FYI: Care Management in ALS (2023)

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physical

1. Muscle Weakness/Skin Conditions

• Evaluate
  • Exercise intensity is assessed; spastic relaxation is present.
  • Assess for the presence of contractures.
  • Assess the skin daily, especially in areas prone to breakouts.
• Common Nursing Diagnosis
  • Potential injury associated with decreased physical mobility.
  • There is a lack of knowledge about injury prevention along with a lack of knowledge to promote mobility and self-care.
  • Change comfort.
• manage
  • Promotes activity and exercise.

• Encourage continuation of daily activities and activities.
• Range of motion (ROM) exercises to prevent joint contractures and pain; first Active ROM, then passive. Consult a physical therapist for an exercise program and refer to the recommended passive exercise descriptions in Managing Amyotrophic Lateral Sclerosis (MALS) Handbook II.
• See a physical therapist or occupational therapist when weakness in a limb begins to affect mobility, safety, or independence.

• Promotes proper positioning to prevent pressure sores

• Use as many different positions as possible while lying in bed. Change positions every two hours or as your skin tolerates. After each change of position, check for redness in the bones
• Protrude and provide eggshell or circulating mattresses when stationary to prevent independent repositioning.
• Reposition on the wheelchair according to the patient's skin tolerance. Use a wheelchair cushion to prevent skin breakage.
• Proper positioning when walking or sitting in a wheelchair, i.e. using a sling when the upper limbs are weak.
• Promotes adequate nutrient intake.

two. Urine function

1. Evaluate
   • Assess voiding patterns and fluid intake patterns.
   • Assess the ability to move to the toilet or toilet, or the ability to stand in males.
   • Evaluate for signs and symptoms of UTI; frequency, urgency, painful urination, fever, etc. (UTI is rare in ALS).
2. Common Nursing Diagnosis
   • 1. Decreased urination associated with:

• gradual loss of mobility
• dehydration

1. manage
   • Unless contraindicated by ability to swallow, 2,500 cc of fluid per day is encouraged.
   • Contact your doctor if you have signs of a UTI.

3. Changes in intestinal function

1. Evaluate
   • Assess for stool pattern (constipation, diarrhea, impaction.
   • Assess diet, fluid intake, and swallowing ability.
   • Assess activity levels.
2. Common Nursing Diagnosis
   • Decreased intestinal excretion.
   • Gradual loss of mobility.
   • Dietary fiber is gradually reduced.
   • Usually mil/moderately dehydrated.
   • The muscles of the trunk gradually fail to support the normal posture that facilitates bowel movements.
3. Management - ideally focused on preventive measures. Unlike neurogenic gut, patients with ALS have reduced gut motility.
4. Encourage patients to keep a daily stool log (including consistency and quantity), especially if constipation changes or the number of bowel movements decreases.
5. Unless contraindicated by ability to swallow, 2,500 cc of fluid per day is encouraged.
6. Provide dietary guidance on the importance of fiber, bran, prune juice, etc. unless contraindicated by swallowing ability.
7. Daily use of stool softeners and Metamucil is usually recommended. Laxatives can be used, but long-term use can damage the gut. Mineral oil should not be used due to the risk of inhalation.
8. As mobility improves and/or changes in diet and fluid intake, a more aggressive daily or every other day bowel program may be required.

Nutrition

1. Nutritional requirements

A. Evaluation

1. Anthropometric measurements:
a) height
b) Premorbid "normal" weight
c) Current weight
d) Weight gain/loss pattern since disease onset
e) "Ideal Body Weight"

2. Laboratory Tests - Indicated if the patient has lost 10% or more of their body weight in the past two months, or 15% or more below their "ideal body weight."

3. Hydration status – determined by carefully recording fluid intake and output and testing urine specific gravity. The average person needs 35cc of fluid/kg of body weight for adequate kidney function. This number can be used to calculate fluid requirements.

B. Dysphagia (dysphagia)

1. Evaluation
a) Assess gag, cough and swallow reflexes, and chewing.
b) Assess the patient's ability to swallow liquids and solids.
c) Continuous assessment of weight.

