Medical interventions and technology have vastly improved the quality of life for people with ALS by assisting with breathing, nutrition, mobility and communication. Proper management of symptoms, and proactive use of medical interventions and equipment can make a positive difference in day-to-day living, and potentially may lengthen survival.
Don't hesitate to discuss any medical or mental concern with your ALS doctor. "Beating" ALS means doing everything possible to cope with symptoms as they occur, if not before.
ALS Care Guidelines
In October 2009, the American Academy of Neurology (AAN) released revised physician guidelines for medical management of ALS. To read them in summary and in full, see the ALS Care Guidelines. The guidelines include information about drug, nutritional and respiratory therapies, along with multidisciplinary care, symptom management and cognitive/behavioral impairment.
The diaphragm is an arched muscle located just beneath the lungs, which moves up and down and allows air to come in and move out. The intercostals are muscles between the ribs that contract and relax and also assist with air movement. As the diaphragm and intercostal muscles weaken due to ALS, the act of breathing, which is entirely automatic for most people, becomes conscious and energy-consuming.
To preserve quality of life — and to prolong life itself in later stages of ALS — it will become necessary to introduce preventive measures. Such symptoms as the inability to cough, shortening of spoken sentences, daytime headaches and sleepiness, exhaustion and weight loss may indicate that breathing problems have advanced to the point where respiratory support will be valuable.
A pulmonologist and respiratory therapist typically are involved in managing the care of individuals with ALS. These professionals are usually available in or near each MDA clinic or MDA/ALS center.
Shortly after an ALS diagnosis is made, many specialists recommend a breathing test called a forced vital capacity (FVC) and other pulmonary function tests (PFTs). These tests give the clinic team baseline measures against which later tests of breathing can be compared.
The physician may recommend noninvasive ventilation to compensate for weakened muscles by assisting the movement of air in and out of the lungs. Generally, supplemental oxygen is not prescribed for individuals with ALS unless there are other medical conditions that require it. It may even do harm, so it should be used with caution, if at all.
Noninvasive ventilation comes in many forms, but usually consists of two basic elements: an “interface,” such as a mask or nose inserts, and air delivered under pressure by a small, portable machine. Usually, there’s one pressure for inhalation and another pressure for exhalation. This type of machine is often called a BiPAP (a registered trademark of Philips Respironics) for bilevel positive airway pressure.
The device does not necessarily require around the clock use. Pressures, masks and other aspects of the device can be changed by the medical team as needed. Ideally, the person with ALS should try several interface options (full-face masks, nasal pillows, etc.) and practice breathing through them to see which are the most comfortable.
The most permanent type of ventilation is the positive-pressure ventilator with a surgically created tracheostomy or trach. A ventilator is attached by a breathing hose to a tracheostomy tube, which delivers air through the neck into the trachea (windpipe) on a timed cycle.
Tracheostomy surgery (to create the opening into the trachea) is usually followed by several days or weeks of rehabilitation, during which caregivers learn how to clean and maintain the tracheostomy tube, change supplies and perform suctioning of mucus.
Many people on total ventilatory support can continue working, traveling, socializing and enjoying life. Today’s vents are small, portable, relatively quiet and can be carried on a wheelchair. For those still able to generate speech, a speaking valve often can be added to the inflatable cuff at the end of the tracheostomy tube, allowing air to travel to the vocal cords and enable speech.
In addition, a tracheostomy can provide a great feeling of safety. Permanent vents have alarms to alert caregivers to congestion or a disconnected tube. And invasive ventilation can return some energy as it relieves the exhaustion of poor sleep, prolonged coughing and labored breathing.
In 2011, a different type of breathing assistance device called a diaphragm pacing system was approved for “humanitarian use” in ALS by the U.S. Food and Drug Administration (FDA). The NeuRx Diaphragm Pacing System (DPS), developed by Synapse Biomedical, rhythmically stimulates breathing through an external pacer unit attached to electrodes that are surgically implanted in the diaphragm. To qualify for the DPS, a patient must have adequate preservation of the diaphragm muscle and phrenic nerves (the nerves that stimulate the diaphragm). The DPS does not slow or stop the progression of ALS, but it may delay the need for a tracheostomy, and may improve sleep and quality of life. Further studies are being conducted of its effectiveness in ALS. “Humanitarian use” approval by the FDA reflects the agency's opinion that the device does not pose "an unreasonable or significant risk of illness or injury, and that the probable benefit to health outweighs the risk of injury or illness from its use." A Synapse video is available.
