The role of the neurologist in the MDA/ALS Clinic is to monitor clinical function, initiate treatment programs as needed, and provide overall support to the patient and family.
Amyotrophic lateral sclerosis is one of the most challenging and frustrating disorders of mankind. For the patient, it is a daily battle that demands inner strength and tremendous courage. For the family, it is a test of caring and patience. For the physician, it is a challenge to make a difference in the absence of a definitive cure.
Over the last decade, we, the neurologist, have been privileged to participate in the care of many individuals with ALS. The experience has been made all the more meaningful because these patients themselves are extremely giving. A deep well of courage fuels their daily actions. Their inner strength, their dignity, and their courage have been a source of inspiration to us all. They have urged us, pleaded with us, cajoled us to find a cure, or even to find something which would slow their disease, not necessarily for themselves, as they are want to say, but for others in the future. The cure is not at hand, but there are increasing number of basic and clinical research studies in ALS and two drugs have for the first time shown an affect on ALS. The fact that the Muscular Dystrophy Association has expanded its efforts in ALS research and clinical care and has established MDA ALS Centers typifies the excitement in ALS investigations. More importantly, it defines a commitment to solve the devastating problems of ALS, and provide help not for the next generation of patients, but for the present generation.
The neurologist has the unique privilege of guiding the ALS patient through the daily challenges and frustrations. The goal is to provide meaningful assistance until the basic research laboratory can fulfill the promises of a disease-free future.
ALS Clinical Nurse Consultant
For PALS who face unresolved problems with phlegm, I recommend contacting your physician and requesting an order for a suction pump and a visiting nurse to educate and demonstrate safe and effective airway secretion clearance. Because training on suction techniques is a skilled nursing procedure, expenses for home visits by a registered nurse are covered by Medicare, Medicaid and most health insurance companies. However, request ONLY an RN with respiratory care experience to come to your home.
Also, request two types of suction catheters /tubes if you have feelings of phlegm stuck in the throat. While physicians may order only Yankauer suction tubes (often referred as "Tonsil Tips), those tubes cannot reach secretions / phlegm effectively down in the throat where phlegm may block the airway. Therefore, a French suction catheter (such as a size 14) is always good to have on hand. This is a long narrow, soft and flexible plastic tube. If this is ordered, learn how to use it safely and effectively to suction the throat if needed, under the training and supervision of a skilled RN from a home care agency.
Although the Cough Assist machine may be effective for selected individuals, not everyone may have insurance coverage or adapt to its use, particularly those with advanced bulbar impairment. However, PALS who do use the device will usually also need a suction pump with appropriate suction catheters / tubes to clear phlegm / secretions from the throat and mouth. A portable suction pump provides a means for quick secretion clearance, and thus, helps to serve as a safety precaution.
However, all suction machines need to be checked regularly to be sure they are working properly and have adequate "suck power." Be sure to always keep portable units charged and ready to use. Some portable units have a tendency to lose their power after a period of time. Therefore, ask your respiratory equipment supplier about your unit and how to check and see if the pressure settings are adequate.
In the past twenty years, I have consulted and followed more than several thousand people with ALS, including hundreds of those with phlegm or excessive secretions in the airway. Having extensive "hands-on" experience in treating this problem, I want to encourage PALS and CALS not to give up! The key to success in clearing secretions is lots of practice.....to try.try again!
I look forward to hearing your victory reports. You may email me at
Pamela A. Cazzolli, RN
ALS Support Network
North Canton, Ohio
Good nutrition is important to everyone, but is of paramount concern for the patient with amyotrophic lateral sclerosis. A proper diet should provide adequate nutrients (proteins, fats, carbohydrates, vitamins, and minerals). With ALS, eating enough of the right foods may become difficult when the ability to chew and swallow becomes impaired.
ALS may cause weakness of the muscles involved in chewing and swallowing and affect jaw movement, tongue mobility, lip closure, or the swallowing reflex. When this occurs, the patient may have increased drooling, inability to form a food bolus, and difficulty moving food posteriorly in the mouth. This could result in coughing, choking episodes, or loss of liquids through the nose. The main concern is to provide adequate nutrients while preventing aspiration of food or liquids into the lungs which could lead to infection and pneumonia.
If swallowing problems occur, modifying the consistency and texture of foods and liquids may be required to decrease the risk of aspiration. According to the patient's swallowing abilities, one of five different diets might be recommended. When changes in consistency of foods become necessary, it is useful to think in terms of familiar foods: Steak consistency diet (no restrictions), pot roast consistency diet (soft, cooked foods and avoidance of foods that contain particles, such as nuts, popcorn, cornbread, and crackers), metalloid consistency diet (finely chopped or ground foods plus thickened liquids), pudding consistency diet (strained, pureed, and blended foods plus thickened liquids), and cream consistency diet (liquid formulas).
