ALS TERM PAPER - Copy.docx

**Term paper** **On** **Amyotrophic** **Lateral** **Sclerosis** **Submitted to** Mrs. Roslin Therese G Asst. Professor HFCON **Submitted by** Tintu John 2^nd^ year MSc(N) HFCON **Submitted on:** **INDEX** **SL.NO** **CONTENT** **PAGE NO:** ----------- ---------------...

**Term paper** **On** **Amyotrophic** **Lateral** **Sclerosis** **Submitted to** Mrs. Roslin Therese G Asst. Professor HFCON **Submitted by** Tintu John 2^nd^ year MSc(N) HFCON **Submitted on:** **INDEX** **SL.NO** **CONTENT** **PAGE NO:** ----------- ------------------------ -------------- 1 Anatomy and Physiology 3-13 2 Introduction 14-15 3 Definition 15 4 ALS and motor neurons 16-17 5 Types 18 6 Risk factors 18-19 7 Causes 19-21 8 Pathophysiology 21-22 9 Clinical Features 22-24 10 Stages 24-27 11 Diagnosis 27-28 12 Treatment 28-31 13 Nursing management 31-34 14 Complications 34-36 15 Research 36-38 16 Conclusion 38 17 Bibliography 38-39 **ANATOMY AND PHYSIOLOGY** **[NERVOUS SYSTEM5]** Nervous System Overview The nervous system is the most complex body system. Constantly alive with electricity, the nervous system is the body's prime communication and coordination network. It is so vast and complex that, an estimate is that all the individual nerves from one body, joined end to end, could reach around the world two and a half times. The Brain and Spinal Cord are the Central Nervous System. Nerves and Sensory Organs Make Up the Peripheral Nervous System.  Together, the central nervous system (CNS) and the peripheral nervous systems (PNS) transmit and process sensory information and coordinate bodily functions. The brain and spinal cord (the CNS) function as the control center. They receive data and feedback from the sensory organs and from nerves throughout the body, process the information, and send commands back out. Nerve pathways of the PNS carry the incoming and outgoing signals 1: The central nervous system (CNS) and peripheral nervous system\... \| Download Scientific Diagram Structure of a Nerve -------------------- A nerve is the primary structure of the peripheral nervous system and is composed of bundles of axons. **Nerve Anatomy** A nerve is an enclosed, cable-like bundle of axons (the projections of neurons) in the peripheral nervous system (PNS). A nerve provides a structured pathway that supports the electrochemical nerve impulses transmitted along each of the axons. In the central nervous system, the analogous structures are known as tracts. Neurons are sometimes referred to as nerve cells, although this term is misleading since many neurons do not occupy nerves, and nerves also include non-neuronal support cells (glial cells) that contribute to the health of enclosed neurons. **Layers** Each nerve contains many axons that are sometimes referred to as fibres. Within a nerve, each axon is surrounded by a layer of connective tissue called the endoneurium. The axons are bundled together into groups called fascicles. Each fascicle is wrapped in a layer of connective tissue called the perineurium. Finally, the entire nerve is wrapped in a layer of connective tissue called the epineurium. See the following illustrations of these structures. The endoneurium consists of an inner sleeve of material called the glycocalyx and a mesh of collagen. Nerves are bundled along with blood vessels, which provide essential nutrients and energy to the enclosed, and metabolically demanding, neurons. Within the endoneurium, individual nerve fibers are surrounded by a liquid called the endoneurial fluid. The endoneurium has properties analogous to the blood--brain barrier. It prevents certain molecules from crossing from the blood into the endoneurial fluid. In this respect, endoneurial fluid is similar to cerebrospinal fluid in the central nervous system. During nerve irritation or injury, the amount of endoneurial fluid may increase at the site of damage. This increase in fluid can be visualized using magnetic resonance neurography to diagnose nerve damage.  Peripheral nerve cross-sectional anatomy. ========================================= Myelin and nerve structure: MedlinePlus Medical Encyclopedia Image Myelin and nerve structure ### Basic Function A nerve conveys information in the form of electrochemical impulses (known as nerve impulses or action potentials) carried by the individual neurons that make up the nerve. These impulses are extremely fast, with some myelinated neurons conducting at speeds up to 120 m/s. The impulses travel from one neuron to another by crossing a synapse, and the message is converted from electrical to chemical and then back to electrical. Nerves can be categorized into two groups based on function: 1. 