specific devices, such as splints (orthoses), braces, walkers, or wheelchairs.

2. Assessment for safety in standing, walking, or transfers, and providing instruction and training for patients and caregivers to perform those activities safely.

3. As some muscles weaken and others become stiff, it is possible to develop a program of exercises to maintain the range of motion of the patients' limbs. This will enable them to use their remaining capabilities to the best extent by preventing permanent shortening of tendons, which would leave the joints fixed in a bent position, or limited in their range of motion.

4. Physical therapy exercises may have a positive impact on patients' morale by emphasizing and helping maintain those functions which can be accommodated safely by the patients' muscle strength.

Physical therapy is not expected to make the patients stronger. Instead, the patients and families may learn how to make the most of what the patients are able to do even if they are losing strength. Performance of physical therapy exercises under the guidance of an enthusiastic professional can be a boost to the patients' morale. However, many physicians and insurance companies will not consider that a sufficient reason to prescribe it on a regular basis, citing the fact that many of the exercises can be performed by family. It should be recognized that in some patients strenuous physical therapy may precipitate cramping, which may be painful; therefore, patients need to judge for themselves if they feel that the benefits of the exercises are worth the time they spend doing them and the possible discomfort which they may cause. It is best if patients who are experiencing difficulties discuss them directly with their physical therapist and the physician prescribing the therapy. There may be some modifications to the program that will give a better balance.

Assistive devices

Assistive devices may enable the patients to extend the time they are able to function independently. For example, ankle-foot orthoses for foot drop will prevent patients from tripping over their own feet and extend the time that they can walk independently. Similarly, if a wheelchair can help someone get around without falling, its use is preferable to falling and breaking a hip. Understandably, the need to use assistive devices is frequently a source of discouragement as it reflects acknowledgment of the loss of independence. However, assistive devices can help conserve patients' energy so that they can do the things that are important to them and may protect them from needless injury.

Speech and communication

If ALS involves the organs of speech, trying to communicate with the outside world becomes progressively harder and a source of great frustration to patients and friends alike. Evaluation by a speech pathologist or a speech therapist may be helpful. There may be an intermediate stage where techniques may be taught to maximize the clarity with which the patient speaks and a later stage where alternate communication devices need to be considered. A note pad or typewriter may serve. Computer-based voice synthesizers, such as those used by Steven Hawking, are available and expensive. A communication board with letters of the alphabet and with commonly used words can be operated by the patients or by the people to whom they are talking. A feature common to most of these devices is that they do slow down communication. However, they extend the time during which some communication is possible.

Nutritional support and saliva control

Patients who maintain adequate hydration and nutrition may feel better and live longer. Usually, chewing and swallowing are done automatically without much thought. If muscles of chewing and swallowing become affected by ALS, eating techniques and diet may change gradually. Usually, the first change the patients notice is that they need to concentrate and slow down when eating and swallowing in order not to choke. Subsequently, there may be difficulty with particular types of food. As chewing becomes harder, softer foods may be easier to eat. Semi-thick foods may be easier to handle than solids or liquids, but there is no pattern of difficulty that is the same for all patients. Frequently, there is difficulty with handling naturally produced saliva due to over-production or to decreased spontaneous swallowing. These difficulties should be discussed with your physician. There are several treatment options which, while not perfect, may provide a measure of relief. They include agents that decrease production of saliva (which work for some patients) and agents that loosen up saliva or respiratory tract secretions (which work for others).

When it is no longer possible to maintain adequate caloric intake through a regular diet, commercially available food supplements often can provide adequate caloric intake as long as the patients are able to swallow them safely.

The chief risk of being unable to swallow properly is aspiration of food, saliva, or upper airway secretions into the lungs, causing pneumonia. A less obvious risk is that swallowing difficulties interfere with patients' hydration and nutrition. Consequently, if patients are choking on food or fluids, take more than one hour for each meal, are losing weight rapidly or are becoming dehydrated, it is appropriate to consider the use of a feeding gastrostomy. This is a feeding tube that enters the stomach directly through the skin of the abdomen. Its use should be considered in the context of the patients' overall condition.

It is better to consider using a gastrostomy tube before significant weight loss has occurred and before, rather than after, patients have gone through several bouts of aspiration pneumonia. However, the placement and adequate maintenance of a feeding gastrostomy has risks. Risks associated with gastrostomies include the risk and discomfort of placing the gastrostomy tube, the risk of a dislodged tube, and the continued risk of breathing food and saliva into the lungs. The risks of placement of a feeding tube are lower if this is done before breathing difficulties set in, or the patient's Forced Vital Capacity (a measure of lung function) drops below 50% of its predicted value. Patients who elect this option should time it accordingly, in consultation with their physicians. The informed decision to have a feeding tube placed should be made after discussion by the patients, their families, and their physicians, taking into consideration the patients' overall condition. It is ultimately the patients' decision.

Sleep disturbances

The two most frequent causes for difficulties in sleeping for patients with ALS are cramps and breathing difficulties. Patients are almost invariably aware of cramps because they are painful and wake them up or prevent them from falling asleep. There are treatments for cramps.

If the patients' breathing ability is declining, it is likely that the first clinically significant effect will occur when they are asleep. Sometimes, it may be possible to identify breathing difficulties during sleep just by observing the patient. Often, however, breathing difficulties during sleep are not so obvious. The first indication that patients are not breathing well while they sleep may be waking up frequently out of sleep, headaches on awakening, or excessive tiredness or sleepiness during the day. If the cause can be shown to be breathing difficulties during sleep, then treatment of those breathing difficulties will result in more efficient sleep and better daytime alertness and function. It is sometimes helpful to document these breathing difficulties either by using a continuous measure of the amount of oxygen in the blood while the patients sleep, checking if there are periods in which the amount drops to low levels, or by obtaining a full-scale sleep study. The specific forms of respiratory support are discussed in the next section.

