Amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig’s disease, is a disease that involves the nervous system and primarily manifests as loss of control of the muscles.
This progressive disease usually starts with slurred speech, weak extremities, and/or muscle twitching. ALS can severely affect the quality of life of patients, especially when it begins to affect the muscular control for eating, speaking, moving, or even breathing.
The cause is unknown, although some medicine experts believe that it is a genetic disorder.
Currently there is no cure for ALS, so treatment is focused on the control of its symptoms in order to maintain the optimal quality of life for patients.
The average life span of an ALS patient in 3-5 years after the symptoms begins, although some patients live for 10 years or more.
Signs and Symptoms of ALS
- Muscle twitching, an/or cramping, usually found in the shoulders, arms, and tongue
- Slurred speech
- Trouble swallowing
- Muscular weakness of the extremities
- Recurrent falls
- Progressive difficulty doing normal daily activities
- Mood, cognitive, and behavioral changes
Causes and Risk Factors of ALS
The root cause of ALS is still unknown. It involves the gradual deterioration of the neurons that control the voluntary muscles. Eventually, these nerve cells die, causing severe loss of muscle control as the messages from these damaged neurons are unable to reach the muscles.
One of the biggest risk factors for ALS is heredity, as 5 to 10% of the people who have ALS have inherited it. The risk also increased with age, particularly above 40 years old. Genetic variations might also put a person at higher risk for developing ALS. Smoking, lead exposure, and military service seemed to have some effect in the development of ALS.
Complications of ALS
- Respiratory Failure. ALS eventually causes paralysis of the muscles that are used for breathing. Assistive breathing devices such as bilevel positive airway pressure (BiPAP) or continuous positive airway pressure (CPAP) may be given to enable the patient to breathe as normal while sleeping. Advanced stages of ALS may require a tracheostomy to breathe better. Respiratory failure is the most common cause of death for ALS patients.
- Malnutrition and dehydration. Difficulty of swallowing can affect the nourishment and hydration of an ALS patient. They are also at higher risk for aspiration pneumonia because of swallowing problems. Feeding tube devices may be needed to provide nutrition and hydration to an advanced ALS patient.
- Dementia. Memory problems and decision-making difficulties may be present in patients with ALS. This can eventually progress to frontotemporal dementia.
- Trouble speaking. Slurred speech is one of the symptoms of ALS. This begins as a mild and occasional trouble speaking, but gradually becomes more apparent and frequent. Communication devices are helpful to understand ALS patients better.
Diagnosis of ALS
- Electromyogram (EMG) – to compare for the electrical activity of muscles at rest versus during muscular contraction by inserting a needle electrode into the skin
- Nerve conduction study – to measure the ability of the nerves in sending impulses to the muscles of the extremities and other parts of the body
- Blood and urine tests – to rule out other possible causes of the signs and symptoms
- MRI – to visualize the brain and the spinal cord
- Lumbar puncture also called a spinal tap – to analyze a sample of the spinal fluids
- Muscle biopsy – to rule out any muscular disease that may have similar symptoms of ALS
Treatment of ALS
- Medications. Riluzole (Rilutek) is an FDA-approved oral medication that can increase the life expectancy of an ALS patient by 3 to 6 months. The main side effects of this drug involve liver function and gastrointestinal conditions. The other drug is Edravone (Radicava), which helps the ALS patient slow down the decline in daily functioning. The patient takes this medication on a daily basis for two weeks and lasts for one month. Other medications can be prescribed to relieve the symptoms of ALS, such as constipation, depression, sleeping problems, and muscle cramps and pain.
- Therapies. Patients with ALS will benefit from respiratory therapy to manage difficulty of breathing, physical therapy for optimal mobility, speech therapy to have improved communication, and occupational therapy to teach them how to manage their activities of daily living. Nutritional, social, and psychological support are also extremely important in the holistic care of ALS.