2. Neurologists usually diagnose disorders of the nerves by a physical examination, including the testing of reflexes, walking and other-directed movements, muscle weakness, proprioception, and the sense of touch. This initial exam can be followed with tests such as nerve conduction study, electromyography, or computed tomography. Classification of Nerves ------------------------ Nerves are primarily classified based on their direction of travel to or from the CNS, but they are also subclassified by other nerve characteristics. #### Direction of Signal Transmission Nerves are categorized into three, primary groups based on the direction of signal transmission within the nervous system. 1. 2. 3.  #### Central Nervous System Connection Nerves can be further categorized based on where they connect to the central nervous system. Spinal nerves innervate much of the body and connect through the spinal column to the spinal cord. Spinal nerves are assigned letter-number designations according to the vertebra where they connect to the spinal column. Cranial nerves innervate parts of the head and connect directly to the brain. Cranial nerves are typically assigned Roman numerals from 0 to 12. #### Diameter, Conduction Velocity, Myelination State Peripheral nerve fibers are grouped based on the diameter, signal conduction velocity, and myelination state of the axons. These classifications apply to both sensory and motor fibers. Fibers of the A group have a large diameter, high conduction velocity, and are myelinated. The A group is further subdivided into four types (A-alpha, A-beta, A-delta, and A-gamma fibers) based on the information carried by the fibers and the tissues they innervate. - - - - Fibers of the B group are myelinated with a small diameter and have a low conduction velocity. The primary role of B fibers is to transmit autonomic information. Fibers of the C group are unmyelinated, have a small diameter, and low conduction velocity. The lack of myelination in the C group is the primary cause of their slow conduction velocity. This is an image of saltatory conduction. It shows the faster propagation of an action potential in myelinated neurons than that of unmyelinated neurons. **Saltatory conduction**: Demonstrates the faster propagation of an action potential in myelinated neurons than that of unmyelinated neurons. C fiber axons are grouped together into what is known as Remak bundles. These occur when an unmyelinated Schwann cell bundles the axons close together by surrounding them. The Schwann cell keeps them from touching each other by squeezing its cytoplasm between the axons. C fibers are considered polymodal because they can often respond to combinations of thermal, mechanical, and chemical stimuli. A-delta and C fibers both contribute to the detection of diverse painful stimuli. Because of their higher conduction velocity, A-delta fibers are responsible for the sensation of a sharp, initial pain and respond to a weaker intensity of stimulus. These nerve fibers are associated with acute pain and therefore constitute the afferent portion of the reflex arc that results in pulling away from noxious stimuli.  An example is the retraction or your hand from a hot stove. Slowly conducting, unmyelinated C fibers, by contrast, carry slow, longer-lasting pain sensations. Motor neurons carry instructions from the brain along axons that stretch from the spinal cord to the muscles in the hands and feet. In diseases eg: spinal muscular atrophy, motor neuron axons become damaged and degenerate, which means signals from the brain never reach the muscles and movement becomes impaired.  Upper and lower motor neuron lesions cause very different clinical findings. - - **AMYOTROPHIC LATERAL SCLEROSIS (ALS)** **[INTRODUCTION ]** Amyotrophic lateral sclerosis (ALS) is a rare neurological disease that primarily affects the nerve cells (neurons) responsible for controlling voluntary muscle movement (those muscles we choose to move). Voluntary muscles produce movements like chewing, walking, and talking. The disease is progressive, meaning the symptoms get worse over time. Currently, there is no cure for ALS and no effective treatment to halt or reverse the progression of the disease. ALS belongs to a wider group of disorders known as motor neuron diseases, which are caused by gradual deterioration (degeneration) and death of motor neurons. Motor neurons are nerve cells that extend from the brain to the spinal cord and to muscles throughout the body. As motor neurons degenerate, they stop sending messages to the muscles and the muscles gradually weaken, start to twitch, and waste away (atrophy). Eventually, the brain loses its ability to initiate and control voluntary movements. Early symptoms of ALS usually include muscle weakness or stiffness. Gradually all voluntary muscles are affected, and individuals lose their strength and the ability to speak, eat, move, and even breathe. Most people with ALS die from respiratory failure, usually within 3 to 5 years from when the symptoms first appear. However, about 10 percent of people with ALS survive for 10 or more years. **[DEFINITION ]** Amyotrophic lateral sclerosis (ALS) is a disease of unknown cause in which there is a loss of motor neurons (nerve cells controlling muscles) in the anterior horns of the spinal cord and the motor nuclei of the lower brain stem. ALS was once commonly known as Lou Gehrig's disease, following the retirement of the famous ballplayer in the 1940s due to the disease.  