Chapter 2 — Daily Care of Your Loved One with ALS
No guide can anticipate all the issues that may come up in day-to-day ALS care, but this chapter offers advice and resources for handling some of the more, and less, common problems that may arise.
Topics include:
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Home modifications make it easier on everyone. Plan ahead and make modifications before they’re needed. Early modifications include taking up anything that can cause tripping, such as scatter rugs or pet toys, and installing handrails in the shower/tub. Later modifications include ramps to the outdoors and increased bathroom space. In two-story houses, a downstairs bedroom space may be necessary.
Modifications range from simple to elaborate: moving furniture, changing to wideropening door hinges, installing an electric door opener, remodeling a bathroom, installing overhead tracks for an electric lift system (see Lifts), building a new bedroom/bathroom suite, etc. Modifications usually are tax deductible to some degree.
Resources:
Ask your MDA clinic about getting a home safety evaluation by an occupational or physical therapist.
Everyday Life with ALS — Chapter 1: Equipment for Daily Living; Chapter 2: Saving Energy; Chapter 3: Home Modifications
Back issues of MDA publications, such as Quest and the MDA/ALS Newsmagazine, can be found online or by calling your local MDA office, (800) 572- 1717.
Jeanine Schierbecker — Physical Therapist, MDA/ALS Newsmagazine, December 2005
ECUs Can Help You Take Control at Home, MDA/ALS Newsmagazine, May 2005
Home Rehabilitation Services: OT/PT Evaluation, MDA/ALS Newsmagazine, October 2002
Adapt My World: Homemade Adaptations for People with Disabilities, by J. Rose Plaxen, Seven Locks Press, 2005
Universal Designed “Smart” Homes for the 21st Century, by Charles Schwab, AIA, Charles Schwab Architects, 2004
Accessibility outside the home
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“When I made my first trip out in my power wheelchair with my sister, she got the last accessible parking space at the mall.
As we were laughing about her struggle to release my chair from the van-locking mechanism, an elderly gentleman approached and proceeded to scold and curse us for taking the last handicapped spot, saying he deserved it more since he was much older than I. To which my sister yelled, ‘Oh yeah? Well, we’re more handicapped than you are!’ |
While the Americans with Disabilities Act (ADA) requires that places open to the public be accessible to people with disabilities, this often is not the case. Whenever possible, check accessibility ahead of time and be ready to be flexible. Notify inaccessible businesses about your problems with access. Get a permit for using handicapped parking spots.
Resources:
Can’t Get In? Work It Out — Accessibility, MDA/ALS Newsmagazine, April 2005
Americans with Disabilities Act Hotline, (800) 514-0301
Disability Business Technical Assistance Center (DBTAC), (800) 949-4232
Institute for Human Centered Design, (617) 695-1225
Nontraditional treatments for ALS — such as nutrition, vitamins, supplements, acupuncture, electrical stimulation, heavy metal chelation and stem cells — currently lack solid scientific proof that they work. Different people react differently, so an alternative therapy may be worth a try, but proceed with caution.
The important thing is to integrate alternative therapies into conventional medical care. Consult your primary care or MDA ALS doctor before starting anything new. If a treatment won’t cause any harm, many physicians are willing to work with you in giving it a try. Alternative therapies usually aren’t covered by insurance and can be pricey.
Anything that’s touted as a miracle cure or that requires a lot of money upfront should be treated very skeptically — or ignored.
Resources:
Memorial Sloan-Kettering Integrative Medicine Service
National Institutes of Health, National Center for Complementary and Alternative Medicine
QuackWatch
University of Texas M.D. Anderson Cancer Center, Complementary/Integrative Medicine/Education Resources
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“My husband would get a daily sponge bath with nice warm water and body soaps. One of us would hold him on his side for the nurse or me to wash his backside. He loved his baths. It also is a form of mild range-of-motion since we were moving the arms and legs. We used big towels rolled lengthwise up next to him while he was on his side to catch any excess moisture during his bath. We patted him dry, then we would rub him down with a good body lotion. His skin was beautiful and everyone including his doctors would comment on how wonderful his skin was.” |
As ALS progresses, bathing techniques change. Early on, handrails, shower chairs and handheld showerheads make it possible for people to continue bathing themselves. In middle stages, more help is needed, especially with limb-onset cases. Later the caregiver is completely overseeing personal hygiene, including tooth brushing, hair combing, shaving and nail trimming.
Mechanical lifts and a shower chair with back and arms make the task easier. Usually a person with late-stage ALS needs a sponge bath, not a full bath, daily or every other day. A bathing sling is used on a standard lift and has a cutout for easier transfer into the tub.
A few other tips: Too-hot water and too vigorous toweling off can cause dry, itchy skin. Similarly, a hot tub or spa may be inadvisable because the heat can overtax the breathing system, and jets can damage weak muscles. For sponge baths, a height-adjustable bed, such as a hospital bed, eases the strain on caregivers’ backs. Look for a norinse shampoo or a shampoo cap that can simplify the task.
Assistance with bathing and daily hygiene can be found through home care aides, Medicare homebound care and hospice staff.
Resources:
See Lifts; Skin; and Chapter 8. Talk with staff at the MDA office or clinic. A visiting nurse can be hired to demonstrate efficient bathing techniques.
Everyday Life with ALS — Chapter 2: Saving Energy; Chapter 3: Home Modifications
Back issues of MDA publications, such as Quest and the MDA/ALS Newsmagazine, can be found online or by calling your local MDA office, (800) 572-1717.
Splish Splash: Easier Ways to Get Clean, Quest, January-February 2008
People with ALS may be at risk for deep vein thrombosis (DVT), a type of blood clot that forms when people are immobile. DVTs are extremely dangerous because they can break off and travel to the lungs, causing a life-threatening pulmonary embolism. Range-of-motion exercises can help to prevent DVT. Your doctor may recommend taking an aspirin daily or wearing elastic stockings.
Signs of a clot in the legs include: redness; heat or discomfort in one leg; one leg that’s more swollen than the other; swelling that doesn’t go down overnight; or pain upon standing or stretching calf muscles. Symptoms of a clot that’s traveled to the lungs include sudden-onset chest pain and shortness of breath.
If you suspect a blood clot, don’t massage the area. Contact a doctor immediately. See Swollen extremities.
Resources:
Don’t Let Leg Swelling Go Untreated, MDA/ALS Newsmagazine, February 2004
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“[My husband] suffered from frontotemporal dementia. Some days he was right as rain, other days he was totally confused, calling me Mom, not knowing our kids or friends, hearing things, highly agitated. I used to take a timeout and walk out on the patio and take a few deep breaths. Then I would go back to him, put on the radio and offer him a massage, cream on his hands, a cool cloth and a hug, whatever, to distract him. Most of the time it worked.” |
Approximately half of all people with ALS won't exhibit any cognitive or bahavioral disruptions throughout the course of their disease.
The other half will exhibit some signs of a condition called frontotemporal dementia (FTD) at some stage in their disease. In most cases (but not all), the signs will be extremely subtle.
FTD refers to changes that occur in the frontal and temporal lobes of the brain, which govern the higher thought processes that make up “executive function.” Such processes include: making or following complicated plans, solving complex problems, following a series of directions and making sound judgments. People with diminished executive function may have varying levels of difficulty completing tasks that require complex planning, forethought or organization.
FTD-associated changes in behavior can include: acting inappropriately in public or toward loved ones and caregivers; loss of motivation (apathy); diminished recognition and response to the feelings and needs of others; repetitive or ritualistic activities or habits; and a change in diet that can include new “favorite” foods or eating too much at one sitting.
Other changes, such as lack of interest, introversion or irritability, may be signs of depression; a doctor can help sort out whether depression treatment will help. Respiratory problems also can contribute to disordered thinking. Counseling can help family members cope with changes in thinking and behavior.
Some tips:
- Organizing and simplifying are important. Have a specific place for commonly used things, such as the television remote and door keys.
- Minimize distractions. If someone is trying to communicate via email or on the phone, make sure that the radio or television is off.
- Encourage doing one thing at a time — not talking on the phone while paying bills, for instance.
- Help the person write notes or use a tape recorder to keep track of things.
- Minimize physical discomfort or pain.
- If the person is stuck on something that’s upsetting him or her, that’s one time when a distraction is a good thing, to divert attention from the upsetting subject.
- Don’t try to convince the person that he or she has changed by pointing out all the changes. That’s usually not effective, wastes energy and can lead to a lot of frustration. It’s not that the person with ALS doesn’t want to see; it’s usually that they aren’t able to see these changes that they’ve gone through.
Resources:
See Pseudobulbar affect; Chapter 3 (respiratory issues) and Dementia in Chapter 6.
When the Thinking Parts of the Brain Go Awry in ALS, MDA/ALS Newsmagazine, November-December 2011
When ALS Affects the Mind, MDA/ALS Newsmagazine, February-March 2005
Cognitive Deficits in ALS Are Usually Subtle, MDA/ALS Newsmagazine, March 2003
This is a very common and frustrating problem in ALS. It may be the result of general immobility; a side effect of some medications; the need for more fiber and liquid in the diet; and/or muscle weakness that makes it hard to bear down to expel the stool.
Constipation isn’t defined by how often a person has a bowel movement, but by whether the stools are hard, dry and difficult to pass. It’s not necessary to have a daily bowel movement, so long as the task can be accomplished without straining.
Common remedies: Gentle dietary fiber is found in raw fruits and vegetables, bran, seeds or high-fiber cereal bars. Bulk or fiber laxatives such as Metamucil or Citrucel are a concentrated form of dietary fiber. Stool softeners, like Colace, keep stools moist and lubricated. Stimulants like Senokot or Smooth Move, an herbal stimulant laxative tea (made by Traditional Medicinals) increase involuntary muscle contractions, moving the stool along more quickly. A daily capful of MiraLax in 8 ounces of water can pull water into the intestines and soften stool. Others swear by a mini-enema called Enemeez or the Magic Bullet suppositories. With a physician’s guidance, keep trying until finding the solution that works best for your situation.
Prescription remedies: Ask your doctor. Mestinon, a drug sometimes used to relieve muscle fatigue in ALS, also has a laxative side effect. It may increase fasciculations, however.
Things to consider:
- Always respond promptly to the urge to defecate. Establish a regular bowel routine, where defecation occurs on a somewhat predictable schedule. Consult your doctor or MDA clinic for suggestions on how to establish a bowel routine.
- Fiber and fluids must be taken together. Without adequate fluid, fiber isn’t effective and in fact can aggravate the problem. This also is true for fiber taken through a feeding tube.