2. Common Nursing Diagnosis
a) Potential hazards.
b) Decreased nutritional status.
c) Lack of knowledge about dietary substitutions, food preparation and alternative procedures for complementary feeding.

3. Management
a) Discuss techniques for protecting the airway, ie. Sit up straight, place your chin on your chest when swallowing, and focus when eating. Avoid adding stimuli, i.e. chatting while eating.
b) Consult a dietitian to determine adequate nutrient intake for continued weight loss.

C. Nutritional Therapy for Patients with Dysphagia

1. Promotes Optimal Body Weight
a) Eat small meals frequently.
b) "Easy to eat" food, i.e. adapted to mechanical soft diet.
c) Increase daily activity through non-fatiguing exercise.
d) Liquid supplements between meals.

breathe

A. Evaluation

1. History: subjective symptoms (craving for air, shortness of breath and relationship to postural changes, fatigue).
2. Assess for: Changes from baseline respiratory rate, depth, pattern, chest expansion; adequate respiratory exchange and effort; intercostal retraction; abdominal breathing; diaphragmatic breathing; nasal expansion and use of cervical accessory muscles for breathing; color (cyanosis); cough, gag, and swallow reflexes.
3. Auscultation: Decreased breath sounds with additional or involuntary sounds (ie, wheezing, wheezing).
B. Common nursing diagnoses
1. Inefficient airway clearance associated with:
a) Decreased/absent gag reflex.
b) Decreased/absent swallowing reflex.
c) Decreased/absent cough/sneeze reflex.
2. Impaired gas exchange associated with:
a) Aspiration secondary to impaired/absent vomiting, swallowing, and cough/sneeze reflexes.
b) Atelectasis secondary to diminished cough/sneeze reflex and/or hypoventilation.
c) Inefficient breathing patterns.
3. Lack of knowledge:
a) Free airway and gas exchange.
b) considerations regarding mechanical life support.

3. Management

1. Keep the airway open
a) Use a suction device to suck out secretions to prevent choking.
b) Use of medication (ie Elvail – 25 mg t.i.d. or 25-75 mg at hs to reduce secretion).
c) If the patient has had a tracheostomy, suction and tracheal care should be provided to prevent accumulation of secretions, possible obstruction and infection.

2. Ensure adequate ventilation
a) Deep breathing and coughing exercises and use of motivational spirometers to encourage lung expansion.
b) Chest physiotherapy: percussion, assisted coughing and postural drainage if indicated.
c) Oxygen, upon request.
d) Elevate the head of the bed with foam wedges or hospital beds to relieve shortness of breath and provide comfort while sleeping.
e) Help monitor and titrate medications that help provide comfort when air starvation is chronic and distressing.

3. Provide information and opportunities to discuss options for mechanical ventilation. In general, the patient and family should be helped to consider life support options if there are early signs of dysphagia, dyspnea, or after the patient has had time to adjust to his diagnosis. The important thing to remember is that the patient needs to think about these decisions before he/she needs them.

4. Provides information and opportunities to discuss medical orders (also known as living wills or medical enduring powers of attorney). The pros and cons of life support should be discussed in detail before the patient completes the medical instructions.

announcement

A. Evaluation
1. Assess loudness and intelligibility of speech. 2. Assess ability to communicate needs with family/significant others.

B. Common nursing diagnoses
1. Language communication impairment associated with:
a) Voice volume changed.
b) Changes in clarity of speech (dysarthria).
c) Loss of speech.

3. Management
1. Dysarthria or complete loss of speech and inability to use upper limbs.
2. There are many options for communication.
3. Refer to a speech and language therapist for assessment and intervention.

psychological adaptation

A. Evaluation
1. Assess the patient's and family's support systems and coping patterns and recognize that the patient and family will continue to grieve as the loss of independence continues.

B. Common nursing diagnoses
1. Ineffective coping strategies
2. Lack of knowledge about mobility and comfort alternatives.
3. Reduced support system.