Respiratory support

There are early symptoms of declining breathing ability. Patients may experience shortness of breath when lying down or when performing simple tasks, such as dressing or eating, or the consequences of breathing difficulties during sleep, discussed above. A Forced Vital Capacity of 50% of the predicted value or less, or a rapid decline in FVC (for example, a 15% of predicted value drop over a period of three months) is often associated with these symptoms, or suggests that they will appear soon, and that respiratory support should be considered. There are two ways to help patients get enough oxygen into their bloodstream: supplemental oxygen and ventilatory support.

1. Supplemental oxygen may help patients who have mild breathing difficulties or whose breathing difficulties are caused by respiratory infection (a cold, bronchitis, or pneumonia). Supplemental oxygen may be given by nasal prongs or by mask. Sometimes, supplemental oxygen may help patients breathe better when they are asleep.

2. Ventilatory support means help with the mechanical task of breathing, namely, helping weak muscles perform the task of breathing. Ventilatory support may be provided using noninvasive or invasive approaches, and may be needed on a temporary or intermittent basis, or permanently.

Noninvasive ventilatory support is accomplished by having patients breathe through a mask placed over their nose or through short plastic tubes placed into their nostrils (nasal pillows). A fan pushes air through the mask or the nasal pillows, and the air pressure is regulated by special valves, giving this type of treatment the name "Continuous Positive Airway Pressure (CPAP) ventilation." The treatment can be modified to provide a higher pressure during inhalation (when air goes into the lungs) and a lower pressure during exhalation (to help air come out of the lungs). This modification is called BiPAP. A simple explanation of how BiPAP helps patients with early breathing difficulties is that it supports the mechanical task of breathing and gives the weakened breathing muscles, which are overworked keeping up with their usual job, an opportunity to rest, and then return to their job with renewed vigor. Therefore, use of BiPAP may decrease the symptoms of early breathing difficulties not only during the hours that patients are using it, but also during the hours in between its use. For example, making the patients' breathing during sleep efficient results in alert patients during the day. Several studies indicate that use of BiPAP increases patient survival by several months. However, BiPAP cannot work if the breathing pathways are obstructed by secretions. Patients in this situation face the difficult choice between use of invasive ventilatory support and resort to comfort measures. Patients who have an acute bout of pneumonia early in the course of their disease may consider temporary invasive ventilatory support (intubation and connection to a breathing machine) in the hope that, after the pneumonia is treated, they will be able to come off ventilatory support. However, most patients later in the course of their disease who request temporary ventilatory support to get through a bout of pneumonia find that they cannot be disconnected from the ventilator and require permanent ventilatory support.

Therefore, patients with ALS need to consider carefully whether they will want to be on permanent ventilatory support when their disease progresses and their breathing muscles are no longer strong enough to breathe for them. This is a decision which they need to discuss with their families and physicians. The usual outcome of these discussions is that the patients' wishes become clarified. A formal way to document these wishes is by writing Advanced Directives for Health Care or a Living Will. Doing so helps reassure patients and families that the patients' wishes will be respected if they are unable to speak for themselves. While most patients do not opt for permanent ventilatory support, a small minority does. Permanent ventilatory support requires the establishment of an opening directly into the windpipe (a tracheostomy). A breathing machine can then be connected to this opening. In my opinion, permanent ventilatory support via tracheostomy makes most sense in those patients in whom the breathing muscles are affected very early in their disease, while they still have control over their limbs and facial muscles. It may also make sense in patients who feel that overcoming the inability to breathe by themselves will enable them to continue in life in a way which is personally and socially meaningful. The situation of patients in whom the inability to breathe occurs late in the course, when they are immobile and depend on others for their care, is different. Home ventilation is elected only by a small minority of such patients. However, it is an expensive option which is taxing for patients and caregivers alike. While most patients who have ended up on a home ventilation program have said that they would opt for it again, most of their caregivers have said that they would not opt for this program for themselves.

Psychological support

Treatment of depression

Depression is common among patients with ALS. In most, it is self-limited. In others, it is not. Specific antidepressant medications should shorten its duration. A common reason for declining such treatment is the false belief that since there is a good reason for the depression (the diagnosis itself or progression of disease), it does not need to be treated. However, antidepressants usually shorten the duration and severity of depression even when it has developed for excellent reasons. Medications may be supplemented by counseling. At the very least, such counseling may facilitate communication between the patients and close loved ones and help them verbalize their reactions to the diagnosis and its implications.

Support groups

Support groups help patients exchange information, may reduce the sense of isolation that patients with an uncommon disease may experience, and provide mutual support through sharing the trials and tribulations of the disease and how they are overcome. Recognizing that caregivers have needs different from those of the patients, separate support groups have been established for caregivers.

Acknowledgments and waiver of copyright

This manual was prepared by Carmel Armon, MD, department of neurology, Loma Linda University School of Medicine, Loma Linda, California, with the help of many patients, families, professional colleagues, and friends. Stacy Turley and Monika Johnson typed (and retyped) the manual. Lee Lesneski, RN, drew the figures. The cover was designed by Bates Moses. The Loma Linda University Medical Center department of marketing produced the second and third editions.

