Introduction
Amyotrophic Lateral Sclerosis (ALS), also known as Lou Gehrig’s disease, is a progressive neurodegenerative disease that affects motor neurons in the brain and spinal cord. This debilitating condition leads to muscle weakness, paralysis, and eventual loss of function. Effective nursing care is crucial for managing the complex needs of individuals with ALS, focusing on maintaining quality of life and addressing various physical, nutritional, respiratory, communication, and psychological challenges. A cornerstone of this care is the accurate identification and management of relevant nursing diagnoses. This article provides a detailed overview of common ALS nursing diagnoses and corresponding interventions, offering a comprehensive guide for healthcare professionals and caregivers.
Physical Care and Associated Nursing Diagnoses
Muscle Weakness and Skin Integrity
Assessment: A thorough initial and ongoing assessment is vital. This includes evaluating motor strength to determine the extent of muscle weakness, noting the presence of spasticity or flaccidity, and assessing for contractures that may develop due to immobility. Daily skin assessments, particularly over bony prominences and areas susceptible to pressure, are essential to prevent skin breakdown.
Common Nursing Diagnoses:
- Potential for Injury related to Impaired Physical Mobility: Muscle weakness inherent in ALS significantly increases the risk of falls and injuries. This diagnosis highlights the patient’s vulnerability due to decreased muscle strength and coordination.
- Knowledge Deficit regarding Prevention of Injury while Promoting Mobility and Self-Care: Patients and caregivers may lack understanding of strategies to maintain safety and prevent injury as mobility declines. This diagnosis addresses the educational needs to promote safe movement and self-care practices.
- Alteration in Comfort: Muscle weakness, spasticity, and immobility contribute to discomfort and pain. This diagnosis acknowledges the patient’s experience of physical discomfort and the need for pain management strategies.
Management:
- Promotion of Activity and Exercise: Encourage patients to maintain their daily routines and activities to the extent possible. Maintaining activity levels helps preserve muscle function and overall well-being for as long as feasible.
- Range-of-Motion (ROM) Exercises: Implement a regular ROM exercise program to prevent contractures and joint pain. Begin with Active ROM exercises, where the patient moves their joints independently, progressing to passive ROM exercises, where a caregiver assists with movement, as weakness progresses. Consulting with a physical therapist is crucial to develop a tailored exercise program.
- Referral to Therapy: When extremity weakness starts to impede mobility, safety, or independence in Activities of Daily Living (ADLs), prompt referral to physical and occupational therapists is necessary. These specialists can provide assistive devices, adaptive strategies, and tailored exercises.
- Promotion of Proper Positioning: Preventing pressure ulcers (decubitus ulcers) is a critical aspect of care. Utilize various positions when the patient is in bed, changing positions at least every two hours or based on individual skin tolerance. After each position change, carefully inspect for redness, particularly over bony prominences. Consider using an eggshell or circulating air mattress to redistribute pressure when independent repositioning is compromised.
- Wheelchair Positioning: Reposition patients in wheelchairs based on their skin tolerance and utilize pressure-relieving wheelchair cushions to minimize the risk of skin breakdown. Ensure proper positioning when ambulating or in a wheelchair, such as using a sling to support a weak upper extremity.
- Nutritional Support: Promote adequate nutritional intake. Proper nutrition is vital for maintaining skin integrity and overall health.
Urinary Function
Assessment: Assess the patient’s urination patterns, including frequency, urgency, and any pain or discomfort. Monitor fluid intake patterns. Evaluate the patient’s ability to transfer to the toilet or commode and, for male patients, their standing ability for urination. Although urinary tract infections (UTIs) are less common in ALS, monitor for signs and symptoms such as increased frequency, urgency, painful urination, and fever.
Common Nursing Diagnoses:
- Impaired Urinary Elimination related to:
- Progressive Loss of Mobility: Difficulty reaching the toilet in time due to mobility limitations can lead to incontinence.
- Dehydration: Inadequate fluid intake, which may occur due to dysphagia or decreased thirst sensation, can affect urinary function.