How Has Stephen Hawking Lived Past 70 with ALS? - Scientific American Physicist Stephen Hawking also has ALS*.* ALS and Motor Neurons --------------------- It's a disease that affects the motor neurons. These nerve cells send messages from the [brain](https://www.webmd.com/brain/ss/slideshow-concussions-brain-injuries) to your spinal cord and then to your muscles. You have two main types: - **Upper motor neurons:** Nerve cells in the [brain](https://www.webmd.com/brain/rm-quiz-amazing-brain). - **Lower motor neurons:** Nerve cells in the spinal cord to muscle. These motor neurons control all the voluntary movements \-- the muscles in the arms, legs, and face. They tell the muscles to contract so you can walk, run, pick up your smartphone, chew and swallow food, and even breathe. ALS is one of a few motor neuron diseases. Some others include: - [primary lateral sclerosis](https://www.webmd.com/brain/primary-lateral-sclerosis-10673) (PLS) - progressive bulbar palsy (PBP) - pseudobulbar palsy What Happens in ALS ------------------- With ALS, motor neurons in the  [brain](https://www.webmd.com/brain/video/brain-training) and spinal cord break down and die. When this happens, the brain can\'t send messages to muscles anymore. Because the muscles don\'t get any signals, they become very weak. This is called atrophy. In time, the muscles no longer work and you lose control over their movement. At first, the muscles get weak or stiff. Person may have more trouble with fine movements \-- such as trying to button a shirt or turn a key. Person may stumble or fall more than usual. After a while, can\'t move your arms, legs, head, or body. Eventually, people with ALS lose control of their [diaphragm](https://www.webmd.com/sex/birth-control/diaphragm-birth-control), the muscles in the chest that help you breathe. Then they can\'t breathe on their own and will need to be on a breathing machine. The loss of breathing causes many people with ALS to die within 3 to 5 years after they\'re diagnosed. Yet some people can live more than 10 years with the disease. People with ALS can still think and learn. They have all of their senses \-- sight, smell, hearing, taste, and touch. Yet the disease can affect their memory and decision-making ability. ALS isn\'t curable. Yet scientists now know more about this disease than ever before. They are studying treatments in [clinical trials](https://www.webmd.com/a-to-z-guides/clincial-trial-guide-patients). **[TYPES]** There are two types of ALS: - **Sporadic ALS** is the most common form. It affects up to 95% of people with the disease. Sporadic means it happens sometimes without a clear cause. - **Familial ALS (FALS) **runs in families. About 5% to 10% of people with ALS have this type. FALS is caused by changes to a gene. Parents pass the faulty gene to their children. If one parent has the gene for ALS, each of their children will have a 50% chance of getting the gene and having the disease. **[RISK FACTORS]** **Risk factors for ALS include**: - - - Environmental factors, such as the following, might trigger ALS. - **Smoking.** Smoking is the only likely environmental risk factor for ALS. The risk seems to be greatest for women, particularly after menopause. - **Environmental toxin exposure.** Some evidence suggests that exposure to lead or other substances in the workplace or at home might be linked to ALS. Much study has been done, but no single agent or chemical has been consistently associated with ALS. - **Military service.** Studies indicate that people who have served in the military are at higher risk of ALS. It\'s unclear what about military service might trigger the development of ALS. It might include exposure to certain metals or chemicals, traumatic injuries, viral infections, and intense exertion. Some studies suggest that military veterans are about 1.5 to 2 times more likely to develop ALS. Although the reason for this is unclear, possible risk factors for veterans include exposure to lead, pesticides, and other environmental toxins. ALS is recognized as a service-connected disease by the U.S. Department of Veterans Affairs. **[CAUSES]** The cause of ALS is not known, and scientists do not yet know why ALS strikes some people and not others. However, scientific evidence suggests that both genetics and environment play a role in motor neuron degeneration and the development of ALS. **Genetics**\ In 1993, scientists supported by the National Institute of Neurological Disorders and Stroke ([NINDS](https://www.ninds.nih.gov/glossary?combine=NINDS)) discovered that mutations in the *SOD1*** **gene were associated with some cases