- Although there’s a danger of becoming dependent on laxatives, stimulants, suppositories or enemas, this issue isn’t as acute for people with ALS. Regular and predictable bowel movements that follow the person’s normal pattern (every day or every three days or whatever) make life more comfortable and easier for everyone. One caveat: With regular laxative use, increase fluids and be alert for signs of dehydration, such as irregular heartbeat, disorientation, extreme thirst and headache (see Chapter 5 for more on dehydration).
- Proper positioning helps move things along. A squatting position is more effective than sitting upright. Carefully elevate your loved one’s feet on a low stool and have him or her lean forward. Provide stability and support as needed. For additional force, a pillow can be hugged to the abdomen or gentle abdominal massage provided. If using a lift sling, ensure the buttocks are the lowest part of the body.
- Assist weak muscles by gently but firmly massaging downward on the lower abdomen for several minutes. Some have reported success by squeezing together the person’s buttocks and massaging downward for several minutes, or by lightly circling the skin around the anus (on the outside) to trigger the colon to move.
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“We tried to handle as much as possible with humor — some days it was either laugh or cry. We decided to laugh. I even had a cheer I would do when he was constipated — ‘You’re the man, if you can’t do it, no one can. Push it out, push it out, way out.’ It didn’t always work but it made it a little easier.” |
When constipation is chronic, fecal impaction may result. This is a large mass of dry, hard stool that can’t be expelled. Symptoms include abdominal cramping and discomfort. Watery stool may move around the mass and leak out, soiling clothes. Don’t mistake this involuntary release as diarrhea and treat your loved one with an anti-diarrheal product.
Left untreated, impaction can be life-threatening and require emergency surgery. Laxatives won’t resolve fecal impaction. If suppositories or enemas don’t work, the mass may have to be manually removed by a health care provider. Suppositories may be given between manual removal attempts to help clear the bowel. If you have any doubts, see your health care provider for a diagnosis.
Resources:
See Toileting.
Regaining the Simple Pleasure of Regularity, MDA/ALS Newsmagazine, September 2003
To remain healthy, joints must be moved through their range of motion on a regular basis. When joints aren’t moved fully, as in ALS, a contracture may develop. This abnormal tightening of muscles and other tissues around a joint immobilizes the joint, causing pain when it’s moved. In ALS, this is especially common and problematic in the shoulder joint. Because of the pain, the person moves the joint even less, further aggravating the problem. Contractures can develop very quickly as muscles become paralyzed. Physical therapy and rangeof-motion exercises are key to preventing contractures. Massage, splints, braces and proper positioning in a bed or chair may help prevent or ease discomfort. Check with your doctor or physical therapist for more information.
Resources:
See Range-of-motion exercises; Pain; and Positioning.
Everyday Life with ALS — Chapter 8: Exercise
Coughing
See Chapter 3: Respiratory Issues.
Cramps
See Pain.
Crying, uncontrolled
See Pseudobulbar affect.
Depression
See Chapter 6: Depression.
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While the person with ALS still can dress without assistance, some techniques can help with weakened hands and arms. Attach a pull, like a key chain, to the ends of zippers. A reacher can help pull up pants or move clothing around. Hooks and loops can be sewn onto skirts or pants. The person should sit for as much of the dressing process as possible; when standing, support should be nearby.
Eventually a caregiver’s help will be needed. Larger sizes are easier to manipulate, and looser underwear makes toileting easier. Your loved one can wear pants or skirts with the back seam opened, sitting on a towel in lieu of underwear. When the wearer is seated, the clothing looks normal. This makes toileting easier as no undressing is required.
A person with ALS will feel “more normal” getting dressed, and not wearing a robe or pajamas all day. People with ALS tend to get cold and may need warmer clothes, including boots and mittens (see Temperature).
Resources:
Dressing Tips and Clothing Resources for Making Life Easier, by Shelley Peterman Schwarz, Attainment Company Publications, 2001
Search “adaptive clothing” online for websites offering products.
Caregivers sometimes worry that their loved ones with ALS no longer should be driving. Although it’s possible to install hand-operated driving controls to compensate for leg and arm weakness, there’s no guarantee that these will remain usable as ALS progresses. Set up a driving evaluation with an occupational therapist or the Association for Driver Rehabilitation Specialists to assess needs and abilities.
Caregivers can help ease the transition to nondriver status by rounding up a supply of readily available drivers, investigating taxi cabs and accessible public transportation, and scheduling trips that accomplish several things at once. If you’re concerned that your loved one is unsafe on the road but won’t give up the car keys, talk to his or her doctor.
Resources:
The Association for Driver Rehabilitation Specialists (ADED), (866) 672-9466
This has been called one of the most annoying symptoms of ALS. The problem isn’t making more saliva, but having less ability to swallow it. This can cause choking, as well as skin irritation, frustration and social isolation due to embarrassment.
In most people with ALS, drooling (sialorrhea) can be controlled or at least brought to a tolerable level. In some cases, an occupational therapist can demonstrate saliva management strategies, such as head posture, using facial muscles and achieving more frequent swallowing.
Physicians may prescribe medications to reduce (not eliminate) the flow of saliva. Common drugs include atropine sulfate (Sal-Tropine), tricyclic antidepressants such as Elavil that cause dry mouth as a side effect, and the scopolamine patch usually used for motion sickness. If these are ineffective, the more potent drug Robinul (glycopyrrolate) may be tried. It has some potential downsides, such as constipation, urinary hesitancy, impaired potency, production of mucus plugs, and worsening of existing glaucoma or mental confusion.
Next up in the arsenal is botulinum toxin (Botox) injections in the parotid (salivary) gland. It may take several injections over a number of weeks to achieve saliva reduction, and the effects are temporary. If this isn’t successful, doctors may recommend radiation of the salivary glands, which also can take several weeks or months to work.
Reducing saliva flow can cause another problem — too-thick saliva, which can be difficult to swallow or cough out. See Choking, for strategies to thin mucus. A dry mouth also can hasten tooth decay.
Remember that drooling and medications that reduce secretions can increase the body’s need for water.
Caregivers have tried various creative ways to handle the flow of saliva:
- To catch the saliva, fold a terry cloth towel or washcloth three or four times, and secure it below the lower lip, tucking it behind the jaw or fastening it with an elastic band behind the head.
- Make or buy rolled gauze pads (like those used by dentists) and tuck them between the gum and cheek.
- Those with arm mobility can wear terry cloth sweat bands on the wrists, for frequent mouth wiping.
- Buy face masks (such as those used by transplant patients or people with allergies) and line the chin area with a strip of absorbent cloth. Cut out the portion that goes over the nose if it causes discomfort.
- Cover bed pillows with a waterproof pad and a folded towel to catch nighttime drool.
- Suck up saliva with a portable suction machine, similar to those used by dentists.
- The person can grip a folded cloth or paper towel between the teeth to absorb saliva.
Natural saliva reduction strategies include:
- Before bed, gargle and swish with warm salt water, especially under the tongue (some recommend kosher salt).
- Reduce or eliminate dairy, sugar (including artificial) and grain/gluten products from the diet.
- Use a few drops of anise oil in the mouth (available at health food stores).
- Drink warm tea.
When using medication to dry up saliva, or when using noninvasive ventilation, mouth dryness and nose bleeds may result. Remedies include increased fluid intake and attaching a humidifier to the ventilator or in the room. Try an over-the-counter dry mouth spray (available in the oral hygiene section) or moisturizing mouthwash, such as Oasis from Sensodyne.
See Choking, for information on dealing with thickened secretions caused by dry mouth.
People with ALS sometimes report a feeling of the ears being plugged, as if they have water in them or need to “pop.” Check with a doctor to rule out infection, sinus problems, wax buildup or other medical causes.
In the absence of these problems, it’s possible the cause may be weakness of the muscles that maintain tone in the Eustachian tubes connecting the mouth and ear canals. In addition, weakness in the muscles that elevate the nostrils (thus opening airways) can lead to a chronic feeling of being stuffed up.
Possible remedies include “nose openers” used to stop snoring, such as Breathe Right nasal strips. These strips of tape go across the nose and hold open the nostrils. Antihistamines and decongestants sometimes help, and some say they can get their ears to pop by using their CoughAssist machine.
For some with ALS, the ears become painfully folded during side-lying. Some caregivers create an “ear pillow” to prevent this problem. Using a piece of foam of the proper thickness to keep the head in alignment (not tilted up too high), cut out a 4-inch hole in the center for the ear, and cover the foam with a pillow case.
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Collect important medical information in one spot to facilitate doctor and emergency room visits. Keep this information current, and post a copy on the refrigerator, where ambulance crews have been trained to look in an emergency. (MDA offers a bright yellow envelope for this purpose.) Information should include:
- The person’s name, birthdate, diagnosis and special information (i.e., can’t speak, can’t move legs, uses letter board, uses BiPAP, etc.)
- Emergency contacts and phone numbers (note if someone holds durable or health care power of attorney)
- Insurance information, including identification numbers, addresses and phone numbers
- Names of doctors and phone numbers
- Daily medications: times and dosages
- Adaptive and supportive equipment
- Allergies
- Special diets
- Advance directives and living will
- An updated history of surgeries and major medical events
Prominently note any special orders such as “Do Not Resuscitate” or “No Tracheostomy” (see Advance directives).
Resources:
National Association of Professional Organizers, (856) 380-6828
Personal Records Organizer, (303) 506-5413
Hurricanes, earthquakes, fires, power outages, terrorist attacks — ALS brings special challenges to any kind of emergency. Some planning will help the family be prepared.
Before an emergency occurs, contact your state and local government’s office of emergency management or local fire department to find out what emergency relief assistance is offered. They’ll tell you whom to contact in event of a disaster, where to go, what to have ready. Some of these offices will have special arrangements for people with disabilities. You may need to register in advance for these services.
Emergency Checklist for Persons with Disabilities:
- Prepare a medical information list (see Emergency Medical Information).
- Send copies of important documents to an out-of-town contact person, in case the originals are lost or destroyed in a disaster.
- Know which shelters are best prepared to accept people with disabilities and special medical needs.
- Have a pet care plan. Shelters don’t always accept pets or service animals. Contact your local animal shelter.
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Make an equipment plan.
- Have an extra battery for a motorized wheelchair or scooter. Know how to recharge the battery.
- Have a patch kit, can of seal-in-air product, and inner tubes for a wheelchair with inflatable tires.
