- Clinic Coordinator
- Clinical Research Coordinator
- Quantitative Disease Progression Study
- Respiratory Therapist
- Speech Pathologist
- Social Worker
- Physical Therapist
- Occupational Therapist
- MDA Representative
- ALS Association Representative
The role of the neurologist in the 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 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 effect on ALS. The fact that the Muscular Dystrophy Association has expanded its efforts in ALS research and clinical care and has established ALS Clinic 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.
The diagnosis of amyotrophic lateral sclerosis comes as a heart-wrenching shock to the patient and the family. Too often, the patient initially encounters the negative attitude from his or her physician that "there is nothing that can be done" and is left terrified by the prospect of his or her mortality and abandoned by the medical profession. Patients may lose their most important asset--the courage and willingness to fight the disease. However, there is much that both the patient and the medical profession can do. The ALS Clinic is one way of bringing help and support to ALS patients and their families.
Our ALS Clinic brings together many different health professionals with expertise in caring for patients with amyotrophic lateral sclerosis. Nurse/Clinic Coordinator is an important member of this team and provides assistance to the patient and their family in learning to live with ALS.
A nurse/clinic coordinator is responsible for the details in running the clinic and is the main contact between the different ALS team members and the patients and their families. The nurse/clinic coordinator in the clinic also provides a multitude of services, including monitoring of vital signs and weight, maintaining the medical records for each clinic visit, assessing general health problems and providing practical solutions, and testing functional changes in the patient's clinical status.
The ALS Team is conducting cutting-edge ALS research in our state-of-the-art facilities. The clinical research coordinators are responsible for conducting clinical trials under the auspices of world-renowned ALS expert, Dr. Stanley Appel and his team of physician-scientists.
The research coordinator assures that all studies follow FDA rules, are conducted ethically, are scientifically sound and puts the patient’s rights, safety and well-being above all else. The office of Neuromuscular Clinical Research is comprised of a Research Manager, Research Nurse, Research Coordinator or Assistant and a Data Manager. The research team will present you with information on current and upcoming clinical trials. Should you be interested, you can discuss this with your family and doctors. These coordinators will assist the physicians in determining if you are a good candidate and meet the requirements for enrolling in a specific trial. They are responsible for scheduling research office visits, testing, and procedures. All aspects of the clinical trial will be monitored such as study medication, test results, exams and how you respond to the trial. The coordinators work very closely with the doctors, team members, boards that approved the study (FDA, IRB) and sponsors (biotech or pharmaceutical companies). Because of the frequent monitoring and visits, research participants receive a high level of medical care. Most research studies are at no cost to the patient or their insurance.
In this study, ALS patients are seen approximately every four months and quantitative evaluations of disease progress are performed at each visit using the Appel ALS Rating Scale (AALS). Standardized information is compiled and entered in a computerized relational database that defines the clinical characteristics of this group of patients.
The course of ALS can be variable, with onset of first symptom to death ranging from less than 1 year to more than 10 years. Individual patients can have vastly different rates of disease progression. However, the rate of change of the AALS total score is a very consistent aspect of the individual's disease in 80-90% of all ALS patients. By following the AALS total score of each patient at regular intervals, his/her disease symptom progression can be monitored and survival time estimated. Information from this study will serve to identify the unique features of individual presentations and permit comparisons with other groups of ALS patients. These data can further provide a historical control group to be used in drug studies in the treatment of ALS. The final goal of this study is to maintain a high level of medical care tailored to the needs of individual patients.
What is the Appel ALS rating scale (AALS)?
The AALS was developed to provide a reliable, quantitative estimate of the functional impairment and symptom progression of patients with ALS. It includes subjectively and objectively quantified assessments of bulbar function (speech and swallowing), respiratory function, muscle strength in arms and legs, upper extremity function, and lower extremity function. The AALS total score is the sum of all five assessments and covers a broad range of disability. One attractive feature of this rating scale is that it provides a single quantitative index of clinical status that documents the variable manifestation and progression of ALS. Data from the individual tests are recorded separately and can be analyzed independently. Haverkamp et al examined 831 patients with classical, sporadic ALS, and the rate of change in the AALS total score was shown to be a significant predictor of survival for these patients.