3. Management
1. Provide an accepting environment where patients and families can share concerns and fears with each other and with members of the healthcare team.
2. Help patients and families anticipate care needs and impacts.
3. Anticipating and supporting emotional responses to loss as normal responses allows families to more effectively receive information and process practical decision-making.
4. Offer encouragement and anticipate unasked questions.
5. Encourage realistic expectations about the disease whenever possible.
6. Consult with a counselor, psychiatrist, psychologist, or psychiatric clinical nurse specialist, if necessary.
7. If available, consult the ALS support team.

-- Excerpted from "Amyotrophic Lateral Sclerosis: A Teaching Manual for Health Professionals," Nancy Konikow, R.N., M.N., ALS Health Support Services, Kirkland, Washington.

Amyotrophic Lateral Sclerosis-Motor Neurone Disease Care Program

problem/requirement
target/standard
nursing intervention
weakness to flaccid paralysis
Emotional responses to diagnosis and prognosis (anxiety, fear, denial, anger).
Keep the patient as active as possible
prevent skin breakouts
Prevention of venous thrombosis and pulmonary embolism
Helping Patients Through the Grieving and Grieving Process
Helping Families Deal with Illness and the Grief Process

• The active and passive range of motion of the affected limb was 3-4 times a day.
• Turn and set every 2 hours.
• Keep skin dry.
• Wash and dry skin after each bowel movement or urination (especially in women).
• Lotion drying areas.
• Beds use air flotation or egg box mattresses.
• Compression stockings (if ordered), especially when the patient is out of bed.
• Encourage expressing feelings.
• Accept behaviors at all stages of the grieving process and allow for grieving.
• Talk to the family about the grieving process and encourage them to accept the patient's behavior and their own reactions.
• Use a positive approach (asking patients and families what they know about the disease) when discussing progression.
• Encourage being independent for as long as possible and doing things on your own as much as possible.
• Teach your family to do the same.
• Arranging transfer activities, overtime, etc. while in the hospital
• Create the same family plan at home.

problem/requirement
target/standard
nursing intervention
Changing body image and self-esteem
(when bulbar symptoms occur)
respiratory insufficiency
difficulty swallowing and chewing
Help patients accept new body image and maintain self-esteem
Maintain adequate O2 delivery to patient; prevent complications of pneumonia-assisted cough
prevent aspiration
Make it easier for patients to eat
Ensure adequate nutrition and fluid balance

• Discuss changes in body image and what it means for the patient.
• Patients are encouraged to discuss this matter with their families.
• Patients are encouraged to focus on positive aspects of themselves and to share these aspects of themselves with others.
• Attract and maintain the patient's airway.
• Manage 02 as needed.
• Always raise the stove to at least 30 degrees
• Auscultate the chest frequently to assess overall airway status.
• Help the sick person to cough and breathe deeply at least every two hours.
• Incentive spirometers or chest physical therapy can be used as needed.
• Wear respiratory protection if necessary.
• Assess the gag reflex before oral fluids or food.
• Turn stove on high for ½ hour during and after meals.
• Soft food, mechanical soft food or puree diet as needed.
• High Protein, High CHO Diet
• Fluid intake 3000 cc/day.
• Consult a nutritionist for the basics of good nutrition and snacking.

problem/requirement
target/standard
nursing intervention
Difficulty speaking
Frequent urination or incontinence due to impingement
establish effective communication
Maintenance of normal bowel and bladder function (usually until advanced disease)

• If the patient can use his hands, start using the magic board.
• If you can't use a weapon, start using a word or letter board.
• The electronic larynx can be used as it is for laryngectomy patients.
• Consult a speech therapist and OT for assistance and further assessment of language needs and ways to meet them.
• If possible, place the urinal/bedpan within the patient's reach.
• Use effective means for the patient to communicate when the patient needs to urinate or have a bowel movement.
• catheterization or as needed
• External cat is useful for noc.
• Affects desired bowel routine; Fleet enemas, Colace, Metamucil, liquids, suppositories if patient can tolerate high fiber diet.

Signs/symptoms of illness, notify the M.D. of:

• Difficulty breathing
• Signs of infection - cold, flu, increased temperature, chills
• hard to swallow
• Difficulty speaking
• Sudden exacerbation of signs and symptoms of pre-existing conditions