- Have a lightweight manual wheelchair for backup.
- Have a converter for your communication device.
- Pack a low-tech communication board and preprinted key phrases in your disaster kit.
- Order an adapter kit for your ventilator so it can be plugged into your car or a marine battery.
- Contact your electricity provider to see if they offer a "priority reconnection service" that will help ensure your power is restored as soon as possible. Even with this service, power could still be out for a long time following a disaster.
- Have a list of the style and serial numbers of medical devices.
- Create a disaster supply kit and escape plan.
A comprehensive checklist for people with disabilities, "Preparing for Disaster for People with Disabilities and other Special Needs" is available from the American Red Cross (search for "disabilities").
In case you must get out of the house quickly, determine the best escape routes and practice them with the family. Remember that you’ll need alternatives to some traditional plans. For example, emergency experts advise heading for the basement in a tornado, but these aren’t usually wheelchair-accessible. Will you be able to carry the person with ALS and their equipment downstairs, or should you go somewhere else?
Assign roles: Who will help the person with ALS? Who will carry supplies? Rehearse, drill, look for other problems. Choose more than one exit in case one is blocked.
It’s also a good idea to discuss a finalized evacuation plan with other family members who may not live with you, as well as with neighbors, friends and home care aides in case anyone other than the primary caregiver needs to assist.
Keep emergency phone numbers in your wallet and near telephones where they’re available to everyone involved in caregiving.
Caregivers who work outside the home should check with supervisors about any emergency plans in effect at the workplace. For example, some places won’t let employees leave for home until an “all clear” has been given by local authorities. Find out whether your home health agency has special provisions during an emergency. Will they continue to provide care and services at another location if your loved one needs to be evacuated?
Resources:
Back issues of MDA publications, such as Quest and the MDA/ALS Newsmagazine, can be found online or by calling your local MDA office, (800) 572-1717.
What Will You Do If the Power Goes Out?, MDA/ALS Newsmagazine, July-August 2007
Alternate Power Resources, MDA/ALS Newsmagazine, July-August 2007
Shelter in a Storm: What Will You Do in a Disaster? Quest, June-July 2007
Plan Ahead for Emergencies, Quest, September-October 2005
Are You Prepared for an Emergency? MDA/ALS Newsmagazine, June 2005
ADA Best Practices Tool Kit for State and Local Government, U.S. Department of Justice
American Red Cross and the Centers for Disease Control and Prevention, Evacuation Planning for Persons with Disabilities and Caregivers, 2006
Individuals with Access & Functional Needs, U.S. Department of Homeland Security Federal Emergency Management Agency (FEMA)
Institution on Community Integration, Impact magazine, Spring/Summer 2007, Feature issue on disaster preparedness for people with disabilities
Interagency Coordinating Council on Emergency Preparedness and Individuals with Disabilities
International Ventilator Users Network
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“Once the medical equipment started to take over the house, it got tougher. We had to face what was coming. We tried to make light of it and realize the end wasn’t going to be tomorrow. The stuff we were bringing into the house was there to help him, not shorten his life.” “By getting the feeding device before she needed it, she was able to get used to it, on her own terms in her own time. Later, when she did have to give up eating, her emotional turmoil was not compounded by having to learn how to use the feeding tube. We approached everything that way, and I still believe that helped us to feel like we were happening to the disease rather than the disease happening to us.” “I wished we had taken everyone’s opinion and got Mom’s wheelchair earlier. It took over 15 months to get the chair and by the time we got it, she could no longer drive it on her own. I was so hoping she might enjoy the independence.” |
ALS leads to muscle fatigue which may manifest as general fatigue. Mental exertion also may be fatiguing because of overall effects of the disease.
Whatever activity a person can do is fine; they should rest when fatigue sets in.
Extreme fatigue may indicate breathing problems, which must be addressed with assisted ventilation. See Chapter 3: Respiratory Issues. In later stages, fatigue may occur even after a passive activity such as being bathed.
Some people with ALS sleep more hours than usual, even 12 or more hours a day. Be sure daytime naps don’t keep the person awake at night. Some doctors are using modafinil (Provigil) to help with daytime sleepiness.
Resources:
Everyday Life with ALS — Chapter 2: Saving Energy
“Get it before you need it” is the mantra of ALS. Some people see using assistive equipment as giving in to the disease, but in fact the opposite is true. Adaptive equipment is like a weapon in the battle against the disease.
Canes, braces, walkers, wheelchairs, communication devices, coughing and suction machines, feeding tubes, lift chairs, hospital beds, alternating pressure mattresses, mechanical lifts and assisted ventilation make it possible to thrive despite the best efforts of ALS to take over. Assistive equipment enables greater independence and safety for your loved one, while making it easier for you to be an effective caregiver. Not using this equipment can result in injuries to the person with ALS and the caregiver.
Another reason to get equipment early is that it takes time to order and get approval from insurance or Medicare. If a move to assisted living, nursing home or hospice is being considered down the line, be sure to get all essential equipment first, as Medicare may not pay for it afterward.
MDA provides financial help toward the repair or modifications of durable medical equipment, including leg braces, wheelchairs and communication devices. It’s possible to borrow equipment through the MDA equipment program; check with your local office.
Resources:
See Chapter 7 for information on help paying for equipment.
Everyday Life with ALS — Chapter 4: Mobility & Support Equipment
Back issues of MDA publications, such as Quest and the MDA/ALS Newsmagazine, can be found online or by calling your local MDA office, (800) 572-1717.
People with ALS Share Personal Experiences with Life-Enhancing Devices, MDA/ALS Newsmagazine, February 2007
Accessing & Acquiring Assistive Technology — Some Options to Make Funding AT Purchases Easier, MDA/ALS Newsmagazine, November-December 2006
Technically Speaking, It’s a Good Time to Have ALS, MDA/ALS Newsmagazine, March 2006
Get Time on Your Side When Obtaining Major Equipment, MDA/ALS Newsmagazine, January 2005
Equipment Survey Can Help with Planning, MDA/ALS Newsmagazine, April 2002
ABLEDATA
RESNA — Alternative Financing Technical Assistance Project, (703) 524-6686
State Departments of Vocational Rehabilitation
It’s not known how much exercise — if any — is valuable for people with ALS. Before beginning an exercise program, consult the doctor or physical therapist about frequency, duration and level of intensity. The goal of gentle exercise in ALS is to help maintain mobility, improve endurance and minimize pain from the effects of muscle wasting. When exercise isn’t possible, physical therapy and range-of-motion can help serve the same purposes.
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Important exercise guidelines include:
- Don’t overdo it: Soreness after exercise is an indication that you’re tearing muscle, which isn’t good.
- Think aerobic: This helps improve heart health and circulation. Possibilities include a stationary bicycle, upper body ergometer, elliptical machines or aquatics, or multiple repetitions of gentle resistance strengthening and stretching exercises.
- With weights, start small: Increase only when appropriate and by a very small percentage.
- Just say no to pain: Forget the motto “no pain, no gain.” This doesn’t apply to people with ALS. Focus on maintaining functional strength, endurance and independence.
- Be flexible: Don’t hold to a rigid program. The person should listen to his or her own body and use common sense.
Resources:
See Range-of-Motion Exercises.
Everyday Life with ALS — Chapter 8: Exercise; Chapter 9: Exercise Instructions
Supervised Resistance Exercise Slowed Functional Loss in Small Study, MDA/ALS Newsmagazine, September 2007
Exercise Has Many Benefits for People with ALS, MDA/ALS Newsmagazine, October 2002
Caregiving, especially lifting, is hard on the back, neck and shoulders. Good body mechanics (like lifting with the legs, not the back) and assistance (using a mechanical lift or helper) significantly reduce the risk of injury and always should be observed. But without proper flexibility and strength in legs, hips, shoulders, abdominal and buttock muscles, an injury can be just one wrong move away.
| “Loads of people have had to give up caregiving due to injury. Then they have to find someone else to provide care, or in some cases find a nursing home. The beauty of exercise is, whatever you can do will help. A little bit here and there all adds up.” |
Regular exercise — say an hour at the gym or a daily walk — is a very good idea for caregivers, with both physical and emotional benefits. Talk with a trainer or doctor about a good program for you. Because it can be hard to find the time for lengthy exercise breaks, consider incorporating several quick exercise periods into daily routines.
The following 30-second stretching and strengthening exercises, performed several times throughout the day, can have a positive cumulative effect. They can be fit into odd moments, like while waiting at a traffic light, cooking or standing in line. Pairing an activity with an exercise — say doing partial squats while waiting for the toast to pop, hamstring stretches during a TV commercial and core muscle strengthening while blow-drying hair — can allow caregivers to improve without even realizing it.
NOTE: Check with your doctor before undertaking any new exercise program.
Flexibility: This is critical to preventing injury during the many caregiving tasks that put the body into unaccustomed positions. Stretch to the point of discomfort but not beyond. Pain or distance isn’t the point. Hold stretches about 15-30 seconds, repeating several times. Never bounce to get a greater stretch.
- Lower back stretch: Tight hamstrings contribute to lower back problems. Stretch hamstrings by sitting in a chair with your feet on a footrest. Keeping knees straight, lean forward until feeling an easy, not painful stretch in the back of the thighs. Or, bend your knees just enough to allow you to touch your toes.
- Shoulder stretch: Scratch your back. Point your elbow in the air and scratch the top of your spine. Next, curl each arm around the front of your neck and scratch over your opposite shoulder. Finally, hold your elbow down by your side and twist your hand back up between your shoulder blades.
- Neck stretch: Lower your ear to your shoulder on either side.
- Strengthening: Muscle strength is important for lifting and for stabilizing the lower back region. To strengthen without using weights, tighten muscles to their maximum, hold for a count of 10, relax for a count of 10 and do several repetitions a day. Be sure you’re stable, either by taking a wide stance or holding onto a secure object.
- Legs and hips: Do partial squats while holding a stable object like a countertop. Keeping your back straight, slowly lower as if sitting down. Go down as far as comfortable then back up. For greater effect, use only one leg at a time. When rising from a chair, use only one leg. Hold onto an object for stability but don’t pull yourself up.
- Lower back: Squeeze buttocks and/or abdominal muscles as tight as possible and hold for a count of 10. To strengthen core muscles, pull your navel, waist and buttocks toward the middle of your body, as if trying to pull your body away from your clothes. Hold, release, repeat.