of familial ALS. Since then, more than a dozen additional genetic mutations have been identified, many through NINDS-supported research. Research on certain gene mutations suggests that changes in the processing of RNA molecules may lead to ALS-related motor neuron degeneration. RNA molecules are involved with the production of molecules in the cell and with gene activity. Other gene mutations indicate there may be defects in protein recycling---a naturally occurring process in which malfunctioning proteins are broken down and used to build new working ones. Still others point to possible defects in the structure and shape of motor neurons, as well as increased susceptibility to environmental toxins. **Environmentalfactors**\ Researchers are studying the impact of environmental factors, such as exposure to toxic or infectious agents, viruses, physical trauma, diet, and behavioral and occupational factors. For example, exposure to toxins during warfare, or strenuous physical activity, are possible reasons for why some veterans and athletes may be at increased risk of developing ALS. Ongoing research may show that some factors are involved in the development or progression of the disease. Scientists are also looking at these other possible causes: - **Glutamate. **This chemical sends signals to and from the brain and nerves. It\'s a type of neurotransmitter. With ALS, glutamate builds up in the spaces around nerve cells and may damage them.\ The [medications](https://www.webmd.com/drugs/index-drugs.aspx) [riluzole](https://www.webmd.com/drugs/drug-12138-riluzole+oral.aspx) ([Rilutek](https://www.webmd.com/drugs/drug-12146-rilutek+oral.aspx)) works by lowering glutamate levels and can help slow the development of the disease.. - **[Immune system](https://www.webmd.com/cold-and-flu/immune-system-function) problems. **Your immune system protects your body from foreign invaders such as bacteria and viruses. In your brain, microglia are the main type of immune cell. They destroy germs and damaged cells.\ With ALS, microglia might also destroy healthy motor neurons. - **Mitochondria problems. **Mitochondria are the parts of your cells where energy is made. A problem with them might lead to ALS or make an existing case worse. - **Oxidative [stress](https://www.webmd.com/balance/stress-management/default.htm).** Your cells use oxygen to make energy. Some of the oxygen your body uses to make energy may form into toxic substances called free radicals, which can damage cells. The medication [edaravone](https://www.webmd.com/drugs/2/drug-173632/edaravone-intravenous/details) ([Radicava](https://www.webmd.com/drugs/2/drug-173679/radicava+intravenous/details)) is an [antioxidant](https://www.webmd.com/food-recipes/antioxidants-your-immune-system-super-foods-optimal-health) that can help control these free radicals. **[PATHOPHYSIOLOGY]** - The exact mechanism of ALS remains unclear due to the uncertainty of causation but it has been determined that it affects the upper and lower motor neurons that provide voluntary movement and muscle power. - The progressive degeneration of the motor neurons leads to inhibition of the action potentials reaching the muscle fibers to promote muscle movement. - It has hypothesized, however, that the motor neurons may be damaged by the increase in glutamate. - The cardiac and smooth muscle of the digestive system are regulated involuntary via the autonomic nervous system. Therefore, the heart and the digestive system are not affected by ALS. - Because ALS primarily involves motor neurons, sensory function is typically preserved. Few patients complain of numbness and paraesthesias. - Skin integrity is usually maintained, primarily due to the combination of preserved sensory function and continued control of bowel and bladder function. **[CLINICAL FEATURES]** The onset of ALS can be so subtle that the symptoms are overlooked but gradually these symptoms develop into more obvious weakness or atrophy. **Early symptoms** include: - - - - - - The first sign of ALS usually appears in the hand or arm and can show as difficulty with simple tasks such as buttoning a shirt, writing, or turning a key in a lock. In other cases, symptoms initially affect one leg. People experience awkwardness when walking or running, or they may trip or stumble more often. When symptoms begin in the arms or legs, it is referred to as "limb onset" ALS, and when individuals first notice speech or swallowing problems, it is termed "bulbar onset" ALS. As the disease progresses, muscle weakness and atrophy spread to other parts of the body. Individuals may develop problems with moving, swallowing (called dysphagia), speaking or forming words (dysarthria), and breathing (dyspnea). Although the sequence of emerging symptoms and the rate of disease progression can vary from person to person, eventually individuals will not be able to stand or walk, get in or out of bed on their own, or use their