23.3 Considerations for Care of Neuromuscular Impairment

Maintaining Respiratory Function

The muscle weakness that occurs with neuromuscular disorders eventually affects the diaphragm and intercostal muscles, resulting in impaired breathing. This typically occurs later in the course of the disease progression, and it is important for the nurse to be prepared to intervene in an effort to maintain respiratory function. First and foremost, it is important to always ensure airway patency. With weak muscles, it may be difficult for the patient to cough effectively and keep the airway clear of secretions. If secretions cannot be cleared, the patient is at high risk for aspiration and subsequent aspiration pneumonia, which can be life-threatening.

In some cases, patients with neuromuscular disorders may require supplemental oxygen to breathe effectively. In more mild situations, the patient may only need a few liters of oxygen via nasal cannula (Figure 23.19).

Figure 23.19 Patients with certain neuromuscular disorders may require supplemental oxygen to be delivered through a nasal cannula. (credit: Nursing Skills [Internet]. 2nd edition/NIH, CC BY 4.0)

In more severe situations, such as with higher level spinal cord injuries, the patient may require lifelong mechanical ventilation through a tracheostomy (Figure 23.20). For patients with tracheostomies, it is important for the nurse to provide “trach” care as ordered to keep the airway clear and prevent infection. Additionally, the nurse should have familiarity with the ventilator settings and be able to intervene appropriately for various alarms.

Figure 23.20 A patient with a neuromuscular disorder may have a tracheostomy, which is connected to a mechanical ventilator. This surgically created opening in the trachea helps provide oxygen to the lungs. (credit: National Heart Lung and Blood Institute/Wikimedia Commons, Public Domain)

Dealing with Immobility Considerations

Patients with muscle weakness from neuromuscular conditions often spend an extended period of time in bed and thus can develop a variety of complications related to loss of muscle strength. Regardless of the cause, immobility can cause degradation of cardiovascular, respiratory, gastrointestinal, and musculoskeletal functioning. Promoting mobility can prevent these complications from occurring. Studies have shown several benefits of mobilization, including less delirium, pain, urinary discomfort, urinary tract infection, fatigue, deep vein thrombosis (DVT), skin breakdown, and pneumonia, as well as an improved ability to void (Alaparthi et al., 2020). (Figure 23.21). Additionally, the use of adaptive equipment and assistive devices (e.g., wheelchairs, walkers, canes) can restore some mobility, allowing for increased independence and participation in self-care activities.

Figure 23.21 The patient’s leg is red and swollen; the arrow points to the deep vein thrombosis that has developed as a complication of prolonged immobility. (credit: Seyahi E, Yurdakul S/NIH, CC BY 2.0)

Nurses should understand the effects of immobility on these body systems as well as the complications. It also is important for nurses to be prepared to implement associated nursing interventions related to immobility (Table 23.6).

Managing Pain

Pain is another common clinical manifestation of neuromuscular disorders. Pain management requires collaboration with the interdisciplinary team, including nurses, healthcare providers, pharmacists, and sometimes pain specialists. There are many different types of pain medications (called analgesics) that can be administered by various routes. Analgesics are classified as nonopioids, opioids, or adjuvants. An adjuvant is a medication that has been found in clinical practice to have either an independent analgesic effect or additive analgesic properties when administered with opioids. Examples of adjuvant medications include antidepressants (e.g., amitriptyline) and anti-seizure medications (e.g., gabapentin). It is important for the nurse to be familiar with analgesic medications, especially their potential side effects before administering them. For example, overuse of opioids can lead to constipation, which can cause more pain for a patient experiencing muscle weakness from a neuromuscular disorder.