- Shoulders and arms: (a) Take a weight such as a shoe or soup can and lift it straight up in the front and to the side, from the waist to over the head. Hold arms straight out to the sides at shoulder height, then bring one arm across in front of the chest, keeping the elbow straight. (b) Force shoulder blades together, then relax, repeating several times. Shrug shoulders up toward the ears then relax.
- Neck: Place your palm in the middle of your forehead and gently press, resisting the push with your neck muscles. Repeat on each side and the back of your head.
Resources:
Exercises to Do on the Run: Simple Ways for Caregivers to Resist Injury,MDA/ALS Newsmagazine, October 2003
Treat Your Own Back and Treat Your Own Neck, by Robin McKenzie, Spinal Publications, 2006
Some people with ALS have burning or dry eyes, or — just the opposite — watery eyes. Either case can be irritating and even painful. This could be caused by lack of blinking or by air blowing from a respirator. Saline eyedrops, or a warm wet washcloth or cold washcloth compress, may help. An antibiotic may be needed if irritation persists. Natural Tears, an over-the-counter product, is recommended. ALS doesn’t affect vision.
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“I was just starting to waken from a delicious sleep, when I heard a terrible crash. I looked over and saw that [my husband] was face down, his glasses bent, and there was blood all over the hardwood floor. (Always considerate, he had somehow missed the carpeting.) The cuts on his face were not serious but there was considerable splatter (thanks, “CSI,” for THAT term). He falls from time to time, so at first, it looked like a matter of checking him out, and perhaps getting the Hoyer lift into play. But he seemed unusually disoriented, he was snoring, and his face seemed slightly purplish. I called 911.” |
This ALS symptom is of great concern to caregivers. Even when a person can walk, trips and falls can occur without warning, and it may be impossible to use the arms to brace for a fall. Injuries from falls range from minor to major and the recovery period can lead to additional loss of physical ability — the last thing anyone with ALS wants.
The best way to avoid falling is to use assistive equipment. However, many people will consent to using a cane or a folding shopping cart, but balk at using more “medical” devices like a walker or wheelchair out of embarrassment or resistance to “giving in to the disease.” Assistive equipment actually allows more independence and fights the disease by preserving precious strength and energy. Caregivers often try to convince their loved ones to use the appropriate equipment, with varying degrees of success.
Some tips:
- Ensure the home environment is safe by eliminating tripping hazards and adding sturdy handholds at strategic locations. Shield sharp corners on tables and counters and add a shower chair in the bathroom.
- Helping someone up off the floor after a fall can be dangerous for both of you. When possible, use a mechanical lift or recruit a neighbor or family member to help. If you can’t get the lift to the person, roll the person onto the lift sling or a sheet and pull to a location where the lift can be used.
- Be careful to use proper body mechanics when lifting. If no help is available, call 911. If you ask the rescuers not to use their sirens, they usually won’t. See the chapter on transfers in Everyday Life with ALS for more on standing up after a fall.
- Ensure your loved one has a cell phone, emergency assistance pager or other signaling device for falls that happen when you’re not around.
- If the person with ALS stays alone for periods of time, ensure rescuers (neighbors or emergency personnel) can get into the house by hiding a key outside or leaving the door unlocked.
- Protective equipment like knee and elbow pads and a helmet can make falls less injurious. Lighter-weight shoes are easier to negotiate.
Resources:
See Lifts and Accessibility at Home.
Back issues of MDA publications, such as Quest and the MDA/ALS Newsmagazine, can be found online or by calling your local MDA office, (800) 572-1717.
Take Falls Seriously to Prevent Further Injuries, MDA/ALS Newsmagazine, October 2002
All Fall Down, Quest, December 2002
| “To keep my husband’s feet from falling to the side, a friend constructed a three-sided box lined with 2 inches of foam. He rests his feet inside the box, which is connected to the bed frame and is adjustable. I tent the blankets and sheets over the box.” |
In addition to exercises recommended by a physical therapist, and/or the use of orthotics (leg braces), feet need protection in bed, as heels can develop pressure sores and the weight of blankets and foot drop (inability to turn the ankle or toes upward) can cause pain. “Float” your loved one’s heels above the bed using small pillows at the ankles. Prevent feet from flopping down or to the side by bracing them with more pillows. Support the weight of sheets and blankets with a blanket lift at the end of the bed. Blanket lifts, which fit between the mattress and box springs and extend upward to support the bedding, can be purchased from medical supply outlets or made at home. Sheepskin pads, knee or elbow pads under the heels, foam boots and heel pads also are helpful.
For foot drop see the medical care team about leg braces, some of which the loved one can sleep in.
By the way, caregivers should protect their feet and knees also. A thick rubber kneeling pad can help a lot if you’re frequently up and down. And watch for repeated banging of knees against furniture when turning or lifting the person with ALS. Knee pads or a new technique may be in order.
Resources:
See Swollen Extremities.
As hands, arms and shoulders weaken, caregivers can seek out various aids to extend dexterity. These include: wraparound lap desks to support the arms; mobile arm supports to allow both horizontal and vertical motion; lightweight wrist splints; eating utensil holders or specially designed eating utensils such as lightweight large-handled cups and plate guards; key holders, doorknob extenders, light switch extension levers; lightweight reachers; card holders (for playing cards); button and zipper hooks; long-handled sponges; Velcro fasteners on pants and shirts; pencil grips; book holders; speaker phones and more. Consult an occupational therapist to help solve specific problems.
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“After losing her speech, my Mom’s left hand was the first thing to be affected. Her fingers also curled, ending up in sort of a claw position, and it was very painful when the fingers were moved. When I put her shirts on, I would cup her hand with mine to make sure the shirt didn’t get caught on her fingers and pull them. At the suggestion of the hospice nurse, we rolled up a washcloth and she would hold it so her hand didn’t become completely clenched.” |
Hand-curling can be managed with hand splints, or by putting a rolled washcloth inside the hand, to keep it in a more natural position and prevent fingernails from digging into palms. Holding small, heated rice bags in the hands for 10 to 20 minutes can make them more comfortable.
Some other techniques to help adapt to changes in hand and arm strength include:
- In addition to range-of-motion exercises for the shoulders and elbows, try the “prayer position” exercise. Put the hands together as if praying and point the fingers up and down; this helps stretch fingers and wrists and releases stiffness in forearms.
- A sling supported with a strap across the back and over the opposite shoulder cradles the arm and can help protect the shoulder joint from uncomfortable stretching.
- Arm rests on chairs may need to be padded in order to be high enough to support weakened shoulder muscles. This is especially true if a pressure relief cushion is put on the seat.
- A lightweight splint, similar to those available at the drugstore for carpal tunnel syndrome, supports the wrist, helping to bend the fingers easily, which maximizes the grip, helping with many tasks of daily living.
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| The Arm Thing, being used by Ron Edwards, co-inventor with his wife, Linda |
- For feeding, the person with ALS can support the elbow on a few books or pillows or with a suspension arm sling. Another help is The Arm Thing, an arm lifter invented by a man with ALS and his wife/caregiver. Long straws make it unnecessary to pick up a glass to drink. Place a stand-up mirror on a table to do makeup, shave, or brush hair or teeth. All of these take the workload off the shoulder muscles.
- Modify tools with foam curlers or tubing to thicken the grip of forks, toothbrushes and other everyday tools.
- Showers instead of baths minimize falls. Use a bath bench or seat while showering. Rest the elbow on the shower wall to wash hair.
- Getting up from a toilet seat is easier and safer with a higher seat. Use a raised toilet seat or a bedside commode positioned over the toilet.
- Prop books or magazines on a table, bookstand or music stand. The eraser end of a pencil can serve as an inexpensive page-turner.
- For working at a desk, rest forearms and elbows on chair arms or desk to relieve stress on the upper arms. A wraparound desk is especially helpful as it supports the elbows without reaching. A keyboard wrist rest can help to support hands, and on-screen keyboards minimize stress on the wrists at the computer.
Resources:
See Accessibility at Home; Dressing; and Swollen Extremities.
Telephone Access for People with Hand & Arm Weakness, MDA/ALS Newsmagazine, October 2006
Arming You with Tips for Living with Arm Weakness, MDA/ALS Newsmagazine, February 2006
Dynamic-Living, (888) 940-0605
Hygiene
See Bathing; Drooling; Skin; Toileting.
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“The first time he used his power wheelchair, I took him in our new converted monster van to Costco. All of a sudden I realized he was no longer with me and in a panic I ran through the store looking for him. Where was he? In the candy aisle, looking for a treat! He felt such a sense of freedom that he could wander through a store alone, which made me feel better.” |
Independence fuels a “fighting spirit.” Help your loved one maintain the highest possible degree of independence, changing strategies as the disease progresses. Figuring out ways around the limitations of ALS is a creative game, in which caregiver and loved one are on the same team and every victory for independence is a blow against the disease. (Note: The use of assistive equipment like wheelchairs is a move toward independence, not away from it.)
- Be patient. Independence for a person with ALS can be slow and painstaking. Sometimes it seems faster and easier for caregivers to do it themselves. Breathe, relax and be grateful for whatever degree of ability the person has.
- Be collaborative. Remember that people with ALS are capable adults even if they can’t communicate clearly. Through whatever means possible, discuss choices, make joint decisions and defer to the loved one’s wishes in decisions regarding their medical care.
- Ask if they want help before helping. Don’t take over tasks that still can be performed if the person is given adaptive devices and time.
- Let your loved one use your hands. When a person with ALS needs help with something, it can be very frustrating to have a helper take over the task and do it their way. The end result is seldom what the person with ALS envisioned. Your loved one doesn’t need a caregiver’s brain to plan things, just a pair of willing hands. Suggestions are fine, but ignoring the person’s ideas is demeaning.
- Help set up your loved one with a computer and Internet access. Help locate and install adaptive technologies that enable computer use when movement is limited or absent. Computers provide entertainment and social interaction and enable the person with ALS to help with household chores such as paying bills, tracking down information, hiring services and grocery shopping.
- Use adaptive devices and strategies. Consult a physical or occupational therapist for suggestions. See Hands, for suggestions of some adaptive devices to assist with activities of daily living (ADLs). Other adaptations include rearranging household objects or furniture and changing the way a task is done (i.e., sliding something rather than carrying it).
- Don’t sacrifice safety for independence. It can be dangerous to leave unattended a loved one with a history of choking or falling. Families have found several ways to monitor loved ones, such as cell phones, baby monitors, emergency response buttons, friendly neighbors, etc. (See Safety.)
Some people with ALS seem to experience greater-than-normal scalp itchiness. There are a variety of possible causes, including dryness, yeast or fungus overgrowth, and stress. Check with a doctor and experiment to find the right remedy. Some things that have worked for others:
- Use gentle hypoallergenic hair care products.