Management:
- Encourage Fluid Intake: Unless contraindicated due to swallowing difficulties, encourage a daily fluid intake of approximately 2,500 cc’s. Adequate hydration supports urinary function and helps prevent constipation.
- UTI Monitoring and Physician Consultation: If signs of a UTI are present, consult the physician for appropriate diagnosis and treatment.
Altered Bowel Function
Assessment: Assess bowel patterns, specifically looking for signs of constipation, diarrhea, or impaction. Evaluate dietary fiber and fluid intake, as well as swallowing ability, as these factors directly impact bowel function. Assess the patient’s activity level, as reduced mobility contributes to bowel dysfunction.
Common Nursing Diagnoses:
- Impaired Bowel Elimination related to:
- Progressive Loss of Mobility: Reduced physical activity slows bowel motility.
- Progressive Decline of Dietary Fiber: Dietary changes due to dysphagia may lead to decreased fiber intake, contributing to constipation.
- Dehydration: Mild to moderate dehydration can worsen constipation.
- Progressive Inability of Trunk Muscles: Weakness of trunk muscles compromises the ability to maintain normal posture for effective defecation.
Management: Focus on preventative measures to manage bowel dysfunction proactively.
- Bowel Movement Record: Encourage the patient to keep a daily record of bowel movements, noting consistency and amount, especially if constipation or less frequent stools occur.
- Fluid Intake: Encourage a daily fluid intake of 2,500 cc’s, unless contraindicated by swallowing difficulties.
- Dietary Fiber Instruction: Provide dietary education regarding the importance of fiber, suggesting bran cereals, prune juice, and other fiber-rich foods, if swallowing is not impaired.
- Stool Softeners and Metamucil: Daily use of stool softeners and fiber supplements like Metamucil is often recommended. Laxatives can be used, but chronic use should be approached cautiously due to potential bowel irritation. Mineral oil should be avoided due to the risk of aspiration.
- Aggressive Bowel Program: As mobility further declines or if diet and fluid intake are significantly altered, a more aggressive bowel program, potentially involving daily or every-other-day interventions, may be necessary. Consult with a healthcare provider to establish an appropriate bowel management plan.
Nutritional Care and Nursing Diagnoses
Nutritional Needs
Assessment:
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Anthropometric Measures: Obtain baseline and ongoing measurements:
- Height
- Pre-morbid “usual” weight
- Current weight
- Weight gain/loss pattern since ALS onset
- “Ideal body weight” calculation
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Laboratory Tests: Consider laboratory tests if the patient has experienced a significant weight loss (ten percent or more of body weight in the last two months) or is significantly underweight (fifteen percent or more below “ideal body weight”).
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Hydration Status: Assess hydration through careful monitoring of fluid intake and output and by measuring urine specific gravity. A general guideline is that an average adult needs approximately 35 cc of fluid per kilogram of body weight daily for adequate renal function. This can be used to estimate individual fluid requirements.
Dysphagia (Difficulty Swallowing)
Assessment:
- Swallowing Assessment:
- Assess gag, cough, and swallowing reflexes, as well as chewing ability.
- Evaluate the patient’s ability to swallow liquids and solids of various consistencies.
- Monitor weight serially to detect weight loss related to dysphagia.
Common Nursing Diagnoses:
- Potential for Injury related to Dysphagia: Dysphagia significantly increases the risk of aspiration, leading to choking or respiratory complications.
- Impaired Nutritional Status: Difficulty swallowing can hinder adequate oral intake, leading to malnutrition and weight loss.
- Knowledge Deficit regarding Alternatives for Diet, Food Preparation, and Supplemental Feeding: Patients and caregivers may need education on modified diets, food preparation techniques, and alternative feeding methods to manage dysphagia effectively.
Management:
- Airway Protection Techniques: Educate patients and caregivers on techniques to protect the airway during swallowing:
- Sitting upright during meals.
- Tucking the chin to the chest while swallowing (chin tuck maneuver).
- Concentrating on eating and avoiding distractions.
- Minimize environmental stimulation during mealtimes.