In addition to medications, numerous nonpharmacological interventions can be used to treat pain associated with neuromuscular disorders. Some of these interventions include the following:

• distraction (e.g., playing games, watching television)
• relaxation through meditation, hypnosis, prayer, breathing, or music therapy
• comfort measures including repositioning and massage as tolerated
• application of heat or cold
• exercise or physical therapy as tolerated
• aromatherapy

Monitoring Nutritional Status

Monitoring nutritional status is important for all patients but is especially important for patients with neuromuscular disorders. These patients experience severe muscle weakness, which can be exacerbated by malnourished states. If the patient is unable to eat enough, the muscles are not getting the nutrients they need, further contributing to their weakened state. To improve this, the nurse should try to encourage the patient to eat when they are able to, and to consume high-calorie, nutrient-dense foods when possible. It may be beneficial for these patients to increase their intake of protein as well to help repair muscle tissue. It is important to note that although consuming more protein and calories can help alleviate some muscle weakness, it is not a cure for the underlying disorder. And in some cases, altering nutrition will still not help symptoms significantly. Some patients with neuromuscular disorders may have difficulty swallowing (known as dysphagia), making it even more difficult to eat. In these cases, enteral or parenteral nutrition therapies may be ordered. It is important for the nurse to collaborate effectively with the care team, including dieticians and speech therapists, to ensure the patient is receiving appropriate nutrition, especially if they are experiencing dysphagia.

Monitoring Elimination

Muscles of the bowel and bladder often become weak in neuromuscular conditions, especially in the later stages of disease progression. This can lead to urinary or bowel incontinence, which is the involuntary expulsion of urine or feces from the body. Nurses should use therapeutic communication with patients experiencing incontinence to help them feel comfortable in expressing their fears, worries, and embarrassment about incontinence and work toward improving their quality of life. Nurses should be prepared to provide education about pelvic floor muscle training exercises, timed voiding, lifestyle modification, and incontinence products. Encourage patients to learn more about their condition so they can optimally manage it and improve their quality of life. In more severe cases where the patient cannot manage the incontinence themselves, it is important for the nurse to check the patient frequently to ensure they are clean and dry to help prevent skin breakdown or the development of a urinary tract infection. If the patient is being discharged home, the nurse should provide education to the family or primary caregiver about the importance of keeping the patient clean and dry to prevent complications.

Monitoring Skin Integrity

Patients with neuromuscular disorders often spend extended periods of time in bed or sitting in wheelchairs because they have muscle weakness, making it difficult or impossible to ambulate. These periods of immobility increase their risk of skin breakdown and pressure injuries. A pressure injury is defined as breakdown of skin integrity caused by prolonged internal pressure against a bony prominence or persistent contact with an external surface (e.g., furniture, medical or other devices) often in combination with shear. Pressure injuries commonly occur on the sacrum, heels, ischial tuberosities, and coccyx and form when a layer of skin tissue gets caught between an external hard surface, such as a bed or chair, and the internal hard surface of a bone. Nursing interventions for the care of a patient with or at risk for pressure injuries include the following:

• assessing and documenting skin status frequently
• applying prophylactic dressings to high-risk areas
• keeping skin free of excessive moisture
• promoting a high-protein diet (if appropriate for patient) and encouraging fluid intake
• repositioning the patient frequently (e.g., every two hours)
• keeping bed linens clean and free from wrinkles

Assisting with ADLs

Muscle weakness often makes it difficult or impossible for patients with neuromuscular disorders to perform ADLs on their own. The nurse should encourage the patient to perform ADLs as independently as possible and participate in prescribed physical therapy. For instance, the nurse should encourage the patient to perform active or passive range of motion exercises as prescribed by the physical therapist or if necessary perform these ADLs for the patient. The nurse should be aware that pain and fear of falling can be major deterrents to a patient’s willingness to ambulate or perform physical therapy, so the nurse should educate the patient about appropriately using assistive devices and adhering to other fall precautions. The nurse should encourage rest between activities. For patients who are bedridden, the nurse should elevate the head of the bed to 30 to 45 degrees, unless medically contraindicated, and turn and reposition the patient every two hours. Furthermore, the nurse should perform hourly rounding to check on the patient’s needs and prevent falls. In some cases, the use of assistive devices may be used to restore some of the patient’s mobility, increasing their ability to participate in performing ADLs. Examples of these devices include canes, walkers, crutches, wheelchairs, mobility scooters, and braces.