Who is eligible to participate?
All patients attending the ALS Clinic in the Stanley H. Appel Department of Neurology, Houston Methodist Neurological Institute are graded every two to three months.
What is involved in the study?
The diagnosis of ALS will need to be confirmed at the Houston Methodist Neurological Institute. The medical history and specific data will be obtained through detailed interviews of both the patient and family, and appropriate laboratory tests, an electromyography, and a muscle biopsy will be performed. Participants will then attend the ALS Clinic at regular intervals to obtain a range of treatments, counseling, and examinations. Clinic personnel with special expertise in caring for patients with ALS include neurologists, nurses, neuropsychologists, social workers, dietitians, speech pathologists, a pulmonary specialist, respiratory, physical and occupational therapists, and representatives from the MDA and ALSA. Quantitative assessments of disease progression, including swallowing and speech ability, muscle strength, forced vital capacity, leg function, and finger and arm use, will be performed. Each clinic visit lasts about four to six hours.
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, but it also can cause changes in the circulatory phase of respiration resulting in reductions in blood oxygen levels and a build-up 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 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 overall 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. Maximum expiratory pressure (MEP) and Maximum inspiratory pressure (MIP) are also reliable assessments of respiratory muscle weakness. Each of these measurements assists the pulmonary team in monitoring the overall pulmonary health of our patients.
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.
Each time ALS patients come to our Clinic, they have the opportunity to participate in counseling educational with one of our neuropsychologist. We begin by establishing a supportive relationship and conveying our availability by phone, in person, and offering referral to a counseling agency in their home communities if needed. Using a psycho-educative approach, we assess coping strategies, signs of depression, maladaptive means of coping, and the nature of the family dynamics. We also assess communication style, quality of relationships, employment history, financial concerns, and level of knowledge and/or experience with ALS. Implicitly understood in working with ALS patients is that ALS changes everything. We make every effort to meet people where they are in the adaptation process. We try not to change them, but to assist them in finding internal resources to help cope with ALS. One of the most effective coping strategies for the patient with ALS is open communication. This includes the ability to articulate needs and wants, the willingness to share feelings with others, and the openness to looking at new ways of communicating with loved ones and health professionals.
The support of family, friends, employers, and the health profession, as well as religious beliefs, have been shown to help patients in coping. Persons seem to do better emotionally if they have close family ties and the family and friends are able to be good listeners and to help physically as needed. It also helps when the patient and family cope as a group, when they "pull" together for help, and have learned they can be intimate and distant at the same time.
Certain personality attributes also appear to assist persons at times of crisis. These attributes include a sense of humor, courage, adaptability/flexibility, an optimistic attitude about life, and previous exposure to adversity. As a member of the ALS team, we marvel at the optimistic and giving spirit of ALS patients. The medical profession can at times be very serious and tend to hide behind their professional demeanor, but our ALS patients teach us to lighten up. They are an inspiration to all.
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, 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 about augmentative communication devices include: one device is perfect for everyone, if it is expensive, then it is better, a simple alphabet board is not as effective as a high-tech computer, 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.
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), meatloaf 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.
In coping with a diagnosis of amyotrophic lateral sclerosis, each individual’s situation is different. Some of the same problems may be encountered, but the resolution will be unique for each person. Often individuals face changing life roles; this upset in the balance can be overwhelming. Change may not always be altered, but one’s reaction to change can. The role of the social worker in the ALS Clinic is to assess the impact of physical, emotional, or financial changes in the person with ALS and how they and the family are coping. Current and future needs are addressed, offering more time to individuals who may be in crisis. We may offer referral to a counseling agency in their home communities. We assess communication style, relationship and role changes, and address any personal or community resources that may be available to meet those needs.
It is important to establish what resources are already in place and help explore and explain benefit eligibility (social security, commercial insurance). Using a psycho-educational approach and strength focused, we discuss coping strategies and use of wellness model in terms of exploring and affirming purpose, goals, adaptability and optimistic attitude about life. Because everyone’s life experience is different, the social worker provides an open line of communication about choices with their family members and with the treatment team. Providing information and assistance in identifying options is a powerful way for the social worker to assist the person with ALS and their families maintain a good quality of life.