In some patients with ALS, even diet modifications are not adequate to maintain nutrition. When choking spells occur with each meal and it becomes unsafe to continue to take nourishment by mouth, alternative feeding methods are recommended.
The role of the dietitian is to devise an individual program to provide adequate nutrients in the context of the patient's swallowing ability.
The respiratory system consists of a ventilatory phase (air sacs and bronchial tubes), a circulatory phase where the exchange of gases occurs, and a mechanical phase determined by the muscles of respiration and by the compliance (stiffness) of the lungs. Amyotrophic lateral sclerosis may directly or indirectly affect all phases of the respiratory system. If there is dysfunction of the mechanical phase of respiration, an ineffective cough may result. This will lead to an accumulation of secretions in the bronchial tubes. With increasing airway secretions and/or inhalation of particles from the mouth and throat into the lungs, an overgrowth of bacteria may develop which leads to infection in the lungs (pneumonia). Not only does pneumonia aggravate the ventilatory phase of respiration, it also can cause changes in the circulatory phase of respiration resulting in reductions in blood oxygen levels and a buildup of carbon dioxide.
The therapy of respiratory dysfunction in ALS is primarily aimed at general supportive measures. Considerations may include ventilatory maneuvers (voluntary or positive pressure) to prevent atelectasis, a cessation of smoking program, instruction in maintaining nutrition and prevention of aspiration, flu vaccinations, and medications to decrease the work of breathing. If oral or pharyngeal secretions become excessive, drugs that decrease saliva production or suction devices to remove secretions may be beneficial. If low blood oxygen levels are documented, supplemental oxygen is given. All infections should be promptly treated with antibiotics.
As a member of the MDA/ALS team, the role of the pulmonary specialist is to carefully monitor the respiratory status and initiate prompt intervention when needed. Careful monitoring of the respiratory system, guidance in ways to lessen complications, and immediate treatment of changes in the respiratory status are critical to the over-all care of patients with amyotrophic lateral sclerosis.
The role of the respiratory therapist is to obtain measurements of respiratory function and instruct the ALS patient and family in the use of therapeutic measures and equipment prescribed by the pulmonary specialist.
Pulmonary function is monitored at each clinic visit. Baseline pulmonary function studies are obtained, including forced vital capacity (FVC) and flow-volume loop or curve. The vital capacity (total amount of air that can be moved in or out of the lung) is the most frequent measurement monitored. This can easily be accomplished by exhaling into a spirometer. Some physicians believe that the maximum expiratory pressure (MEP) is the most reliable assessment of respiratory muscle weakness, but is more difficult to perform in the outpatient setting. Also the sensitivity of this test frequently leads to false-positive findings. We find the FVC is easier to perform and is a meaningful indicator of changing respiratory status in the ALS patient.
Evidence of respiratory involvement might include shortness of breath with or without exertion, increased lethargy, poor cough, difficulty lying flat, and headaches. The respiratory therapist assists in the instruction of therapeutic measures ordered by the pulmonary specialist such as incentive spirometry, assistive coughing and breathing exercises, suction, intermittent positive pressure breathing, and postural drainage.
The importance of careful monitoring of respiratory function and proper instruction in therapeutic measures is essential in the overall care of patients with amyotrophic lateral sclerosis.
To communicate we convey our thoughts by speaking, writing, or gesturing. Communication deficits may result when ALS impairs the muscles involved in producing speech and voice and/or movement of the arms and hands. The role of the speech/language pathologist is to assess the patient's communication skills, implement strategies to enhance communication, and provide patient/family education.
If there is muscle weakness in the lips, tongue, or palate, the patient may not be able to move their mouth precisely or fast enough which results in slurred speech. This is called dysarthria, dys meaning disorder and arthria referring to speech production. Instruction in the use of compensatory strategies (such as slowing the rate of speech, separating the syllables, and over-enunciating the speech sounds) can be helpful.
If the muscles involved in breath support for speech and voice production are affected, the patient may complain that they cannot project their voice in a noisy room, their voice sounds hoarse, or just quits after a long period of talking. This is called dysphonia, dys referring to disorder and phonia to voice production. Strategies for dysphonia include trying to speak louder by taking in more air and using shorter phrases. An amplification device may also be recommended to help project the voice and conserve energy.
Sometimes, even with maximal effort and the use of compensatory strategies, ALS patients may continue to have difficulty with slurred speech and impaired voice production. Under these circumstances, an alternative system to supplement or support speech is required. There are many different augmentative communication devices available, such as magic writing slates, alphabet boards, picture boards, eye scanning boards, electronic devices with voice output, and even high-tech computerized devices. Careful examination of the features, benefits, and liabilities of each device is necessary, and an individualized program must be developed to assure effective communication.