- Shampoo hair in lukewarm water — hot water strips away protective scalp oils. Towel dry gently, not vigorously. Don’t wash too frequently or too infrequently.
- Use a humidifier.
- Over-the-counter remedies include Benadryl, Absorbine Jr. (applied liberally to the scalp), shampoos containing ketoconazole (such as Nizoral) or coal tar (such as T-Gel). Scalpicin Anti-Itch Scalp Treatment is a spray that can be used several times a day.
- In some cases of chronic itching or skin sensitivity, a physician may prescribe an anti-epileptic drug such as carbamazepine (Tegretol) or gabapentin (Neurontin), or one of the tricyclic antidepressants.
Resources:
See Skin.
An uncomfortable tightening or chattering of the jaw in response to cold, anxiety or pain may occur in ALS. Relaxing medications such as diazepam (Valium) may help. Also see Pseudobulbar Affect.
Laughing, uncontrolled
See Pseudobulbar Affect.
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As ALS progresses, mechanical lifts can save caregivers’ backs, necks and shoulders from injury. Lifts are useful in transfers, such as from bed to wheelchair, or from wheelchair to toilet or bath, and in getting someone up off the floor after a fall. (See Everyday Life with ALS — Chapter 7, for ways of moving the person before a lift is needed.)
Lifts are operated mechanically or with motors, and have a sling of sturdy material on which the person with ALS sits. There are several types of slings, geared for different uses. Besides freestanding lifts, some operate on ceiling tracks.
Sometimes people with ALS are leery of lifts, feeling insecure and vulnerable swinging in this new contraption. Practice on other family members to get a feel for it and to allow your loved one to see it in action. Once the mechanics have been mastered, this is a terrific piece of assistive equipment.
Motorized lift chairs and uplift seat cushions (with a spring-powered seat) can boost a person upright when arms are too weak to help push up. Be sure the chair is correct for the person’s height. These aren’t likely to be covered by insurance policies, while mechanical lifts usually are. Check your policy.
| “Even with the narrow hospital bed, I have to stretch when turning and caring for [my husband], and as a result my knees have been injured by pressing against the bedrail. I’m very cautious now and use slippery plastic under his shoulder and buttocks and a lengthwise-folded towel as a strap to pull his bent lower knee when turning him. I also pad my knees against the bed with a pillow.” |
To go upstairs and downstairs, a chair lift can be installed on the home’s major staircase.
Resources:
Check with insurance, Medicare or the MDA equipment program for information on obtaining a lift.
Give Me a Lift: The Right Tools for the Transfer, Quest, March-April 2006
For permanent or portable lifts, stairlifts or lift chairs, see ads in Quest or go online.
Regular massage by a professional or a caregiver is physically and emotionally therapeutic for people with ALS, and also can help them sleep. Gentle massage is preferred to deep muscle massage. Caregivers also will find a massage for themselves is a great way to ease stress.
It’s wise to have a primary care physician for care that doesn’t involve ALS, and to have this doctor consult with your ALS physician when necessary. Regular medical checkups, flu shots and pneumonia vaccinations are essential to preventing respiratory complications. Conditions such as diabetes, cancer, hypertension, Alzheimer’s, heart disease, etc., may affect what medications or treatments a person can take. For example, a respiratory system weakened by ALS may make surgery more risky.
Keep your ALS doctor and other doctors informed about all of your loved one’s medications and conditions. Knowing as much as possible about ALS will help a caregiver determine whether a symptom is related to the disease or has some other cause. When in doubt, call someone on the ALS health care team.
Only one medication has been developed specifically for ALS — riluzole (Rilutek). When started early in the disease course, it may add at least a few months to life expectancy, possibly by interfering with glutamate, a carrier of signals in the nervous system which may be overactive in ALS. Sanofi-Aventis, headquartered in Paris, now holds a patent on the medication, but at least one other company is working on a generic version.
| “I was totally shocked at how much teaching I would have to do. The student nurses, the full-fledged nurses, all the CNAs, aides, hospital PTs, you name it. I had to enlighten them on ALS and teach them how to use the gait belt properly. I had to explain what happens to your body with ALS. Most were very receptive and wanted to learn.” |
A few people have found that Rilutek caused upset stomach or skin allergies. An ALS doctor can help decide whether the drug is beneficial. Many believe it has significantly slowed the progression of their ALS.
Rilutek can cost up to $1,000 a month. Medicare and most insurance companies cover it, and the National Organization for Rare Disorders (NORD) has a Rilutek patient assistance program that can help with the cost. MDA and your health care team can help you explore all sources of coverage.
A number of other drugs are prescribed in ALS to help with symptoms such as coughing, depression, pain, pseudobulbar affect, etc. Insurance often covers these.
Cholesterol-lowering drugs known as statins may have some role in neuromuscular disease, but that role isn’t clear. Some studies suggest that taking statins may lead to ALS symptoms; others that the drugs may have anti-inflammatory benefit in the disease. Until the information is further clarified, ALS patients with high cholesterol should carefully consult with their doctors to determine the best therapies.
MDA-funded scientists are developing and testing several potential ALS drugs. Find out which drugs are being tested, and which clinical trials are open to participants, at clinicaltrials.gov.
Resources:
See Prescription Drugs and Supplements.
ClinicalTrials.gov. Federal site offering current and archived information about clinical trials in ALS.
Neck muscle weakness in ALS makes it hard to turn or hold up the head, leading to decreased mobility and — if untreated — pain, especially when turning the head, raising and lowering into bed, or rolling over. Poor neck posture also can impair breathing, swallowing and communication. Effective management of neck weakness can prevent or treat pain and injury. The key is to find the best methods of supporting the head.
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Be aware that neck weakness makes the person more vulnerable to injury during transfers and when riding in a vehicle. If the head suddenly flops down, back or to the side, muscles and ligaments in the neck can tear, or the cervical spinal cord or neck vertebrae can be seriously injured. For some, even a slight jerk is all it takes to tear muscles.
Encourage and assist neck-stretching exercises (after consulting a physical therapist).
Two simple exercises:
- Slowly turn the head to each side as far as possible without pain, hold and return to the center.
- Tilt the head sideways on each side, so the ear points down toward the shoulder; hold and return to center.
Use a lumbar roll or cushion behind the lower back to prevent slumping when sitting, and to prevent the head and shoulders from tipping forward. Pillows under each arm also promote upright head position when sitting.
Work with a physical therapist to investigate different types of soft collars, neck braces and head supports such as a band around the forehead that attaches to a headrest. Alternating the use of collars and head support systems helps reduce pressure points and skin breakdown. Thin-cushioned skin dressings (e.g., Duoderm) also protect the skin.
Ensure the person’s bed pillow isn’t too thick, as that can cause neck strain. Try placing a rolled towel under the back of the neck, coupled with a thin pillow for the head.
Terrible nightmares, coupled with morning headaches and mental fuzziness, may indicate respiratory problems in ALS. See Respiratory issues for more information.
Although ALS doesn’t directly cause pain, it leads to some painful secondary conditions. It’s estimated about two-thirds of people with ALS experience chronic discomfort or pain, primarily due to muscle cramps, pressure sores, stiff joints, overstretched muscles and spasticity (jerky movements caused by rigid muscles). Smaller involuntary contractions (twitches) are called fasciculations.
There are several avenues to pain relief:
- Rest and sleep. Lack of sleep can make anyone especially sensitive to discomfort. A good night’s sleep or a few days’ rest from overexertion sometimes can relieve aching and cramps. See Sleep, for ideas on more comfortable sleep.
- Check equipment. As the person becomes less mobile, pain can arise from ill-fitting equipment, such as incorrect wheelchair seating. Any prolonged position can contribute to pressure sores. See Positioning; Pressure Sores; and Sleep, for more.
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Ensure your loved one always is positioned correctly in the bed, chair or wheelchair. Experiment with cushions, mattresses and pillows until the right support is found. If it’s impossible to get comfortable, investigate a change in bed or wheelchair.
Some people with ALS become profoundly sensitive to anything touching them. A light blanket may feel like a heavy weight or an annoying sensation when the person can’t move to get comfortable.
- Relieve coughing. Prolonged coughing from the flu or from weakened respiratory muscles can become exhausting and make the chest muscles sore and achy. See Assisted Coughing, to learn how to help relieve this kind of pain.
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Movement and touch. Stretching, exercise and laying on of hands can help dissolve pain. Every day, encourage, assist or perform range-of-motion exercises — these are critical for preventing or easing pain. Range-of-motion, if not too strenuous, can feel like a gentle massage. (See Contracture, and Range-of-motion.)
Applying heat, such as microwaveable moist heat pads, directly to the area of discomfort can provide relief. Warm baths or showers may work.
Don’t forget massage. Gently massage the painful area till it relaxes, or have the person sit facing the back of the chair or lie down for a relaxing back rub.
Doctors have recommended leaning over and pretending to stir a big pot as a way to work out cramps. Moving the arm as if crawling up a wall can ease frozen shoulder joints. A good stretch for the arms is pretending to pull up a zipper behind the back.
- Medicate. Sometimes it’s necessary to consult a doctor about medications for spasticity and cramps. It can take a while to find the right medication and dosage that reduces spasticity without increasing weakness. Some common medications include Baclofen, Zanaflex, Neurontin, Lyrica, quinine (Qualaquin), magnesium and clonazepam (Klonopin). Arthritis medications or over-the-counter nonsteroidal anti-inflammatories (Ibuprofen, Aleve) sometimes work. In states where marinol (medical marijuana) is legal, it’s been found to relieve some pain, accumulation of mucus and spasticity. In advanced cases, such as for those in hospice care, narcotic pain medication may be prescribed.
Resources:
Marijuana as Wonder Drug, Boston Globe, March 1, 2007
Living with Chronic Pain: The Complete Health Guide, by Jennifer P Schneider, M.D., Ph.D., Hatherleigh Press, 2009
Making Friends with Pain: Learning to Live Well with Chronic Illness, by Elizabeth Flora, Sadie Books, 1999
| “An adult who can be a rock during tough times doesn’t have to be the parent. Our kids got close to our social worker and also had friends whose parents listened as well. Sometimes it’s best if it is someone not right in the middle of the situation.” |
ALS can strike as early as the teens and as late as the 80s. It very often hits those in their prime years, who are raising children or planning families.