hands and arms. Individuals with ALS usually have difficulty swallowing and chewing food, which makes it hard to eat. They also burn calories at a faster rate than most people without ALS. Due to these factors, people with ALS tend to lose weight rapidly and can become malnourished. Because people with ALS usually can perform higher mental processes such as reasoning, remembering, understanding, and problem solving, they are aware of their progressive loss of function and may become anxious and depressed. A small percentage of individuals may experience problems with language or decision-making, and there is growing evidence that some may even develop a form of dementia over time. Individuals with ALS eventually lose the ability to breathe on their own and must depend on a ventilator. Affected individuals also face an increased risk of pneumonia during later stages of the disease. Besides muscle cramps that may cause discomfort, some individuals with ALS may develop painful neuropathy (nerve disease or damage).  --------------------------------------------------------------------- **[STAGES OF ALS]** ALS is a relentlessly progressive disorder. The rate of progression between individuals is variable and the history generally reflects gradual and progressive worsening over time until death occurs. ### Early stages #### *Muscles* - Muscles may be weak and soft, or they may be stiff, tight, and spastic. Muscle cramping and twitching (*fasciculation*) occurs, as does loss of muscle bulk (*atrophy*). - Symptoms may be limited to a single body region or mild symptoms may affect more than one region. #### *Physical effects* - The person may experience fatigue, poor balance, slurred words, a weak grip, tripping when walking, or other minor symptoms. - Sometimes this stage occurs before a diagnosis is made. ### Middle stages #### *Muscles* - Symptoms become more widespread. - Some muscles are paralyzed, while others are weakened or unaffected. Fasciculations may continue. #### *Physical effects* - Unused muscles may cause *contractures*, in which the joints become rigid, painful, and sometimes deformed. - If a fall occurs, the person may not be able to stand back up alone. - Driving is relinquished. - Weakness in swallowing muscles may cause choking and greater difficulty eating and managing saliva. - Weakness in breathing muscles can cause respiratory insufficiency, especially when lying down. - Some people experience bouts of uncontrolled and inappropriate laughing or crying (*pseudobulbar affect*). Despite how it seems, the person usually doesn't feel particularly sad or happy. ### Late stages #### *Muscles* - Most voluntary muscles are paralyzed. - The muscles that help move air in and out of the lungs are severely compromised. #### *Physical effects* - Mobility is extremely limited, and help is needed in caring for most personal needs. - Poor respiration may cause fatigue, fuzzy thinking, headaches, and susceptibility to pneumonia. (Respiratory insufficiency is a leading cause of death in ALS.) - Speech, or eating and drinking by mouth, may not be possible. ### End stage - The vast majority of deaths in ALS are the result of respiratory failure, a process that progresses slowly over months. Medications can relieve discomfort, anxiety, and fear caused by respiratory insufficiency. - Far less-common causes of death in ALS include malnutrition as a result of swallowing problems, *pulmonary embolism* (a blockage in one of the arteries of the lungs), abnormalities in the heart's electrical pacing system called *cardiac arrhythmias*, and pneumonia as the result of aspiration (when food or fluid gets into the lungs). - [Hospice care](https://www.mda.org/alsn/article/not-gloom-and-doom-demystifying-hospice) (in a facility or in the home) focuses on providing comfort and maintaining quality of life by supporting the physical, emotional, and spiritual needs of the individual with ALS and their family members. Families should contact hospice early on to see what in-home services are available even before the most advanced stage. Flowchart of ALS staging systems and their definitions (King\'s staging\... \| Download Scientific Diagram **[DIAGNOSIS]** There is no single test that provides a definitive diagnosis of ALS. It is primarily diagnosed based on a detailed history of the symptoms observed by a physician during physical examination, along with a review of the individual's full medical history and a series of tests to rule out other diseases. A neurologic examination at regular intervals can assess whether symptoms such as muscle weakness, muscle wasting, and spasticity are progressively getting worse. **Muscle and imaging tests** - - - - - - **Spinal tap (lumbar puncture).