Another aspect of respiratory care that’s important in ALS is assisted coughing. As the coughing muscles weaken, it becomes harder to clear mucus from the airways and life-threatening mucus plugs can form. An assisted coughing device, which pushes air into the airways through a mask and then quickly reverses air flow, can help clear the airways and prevent infection. Doctors may recommend other methods to assist with coughing and clearing secretions from the airways.
Cognitive and behavior changes
Although ALS is considered a disease of the motor (movement) system, cognitive (thinking) and behavioral changes can also occur in this disease. Some changes may be in response to the devastating nature of the disease, while others appear to be neurological in origin and to be part of the disease process itself.
Many patients do experience alterations in thinking or behavior although in most instances, symptoms are minimal and may be more distressing to relatives and caregivers than to the affected person. Although the medical profession often refers to these changes as frontotemporal dementia (FTD), which connotes memory loss (as in Alzheimer's disease), memory is generally well-preserved in ALS. Instead, the person with ALS may become unduly angry or irritable, or may be less considerate of others than one might expect him or her to be, or may exhibit poor judgment, apathy, ritualistic habits, new dietary preferences or other uncharacteristic behavior.
Some people with FTD symptoms lose insight into their actions and may not realize (even when it's pointed out to them) that they aren't thinking as clearly as before or that their behavior may be problematic.
Another phenomenon that sometimes occurs in ALS is known as pseudobulbar affect (PBA), in which the person experiences uncontrollable bouts of laughing or crying out of proportion to the situation. In 2010, the drug Nuedexta was approved to treat this symptom.
It's important for caregivers to know about possible cognitive symptoms in ALS, so that they can recognize the signs and not think that their loved one is simply "being difficult." Families can deal with the cognitive and behavioral changes associated with FTD by making modifications to the environment of the person with ALS that improve safety and organization; carefully describing symptoms to health care providers; helping their loved one make important decisions as early in the disease process as possible; and seeking support from others affected by ALS through online or in-person groups.
Speaking ability is lost when ALS affects the muscles of the mouth and throat that control speech and the muscles that help move air over the vocal cords.
For this reason, speech therapists and speech-language pathologists are vital members of the ALS care team. A speech therapist can teach the person with ALS special techniques for conserving energy and making speech more understandable. In some cases, a dentist can make a device called a palatal lift that can help compensate for certain types of weakness in the roof of the mouth.
In the early stages of ALS, speech therapists may suggest voice banking, which involves recording a number of common phrases that later can be programmed into a computer or communication device, enabling individuals with ALS to continue speaking in their own voice when they communicate via assistive technology.
Many therapists recommend being proactive about exploring communication options well before assistive technology is needed. A speech therapist can demonstrate how to use an alternative augmentative communication device, or AAC device.
Because learning to use such a device at later stages of ALS may be harder, individuals are encouraged to discuss options with their MDA clinic team and speech therapist early on, in order to proactively plan ways to stay in the conversation.
Cramps, twitches and spasticity
Muscle cramps, twitches and spasticity (tightness) are common in ALS.
The October 2009 American Academy of Neurology ALS Care Guidelines found insufficient data to support or refute any specific interventions for the treatment of these problems in ALS. However, the authors noted that in diseases such as multiple sclerosis and cerebral palsy, effective treatments for these problems include benzodiazepam, baclofen, dantrolene and tizanidine. These medications require a doctor's prescription.
Rest, repositioning, heat (such as from a microwaveable pad or warm bath) or gentle massage often are helpful in relieving the discomfort associated with muscle cramps, twitching and tightness.
Studies have estimated the prevalence of depression in people with ALS to be anywhere from 0 to 44 percent. However, when carefully structured interviews were used, the rate was consistently 9 to 11 percent.