Men and women with ALS have conceived and borne children after diagnosis. Sexual organs aren’t directly affected, though sexual activity may be influenced by immobility or discomfort (see Intimacy and Sex).
Mothers and fathers with ALS find many ways to relate to their children. Activities may change, but children’s need for a parent’s love and attention does not. For some parents with ALS, having a child provides a reason to keep living and fighting the disease.
Resources:
See Pregnancy.
Children and Parenting in Chapter 6 explore emotional and practical issues of parenting.
MDA/ALS Newsmagazine website's category on Parenting
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“What helps me is being in a wheelchair with armrests to support my arms and a lap tray across the armrests to put my hands on. The armrests are cupped which helps keep my elbows from falling off. If I get too low in the chair the upward pressure of my arms in my shoulder joints gets painful, so I need to be pulled up. Some non-skid material on my cushion helps prevent sliding down so I don’t need to be pulled up often.” |
Proper body alignment and support can forestall a variety of problems, like pressure sores and joint pain. As noted by one woman with ALS, “One hour spent with an arm unsupported is miserable and can result in days of shoulder pain and sleepless nights.” In general, the head, shoulders, hips and feet should be aligned and not too flexed or overextended. Caregivers have several strategies to achieve proper positioning.
- Lots of pillows, in different sizes and firmness, stabilize a person in comfortable positions. When side-lying, put a pillow between the person’s knees to keep hips in alignment. For back-lying, put a pillow under the knees and elevate the arms slightly with pillows. Use more pillows to “float” heels above the bed and keep feet from flopping to the sides or downward (see Feet). Large body pillows, rolled towels and air cushions work for some.
- There’s no such thing as a one-size-fits-all wheelchair. Consult an OT or PT to customize a chair to your loved one’s unique body proportions. Important measurements include depth of the seat, position and height of the headrest, distance from seat to footrest, height of armrests, and backrest trunk support. Finding and fitting the proper wheelchair cushion is a crucial element.
- A wheelchair wraparound lap desk helps support the trunk, arms and hands.
- In bed, a drawsheet is a key positioning aid. The drawsheet is placed on top of the bottom sheet, extending from the person’s shoulder to buttocks with at least 6 inches of sheet remaining on each side. Grasping and pulling on the sheet (alone or with a helper) allows even a large person to be rolled to the side or hoisted higher up on the mattress. Ask an OT or PT for a demonstration.
- Silky sheets and pajamas can make positioning easier.
- No one should have to sit in the same position all day or lie in one spot all night. When your loved one no longer can change positions, an important part of caregiving is helping shift their limbs or entire body.
- If the person keeps sliding out of the wheelchair, try a seat belt or rubbery shelf liner on the seat and foot supports.
Resources:
See Pressure sores; and Sleep.
Everyday Life with ALS — Chapter 7: Transfers
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“When we first had him, I had this fear that he would know that I was different and that he would not bond with me, but they’re just so adaptable and they love you for who you are. I guess I associated being a mother so much with the action, changing a diaper and so forth. It’s almost as if I’m half sister, half mother, because he and I are always being pulled around by Dad. We laugh together all the time. I sense that he does know that I’m his mom. So it really has been eye-opening and humbling to me to see the love there. What I wasn’t really aware of was just the everyday miracle of having a child in the house. Instead of just the sound of the TV or silence, it’s so cute to hear him laughing and talking to himself. I find it wonderful medicine for me.” |
Women with ALS who are considering pregnancy should take into account these factors:
- The enlarging uterus will push up against the diaphragm, squeezing the lungs, which can exacerbate weak breathing.
- If a Caesarean section is required, certain types of anesthesia may be a problem because of the risk to respiration.
- Vaginal delivery requires strong muscles in the lower body.
- ALS in some cases may be hereditary.
- The child may lose his or her mother in childhood.
- Prolonged bed rest can lead to significant loss of muscle mass, which probably won’t be regained in ALS.
However, the urge to have a child is powerful, and many women with ALS have given birth. A child may give both parents joy and hope that will strengthen them in the battle against ALS. Consulting many experts may help couples make this very personal decision.
Resources:
See Parenting above and Parenting in Chapter 6.
MDA/ALS Newsmagazine website's category on Parenting
Whenever skin is under prolonged pressure, tiny blood vessels are compressed, the supply of oxygen and water is interrupted, and skin starts to die. Pressure sores (decubiti) are caused by staying too long in one position and by medical equipment such as a ventilator mask or neck brace. Sores can lead to infection, long and inconvenient recovery periods, and in extreme cases, death.
Some cautions to observe:
- Nutritional or respiratory problems can cause skin to become very fragile.
- People who are immobile should have their skin checked thoroughly at least once a day. For light-colored skin, an area that stays red longer than 15 minutes after pressure is removed is the beginning of a pressure sore. For dark-colored skin, look for areas that are darker or purplish-blue in color.
- A developing pressure sore causes a burning, itching pain. Even if there is little or no redness at the spot, treat the area as a pressure sore by reducing pressure and cleaning the area. Prompt response to this early sign can make a huge difference in just a few days.
- Ensure your loved one always is properly positioned in bed, chair and wheelchair. Reposition at least every two hours or whenever asked by the person due to discomfort. (See Positioning.)
- Protect body parts that get a lot of pressure (especially skin over bony areas like elbows, shoulders, heels, tailbone, shoulder blades, ears and the back of the head) with pillows, pads, specially designed cushions or cushioned skin dressings such as Duoderm.
- When making a foam pad to sit on, don’t cut out the center (as is done for corns or bunions) as this can further decrease circulation.
- When people no longer can reposition themselves in a chair or wheelchair, get a pressure relief cushion, like a ROHO or Jay cushion, as ordinary foam or air cushions aren’t adequate to prevent pressure sores.
- Experiment to find the most comfortable and effective pressure reduction in bed, such as standard or alternating pressure air mattresses, real or synthetic sheepskin, foam mattress overlays and automatic turning mattresses.
- Beware of recliners, where people with ALS tend to spend a lot of time. Outfit the recliner with a pressure cushion or sheepskin padding; protect elbows and heels; and keep a close eye out for problems.
- When ventilator masks and neck supports cause skin breakdown, alternate with different masks or head support systems to give skin a rest.
Resources:
See Skin; and Sleep.
Check out ads in Quest, and search the Internet for “wheelchair cushions” or “pressure relief cushions.”
Uncontrollable crying and laughing, out of proportion to the situation and sometimes out of the blue, is a symptom affecting 15 to 45 percent of people with ALS. Although not well understood, the problem (also called emotional lability or involuntary emotional expression disorder) seems to be related to degeneration of neurological pathways that modulate emotional expression. This degeneration appears to cause a "disconnect" between a person's actual mood and his or her facial expressions.
PBA’s emotional outbursts cause some with ALS to shun social situations and to worry that they’re “losing it.”
| “Dad just keeps on weeping all day. So much so that it’s difficult for us to know if he is happy or sad. I am trying to do all that I can to make him a bit more comfy, but he just doesn’t seem to like it.” |
Excessive yawning and jaw clenching also may be symptoms of PBA.
In October 2010, the U.S. Food and Drug Administration (FDA) approved the drug Nuedexta, made by Avanir Pharmaceuticals, specifically for the treatment of PBA in ALS and multiple sclerosis.
PBA is tough on caregivers. Excessive crying is very upsetting, while inappropriate laughing can feel insulting in some situations. It’s important for everyone to remember that this symptom isn’t related to true emotions, but to neurological "disconnects."
- Is it PBA or depression? Check with a doctor for a questionnaire that rates episodes for frequency, duration, voluntary control and appropriateness.
- Medical treatments include Nuedexta, which is approved specifically to treat PBA in ALS; and antidepressants, which may work even though depression isn’t the issue. Talk with your MDA clinic doctor.
- Caregivers should try to remain emotionally neutral during a PBA crying episode. Although the natural response is to comfort a crying person, that may actually make it harder to regain control. Change the subject or take a break.
- Sometimes people can shorten their PBA episodes by focusing on breathing patterns. Focus on breathing in for laughing and breathing out for crying.
- In social situations, briefly explain to others that this is something that happens with ALS, that it doesn’t represent true emotions, and that it will pass shortly. Don’t allow your loved one to become socially isolated due to this symptom.
Resources:
About Nuedexta, Avanir Pharmaceuticals, 2011
Emotional Expression Medication Approved for Use in ALS, MDA/ALS Newsmagazine, November 1, 2010
PBA Symptoms No Laughing Matter, MDA/ALS Newsmagazine, March 2006
Range-of-motion (ROM) exercises
| “Range-of-motion is extremely important. [My husband] got ROM twice daily with touching and massaging of hands and feet whenever we were with him. This was for our benefit as much as his, so we felt close to him. Our children were great about massaging their dad’s hands and feet.” |
 |
| Physical therapist Jeanine Schierbecker tests the strength of ALS patient Glen Houston, who was in a clinical trial at Washington University. |
Stretching and moving muscles and joints is essential to maximizing movement and minimizing pain. Blood clots, pressure sores, discomfort, sleeplessness and contractures are some of the potential consequences of immobility. Caregivers can learn ROM from a physical therapist. If a loved one still can perform exercises alone, encourage daily practice. When assistance is needed, caregivers should assist only to the point at which the person can do it alone. Once voluntary movement is gone, passive ROM should be performed by a caregiver every day.
Resources:
Instructions and diagrams for stretching and range-of-motion exercises can be found in Everyday Life with ALS — Chapter 9: Exercise Instructions.
|
“When I have to use the alarm button, it rings into the company. They answer quickly and ask, ‘What is your emergency?’ Well, most of the time it is my cat that has pushed the emergency button. No problem, the medic alert is there when I need it. There is even a reminder alarm for my medications. I never forget anymore. It is great!” |
In addition to being prepared for the unexpected (see Emergency Preparation), there are some precautions to keep in mind for everyday safety.
Keep the house free of obstacles (see Accessibility at Home). Remember that weak arms make it difficult or impossible to brace for a fall (see Falling), and know that knees abruptly can give way without warning. Be available to lend an arm during walking, and encourage the use of assistive equipment.
Using a wheelchair is much safer than attempting to walk when each step is a struggle. Some people with ALS resist the wheelchair but may be willing to use it when a lot of walking is required. Keep the seat belt on whenever the person is in the wheelchair — at home, in public, in the car, etc. A slight bump, a steep ramp or uneven terrain can make someone tumble out of the chair.
The caregiver and person with ALS also should carry cell phones with important and emergency numbers programmed in. A medic alert bracelet or medallion will alert strangers to call the doctor.