** This involves removing a sample of the spinal fluid for laboratory testing using a small needle inserted between two vertebrae in the lower back. **[TREATMENT]** There is no treatment to reverse damage to motor neurons or cure ALS. However, treatments can help control symptoms, prevent unnecessary complications, and make living with the disease easier. Supportive health care is best provided by multidisciplinary teams of professionals such as physicians; pharmacists; physical, occupational, speech, and respiratory therapists; nutritionists; social workers; clinical psychologists; and home care and hospice nurses. These teams can design an individualized treatment plan and provide special equipment aimed at keeping people as mobile, comfortable, and independent as possible. **Medications** The U.S. Food and Drug Administration (FDA) has approved drugs to treat ALS: - - Physicians can also prescribe medications to help manage symptoms of ALS, including muscle cramps, stiffness, excess saliva and phlegm, and the pseudobulbar affect (involuntary or uncontrollable episodes of crying and/or laughing, or other emotional displays). Drugs also are available to help individuals with pain, depression, sleep disturbances, and constipation. **Physical and occupational therapy** Physical therapy and special equipment can enhance an individual's independence and safety throughout the course of ALS. Gentle, low-impact aerobic exercise such as walking, swimming, and stationary bicycling can strengthen unaffected muscles and range of motion and stretching exercises can help prevent painful spasticity and shortening (contracture) of muscles. Physical therapists can recommend exercises that provide these benefits without overworking muscles. Occupational therapists can suggest devices such as ramps, braces, walkers, and wheelchairs that help individuals conserve energy and remain mobile. **Communications support** People with ALS who have difficulty speaking may benefit from working with a speech therapist, who can teach adaptive strategies to speak louder and more clearly. As ALS progresses, speech therapists can help people maintain the ability to communicate. Devices such as **computer-based speech synthesizers** use eye-tracking technology and can help people develop ways for responding to yes-or-no questions with their eyes or by other nonverbal means. Some people with ALS may choose to use **voice banking** while they are still able to speak as a process of storing their own voice for future use in computer-based speech synthesizers.   A **brain-computer interface (BCI)** is a system that allows individuals with ALS to communicate or control equipment such as a wheelchair using only brain activity. Researchers are developing more efficient, mobile, and even some auditory-based BCIs for those with severe paralysis and/or visual impairments. **Nutritional support** Nutritionists can teach individuals and caregivers how to plan and prepare small meals throughout the day that provide enough calories, fiber, and fluid and how to avoid foods that are difficult to swallow. People may begin using suction devices to remove excess fluids or saliva and prevent choking. When individuals can no longer eat, doctors may advise inserting a feeding tube, which reduces the risk of choking and pneumonia that can result from inhaling liquids into the lungs. **Breathing support** As the muscles responsible for breathing start to weaken, people may experience shortness of breath during physical activity and difficulty breathing at night or when lying down. **Noninvasive ventilation (NIV)** refers to breathing support that is usually delivered through a mask over the nose and/or mouth. Initially, NIV may only be necessary at night but may eventually be used full time. NIV improves the quality of life and prolongs survival for many people with ALS. Because the muscles that control breathing become weak, individuals with ALS may also have trouble generating a strong cough. There are several techniques to help people increase forceful coughing, including mechanical cough assistive devices. As the disease progresses, individuals may need **mechanical ventilation (respirators)** in which a machine inflates and deflates the lungs. Doctors may place a breathing tube through the mouth or may surgically create a hole at the front of the neck and insert a tube leading to the windpipe (tracheostomy). Although ventilation support can ease breathing problems and prolong survival, it does not affect the progression of ALS. **NURSING MANAGEMENT** 1. 2. 3. 4. 5. 6. - - - 7. 