According to the 2009 ALS Care Guidelines, there have been no controlled trials of treatment for depression in ALS, although there is consensus among experts that it should be treated. Many clinicians have found that antidepressants or anti-anxiety medication can have a positive effect.
Drooling, also known as sialorrhea, is common in ALS, because of weakness of the muscles of the mouth and throat. The October 2009 American Academy of Neurology ALS Care Guidelines say that the oral medicationamitriptyline may be helpful in drying up saliva (thereby reducing drooling), as may injections of botulinum toxin type A into the salivary glands.
Radiation of the salivary glands to reduce saliva production also has been used for treatment of this condition when other measures fail. However, the guidelines note that side effects included redness, sore throat and nausea.
Eating, drinking and nutrition
Recent evidence shows that maintaining one's weight may increase survival with ALS. Severe weight loss means muscle loss. Adequate fluid intake also is essential, for hydration, keeping saliva and mucus thin, and avoiding constipation.
Swallowing difficulties (dysphagia) are a prime cause of weight loss. As the muscles involved in chewing, moving food toward the back of the mouth, and swallowing weaken in ALS, eating and drinking become less pleasurable and more hazardous and time-consuming. Mouth and throat weakness can lead to choking and aspiration (inhaling food or liquid into the lungs), which can cause respiratory infection.
Other challenges include arm/hand weakness that limits self-feeding, decreased appetite, constipation, shortness of breath and nausea after eating, or fatigue due to the long and tiring process of eating.
Speech-language pathologists or therapists are also specialists in swallowing, since these functions involve the same muscles as speech. Some therapists specialize more in speech and others more in swallowing. Early solutions involve changing the consistency of food and liquids — usually thickening the liquids and avoiding large pieces of food — as well as changing swallowing techniques. (For more tips, see Nutrition Issues in the MDA ALS Caregiver's Guide and Meals for Easy Swallowing.)
If swallowing becomes difficult or unsafe and/or if eating takes a great deal of time and energy, a feeding tube may be recommended, often called a gastrostomy tube, g tube or PEG (percutaneous endoscopic gastrostomy) tube. The term "gastrostomy" refers to making a small incision in the stomach. It’s usually done percutaneously, which means “through the skin,” with the help of an endoscope, a medical instrument.
An October 2009 American Academy of Neurology report (see ALS Care Guidelines) found no ALS-specific indications for the timing of feeding tube placement. However, the report said the risk of placement increased when respiratory function declined below 50 percent of normal, and suggested that those with swallowing difficulties will be exposed to less risk if a feeding tube is placed when respiratory function is above 50 percent of normal.
If it’s still possible to swallow some foods or liquids safely, the person with ALS can continue to eat and drink by mouth even after placement of a feeding tube. But eating by mouth no longer has to be relied on as the only way to get adequate nutrition. This can be a relief to those who can’t take in enough calories by mouth because they get too tired or are afraid of choking or aspirating food.
Although getting a feeding tube initially feels like "ALS is winning," those who get them find they regain time and energy, and reduce the strain on their caregivers.
Occupational therapists specialize in helping people find and use tools to cope with progressive weakness in hand muscles. Special grips for writing and eating utensils, devices that fit over keys to make them easier to turn, zipper pulls and button hooks can help make weakening hands more functional, and help preserve independence in activities of daily living. Please consult with the occupational therapist on your MDA clinic team for more information.
The AAN guidelines found that visiting a multidisciplinary ALS clinic (one with many types of health professionals, such as an MDA/ALS center) can help people with ALS get the best possible care. The evidence showed that people with ALS who get care at a multidisciplinary clinic live longer, and may have a better quality of life than those who don't.
The drug riluzole (Rilutek) has a modest effect on slowing disease progression and prolonging survival. Four clinical trials have shown the drug prolongs survival by two to three months. However, five other studies using large databases spanning five to 10 years have suggested that riluzole might be associated with a prolonged survival of six months or even longer.
The drug appears to be safe, but it is expensive, and can cause fatigue, nausea and liver damage. The manufacturer suggests that people taking riluzole should avoid excessive intake of alcohol to minimize the risk of liver damage.