Resources:
Everyday Life with ALS — Chapter 3: Home Modifications
Keys to Safety, MDA/ALS Newsmagazine, April 2006
Saliva management
See Drooling.
Immobility can cause a painful condition called frozen shoulder. Although the shoulder may move, it’s stiff and movement causes pain. Range-of-motion exercises prevent this condition and are a component in its treatment.
Shoulder pain also occurs when the weight of the arms isn’t supported, causing a constant pull on weakened muscles and the shoulder joints. Carrying heavy objects can cause the arm to be subluxed (dislocated). Ensure proper positioning by supporting arms with armrests or pillows. Caregivers also need to protect the loved one’s shoulders during transfers, by not pulling on their arms to move them.
Resources:
See Exercise; Hands; Positioning.
Everyday Life with ALS — Chapter 7: Transfers; Chapter 8: Exercise; Chapter 9: Exercise Instructions
Some skin changes have been noted in ALS, such as changes in the biochemical properties of collagen and elastin which run through the dermis, or middle layer of skin. Blood vessels in the dermis also display irregularities and protein deposits as ALS progresses. Poor nutrition and respiration can make skin more fragile.
Caregivers can take several steps to ease skin woes for loved ones with ALS.
For dry itchy skin:
- Moisturize the air with a humidifier.
- Hot water strips the skin of moisture; use lukewarm water for baths and showers. If a hot soak is a must, use bath oil.
- From the standpoint of cleanliness, it’s usually not necessary to take a full bath every day; a “parts” cleanup with a sponge usually is sufficient.
- Use mild or soap-free cleansers. Avoid deodorant soap.
- Pat skin dry with a towel; don’t rub.
- Immediately after patting dry, seal in moisture with the greasiest lotion tolerated.
- Shave with lotion instead of foam.
- Try over-the-counter itchy skin remedies, such as those containing cortisone. (See Itchy scalp.)
- In some cases of chronic itching or skin sensitivity, a physician may prescribe an anti-epileptic drug such as carbamazepine (Tegretol) or gabapentin (Neurontin), or one of the tricyclic antidepressants.
For skin infections:
Fungal infections like “jock itch” can be caused by being seated all day, which creates warm, damp pockets in underarms, groin and skin folds. To combat infections:
- Dry skin completely after washing, using a hair dryer on the cool setting.
- Buy clothing that wicks moisture away from the skin.
- Use over-the-counter antifungal sprays, powders and creams; avoid cornstarch, talcum or other nonmedicated powders.
- Herbal/natural remedies include applying apple cider vinegar or vitamin E; eating six cloves of fresh garlic or six to nine garlic capsules a day; eating yogurt with live cultures; and taking more B-complex vitamins.
Healthy-skin nutrition includes:
- Drinking eight to 10 glasses of water a day; taking a daily multivitamin-mineral supplement; eating daily servings of foods rich in linoleic and essential fatty acids such as safflower oil, nuts, avocado, seeds, soybeans, salmon, tuna, shrimp and corn oils.
- Skin damaged by pressure sores requires extra protein, zinc and vitamins A, C and K. Vitamin C also builds collagen, which is adversely affected by ALS.
Resources:
Protection and Prevention are Keys to Comfortable Skin, MDA/ALS Newsmagazine, December 2002
Helping the person with ALS sleep well also helps the caregiver sleep well. Taking extra time to ensure comfort when a loved one goes to bed can cut down on call-backs for repositioning later in the night. Some caregivers create a checklist of bedtime adjustments to make sure nothing is forgotten. Although comfort is an individual thing, common elements are pillows for stability and to prevent pressure sores, a blanket lifter to keep weight off the feet, and blankets that don’t restrict weakened movements by being too heavy or tight (see Feet).
| “[My daughter] says the Mattress Genie ‘is wonderful because it allows me to continue using my bed instead of replacing it with a hospital bed.’” |
A comfortable mattress is essential. Some people prefer “memory foam” mattresses or mattress toppers; others use air mattress toppers or invest in automatic turning or alternating pressure mattresses. Automatic hospital beds allow people who can operate a remote to reposition themselves; the height-adjustable feature protects the caregiver’s back and makes caregiving a little easier.
When sleep is difficult, over-the-counter or prescription sleep aids such as Tylenol PM, Ativan, Ambien, Lunesta or Unisom may be in order (check with your ALS doctor first). Other meds have drowsiness as a side effect, such as allergy medications like Benadryl or the herb valerian. Tolerance to these substances can develop, so they should only be used if necessary.
To deal with nighttime saliva, try elevating the head off the bed. A tricyclic antidepressant such as Elavil can both dry up secretions and cause drowsiness. Other suggestions can be found under Drooling.
Resources:
Sleep Aids: Low-Tech Strategies for Improving Sleep Comfort, MDA/ALS Newsmagazine, March 2007
One Good Turn, Quest, September/October 2006
Sleep deprivation (for caregivers)
ALS caregivers may get up numerous times a night to reposition or help their loved ones, leading to chronic sleep deprivation. Often the problem isn’t getting up, but the inability to fall back to sleep afterwards.
Researchers say chronic sleep deprivation can cause depression, fatigue, forgetfulness, lowered alertness, reduced creativity, inability to speak and write clearly, lowered resistance to disease, weight gain and increased risk of stroke, heart attack and adult-onset diabetes. Sleep-deprived people also are more likely to verbally and physically abuse their children, and are more prone to falling asleep while driving. It’s a problem that needs to be solved quickly. (See Caregiver emotions and stress.)
Strategies to get more rest while still providing nighttime care include:
- Make it quick and quiet. When getting up, don’t turn on the lights (use a low-level nightlight if necessary), don’t have a conversation or do anything mentally stimulating, and stay up the minimum amount of time necessary.
- Don't try too hard. If you can’t fall back to sleep within 10 or 15 minutes, get up and do something relaxing, then return to bed as you feel yourself getting drowsy. Performing a good all-over body stretch can add in relaxation.
- Decrease caffeine, alcohol and nicotine. Especially avoid caffeine in the afternoon, as it can contribute to sleeplessness at night. Although some people find that an alcoholic drink before bed helps them fall asleep, alcohol increases the likelihood of waking later in the night.
- Power nap. Aim for a short (15-to-30-minute) nap sometime during the “midday trough” between 1 p.m. and 3 p.m., when your body naturally wants to rest. A longer nap may leave you groggy and unable to sleep at night. If you can’t fall asleep, just rest quietly with eyes closed for a brief period.
- Go to bed. A simple way to get more sleep is to go to bed a little earlier. It sounds obvious, but tired people often stay up to watch the late show rather than turn in earlier. Record favorite late-night shows and try to go to bed and get up about the same time every day.
- Check out your sleep space. An uncomfortable mattress, snoring spouse, too much light, being too hot or too cold, pets who jump on and off the bed, and outside noises all are subtle distractions that make it hard to settle back down. Try eyeshades, putting pets elsewhere or buying a more comfortable mattress.
- Three on, three off. If possible, share nighttime caregiving duties. For optimum benefits, sleep experts recommend a three-nights-on, three-nights-off schedule, rather than switching with someone every other night.
- Talk to your doctor. Not all caregiver sleep problems are caused by stress or getting up in the night. Schedule an appointment if nothing else is working.
Resources:
Give It a Rest: Tips for Sleep-Deprived Caregivers, MDA/ALS Newsmagazine, December 2001
Isolation is a risk for those with ALS. Symptoms such as drooling, immobility, pseudobulbar affect or difficulty in communicating may make people reluctant to see friends or go out. But it’s important to keep up social activity and be part of the world and the community, and it’s possible to find ways to adapt in almost any circumstance. Socializing helps fight off depression and enables the person with ALS to make a contribution. It also can help relieve caregiver burnout when others can keep your loved one company. Continued involvement with the larger world makes ALS only a new part of life, not an end to old interests.
Sometimes friends are reluctant to stay in touch if the disease makes them uncomfortable. If friends or family members seem uncertain how to relate to the person with ALS, remind them he or she is still the same person, and encourage them to talk about things other than ALS. Your loved one will want to keep up the same interests as before — sports, politics, movies, etc. Friends can come over to watch a ball game or concert on TV as a way of simply being together.
| “My mom refused to be seen outside with her walker and as a result she gave up shopping, going to parties, and other fun things that she loved to do. I tried to talk her into using her walker to do things outside the home, but she refused. She lost a lot of good years that way.” |
Friends also can spell caregivers at times so they can take a break or get other things done. Make it clear that they aren’t being asked to provide personal or medical care, but rather simply to be there to talk or call for help if there’s an emergency. The MDA clinic or support group is a new source of friends who can share the ALS experience — for both patient and caregiver. Some develop a whole new family or community that will be deeply appreciated throughout the ALS journey.
Going to public events is feasible and enjoyable. Most public places are accessible to wheelchair users, thanks to the Americans with Disabilities Act (see Accessibility outside the home). With an adapted vehicle or public transportation, people with ALS can continue to work, go to movies, ball games, kids’ activities, church, family events and restaurants.
Resources:
For more about social relationships, see Friends in Chapter 6.
The Company of Others: Stories of Belonging, by Sandra Shields and David Campion, PLAN Institute for Caring Citizenship, 2005
Making the Moments Count: Leisure Activities for Caregiving Relationships, by Joanne Ardolf Decker, Ph.D., Johns Hopkins University Press, 1997
Yes You Can!!! Go Beyond Physical Adversity and Live Life to Its Fullest, by Janis Dietz, Ph.D., Demos Medical Publishing, 2000
Spasms
See Pain.
Stairlift
See Lifts.
In some people with ALS, fingers, hands, arms, ankles, feet or legs may appear swollen, sometimes extremely so. This is edema, the retention of fluid, and it has several causes. Often medications used to control drooling cause the body to retain fluids, leading to edema.
| “My legs are ghastly. They look fine when I am lying down, but the minute I sit up they turn hideous shades of red and purple and blue and gray. If I am not careful to prevent as much swelling as I can, they are swollen like sausages by noon and miserably uncomfortable, and absolutely painful by evening.” |
Most commonly in ALS, edema is due to being immobile. Muscle activity helps push blood through the veins to the heart. Without it, blood pools in the veins, and the pressure causes water in the blood to leak out into surrounding tissue, causing swelling.
Edema can be very uncomfortable. While the person is in bed, elevating the swollen parts above the level of the heart can reduce edema; prop the hands, knees and feet on pillows. If using an adjustable hospital bed, note that it raises the knees, not the feet. It’s necessary to put a pillow at the foot of the mattress to bring the feet up level with the knees.