The goals of the physical therapist are in three main areas: exercise, equipment, and safety. For each patient, we seek to promote the highest level of possible function, promote safety in all aspects of life, and provide instruction for exercise and equipment.
Exercise guidelines are in accord with the patient's energy level and the extent of exercise must be reduced if the patient becomes too fatigued. Aerobic exercises, such as water exercise, stationary biking, and walking on a flat terrain, are recommended for endurance and conditioning if the patient is able to safely perform these activities. Instructions for stretching exercises, either self-stretching or assisted stretching by a family member or caretaker, will help maintain flexibility and reduce cramping. Range of motion should be performed and include all the major joints of the body. Muscle weakness leads to decreased joint mobility which can, in turn, impair mobility and activities of daily living. Patients are instructed to perform range of motion in different positions and to use the mechanical advantage that some positions provide. For example, some patients may not be able to raise their arms over their head while sitting, but they can do so in a supine position. Limitation of range of motion and pain in shoulder flexion, abduction, and external rotation, may indicate adhesive capsulitis which occurs in some patients with ALS. Therapy consists of joint mobilization to restore joint alignment and to free adhesions and instruction in appropriate exercise to maintain range of motion. Some cases of adhesive capsulitis require cortisone injections to assist with the healing process. Our team includes an orthopedist who specializes in the treatment of ALS related shoulder conditions and consults with our physical therapists to determine the best treatment approach.
Proper equipment is the key to independent living. Recommendations are made based on each patient's specific needs. There are a multitude of devices and equipment available to help in maintaining mobility and independence. These may include an ankle-foot-orthosis, a wheelchair, or aids for transfers and safety in the home environment.
Safety issues include prevention of fatigue and changes that may need to be made in the work or home environment. Issues to be addressed include architectural barriers, potential safety hazards, and changes that need to be made in the daily routine or the environment for energy conservation. Examples that might be recommended include shower chairs, grab bars, non-slip surfaces, removal of scatter rugs, and moving items to a lower shelf for easier access. Instruction in proper utilization of body mechanics is provided to both the patient and the family.
It is our goal to help the patient adjust to the many ways in which their lifestyle may be changed by ALS and to assure maximum independence and safety. Our simple philosophy is to encourage each patient to do as much as they can for as long as they can, and to rest when they are tired.
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. At each clinic visit, 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. The OT also assesses and provides upper extremity and neckbracing.
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 when needed the OT will complete power w/c setting assess. The goals of occupational therapy must coincide with the goals of the ALS patient and their perception of what independence means to them.
The ALS Clinic is sponsored by the Muscular Dystrophy Association (MDA). The MDA representative helps the patient in understanding MDA's services program which is dedicated to helping patients and families cope with the monumental challenges posed by amyotrophic lateral sclerosis. Through the clinic, the MDA offers follow-up examinations; physical, occupational, and respiratory therapy consultations; information and referrals; flu shots; practical advice; information on the latest research and clinical trials; and educational materials for patients and families.
The MDA-supported ALS Clinic provides an opportunity for networking with other families and sharing solutions to some of the problems that arise as a result of the disease. In addition, through its local chapters, MDA sponsors support/interaction groups designed to help patients, caregivers, friends, and families cope with a diagnosis of amyotrophic lateral sclerosis. Support groups address topics related to living with ALS, such as transportation, communication techniques, legal matters, personal care, nutrition, physical and occupational therapy, respiratory care, expenses, medical equipment, adjustments in lifestyle, or emotions experienced by all members of the family.
The ALS Association is the only national not-for-profit organization completely dedicated to fighting ALS. We are committed to providing ALS patients and their caregivers, medical professionals, and families the most comprehensive, up-to-date information on the disease alongside top-quality medical care at our ALS Association Certified Treatment Centers and Clinics. Some of our care services include community support groups & counseling, transportation assistance, an Equipment Loan Bank, where patients can borrow assistive equipment on a long-term basis; assistance with finding and obtaining community or government resources and multidisciplinary medical care under one roof.