Printing and distribution costs of the first and third editions were borne by the Friends of Loma Linda University ALS Patients' Fund and the Neurology Clinical Research Center. Major support to fund the printing of the second edition was received from the Loma Linda University Medical Center Employees' Contributions Fund. Additional support was received from the Center for Neurologic Study, San Diego, California, and the Loma Linda University Neurology Clinical Research Center.

This manual is provided at no cost to patients and their families while supplies last. A donation to the Friends of Loma Linda University ALS Patients’ Fund will always be appreciated.

The material in this manual is not copyrighted and may be reproduced, unaltered, in whole or in part, in any form, provided that the entire text of the Acknowledgments and Waiver of Copyright is attached to it and provided that this condition is adhered to by anyone who receives or disseminates this material.

ALS 1996 and Beyond: New Hopes and Challenges
A manual for patients, families, and friends

Third ("Year 2000") Edition
Author: Carmel Armon, MD

ISBN 0-9655186-0-4

Published by the Department of Neurology, Loma Linda University School of Medicine, Loma Linda, California.

First Edition - May 1996
Second Edition - October 1996
Third Edition - November 1999

New drug lessens outbursts in ALS patients

Los Angeles Times

As far back as 1872, British naturalist Charles Darwin observed that people with brain injuries or illnesses were sometimes stricken with uncontrollable, and often inappropriate, outbursts of anger, laughter or grief. "Certain brain diseases ... have a special tendency to induce weeping," he wrote.

Even in modern times, there have been no specific treatments for the mysterious problem, now called "pseudobulbar effect."

The condition occurs in people who have had strokes or brain injuries or who suffer from neurodegenerative diseases such as multiple sclerosis, Alzheimer's disease, Lou Gehrig's disease and Parkinson's disease. The causes remain unknown, but more than 130 years after the condition's discovery, sufferers may have a medication to treat it.

A new drug, Neurodex, has been shown to reduce emotional outbursts in people with Lou Gehrig's disease (amyotrophic lateral sclerosis).

It's now being tested on MS patients at 53 centers nationwide and on people with brain injuries and neurodegenerative disorders, says the drug's manufacturer, Avanir Pharmaceuticals of San Diego.

"It usually takes a long time for a drug to get from the lab to patients," said Dr. Richard Alan Smith, director of the Scripps Center for Neurologic Study in La Jolla, Calif. "But this has moved along at a pretty rapid pace."

The drug's effects on emotional disturbances was discovered by accident about 10 years ago.

Smith was studying Lou Gehrig's disease, which attacks nerve cells in the brain and spinal cord, and had begun working with dextromethorphan, the active ingredient in cough syrups. He thought dextromethorphan might help stop the attacks on nerve cells by blocking a chemical in the brain called glutamate.

After altering the drug so that it would remain in the body longer, Smith gave it to patients. The drug didn't stop the disease process, but patients quickly reported feeling more emotional stability.

The uncontrollable outbursts of laughing or crying that are hallmarks of the disorder, as well as displays of anger can make family relationships difficult, sidetracking rehabilitation efforts.

Life on the Vent: The Other Side of the Mountain

Copyright Muscular Dystrophy Association
QUEST Volume 8, Number 3, June 2001

by Erin Brady Worsham

Macy's Thanksgiving Day Parade, 1997
The Macy's Thanksgiving Day Parade has long heralded the start of the Christmas shopping season. In 1997, it marked the beginning of a new life for me. It had been just over three years since my husband, Curry, and I received my diagnosis of amyotrophic lateral sclerosis (ALS) and I was given three to five years to live.

I lay in bed in my Nashville home that morning, as I had done the two previous days, too exhausted to get up, and watched the televised parade. Helium-bloated Garfield, Bullwinkle and Blue from "Blue's Clues" floated ponderously down the streets of New York.

An unnatural stillness in my body suddenly made me conscious that my heart was pounding madly. I had stopped breathing, just like that. After three years of watching and waiting for this moment, death was slipping past my vigilant guard.

My body rejected death's invitation to rest and fought for every breath.

Curry was in the room when I went into respiratory arrest. I couldn't find the breath to tell him I couldn't breathe. He initially left the room and called the nurse who handled our case. But then, seeing my blue lips, he called 911 and the paramedics were there in five minutes.

Our 2-year-old son, Daniel, was bundled off to the neighbor's house. I wondered when would I see him again.

After their record-setting arrival, the paramedics took their time setting up. They certainly weren't panicking — why should I? They started me on oxygen and transported Curry and me to Vanderbilt Hospital. As the oxygen flowed, my fear melted and I gave thanks.

The Plan Goes Forward
What happened that Thanksgiving morning may have surprised us, but how we chose to proceed had been in the planning for a long time. We'd already discussed with my pulmonary doctor, James Snell, the time frame for my getting a tracheostomy and ventilator. He must have known it wouldn't be long when he told Curry to call 911 if anything happened over the holiday.

Three years earlier, I wasn't sure I wanted to continue living with ALS. Following my diagnosis, I staunchly declared that I would not live on a machine.

That was before we discovered I had gotten pregnant the day after my diagnosis. For six years we'd tried to have a family. How could I think of dying while carrying such a precious gift inside me? So I decided to use whatever means possible to stick around and watch this miracle child grow up.

That meant getting a feeding tube, a tracheostomy and a ventilator when the time was right for each. Curry supported this decision. We made sure everyone working with us was aware of our choices.