When the person is out of bed, correct positioning is critical. Recliner-type chairs can contribute to swelling of feet and ankles because, with the footrest up, all the weight of the legs is on the calves, reducing circulation. Adding pillows in the gap between the chair and leg rest can help distribute the weight and improve circulation.
Whenever the person is sitting upright, allowing the legs to hang without support will cause severe swelling. Adjust the length of wheelchair footrests or put a box or cushion under the feet to reduce pressure at the thigh and back of the knee.
Swelling of the hands can be minimized by using a lap tray to support the arms. The best lap trays (easily cut from plywood or plexiglass) surround the waist and extend back at the sides to support the elbows. Elbow pads will be needed, and the hands can be raised on a pillow.
Any exercise possible also will help, whether it’s being assisted to walk a little, tightening and pushing with the calf muscles, or just range-of-motion exercises.
Wearing compression hose can help, and shoes that lace are far better than slippers at controlling edema. Keeping the legs cool also is more comfortable. Reducing salt intake sometimes is recommended, although it’s important to keep up fluid intake.
Diuretics, which increase the production of urine, often are prescribed but should be used only if other methods aren’t enough to prevent discomfort. Diuretics can counteract medications used to control drooling, can deplete fluids and require additional trips to the bathroom — all problems for people with ALS. The family and medical team need to figure out a balance between controlling edema and excess saliva.
Edema also can be a sign of congestive heart condition, cirrhosis or kidney problems, so be sure medical personnel check for these, especially if the eyes are puffy. Edema just in a leg can be a sign of a blood clot.
Dental care for a person with ALS eventually will require caregiver assistance.
As it gets harder to hold a toothbrush or hold the mouth open, the first step is an electric toothbrush, sometimes with a Waterpik. A child’s toothbrush or foam-tipped swabs such as Toothette Plus Oral Swabs with Mouth Refresh Solution can be used without water and are easier to get into a tight mouth. These are available at many pharmacies and medical supply stores. Biotene toothpaste foams less than others and is easier to swallow or spit out.
Some people with ALS bite their cheeks, lips and tongues. A strong mouthwash can help heal the sores. A variety of mouth guards, such as SleepRight night guards or plastic sports mouth guards, can be found in drugstores. A dentist can order a rubber bite block or a custom mouth guard. These guards also can hold the mouth open while teeth are brushed and be worn at night to prevent teeth grinding.
Massage may help relax a clenched jaw. Be careful not to force anything into the jaw, because the person may inadvertently bite down hard on a toothbrush or a finger.
Getting teeth professionally cleaned can be tricky when the person with ALS has excess saliva, a tight jaw, or trouble sitting up or lying back. Any serious dental work should be done as early as possible after the diagnosis to minimize these complications. In most communities there are dentists who specialize in helping people with disabilities; the regular dentist or county dental society may be able to recommend someone.
During a dental checkup or cleaning, strongly remind the hygienist to suction constantly and thoroughly to avoid choking on saliva. Sometimes caregivers take over the suctioning because they’re more familiar with signs from the patient and have to suction the mouth frequently at home.
Medicare and some private health insurance may cover dental care. A prescription or letter from the ALS specialist can help ensure the coverage, though it’s not a guarantee.
Resources:
For more on suctioning, see Chapter 3.
Back issues of MDA publications, such as Quest and the MDA/ALS Newsmagazine, can be found online or by calling your local MDA office, (800) 572-1717.
Those Teeth Are Made for Brushing: Open Wide and Say Ahhh, Quest, December 2002
Disability and Oral Care, ed. by Dr. June Nunn, FDI World Dental Press, 2000
Cold extremities — feet and hands — affect some people with ALS. If these problems persist, practical solutions such as extra socks and gloves, room heaters, massage, range-of-motion, hot water bottles or hot packs can help.
In hot temperatures, breathing can be affected. Extreme hot or cold environments can affect the functioning of ventilators (see Chapter 3).
Naturally, people with ALS prefer to take care of their own bathroom needs for as long as possible. This can be a touchy subject and is best handled step by step as needs increase — and with humor and lack of embarrassment.
- Remodel: At home the bathroom should have a door wide enough for a wheelchair to enter, and enough space for it to turn around, i.e., a 5-foot radius. Removing the door and hanging a curtain instead may be all that’s needed. Special hinges also enable wider door openings. Add grab bars around the tub and toilet.
- Stay regular: As managing the bathroom becomes more difficult, people are sometimes tempted to cut back on their liquid intake or “hold it in” as long as possible to minimize trips. This is a bad idea. Dehydration can lead to constipation (see Constipation) and swallowing problems, which are uncomfortable and unhealthy. People with ALS have bladder and bowel control for the most part — they know when they need to go — (see Urinary Urgency), and always should go when they need to.
- Raise the seat: Even for those who can transfer onto and off the toilet independently, getting up from a seated position can be a problem. Many raised toilets are available, including seats to be added to an existing toilet, raising the height by 2 to 13 inches. Get the highest one that works because your loved one will need more height as ALS progresses. A new toilet with a very high seat can be installed; there are self-contained units that don’t require drilling into plumbing. A less expensive option is a portable, adjustableheight bedside commode chair that can be placed over the toilet. Some commode/shower chairs have wheels, allowing transfers to take place in another room.
- Lift with a sling: A hygiene sling has a large cutout that makes toileting possible from the lift.
- Say hey to the bidet: Arm weakness may make it difficult to use toilet paper effectively (hand-held toilet paper extenders are available). One independence strategy is the bidet. Several models, including the Lubidet, Brondell or Toto Jasmine Washlet, can be installed in place of the seat on the existing toilet. They provide a gentle warm water wash and warm air drying. Some have remote controls that can be operated by hand or foot, or by someone outside the door. Bidets run from $400 to $800 and usually can be installed by the homeowner. Bidet wands are good for traveling or public restrooms.
- Portable urinals: Portable urinals can save several bathroom trips a day. A bottleshaped urinal is a good solution for men. Portable urinals for women have larger openings and handles. These can be used independently or with a caregiver’s help, in standing, lying or seated positions. It’s best to put down a waterproof pad first.
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Catheters: Men can use external condom catheters; some experimenting may be necessary to find the right type. Foley catheters involve inserting a tube into the urethra and connecting to a drainage bag. These indwelling catheters are used by men and women, and don’t interfere with movement or transfer. The bag has to be checked and emptied, and the insertion site needs to be cleaned daily. If the catheter becomes clogged, painful, leaky or shows signs of infection, the doctor may replace it. The drainage bag should be cleaned periodically with two parts vinegar or chlorine beach and three parts water.
A suprapubic catheter may be used when the Foley is too uncomfortable or causes continued infections. It’s inserted directly into the bladder through the abdomen wall.
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Cleansing: The time may come when your loved one with ALS is unable to transfer to the toilet at all and must perform bladder and bowel movements while in bed. Adult diapers (which also may be worn under clothes when going out) or disposable bed pads under the person in bed are the usual solution.
Thorough, gentle cleaning is important after a bowel movement to protect against bedsores. Wearing rubber gloves, turn the person onto one side and wash gently with baby wipes and a very mild soap such as Johnson’s Bedtime Bath. Dry carefully and use some Desitin or other powder. A mix of Desitin with zinc oxide is comfortable and protective. To clean sensitive areas in the shower, hang two enema bags, one containing liquid soap and warm water, and one with warm water. Use one to clean the area, then the other to rinse.
- Public restrooms: In public places, there’s usually a toilet stall which is big enough for manipulating a wheelchair. Still, your loved one may need assistance to transfer to the toilet and adjust clothing; a bidet wand may be convenient to carry. If the caregiver is the opposite sex from the person with ALS, ensure privacy by asking a bystander to stand guard or announcing “man (or woman) in the room” whenever someone enters. Or carry along a sign saying “Handicapped — opposite sex using restroom,” and attach it to the door. See “Dressing”, for other techniques that will make using a public bathroom easier.
Resources:
Splish Splash: Easier Ways to Get Clean, Quest, January-February 2008
Transfers
See Everyday Life with ALS — Chapter 7.
People with all kinds of disabilities travel the world. To get around in town, investigate local public transportation and services for those with disabilities, or look into buying/renting a van that can accommodate a wheelchair.
Airlines, hotels and tourist sites have accommodations. Definitions of “handicapped accessible” vary widely, so it’s best to call in advance and find out exactly what’s available.
Cruises are a particularly comfortable way for people with disabilities to travel.
Airlines allow transportation of wheelchairs, respirators and other equipment, but usually as luggage. Damage to power chairs is not uncommon. If you’re flying to a distant destination, you may be able to get a loaner wheelchair through the MDA loan closet in the city you’re visiting. Talk with your local MDA office about this before the trip.
Resources:
See To Boldly Go columns in back issues of MDA’s Quest for accessible destinations.
Oh the Places You Can Go, Quest, January-February 2007
Travel Tips from People on the Go, MDA/ALS Newsmagazine, April 2005
101 Accessible Vacations, by Candy B. Harrington, Demos Health, 2007
Barrier-Free Travel, 3rd ed., by Candy B. Harrington, Demos Health, 2009
There Is Room at the Inn, by Candy B. Harrington, Demos Health, 2006
Able to Travel
Society for Accessible Travel & Hospitality
Incontinence isn’t a feature of ALS because in general the smooth muscle of the bladder and bowel aren’t affected by the disease. But other muscles and nerves are involved and they may be weakened, making it harder to tighten the sphincter. At times the bladder may have a spasm, causing a powerful urge to urinate. Some medications may lead to more frequent urination.
Urinary urgency may prove temporary. Ditropan (oxybutynin) is often prescribed to relax the bladder and lessen urinary symptoms — watch for side effects including headache, dry mouth, constipation or diarrhea. Urinary urgency also can be a sign of a urinary tract infection, prostate trouble, fibroids in the uterus, or other causes, so be sure to have a thorough medical checkup.
Frequent awakening at night, followed by a need to urinate, may be a sign of respiratory problems; check with a doctor.
Excessive yawning in ALS doesn’t mean tiredness. It can occur in half or more ALS patients, especially those with the bulbaronset form. Yawning also can be a side effect of some drugs such as the antidepressant Lexapro.
Some people find sucking on a hard candy between the cheek and gums or chewing gum can stop the yawning — but beware if choking is a problem. Medications such as Effexor, Ativan or Klonopin may be prescribed. This symptom may be part of pseudobulbar affect.
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