Nursing Diagnosis for Amyotrophic Lateral Sclerosis
ALS Nursing Care Plan 1
Nursing Diagnosis: Deficient Knowledge related to new diagnosis of ALS as evidenced by patient’s verbalization of “I want to know more about my new diagnosis and care”
Desired Outcome: At the end of the health teaching session, the patient will be able to demonstrate sufficient knowledge of ALS and its management.
Amyotrophic Lateral Sclerosis Nursing Interventions | Rationales |
Assess the patient’s readiness to learn, misconceptions, and blocks to learning (e.g. denial of diagnosis or poor lifestyle habits)To address the patient’s cognition and mental status towards the new diagnosis of ALS and to help the patient overcome blocks to learning. | |
Explain what ALS is, and how it affects the body. Avoid using medical jargons and explain in layman’s terms. | To provide information on ALS and its pathophysiology in the simplest way possible. |
Inform the patient the details about the prescribed medications (e.g. drug class, use, benefits, side effects, and risks) to promote sodium and water resorption, to reduce potassium levels, and to manage symptoms of ALS, and explain how to properly self-administer each of them. Ask the patient to repeat or demonstrate the self-administration details to you. | To inform the patient of each prescribed drug and to ensure that the patient fully understands the purpose, possible side effects, adverse events, and self-administration details. The two known ALS medications are Riluzole and Evradone, while the physician may prescribe several supportive medications for symptom relief. |
Use open-ended questions to explore the patient’s lifestyle choices and behaviors that can help in the management of ALS. | To assist the patient managing nutrition, diet, and exercise as related to the care and management of ALS. |
ALS Nursing Care Plan 2
Nursing Diagnosis: Imbalanced Nutrition: Less than Body Requirements related to decrease food intake secondary to ALS, as evidenced by weight loss, poor muscle tone, swallowing difficulty, and lack of appetite
Desired Outcome: The patient will be able to achieve a weight within his/her normal BMI range, demonstrating healthy eating patterns and choices.
Amyotrophic Lateral Sclerosis Nursing Interventions | Rationale |
Create a daily weight chart and a food and fluid chart. Discuss with the patient the short term and long-term goals of weight gain. | To effectively monitory the patient’s daily nutritional intake and progress in weight goals. |
Help the patient to select appropriate dietary choices that are easy to swallow. | ALS patients often require nutritional support because the nerve cells that control the muscles for swallowing are progressively getting damaged. Foods that are easy to swallow are recommended for ALS patients. |
Prepare the patient for feeding tube insertion as prescribed. | To provide nutritional support if swallowing is too difficult for the patient. |
Refer the patient to the dietitian and the speech and language therapist. | Dietitian: To provide a more specialized care for the patient in terms of nutrition and diet. Speech and Language Therapist (SALT): To accurately assess the swallowing and communication difficulties of the ALS patient. |
ALS Nursing Care Plan 3
Nursing Diagnosis: Impaired Mobility related to muscle wasting secondary to Amyotrophic Lateral Sclerosis (ALS) as evidenced by the inability to move freely within the physical surroundings, involving bed mobility, transfers, and walking.
Desired Outcomes:
- The patient will engage in a physical activity independently or within the disease’s limitations.
- The patient will exhibit mobility-improving measures.
- The patient will show how to use adaptive devices to improve mobility.