I got my feeding tube in June 1997, when eating and drinking became too much of an effort and I lost 20 pounds. It took some time for my stomach to adjust, but then my body blossomed with the balanced nutrition and unlimited fluids I could have without fear of choking.

For three years I had climbed the mountain of ALS. My muscles weakened from the exertion and eventually gave out. The longer and higher I climbed, the thinner the air became, until I reached the summit on that Thanksgiving Day and couldn't breathe at all. The next day I fell gently to sleep under the anesthesia and awoke on the other side of the mountain.

A New Therapy
Breath in, breath out, breath in, breath out, breath in, breath out. Six times a minute the ventilator filled my lungs. The rhythmical whoosh of the ventilator was now my constant companion. I soon found I didn't mind its steady cadence.

I naively thought that once I was plugged into the ventilator, I could go home and resume my life with my family. But there were arrangements to be made for my homecoming, Curry needed training with the ventilator, and I needed rehab.

Rehabilitation isn't usually associated with ALS, the logic being that there's no going back after the muscles have atrophied. No one told the therapists this at the Vanderbilt Stallworth Rehabilitation Hospital. They took me on as their first ALS patient.

People with ALS and other neuromuscular diseases have healthy lungs. What we lack is the diaphragm power to operate them properly. As the diaphragm (a muscle) weakens, it becomes harder to breathe deeply and cough effectively. Secretions build up in the lungs, which can lead to pneumonia and death. When I went on the ventilator, I was using only 8 percent to 10 percent of my lung capacity.

For two weeks the staff of respiratory therapists, under the direction of Stephen Lampe and Brenda Butka, worked me over mercilessly. I knew I was in trouble shortly after my arrival when two therapists came into the room with an Ambu bag, a balloonlike device that's used to pump air into the lungs for resuscitation or suctioning.

"You're not going to like this," they said. "But when we get finished with you, you will love it!"

Stallworth uses a very simple, but effective, therapy that was developed at Dallas Rehab (now part of Healthnet Hospital). The therapist covers the exhaust valve of the Ambu bag and hyperinflates the lungs. Then with the butt of the palm of one hand, he delivers a powerful push to the diaphragm as the air is released. This causes secretions from deep in the lungs to come flying out of the trachea. Saline helps loosen the secretions.

The first time I received this therapy, the pain was excruciating. The walls of my lungs had grown stiff from disuse and protested mightily at the hyperinflation. But each day they became more pliable and my capacity increased. I remember the morning the last big plug flew out and made my therapists very happy. As they had predicted, I soon began asking for the therapy.

Healing and Grief
Though my lungs healed, my emotions remained in turmoil. Everything turned me into a puddle. Curry, who was staying with me in the hospital, would go and fetch Daniel every day. It comforted me to watch them eat supper together, or to have Daniel lie down and nap with me. I didn't want him to be afraid of his mama's new equipment.

From the moment we're born and take our first breath, breathing seems to be our God-given right. Having to rely on a machine for every breath, I relinquished that right forever. And so I grieved for that loss and began the process of learning to trust my ventilator and accept it as a part of my body.

One hurdle remained before I could go home. I had to get out of bed. It sounds simple enough, but after three weeks of lying down, I was terrified. It took four people to transfer me and then the ventilator to the wheelchair. For the next few days Curry wheeled Daniel and me about the halls of Stallworth.

An outstanding moment in our Stallworth stay was when I rediscovered my voice. Before having the tracheostomy and ventilator, I'd lost the ability to speak for lack of air. My new trachea was equipped with a small, inflatable collar, called a cuff. When inflated, the cuff prevented my making any sound, and it was kept inflated for some days after the surgery.

When the therapists at Stallworth deflated the cuff, my voice erupted from my throat in a loud, liberating shout! We called my family in Louisville, Ky., and blew them away when I said "hi."

During our two weeks at Stallworth, Curry closely watched the therapists as they worked. They began allowing him to do the therapy and the suctioning while they observed. His confidence was growing.

As the ambulance pulled into our driveway, my heart pounded, this time in happy anticipation of our homecoming. The EMTs wheeled me into the house on a stretcher. Standing in the living room, without a bauble on it, was the prettiest Christmas tree I'd ever seen. Curry had slipped out of the hospital and gotten it. We were home for Christmas!

The Search for Florence Nightingale
We thought we had a good plan for my nursing care. I'm covered by the state-subsidized Tenncare. Snell prescribed 12 hours of nursing care a day to give my husband a rest. We had no reason to think this would be denied, but it had not been approved by the day I was released from Stallworth.

This necessitated our getting financial help from the family to hire people on our own. Though we talked to everyone we met in the hospital, trying to enlist nurses on our own was a bust. We couldn't afford to hire a nurse through an agency, so we had to settle for hiring a sitter. At least she could wake Curry up if there was a problem.

Our first sitter arrived at our house dressed in a leopard-printed outfit and confidentially told me this was her very first job as a sitter. My heart sank.

During the night, I awoke in a panic because the air was escaping from my open mouth as I slept. In my fear, I could make little sound. My guardian angel must have roused Curry, for he suddenly woke up. He set things to rights and went into the living room, where he found the sitter curled up and sleeping like a baby on the couch.

Incidentally, we solved the problem of the escaping air. Had I had my cuff inflated, it never would have happened, but I'm not comfortable with the idea of not being able to make any sound. We came up with a low-tech alternative. Now we leave the cuff deflated and I sleep with a washcloth between my teeth and cotton in my nose to prevent air from escaping.