Myths to avoid include :
1) one device is perfect for everyone
2) if it is expensive, then it is better
3) a simple alphabet board is not as effective as a high-tech computer
4) a device is the perfect replacement for speech
When ALS affects communication, the role of the speech/pathologist is to assess the speech and communication skills, provide compensatory strategies, and assure that ALS patients can express their feelings, thoughts, and needs in diverse physical and social environments.
The role of physical therapy in ALS begins before there is any significant loss of strength or function, and continues throughout the person's lifetime.
Physical therapy involves:
1. Maintaining the person's normal joint flexibility, or range of motion
2. Maintaining muscle strength as long as possible
3. Maintaining function as long as possible
4. Decreasing pain
The therapist achieves these goals by evaluating the individual on a regular basis and, based on the findings, instructing the patient and/or caregiver in appropriate exercises, procuring adaptive equipment, and assessing ADL (activities of daily living) needs.
When any joint of the body is not moved through its normal motion for any appreciable length of time, this connective tissue becomes less elastic and malleable. It can quickly (sometimes within days) tighten, become fibrotic, and restrict motion. If the joint is then forcibly moved towards its normal range (past the point which is now not allowed), the patient experiences immediate sharp pain, and an inflammation response is produced by the body.
A vicious cycle now begins. The patient does not wish to move the joint, as this causes pain, but increased immobility leads to more restriction and more pain. Because the shoulder joint is the most common joint to be involved, this causes real problems with activities of daily living. Just the attempt by the patient or a caregiver to lift the arm for bathing, or the attempt to pull on or off a shirt or jacket, can cause excruciating pain. Eventually, just sitting upright with the arm unsupported will be intolerable.
To attempt to avoid this scenario, it is necessary to begin daily active, active assisted, or passive (the limb is moved by someone other than the patient) range of motion exercises before the patient has lost any mobility. At the first signs of weakness of the shoulder musculature, the patient and caregiver should be instructed in flexibility (range of motion) exercises.
The patient should attempt to complete these independently for as long as possible, by lying on his/her back and using a cane or dowel to allow the opposite arm to supply the strength. Eventually, if the patient can no longer lift the arm, the caregiver should still gently move the shoulder joint through its available range. It is important that the patient and family are instructed by a therapist in the correct method to perform the exercises, as real damage can be done if the exercises are done incorrectly. In some cases, a caregiver may be instructed in gentle joint mobilization. This is an exercise technique which takes some training to learn, but if performed correctly, can help prevent adhesive capsulitis.
If, in spite of all efforts, if adhesive capsulitis is suspected, intense physical therapy should begin at once. The combination of deep heat or ice, anti-inflammatory and pain medications, and gentle mobilization techniques may still restore normal flexibility and eliminate pain.
The patient with ALS will benefit from physical therapy intervention at all stages of the disease. It is imperative, however, that each patient be evaluated by a physical therapist regularly, and that appropriate therapy be initiated before serious complications begin.
The role of the occupational therapist is to provide the ALS patient with options, resources, and information for maintaining independence in activities of daily living. The occupational therapist assesses the patient's functional abilities and activities of daily living (ADL). These include range of motion, muscle strength, daily activity levels, mobility, and performance in ADL such as dressing, feeding, hygiene, and in the work environment. Intervention consists of instruction in motion economy, pacing techniques, methods to decrease resistance to muscle groups, and body mechanics for use in the home and workplace.
Motion economy is the elimination of unnecessary steps in an activity. The patient can break down the components and motions involved in a task, change the sequence of steps in the task, modify the task, and intersperse heavy with lightweight work.
ALS patients are encouraged to pace themselves, alternating activity with rest periods. It is best not to work at a constant rate to decrease the effect of local muscle fatigue. Decreasing the amount of resistance placed on muscle groups also helps to prevent overwork fatigue. Using a lightweight thermal coffee cup instead of stoneware and a paperback book rather than a hardback book are examples of ways to decrease resistance.
Adaptive devices can assist weakened muscles, reduce fatigue, promote safety, and enhance life quality by helping to maintain maximum independence. There are a multitude of equipment choices (such as rocker knives, card holders, button hooks, handwriting aids, book holders, zipper pulls, key holders, reachers, grab bars). Muscle weakness can make certain self-care, household, and occupational tasks more difficult. This weakness, combined with general fatigue, may require an assistive device to help maintain independence. The goals of occupational therapy must coincide with the goals of the ALS patient and their perception of what independence means to them.