Amyotrophic Lateral Sclerosis Nursing Interventions | Rationale |
Identify the present activity levels and physical features. Use a 0–4 scale to assess the range of functional impairment. | Provides information for developing a rehabilitation care plan. Motor problems are less likely to resolve than sensory issues. |
Evaluate how safe the surroundings are. Put bed rails up, the bed in the bottom position, and essential items nearby. | These precautions ensure a safe and secure setting and may decrease the likelihood of falls. Blockages in the area, such as throw rugs, toys for children, and pets, might further restrict and hinder one’s capacity to ambulate safely. |
Examine the emotional experience of the incapacity or limitation. | Individuals’ tolerance for temporary or long-term disabilities can differ significantly. Each individual has a unique view of what constitutes an acceptable standard of living. |
Recognize the necessity for home care (e.g., physical therapy, speech pathology, nutritionist, respiratory therapy, doctors, etc.). | Providing adequate guidance or support for the client can promote safe and effective development activities. |
6. Enable as much independence as possible while assisting based on the degree of impairment. | Involvement in self-care helps alleviate the distress associated with the loss of autonomy. |
Exercise all extremities using a passive or active assisted range of motion or ROM exercises. | Exercise improves blood circulation, prevents stiffness, and helps balance muscular strength and endurance. It also prevents contracture deformity, which can accumulate quickly and hinder prosthetic usage. |
7. Have the following mobility aids and devices as indicated: shower chair and elevated toilet seat with arm supports. | These types of equipment can substitute for functional impairment and increase activity levels. Utilizing such devices is to ensure safety, improves mobility, prevents accidents, and saves energy. |
8. Provide positive encouragement during activities. | Fear of falling may cause patients to be afraid To move or begin new activities. Positive feedback improves the patient’s chances of recovery and self-esteem. |
9. Allow the patient to rest in between activities. Think about energy-saving methods. | Rest times are necessary for energy efficiency. The patient must recognize and accept her or her limitations. |
10. Administer medications as necessary. | Antispasmodic medications can assist with muscle spasms or spasticity inhibiting mobility, while analgesics can relieve pain, limiting movement. |
11. Move and reposition the patient every two hours or as needed. | Adjustments in a position provide pressure relief and blood flow to all body tissue. |
12. Maintain functional alignment of extremities with one or more of the following: cushions, sandbags, wedges, or prefabricated Splints. | Maintaining functional alignment prevents foot drop and excessive plantar flexion or stiffness. Maintain the dorsiflexed position of the feet. |
13. Promote coughing and deep breathing exercises. If necessary, use suction. Use an incentive spirometer. | Coughing and breathing help to keep secretions under control. Incentive spirometry promotes lung capacity. |
14. Make dietary recommendations to meet the client’s energy and metabolic needs. | Maintaining a sufficient level of energy requires proper nourishment. To provide the client with energy resources, they must consume an adequate amount of these nutrients in a balanced diet. |
15. Implement a bowel program as necessary, including enough water intake, exercise, stool softeners, and laxatives. | Take note of the client’s bowel activity levels. A sedentary lifestyle worsens constipation. Use a variety of interventions to support regular elimination. |
16. Provide a wide range of activities. Take note of any emotional or behavioral responses to immobility. | Forced immobility can aggravate uneasiness and frustration. Diversional activity promotes coping with restrictions and assists in refocusing attention. |
17. Make objectives with the patient or loved ones for participation in activities, exercise, and posture changes. | To increase the anticipation of growth or advancement and provides some feeling of control or freedom. |
18. Teach the patient or household how to keep the home secure and free of hazards. | A safe atmosphere will help to reduce the risk of falls. Home improvements can assist the patient in retaining the optimal level of functional independence and lessen activity-related exhaustion. |
19. Give the patient an update on the status of their progressive activities. | Setting small, achievable goals promotes self-confidence and minimizes frustration. |
ALS Nursing Care Plan 4
Nursing Diagnosis: Risk for Aspiration related to impaired swallowing secondary to Amyotrophic Lateral Sclerosis (ALS).
Desired Outcomes:
- The client will not experience aspiration, as evidenced by clear breath sounds, unlabored respiration, and oxygen saturation within normal ranges.
- The client will show appropriate aspiration prevention practices.
- The client will discuss possible risk factors for aspiration.