Eventually, our social worker told us our nursing care had been denied. But we never received any direct notification from the state, nor any word about how to make an appeal. For six months we paid for a sitter out of pocket. The social worker encouraged us to get a lawyer and contest the state's decision.

Michelle Johnson with the Tennessee Justice Center thought we had a good case. She wrote a powerful letter to the state and we won, without ever going to court. Our case became part of a class action that resulted in the state's having to change its appeals process.

Being eligible for 12 hours of nursing care a day and actually filling those hours with nurses are two different things. Good nurses are a treasure!

We've been blessed with some excellent nurses over the years, but keeping our schedule filled is an ongoing battle. It's a fight we're more than willing to wage, because their help is indispensable in our lives.

Epilogue: The Big, Blue Van
It's been more than three years since that Thanksgiving morning when I confronted my own mortality and chose to fight for my life. Daniel is an impudent young man of 5, who shamelessly bosses his mother around. Our big, blue van takes us everywhere around Nashville, Middle Tennessee, and as far away as Virginia and Michigan.

It hasn't all gone smoothly. There was the time Curry forgot the canister on the suction machine and had to fashion one out of a Styrofoam cup and duct tape. It was nothing to write the patent office about, but it temporarily did the job until we got home.

And who can forget the day we went to the Russian exhibit at the museum and my ventilator tube caught on the van's wheelchair lift and popped apart? Curry truly amazed me that day.

While "bagging" me with the Ambu bag, he lowered me on the lift and tried to reconstruct my tube. A fierce storm was blowing up around us. Curry took me inside the museum, put together my spare ventilator circuit and got me back on track, with time left to see the exhibit. It's always frightening when you can't breathe, but each crisis teaches us more about the ventilator and how to solve various problems.

Using the Stallworth therapy, I've remained free from pneumonia. Before leaving Stallworth, Lampe gave us some good advice: Keep it simple!

My ventilator and feeding tube keep me going, and my Liberator communication device interfacing with our computer allows me to speak, write and draw. I was an artist before I became sick. After the diagnosis, I lost the heart to create. When I found it again, I'd lost the muscle! With the Liberator, I can create again.

The other side of the mountain is more beautiful than I could ever have imagined. I wouldn't have missed this adventure with Curry and Daniel for the world.

People are mistaken when they say they can't live hooked to a machine. Curry likes to remind me that I'm not hooked to the ventilator, it's hooked to me! Together, we're living a good life.

The Lurking Danger of Pressure Sores

Copyright Muscular Dystrophy Association
Volume 6, Number 1, February 1999

by Phil Ivory

2/99 - Pressure sores and bed sores are terms that basically refer to the same problem, the formation of a wound due to prolonged pressure on a particular point on the body.

The term pressure sores is perhaps more useful than bed sores, since it includes injuries caused by being in bed as well as those that result from sitting in a wheelchair or using an orthopedic device that presses against the skin. Decubitus ulcer is a term doctors use to describe any such sore.

The problem is a common one in nursing homes and among people with disabilities. But just because it's common doesn't mean it isn't also quite serious.

Once formed, pressure sores can take months to heal, can be quite painful, and can complicate and add to existing health problems. In worst cases, they can actually become life-threatening.

However, there are steps that can be taken to stop them from getting worse once they've formed or, better yet, to prevent them from forming in the first place.

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NATURAL DEFENSES
In a person whose nerves and muscles are healthy, the nervous system conveys a signal of discomfort to the brain whenever a part of the body has remained in one place too long and is receiving excess pressure. In response, the person shifts sitting position or turns over in bed, relieving the pressure.

In those with spinal cord injuries and other conditions associated with a loss of mobility and a loss of sensation, the individual may not be able to move to relieve the pressure and may not even be aware that a part of the body is under duress.

In contrast, in certain neuromuscular diseases such as Duchenne muscular dystrophy (DMD) or amyotrophic lateral sclerosis (ALS), sensory function largely remains intact. It may be partly for this reason that in patients with neuromuscular diseases, pressure sores aren't reported as often as might be expected.

In Amyotrophic Lateral Sclerosis (1998, F.A. Davis Company), Hiroshi Mitsumoto, David A. Chad and Erik P. Pioro cite a study which suggests that the biochemical properties of a protein called collagen (which is present in the skin) are different in people with ALS, and this difference may help protect them from pressure sores. Nonetheless, pressure sores can and do occur in ALS, particularly in later stages of the disease.

Certainly, even if sensory perceptions are intact, the loss of mobility associated with progressive neuromuscular disease will always create a danger of pressure sores.

In addition, unwanted curvature of the spine, or scoliosis, is a complication that often accompanies neuromuscular conditions. It can contribute to pressure sores because it causes the individual to lean to one side, placing disproportionate weight on one buttock.

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CAUSATIVE FACTORS
Pressure sores are most likely to form at a point where the bone is close to the skin.

Prolonged pressure from a bed or chair on one side and bone on the other makes it impossible for the affected area of skin to be properly nourished by tiny blood vessels called capillaries. The area of skin starts to die. The greater the pressure, the more likely damage will occur.

Friction is another potential cause. Movement that causes skin to rub roughly against bedding may damage the capillaries and diminish blood supply at a particular point. Dragging someone across a surface instead of lifting the person can cause this.

Excess moisture on the skin that results if the person suffers from incontinence of the bowels or bladder can also contribute to skin breakdown. Perspiration caused by wearing a brace or other orthopedic device can have a similar effect.

General poor health, undernourishment and obesity are other possible factors leading to pressure sores.