8. - - - ##### **Nursing Diagnosis** - - - - **End-of-life care** Patients with ALS may live up to 5 years after symptom onset. Shortly after onset, they should discuss advanced planning with their families and caregivers and begin to make decisions regarding end-of-life care. Nurses are well situated to provide holistic, individualized end-of-life care to patients with ALS and their families. Patients with ALS often retain the mental capacity to make decisions even when close to death. However, the disease can impair their communication abilities, so patients and families must plan for end-of-life care before these abilities deteriorate. Patients should be offered the option to create a living will and/or advance directives to detail their end-of-life preferences while still in the early stages of ALS. For example, they should consider whether they would want mechanical ventilation via an artificial airway such as a tracheostomy, or if they would be unhappy with this quality of life. Encourage patients to remain involved in decision-making regarding the pursuit or rejection of life-sustaining treatments. Patients may struggle emotionally with facing death and require emotional support from their family, friends, and caregivers as they cope with and process their fears of the future. Nurses and other healthcare professionals should anticipate these needs. Discovering and understanding how individuals cope allows healthcare providers to collaborate with patients to form an acceptable plan for the end-of-life phase. The coping process during any degenerative disease varies among patients. Keeping communication channels open between clinicians, the patient, and family is critical to delivering optimal patient care at the end-of-life phase of ALS. Because the presentation and progression of ALS may vary, nurses should discuss the comprehensive aspects of ALS to learn how each patient wants treatment to proceed. Patients may resent relinquishing control of various facets of their lives to their families or caregivers. Timing is vital when planning discussions about life-sustaining measures. Clinicians should remain alert to all aspects of each patient\'s ALS journey. For example, some patients prefer to die at home in the presence of family rather than in a healthcare facility. Healthcare providers should explore and facilitate this option and help coordinate it when possible. Encouraging patients to participate in the decision-making process early gives them a sense of control over their end-of-life process. Patient decisions about life-sustaining support are essential and should include a complete disclosure of their options and the corresponding risks and benefits by a healthcare provider, which will help them make the best decisions according to their preferences. Healthcare professionals must take care to communicate with the patient and/or family or caregivers in an objective way that does not pressure them to make life-sustaining decisions that may not be acceptable to them. A consultation with a palliative care specialist could improve communication and interactions for patients, families, and caregivers. Most patients with ALS lived independently before their diagnosis, and some may be resistant to advice or assistance from healthcare providers. The reality of their physical deterioration means they cannot continue living the same way, and this is a significant emotional hurdle that healthcare providers must recognize and respond to in a sensitive and patient-centered manner. The challenges associated with a loss of independence can be exacerbated if their healthcare providers are not giving clear information in a sensitive and respectful manner. Comprehensive nursing care along with holistic elements (focusing on mind, body, spirit, and emotional well-being) can help nurses positively impact patients with ALS at the end of life. Holistic care can assist patients and generate satisfaction and an overall sense of peace in daily functioning for these individuals and their families. This approach can provide comfort during disease progression, especially during the end-of-life phase. **[COMPLICATIONS]** As the disease progresses, ALS causes complications, such as: ### Breathing problems - Over time, ALS paralyzes the muscles you use to breathe. You might need a device to help you breathe at night, similar to what someone with sleep apnea might wear. For example, you may be given a bilevel positive airway pressure (BiPAP) device to help with your breathing at night. This type of device supports your breathing through a mask worn over your nose, your mouth or both. - Some people with advanced ALS choose to have a tracheostomy --- a surgically created hole at the front of the neck leading to the windpipe (trachea) --- for full-time use of a respirator that inflates and deflates their lungs. - The most common cause of death for people with ALS is respiratory failure. On average, death occurs within 3 to 5 years after symptoms begin. However, some people with ALS live 10 or more years. ### Speaking problems - Most people with ALS develop trouble speaking. This usually starts as occasional, mild slurring of words, but becomes more severe. Speech eventually becomes difficult for others to understand, and people with ALS often rely on other communication technologies to communicate. ### Eating problems - People with ALS can develop malnutrition and dehydration from damage to the muscles that control swallowing. They are also at higher