Amyotrophic Lateral Sclerosis Nursing Interventions | Rationale |
Evaluate the client’s level of consciousness. | Unconscious clients are at higher risk of aspiration. Patients with disabilities may be incapable of removing secretions by themselves. |
Evaluate the client’s gag reflex. | Examine teeth and the capacity to seal the lips, control tongue movement, facial symmetry, and cough. The nurse can first evaluate the patient’s speech and any problem in speaking that may indicate subsequent issues. The nurse can assess the gag reflex by touching the back of the patient’s throat with a tongue blade or cotton swab. As a positive response, the patient may cough or begin swallowing. If not, do not provide any details orally and request further evaluation. |
Assess swallowing abilities by looking at the following: Coughing, choking, throat clearing, gurgling, or having a “wet” voice while swallowing. Food residue in the mouth after eating.Food or liquid regurgitation through the nares | There’s a greater risk of aspiration when swallowing is impaired. There is still a need for reliable and simple screening for aspiration risk. |
Check for the presence of nausea or vomiting. | Patients who experience nausea or vomiting run a high risk of aspiration, particularly if their consciousness level is impaired. Antiemetics prevents the aspiration of regurgitated stomach contents. |
In patients with tracheostomies, check for food particles in their tracheal secretions. | Some clients with advanced ALS undergo a tracheostomy using a respirator to inflate and deflate their lungs continuously. Food should never be allowed to pass the tracheobronchial passageways. It represents aspirated materials. |
Evaluate respiratory status by auscultating breath sounds. | ALS gradually paralyzes the breathing muscles. With aspiration pneumonia, it detects abnormal lung sounds such as crackles or rhonchi. Any alteration in respiratory status, such as increased rate, effort, or a decrease in SaO2 level, requires immediate attention. |
When feeding high-risk patients, keep a suction machine on hand. If aspiration occurs, suction quickly. | Clients with ALS are at risk for aspiration. If aspiration occurs, the need for immediate suctioning and additional life-saving measures like intubation is important. |
Raise the head of the bed after feeding. | The head of the bed should be elevated for 30 minutes to an hour following self-feeding, assisting with meals, administering medications, or tube feedings. |
Position the clients on their sides if there’s a decrease in their level of consciousness. | This placement (rescue positioning) reduces the risk of aspiration by encouraging secretions to drain out of the mouth rather than down the pharynx, where they could cause aspiration. |
Provide foods that are easy for the patient to swallow. If a speech pathologist or a dietitian recommends it, use thickening agents. | Thickened semisolid foods, such as pudding and hot cereal, are the easiest to swallow and are less likely to be aspirated. Patients with dysphagia have the most difficulty with liquids and thin foods (e.g., creamed soups). |
Use other feeding methods. | Remind the client to chew and swallow the food. Give a small amount of serving if the client requires feeding assistance. Allow time for rest before feedings to reduce the client’s trouble swallowing. Do not allow the client to talk or be distracted during chewing or swallowing. |
Observe any new onset abdominal distention or increasing abdominal rigidity. | Abdominal distension or rigidity may be related to paralytic or mechanical blockage and an increased risk of vomiting and aspiration. |
Seek advice from speech therapy. Impeded swallowing requires additional patient testing. | A speech-language pathologist (SLP) can perform swallowing tests with various foods and liquids. Additionally, they can guide the patient in swallowing-reduction strategies such as the “chin-tuck” procedure. |
Include tablets in soft foods, whole or crushed (e.g., custard). | Mixing tablets with food lowers the risk of aspiration. However, before breaking the pill, consult a pharmacist first. |
Perform oral care before and after meals. | Mouth hygiene before meals lowers bacterial levels in the oral cavity. Oral hygiene after eating removes the remaining particles. Any leftover food in the mouth is a potential risk for aspiration. |
ALS Nursing Care Plan 5
Nursing Diagnosis: Ineffective Breathing Pattern related to impaired breathing muscles secondary to Amyotrophic Lateral Sclerosis (ALS) as evidenced by difficulty breathing, tachypnea at 30cpm, nasal flaring, and poor oxygen saturation at 92%
Desired Outcomes:
- The patient will maintain an efficient breathing pattern, as shown by relaxed breathing at a standard rate and depth and the absence of dyspnea.
- The patient’s arterial blood gas or ABG levels will improve from 92% to 96% and continue within specified limits.
- The patient will show good breathing patterns and maximum lung capacity.