In A Clinician's View of Neuromuscular Diseases (1986, William & Wilkins), Michael H. Brooke writes that pressure sores may form on the heels of boys with DMD whose legs have been temporarily immobilized after tendon-release surgery.

Recent evidence suggests that any patient undergoing surgery for more than three hours faces a risk of developing pressure sores, caused by immobility during the operation and the use of anesthesia.

Damage to the skin may occur during surgery but not develop into full-blown pressure sores until some time after the procedure. As a result, sometimes the surgery's causative role may be missed.

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FROM BAD TO WORSE
In light-colored skin, the first warning sign of an impending pressure sore may be a soft, reddened area with no broken skin. If redness remains 30 minutes after the pressure is relieved, enough damage may have already been done that formation of a sore is unavoidable.

When seeking this sign in people with darker pigmented skin, it may be necessary to look for increased darkness rather than redness, or to check for a rise in skin temperature instead of a change in color.

It's imperative after noting this initial symptom to call in a nurse, doctor or other health professional, if one isn't involved already.

If a pressure sore continues unabated, the area will become blistered and then ulcerous, with a shallow opening in the skin, possibly accompanied by fluids draining from the site.

After that, the wound can become deeper, with destruction not only of the outer skin layer but also of the fat and muscle beneath, with increasing likelihood of pain and drainage.

In severe cases, if untreated, the wound can extend all the way down to the bone. At this point, there'll most likely be an excess of pus and dead tissue appearing in the site, with accompanying pain.

Fever and increased warmth around the wound are possible signs of infection, as is the presence of a green or yellow discharge. Once the site is infected, the surrounding tissues and the bone are also in danger of infection. Sepsis -- the general spread of bacteria throughout the body -- is a further possibility, paving the way for potentially fatal complications.

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TREATMENT
The initial step in treating a pressure sore is to make sure that no additional pressure is placed on the site until it has had a chance to fully heal. The placement of special foam pads and pillows can help a person lie in bed without putting pressure on a wound.

A wound isn't considered to be healed unless the skin is once again unbroken and normal color is restored, aside from permanent changes in skin color due to scarring. Once the spot is healed, pressure must be placed on it for short trial intervals only, with careful checks made for any ill effects.

For an open wound, a doctor or nurse may need to show the affected individual or caregiver how to tend the injury. This care may involve cleaning or irrigating the wound, removing dead tissue and applying a dressing.

Saline solution may be adequate for cleaning some wounds. It can be bought or made at home. Deeper wounds may require more stringent cleaning and more elaborate care, with careful monitoring for any signs of infection.

Antibiotics may be in order if infection is detected. In extreme cases, surgery or other procedures may be necessary to remove dead tissue to enable the wound to heal.

A new kind of treatment not yet widely used is called vacuum-assisted closure therapy. Through application of an air-tight foam dressing and a vacuum pump, negative pressure is created around a wound to stimulate blood flow and encourage healing.

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PREVENTION
Anyone who uses a wheelchair or must remain in bed for extended periods must be sure to change position regularly. Pressure sores can form in only a few hours.

Carol Stumpf, a physical therapist and manager of rehabilitation at University Medical Center in Tucson, Ariz., says a bed-bound patient should move or be moved at least once every two hours, if not more often.

A bed-positioning program might have a person lie on the left side, then on the back, then on the right side, all at two-hour intervals.

Using a specially designed bed, bed cover or mattress can help. These include air-filled, alternating-pressure mattresses; sponge-rubber mattresses with "egg crate" shaped modeling; and silicone gel or water mattresses. They adjust to the body's shape and help to spread pressure over a wider area. They don't, however, eliminate the need to change position every two hours.

One common sense strategy to prevent a person in bed from placing too much stress on one side of the body is to make sure that a TV is placed directly in front of the person, not on one side or the other.

For wheelchair users, pressure-relieving cushions filled with air or gel can increase comfort and guard against sores. But whether they're used or not, the individual will need to shift position as often as every 10 to 15 minutes. If he can't move himself, he should be moved at least once an hour.

Wheelchair users with sufficient upper body strength may be able to use a self-lifting exercise known as "wheelchair pushups," with doctor's approval.

People with neuromuscular diseases may benefit from using standing wheelchairs to occasionally relieve the pelvic area of the pressure caused by sitting. (See "Taking a Stand," Quest Vol. 5, no. 3.)

For users of braces and other orthopedic devices, special protective pads or a layer of cotton clothing worn underneath the appliance may help discourage sore formation.

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CHECKING THE SKIN
Good hygiene is crucially important. For unbroken skin, cleaning gently with mild soap and water is appropriate, followed by gentle drying (patting, not rubbing). Skin shouldn't be allowed to be too moist or too dry. Overrigorous cleaning can chafe or cause excess dryness.

Ask your doctor if he recommends the use of any particular oils or lotions to help maintain skin quality. Shop wisely. No one agent is acknowledged to be universally superior in prevention or treatment of pressure sores.

Those in danger of developing pressure sores will need to create a daily skin-checking regimen. A parent of a child who uses a wheelchair should check the trouble spots in the morning when the child gets up and also whenever the child is taken out of the wheelchair or when an orthopedic appliance is removed. Adequate light should be available.

If the child wishes to be independent, he'll need to learn how to do this routine by himself, using mirrors if necessary.

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OTHER FACTORS
Bedding should be changed frequently and kept clean, dry and smoothed out. Bedding that is wrinkled or bunched can cause friction against skin and contribute to wound formation. Lying on a sheepskin or synthetic sheepskin seems to help protect against friction.