Provide Patient and Family Support

Many patients with neuromuscular disorders have family caregivers who are an extension of the healthcare team and constantly work with the patient. It is important for nurses to also assess the caregiver when seeing them with the patient in the home, clinic, hospital, or long-term care setting and provide encouragement. What do caregivers want? Research shows that caregivers want and need the following (Lowey, 2024):

• support, assistance, and practical help (e.g., finding others to assist with grocery shopping, going to the pharmacy, and food preparation)
• honest conversations with the healthcare team
• assurance that their loved one is being honored
• inclusion in decision-making
• desire to be listened to and their concerns heard
• remembrance as a good and compassionate caregiver
• assurance that they did all they possibly could for their loved one

Nurses should assess caregivers’ social support network and any needs for further assistance. This assessment includes assessing the caregiver’s physical needs, sleep patterns, and ability to perform other responsibilities. Nurses should watch for signs of declining health, clinical depression, or increased use of alcohol and drugs. Listening to their stories and providing presence, active listening, and touch contributes greatly to the caregiver’s sense of worth. The nurse should help the patient’s caregiver in identifying and using support systems and should refer them to resources and support groups in the community as needed.

Teach Coping Strategies

Interventions to enhance coping can be implemented for patients with neuromuscular disorders as well as for family members. Sample interventions that the nurse should consider implementing include the following:

• Assist the patient in identifying short- and long-term goals.
• Help the patient find available resources to meet the goals.
• Encourage relationships with others who have common interests and goals.
• Help the patient solve problems in a constructive manner.
• Assist the patient in breaking down complex tasks into small, manageable steps.
• Help the patient identify alternative responses to difficult situations.
• Use a calm, reassuring approach.
• Provide an atmosphere of acceptance.
• Help the patient identify information they are most interested in obtaining.
• Provide factual information regarding medical diagnosis, treatment, and prognosis.
• Provide the patient with realistic choices about certain aspects of care.
• Encourage an attitude of realistic hope as a way of dealing with hopelessness.
• Seek to understand the patient’s perspective of a stressful situation.
• Discourage decision-making when the patient is under severe stress.
• Acknowledge the patient’s cultural and spiritual background and encourage use of spiritual resources, if desired.
• Encourage verbalization of feelings, perceptions, and fears.
• Encourage family involvement, as appropriate.
• Help the patient identify positive strategies to deal with limitations and manage needed lifestyle or role changes.
• Instruct the patient on the use of relaxation techniques.

Provide Education

Parents of children with newly diagnosed neuromuscular disorders are often scared and anxious about many aspects of their new reality with a sick child. For this reason, it is important that the nurse provides thorough education regarding the diagnosis itself and how to care for the child in the home setting. Additionally, the nurse will need to educate patients and families about typical developmental milestones and how neuromuscular conditions may affect the attainment of these milestones. The nurse should emphasize the importance of early intervention services and explain how these therapies can help promote optimal development, improve functional abilities, and enhance quality of life. The nurse can also provide information about adaptive strategies, assistive devices, and mobility aids that can support independence and participation in daily activities. Thinking on a bigger scale, the nurse can also educate families and educators about the educational rights and needs of individuals with neuromuscular conditions to promote advocacy.

The nurse must also be prepared to address any other questions the parents or guardians may have. It is important to remember that parents may require repetition of information because often they are anxious, making it difficult for them to process large amounts of information. Most importantly, the nurse should be patient, kind, and nonjudgmental when providing education and interacting with both the child and family.

Promote Self-Care Regimens

Often, young children are unable to participate in a self-care regimen, but as they get older, the nurse should emphasize the importance of allowing children to perform as much of their own self-care as possible. Having a neuromuscular disorder takes away much of a person’s independence, so they should be encouraged to perform as much care for themselves as possible in an effort to restore some of that autonomy. In the hospital setting, the nurse should allow the patient to do as much on their own as possible while still remaining in close proximity to be prepared to help if the patient requires assistance.