- Dietician Referral: Refer patients to a registered dietician for a comprehensive nutritional assessment and to develop an individualized dietary plan to ensure adequate nutritional intake while managing dysphagia and weight loss.
Nutritional Management of the Dysphagic Patient
- Promotion of Optimum Weight:
- Smaller, More Frequent Meals: Smaller meals can be easier to manage and less overwhelming for patients with dysphagia.
- “Easy-to-Eat” Foods: Recommend mechanically altered diets, such as a mechanical soft diet, to adjust food textures to facilitate easier swallowing.
- Limited Activity with Non-Fatiguing Exercise: Encourage gentle, non-fatiguing exercise to help maintain muscle mass and appetite without excessive energy expenditure.
- Liquid Supplements: Consider liquid nutritional supplements between meals to augment caloric intake and address nutritional deficits.
Respiratory Care and Nursing Diagnoses
Respiratory Assessment
Assessment:
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History: Obtain a detailed history of subjective respiratory symptoms, such as:
- “Starving for air” sensation
- Shortness of breath (dyspnea) and its relationship to positional changes (orthopnea)
- Fatigue
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Physical Assessment: Thoroughly assess:
- Changes from baseline respiratory rate, depth, and pattern.
- Chest expansion symmetry and adequacy.
- Respiratory effort and signs of distress, such as retraction of intercostal spaces, abdominal breathing, diaphragmatic breathing, nasal flaring, and use of cervical accessory muscles of respiration.
- Skin color for cyanosis (bluish discoloration indicating poor oxygenation).
- Cough effectiveness, and gag and swallow reflexes.
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Auscultation: Auscultate lung sounds for:
- Decreased breath sounds, indicating reduced airflow.
- Presence of adventitious or extra sounds, such as rales (crackles) or wheezes, which may indicate fluid or airway obstruction.
Common Nursing Diagnoses:
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Ineffective Airway Clearance related to:
- Impaired/Absent Gag Reflex: Increased risk of aspiration due to inability to protect the airway.
- Impaired/Absent Swallowing Reflex: Difficulty managing secretions and food/fluids in the mouth.
- Impaired/Absent Cough/Sneeze Reflex: Reduced ability to clear secretions from the airway.
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Impaired Gas Exchange related to:
- Aspiration secondary to impaired/absent gag, swallowing, and cough/sneeze reflexes: Aspiration leads to potential lung infection and impaired oxygen exchange.
- Atelectasis secondary to impaired cough/sneeze reflexes and/or hypoventilation: Inability to effectively clear secretions and take deep breaths can lead to lung collapse.
- Ineffective Breathing Pattern: Muscle weakness affecting respiratory muscles leads to shallow and ineffective breathing.
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Knowledge Deficit regarding:
- Airway Clearance and Gas Exchange: Patients and caregivers need to understand strategies for maintaining clear airways and optimizing breathing.
- Considerations regarding Mechanical Life Supports: Understanding options for mechanical ventilation is crucial for informed decision-making.
Management:
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Maintain Airway Patency:
- Aspirator Use: Utilize an aspirator (suction device) to remove secretions from the mouth and airway to prevent choking and aspiration.
- Medications: Consider medications to reduce excessive secretions (e.g., amitriptyline [Elavil] in low doses – as prescribed and monitored by a physician).
- Tracheostomy Care: If the patient has a tracheostomy, perform regular suctioning and “trach care” to prevent secretion buildup, potential obstruction, and infection.
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Maintain Adequate Ventilation:
- Deep Breathing and Coughing Exercises: Encourage deep breathing and coughing exercises and the use of an incentive spirometer to promote lung expansion.
- Chest Physiotherapy: Administer chest physiotherapy, including percussion, assisted cough techniques, and postural drainage, when indicated to mobilize and clear secretions.
- Oxygen Therapy: Administer supplemental oxygen as prescribed by a physician to maintain adequate oxygen saturation.
- Head of Bed Elevation: Elevate the head of the bed using a foam wedge or hospital bed to ease shortness of breath and enhance comfort, particularly during sleep.