Good nutrition, too, is important in that it will help maintain healthy skin and make it more resistant to breaking down. Vitamins and mineral supplements might help.

Since circulation is a factor, it's possible that gentle massages and whirlpool treatments may be of value as preventative measures against pressure sores, although special gentleness must be used near the trouble spots.

More information on pressure sores is available online at the U.S. National Library of Medicine Web site at www.nlm.nih.gov. Go to the Health Info section, then search Health Services/Technology Assessment Text for "pressure sores" and select "Pressure Ulcer Treatment (Consumer's Guide)."

You can also contact the National Pressure Ulcer Advisory Panel (NPUAP) by writing to NPUAP, 1321 Duke St., Suite 304, Alexandria, VA 22314-3563 or by calling (703) 548-3100. The e-mail address is khsnpuap@aol.com and the Web site is www.npuap.org.

All Fall Down: Staying Upright With a Neuromuscular Disease

Copyright Muscular Dystrophy Association
QUEST Volume 9, Number 6, December 2002

by Christina Medvescek

Some people have nightmares about falling off cliffs. Brad Williams has nightmares about falling — period.

“Whenever I’m walking, falling down is always the major thought on my mind,” says Williams, 39, of Alexandria, Va. “It has to be on my mind a lot for me to be dreaming about it.”

Williams has Miyoshi distal myopathy, a slowly progressive form of muscular dystrophy that primarily affects the extremities. He hosts an MDA Internet chat under the nickname “dysf,” and notes that other chat participants also have reported falling nightmares.

“It’s like a flying-falling dream, except it’s just about falling down.”

For many people with neuromuscular diseases, falling is a common waking nightmare. Although most falls are merely frustrating — resulting in cuts, bruises and embarrassment — some are far more serious, breaking bones, causing head injuries and sometimes putting an end to walking.

What causes falling? More important, what “causes” stability? Are there ways to protect against injury? When is it time to go to a wheelchair? This article addresses those questions by culling the expertise of physicians and physical therapists, and the hard-knocks wisdom of people who fall.

Causes of Falls
Among the causes of falling for those with weak muscles, environmental hazards play a big part, of course: ice, snow, uneven ground, wind, crowds, gravel, toddlers, dogs and cats, even well-meaning friends.

“It’s when somebody pats you on the back to say hi that you go down,” wryly observes Jon Nitz, 47, of Tucson, Ariz., who has spinal muscular atrophy (SMA) type 3.

Whatever’s happening around you, your muscle problems are the underlying cause of falls. Weakness in leg muscles can lead to foot drop and tripping, or the inability to straighten your knee on uneven ground. Arm and neck weakness can cause balance problems.

Myotonia, or lack of muscle relaxation, which occurs at times in amyotrophic lateral sclerosis (ALS), myotonic dystrophy, myotonia congenita and paramyotonia congenita, makes muscles hard to control. Muscle cramps or too-tired muscles can drop a person in mid-stride.

Contractures, lack of flexibility and poor range of motion — common to many neuromuscular disorders — make it tougher to stay upright. Difficulties with proprioception — your awareness of your posture, movement and equilibrium — can make it hard to properly place the foot while taking a step.

Body habits are another big cause of falls, says Wendy King, adjunct professor of neurology at Ohio State University in Columbus and a physical therapist in the MDA clinic there.

“Often, people just make a move too suddenly,” she explains. “They’re doing something they’ve always done, like working in the kitchen, and they turn quickly to grab something out of a drawer, and down they go.”

Concentration is critical, agrees Todd Allen, 38, of Chicago. Allen has Kennedy’s disease (spinal-bulbar muscular atrophy), which is similar to ALS but with a slower progression.

“My subconscious still believes my body works on autopilot. If I’m concentrating, I can walk across a freshly plowed field, but if I’m distracted by talking to a friend, I can easily trip on a crack in the sidewalk,” Allen says.

Moving too quickly after prolonged sitting can cause a crash. For example, in January, Brad Williams stood up after a session at his home computer. His muscles were stiff and unready and his knee buckled, resulting in a severely broken leg.

The mind plays a part as well. In spite of progressive weakness from his SMA, Nitz stood all day in his job as an auto repair facility manager by leaning against the counter.

“Psychologically, you need that wall or counter to hang onto,” he says. “Take it away and psychologically you think you’re going to fall. You kind of cause yourself to lose your balance.”

Assistance and Resistance
Walking is a valuable activity for people with neuromuscular diseases who are still ambulatory. Being on your feet enhances bone growth and strength, helps prevent osteoporosis, improves muscle tone and makes it easier to interact professionally and socially.

But when falling is an issue, walking has the potential to do more harm than good. The trick is to safely maximize the benefits of walking while minimizing the dangers of falling. That’s what assistive devices are for.

Braces, canes, crutches, walkers, scooters and wheelchairs can be a big help — if you use them.

“Pride goeth before a fall,” King quotes. “For many people, it’s worth the risk of a fall not to use a device. The most common problem is not that people refuse any device, but that they use a single-point cane when they need much more than that.”

People may resist using a device because they think it looks funny, feels funny or makes them feel they’re giving up the fight to walk independently.

Safety First
But the bottom line, doctors and therapists say, is safety.

“Don’t give up hope, don’t stop fighting the disease, but be smart about it!” says Anne Clark, research physical therapist at the Eleanor and Lou Gehrig MDA/ALS Center at Columbia Presbyterian Medical Center in New York. “If you’re falling all over the place, do you continue to fight [the disease]? Absolutely! But you need to stay safe, too.”