The damage ALS causes to the nervous system can lead to uncomfortable symptoms such as cramps and muscle twitching. Also, the immobility that results from ALS may put stress on muscles and joints, causing what is known as secondary pain.
Physicians may prescribe medications to treat these symptoms, as well as other symptoms often associated with ALS — including drooling, anxiety and depression, constipation (the result of reduced mobility and/or weakened abdominal muscles), sleep difficulties and pain associated with prolonged immobility.
Vitamin supplements may be recommended if swallowing difficulties result in reduced intake of nutrients. Reasonable doses of antioxidant supplements, such as vitamins C and E, are thought to have beneficial effects on the nervous system and overall health. However, high doses can do more harm than good.
In 2010, a medication called Nuedexta was approved specifically for an aspect of ALS called pseudobulbar affect.
Talk with your MDA clinic physician about medications and/or vitamin supplements that may be beneficial, and discuss dosage levels of supplements.
Fatigue, falling and increased difficulty walking often are experienced as ALS progresses. Avoiding falls is of paramount importance and can prevent trauma that could accelerate ALS disease progression. In addition to using mobility equipment to avoid falls, be sure to move area rugs, install grab bars and eliminate clutter wherever possible. Carry a cell phone when walking alone, to call for help if necessary.
In the early stages of ALS, mobility equipment such as a cane, walker or a supportive brace (orthosis) provide help in getting around. Weakness of muscles controlling the foot make it hard to move the foot or toe at will, causing foot drop that can lead to trips and falls. A lightweight ankle-foot orthosis, or AFO, keeps the foot from dropping and adds steadiness when walking. It can be slipped into different styles of shoes and concealed by wearing a sock over the brace, but it’s best worn with a supportive tie-up shoe.
When walking becomes difficult, riding in a manual wheelchair for long distances can conserve energy for short-distance walking, and also help prevent injury.
In later stages of the disease, a power wheelchair is usually the preferred means of mobility. When the time comes to use a power wheelchair full time, many with ALS find that they recover a large degree of independence.
Power chairs can be driven by a variety of means besides by hand, including by eyegaze and by "sip and puff" breath control. A “tilt-in-space” option on a wheelchair allows the seat to be positioned at a variety of angles, relieving pressure and helping prevent skin breakdown. Other valuable power chair options include elevating seats, chairs that turn into standers, motorized leg rests, custom seating and more.
Physical therapists, occupational therapists and equipment specialists have specialized knowledge about maintaining mobility and using equipment, and should be consulted prior to buying. Remember that insurance often will pay for only one mobility device during a set period of time, so it's important to consider upcoming needs before buying. If possible, it may be wise to borrow interim equipment, such as a mobility scooter or manual wheelchair, perhaps from your local MDA equipment program, and save insurance for big-ticket items like a power wheelchair.
Weakness in neck muscles also is associated with ALS. This results in difficulties in controlling or holding up the head, which leads to excessive fatigue and discomfort, not to mention frustration. Cervical collars may be recommended for support.
Very little research has been done on the subject of exercise and its role in ALS. In fact, it isn’t known whether exercises are beneficial for increasing muscle strength for people with ALS.
However, it’s widely accepted among physicians and therapists that specific kinds of exercise help prevent the development of painful contractures (the permanent tightening of muscles) and can decrease the spasticity(intermittent or constant muscle tightness or spasms) that’s common in ALS.
Practicing the healthiest type of exercise for each stage of ALS will help maintain comfort and mobility. For some people, a moderate amount of daily walking in the early stages of ALS may be all that’s advisable. As the disease advances, it's very important to do daily range-of-motion and stretching exercises, either independently or with the help of a caregiver. Consult a physical therapist for the best exercise regime.
For more information, see the following sections in Chapter 2 of the MDA ALS Caregiver's Guide:
- Exercises for people with ALS
- Range-of-motion (ROM) exercises
Planning early for the inevitable changes that occur over the course of ALS goes a long way toward maintaining function and independence for as long as possible and maintaining the highest possible quality of life. Such plans include addressing how home accessibility may be affected as the disease progresses. There are many ways to make adaptations or modifications that promote independence and safety in the home. A home visit by an occupational therapist to assess the environment is very helpful.
For more information, see your MDA clinic team.