risk of getting food, liquids or saliva into the lungs, which can cause pneumonia. A feeding tube can reduce these risks and ensure proper hydration and nutrition. ### Dementia - Some people with ALS have problems with memory and decision-making, and some are eventually diagnosed with a form of dementia called frontotemporal dementia. **RESEARCH** The National Institute of Neurological Disorders and Stroke ([NINDS](https://www.ninds.nih.gov/glossary?combine=NINDS)) is the primary federal funder of research on the brain and nervous system, including disorders such as ALS. NINDS is a component of the National Institutes of Health ([NIH](https://www.ninds.nih.gov/glossary?combine=NIH)), the leading supporter of biomedical research in the world.  The goals of [NINDS](https://www.ninds.nih.gov/glossary?combine=NINDS)'s ALS research are to understand the cellular mechanisms involved in the development and progression of the disease, investigate the influence of genetics and other potential risk factors, identify biomarkers, and develop new treatments. **Cellular defects**. Ongoing studies seek to understand the mechanisms that selectively trigger motor neurons to degenerate in ALS, which may lead to effective approaches to halt this process. Research using cellular culture systems and animal models suggests that motor neuron death is caused by a variety of cellular defects, including those involved in protein recycling and gene regulation, as well as structural impairments of motor neurons. Increasing evidence also suggests that glial support cells and inflammation cells of the nervous system may play an important role in ALS. **Stem cells**. Scientists are turning skin cells of people with ALS into stem cells that are capable of becoming any cell type, including motor neurons and other cells which may be involved in the disease. NINDS-funded scientists are using stem cells to grow human spinal cord sections on tissue chips to help better understand the function of neurons involved in ALS. **Genetics and epigenetics. **Clinical research studies supported by [NINDS](https://www.ninds.nih.gov/glossary?combine=NINDS) are looking into how ALS symptoms change over time in people with *C9ORF72* mutations. Other studies are working to identify additional genes that may cause or put a person at risk for either familial or sporadic ALS. A large-scale collaborative research effort supported by [NINDS](https://www.ninds.nih.gov/glossary?combine=NINDS), other [NIH](https://www.ninds.nih.gov/glossary?combine=NIH) institutes, and several public and private organizations is analyzing genetic data from thousands of individuals with ALS to discover new genes involved in the disease. By using novel gene editing tools, researchers are now able to rapidly identify new genes in the human genome involved in ALS and other neurodegenerative diseases. Additionally, researchers are looking at the potential role of epigenetics in ALS development. Epigenetic changes can switch genes on and off, which can greatly impact both health and disease. Although this research is exploratory, scientists hope that understanding epigenetics can offer new information about how ALS develops. **Biomarkers**. [NINDS](https://www.ninds.nih.gov/glossary?combine=NINDS) supports research on the development of biomarkers---biological measures that help identify the presence or rate of progression of a disease or the effectiveness of a therapeutic intervention. Biomarkers can be molecules derived from a bodily fluid (blood or cerebrospinal fluid), an image of the brain or spinal cord, or a measure of the ability of a nerve or muscle to process electrical signals. **New treatment options**. This work involves tests of drug-like compounds, gene therapy approaches, antibodies, and cell-based therapies in a range of disease models. Additionally, a number of exploratory treatments are being tested in people with ALS. **[CONCLUSION]** ALS is a progressive degenerative disease. Although more research needed, it is evident that occupational therapy intervention is beneficial in managing the symptoms of ALS. The main goal of OT intervention for a client with ALS is to enhance the quality of his/her life. **BIBLIOGRAPHY** - Nerves \| Boundless Anatomy and Physiology \[Internet\]. Courses.lumenlearning.com. 2022 Available from: - Melinosky C. What is ALS? What Are the Types and Causes? \[Internet\]. WebMD. 2021 \[cited 14 November 2021\]. Available from: - Mda.org. Available from: - McIntosh J, Han S. Amyotrophic lateral sclerosis (ALS): Causes, symptoms, and treatment \[Internet\]. Medicalnewstoday.com. 2022 \[cited 13 March 2022\]. Available from: - Amyotrophic lateral sclerosis (ALS) - Symptoms and causes \[Internet\]. Mayo Clinic. 2022 \[c Available from:. - Rnpedia. Amyotrophic lateral sclerosis (ALS) Nursing Care Plan & Management \[Internet\]. RNpedia. 2018 \[cited 2022Nov28\]. 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