- Comfort Medications: When air hunger becomes chronic and distressing, work with the physician to monitor and titrate medications (e.g., opioids) to provide comfort and manage symptoms.
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Mechanical Ventilation Discussion: Provide comprehensive information and facilitate discussions about options for mechanical ventilation. Ideally, these discussions should occur early, particularly if there are early signs of dysphagia or dyspnea, or after the patient has had time to adjust to the ALS diagnosis. Proactive consideration of these decisions is crucial, well before the immediate need arises.
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Physician’s Directive Discussion: Provide information and facilitate discussions about advance care planning documents, such as a Physician’s Directive (Living Will or Durable Power of Attorney for Medical Care). Thoroughly discuss the pros and cons of life support options before the patient completes a Physician’s Directive, ensuring informed decision-making aligned with their values and wishes.
Communication Care and Nursing Diagnoses
Assessment:
- Speech Assessment: Assess the volume and clarity of speech. Note any changes or difficulties.
- Communication Needs Assessment: Evaluate the patient’s ability to effectively communicate their needs to family members and significant others.
Common Nursing Diagnoses:
- Impaired Verbal Communication related to:
- Altered Volume of Speech: Speech may become softer and difficult to hear.
- Altered Clarity of Speech (Dysarthria): Slurred or distorted speech due to muscle weakness affecting speech articulation.
- Loss of Speech (Anarthria): Complete inability to produce spoken language as ALS progresses.
Management:
- Alternative Communication Methods: For patients with dysarthria or total speech loss, especially those without upper extremity use, explore and implement various alternative communication methods.
- Speech Pathologist Referral: Refer patients to a speech-language pathologist for comprehensive evaluation and intervention. A speech pathologist can assess communication abilities, recommend appropriate augmentative and alternative communication (AAC) devices and strategies, and provide therapy to maximize communication effectiveness.
Psychological Adaptation and Nursing Diagnoses
Assessment:
- Psychosocial Assessment: Evaluate the patient and family’s support systems and coping patterns. Recognize that ongoing loss of independence will lead to continuous grieving for both the patient and their family.
Common Nursing Diagnoses:
- Ineffective Coping Strategies: ALS diagnosis and progression can overwhelm coping mechanisms, leading to distress and maladaptive coping.
- Knowledge Deficit regarding Alternatives for Mobility and Comfort: Patients and families may need information and support to adapt to changing mobility and comfort needs.
- Impaired Support System: ALS can strain existing support systems, and patients may experience a lack of adequate emotional, practical, or social support.
Management:
- Accepting Environment: Create a supportive and accepting environment where the patient and family feel comfortable sharing concerns and fears with each other and the healthcare team.
- Anticipate Care Needs: Help the patient and family anticipate future care needs and the implications of disease progression. Provide proactive guidance and resources.
- Emotional Support: Acknowledge and support emotional responses to loss as a normal reaction to ALS. This validation can help families process information and cope more effectively with practical decision-making.
- Encouragement and Anticipation: Provide ongoing encouragement and be attentive to unasked questions and unspoken concerns.
- Realistic Expectations: Foster realistic expectations about the disease progression and its impact, while emphasizing available support and management strategies.
- Referrals for Counseling: If needed, refer the patient and/or family to a counselor, psychiatrist, psychologist, or psychiatric clinical nurse specialist for specialized psychological support.
- ALS Support Groups: Recommend and facilitate connection with local ALS support groups, if available. Support groups provide valuable peer support, shared experiences, and resources.
Conclusion
Nursing care for individuals with ALS requires a holistic and proactive approach, centered on addressing the multifaceted challenges posed by this progressive disease. By accurately identifying and managing relevant nursing diagnoses, healthcare professionals can develop comprehensive care plans that prioritize patient comfort, quality of life, and optimal functioning throughout the course of ALS. Continuous assessment, tailored interventions, and strong interdisciplinary collaboration are essential to providing effective and compassionate care for individuals and families affected by ALS.
References
Konikow, Nancy R.N., M.N. “Amyotrophic Lateral Sclerosis: A Teaching Manual for Health Professionals”. ALS Health Support Services, Kirkland, Washington.