Persistence doesn’t mean you should continue to use a device that doesn’t seem to help. MDA offers annual PT and OT evaluations, which may include an assessment of walking ability, as part of its clinic program.

“Each individual is different. There is some trial and error involved,” says Carol Marulic, a physical therapist and manager of therapeutic and diagnostic services for University Medical Center in Tucson. For example, if your leg braces or AFOs make it harder to walk, then go back and work with the therapist on another solution, rather than giving up on assistive devices entirely.

In the same vein, resist the urge to buy a decorative cane at a craft fair or a used walker at a yard sale. Not only might you be wasting money because the device won’t be effective for your situation, but you may be putting yourself in danger.

Devices need to be professionally fitted, and some materials are stronger than others. Wooden canes, for example, can have dangerous stress fractures that cause them to fail when you need them most.

If you’re falling frequently — once a week in different situations — or if you’re severely limiting your activities out of fear of falling, it’s time to consider a wheelchair or scooter. Therapists often recommend using a chair or scooter to get to an activity, then walking once you get there. This not only is safer, but it helps conserve energy and ultimately may keep you walking longer.

For more about deciding between a scooter and a wheelchair, see “As the Wheel Turns.”

Children tend to fall more frequently than adults but sustain fewer fractures.

“Children definitely will fall more because they’re less careful, but they’re more agile and able to pop back up. We encourage them to be active,” Marulic says. “When considering a wheelchair, the questions I always ask parents are: How often are they falling, how far can they walk safely and what is the school saying?”

Safety is key — but so is sensitivity to the affected person’s feelings about using a wheelchair or scooter. Marulic recalls a teen-ager with facioscapulohumeral muscular dystrophy who had needed a wheelchair for quite a while but absolutely refused to get one.

Marulic and the girl’s parents both thought she was in denial, “but I wasn’t listening to her as closely as I should have been,” Marulic confesses. “One day she told me in tears, ‘Just let me graduate out of the chair.’ She just wanted to walk down the aisle at graduation. You really have to listen to the patient and what is going on in their lives.”

Taking Precautions
Making simple home modifications and planning ahead are as useful in safe walking as are assistive devices.

Eliminate throw rugs and toys scattered on the floor. Even out variations in floor height or add handholds. Make sure furniture arms are sturdy enough to provide support when standing up. Replace a squishy deep pile carpet with a thinner, firmer one. Installing a new or used stair lift can make it safer, and easier, to go from one floor to another.

“I always ask adults, ‘Is there a rug or steps that you always trip over?’ It’s amazing how often they say yes,” says Petra Kaufmann, assistant professor of neurology, and associate director of the Pediatric MDA Neuromuscular Center at Columbia University in New York.

“They think they’ll take care of it one day but not this week. I always emphasize the importance of taking care of it right away, and recommend that they get a home evaluation.”

Professional home evaluations, offered by occupational or physical therapists, look at ways to make your house more user-friendly, both now and in the future.

Sometimes private insurance will authorize an in-home evaluation. You can do your own informal evaluation of your environment, too. Anticipate a fall, King advises.

“Look at your common pathways or places where you stand, like the kitchen, and do a ‘360.’ Ask yourself, ‘If I fell in this direction what would I hit? How about this direction?’ Then remove sharp corners or whatever.”

Children need more teaching and supervision than adults to learn caution, Kaufmann says. “It’s more of a challenge to keep them safe because they want to do the same things as everybody else,” she notes.

Review situations in which your child may need help — the playground, stairs in school or carrying books — and work together to create alternate strategies the child can live with.

Rest and sleep are crucial to fall prevention, says Patrick Griffin, 52, of Washington, Kan. Griffin’s Kennedy’s disease has given him many opportunities to “inspect the ground up close and personal.”

A former Army medic and mountain climber, Griffin now has to “listen to my body and rest before it becomes absolutely necessary,” even if it’s only a brief sit after walking across a parking lot.

He now allows himself to be helped and uses handicapped parking spaces.

“I’ve accepted that being seen as a ‘wimp’ or ‘lazy’ is preferable to falling and sustaining a concussion, or overtiring myself so that I need three days of intensive rest afterward,” Griffin says.

Falling Down and Standing Up
Falling hurts. As muscles weaken, it becomes more and more difficult to avoid injury.

One way is to fight the instinct to put out your hands to catch yourself, which can lead to arm or wrist fractures. As unnatural as it sounds, when you’re about to fall, “relax,” Williams advises. “You’re going to fall anyway and if you’re not rigid, it makes it less likely you’ll injure yourself.”

Allen agrees. “I’ve never been severely hurt when I simply let myself go down, curling up or rolling to take the impact in the fleshier parts of my body, such as the butt and shoulders.”

Allen finds in-line skating protective gear, such as wrist guards and knee and elbow pads, a practical way to avoid injury when romping with his dogs.

Judo taught Griffin how to fall as a youngster and he finds the
technique useful now. Whenever possible, roll with the fall instead of landing flat, he advises.

Tuck in your chin so your head doesn’t smash into the ground, he adds. When falling forward, extend an arm in a curve and tuck your head into it. Use the arm as a curve upon which to roll.

Often the worst part of a fall is getting back up again.

“It really helps to not get back up too quickly,” Allen says. “It helps to rest a bit and then think through exactly how to get back up.”

Williams has an armchair with a lift in it that raises him from sitting to standing position. More than once after a fall he’s made his way over to the footrest of the lift cha