Amyotrophic Lateral Sclerosis Nursing Interventions | Rationale |
Check and record the client’s respiration rate and rhythm every 4 hours. | Adults breathe at an average pace of 10 to 20 breaths per minute. When there is a change in breathing patterns, it is essential to intervene to recognize early indicators of respiratory system damage. |
Check the client’s breath sounds and other vital signs. | Check the client’s breath sounds and other vital signs. Closely monitor alterations in lung sounds, respiratory rate and depth, and oxygen saturation for deterioration or improvement. |
Keep an eye out for any signs of anxiety or a change in mental state. | As oxygen in the brain diminishes, the patient may get confused or lose consciousness. Breathing difficulties may cause panic and may trigger hyperventilation. Observe for any changes in behavior or illogical responses. |
Evaluate the function of the accessory muscle. As lung capacity declines, the work of breathing increases significantly. | As lung capacity declines, the work of breathing increases significantly. |
Check for diaphragmatic muscle fatigue or tightness (paradoxical motion). | The inward vs. outward movement of the belly during inspiration is a sign of respiratory muscle strain and weakening. |
Adjust the patient’s position. | Patients unable to move may slump in bed, preventing normal lung expansion. Elevate the HOB and maintain the patient in the Semi-or Fowler’s High-position Fowler’s as tolerated to improve oxygenation. |
Instruct the patient to breathe with their lips pursed. | Pursed-lip breathing is a technique for controlling breathing. Inhale through the nose, then exhale slowly through pursed lips, allowing a more prolonged expiration. According to research, this may help to expand the airways and avoid CO2 trapping. |
Promote the use of an incentive spirometer. | Using incentive spirometers encourages deep, slow breathing and lung expansion. Incentive Spirometers can help prevent respiratory diseases such as pneumonia. |
Maintain a cool, calm, and comfortable environment. | To alleviate anxiousness, the nurse can employ relaxing approaches such as a calm voice and soothing music. A fan blowing on the patient helps lessen the sensation of dyspnea. Overheating can worsen breathlessness. Therefore, the usual choice is a cold and comfortable room. |
Encourage the client to conserve energy. | Instruct the client to complete the most strenuous or essential activities, such as bathing, when energy levels peak. Rest as necessary and pause between movements to avoid exhaustion. |
Encourage the client to stop smoking. | Educate the client on the connection between smoking and respiratory function. Assist the patient in developing a plan and goals for quitting smoking. |
Suction secretions or provide expectorants as directed. | Expectorants can help people who can cough efficiently break up mucus so they can cough it up. If the patient cannot cough independently, regular secretion suctioning helps avoid aspiration or poor oxygenation. Anticholinergic medicines can also cause saliva and secretions to dry up. |
Examine the presence of sputum for quantity, color, and consistency. | These could indicate a cause for the alteration in respiration. |
Monitor for changes in the level of consciousness. | Inadequate oxygenation to the brain can cause restlessness, disorientation, and irritation. |
Other Nursing Diagnoses for Amyotrophic Lateral Sclerosis :
Nursing References
Ackley, B. J., Ladwig, G. B., Makic, M. B., Martinez-Kratz, M. R., & Zanotti, M. (2020). Nursing diagnoses handbook: An evidence-based guide to planning care. St. Louis, MO: Elsevier. Buy on Amazon
Gulanick, M., & Myers, J. L. (2022). Nursing care plans: Diagnoses, interventions, & outcomes. St. Louis, MO: Elsevier. Buy on Amazon
Ignatavicius, D. D., Workman, M. L., Rebar, C. R., & Heimgartner, N. M. (2020). Medical-surgical nursing: Concepts for interprofessional collaborative care. St. Louis, MO: Elsevier. Buy on Amazon
Silvestri, L. A. (2020). Saunders comprehensive review for the NCLEX-RN examination. St. Louis, MO: Elsevier. Buy on Amazon
Disclaimer:
Please follow your facilities guidelines and policies and procedures. The medical information on this site is provided as an information resource only and is not to be used or relied on for any diagnostic or treatment purposes. This information is not intended to be nursing education and should not be used as a substitute for professional diagnosis and treatment.