A number of neuromuscular disorders may be acquired during childhood or adolescence. These include dis- orders resulting from trauma as well as those that are autoimmune and infectious in nature. Trauma or unin- tentional injury is a leading cause of childhood morbidity
and mortality in the United States (Centers for Disease Control and Prevention, 2009). The child is at increased risk for trauma based on the developmental factors of physical and emotional immaturity; additionally, adoles- cents often display belief of invincibility. The developing neuromuscular system, if injured, may be irreparable, so the injury may result in life-threatening or lifelong effects.
Neuromuscular trauma includes spinal cord injury and birth trauma (refer to chapter 24 for information related to birth trauma). Autoimmune neuromuscular disorders include Guillain-Barré syndrome, myasthenia gravis, and dermatomyositis. Botulism, though uncommon in devel- oped countries, is an important neuromuscular disorder resulting from infection.
Spinal Cord Injury
Spinal cord injury is damage to the spinal cord that results in loss of function. Frequent causes are trauma, such as car accidents, falls, diving into shallow water, gunshot or stab wounds, sports injuries, child abuse, or birth injuries.
Spinal cord injuries are relatively uncommon in children, but when they do occur they have a devastating impact on the child’s physical and functional status, social and emotional development, and family functioning. More spinal cord injuries are seen in people 16 to 30 years of age due to their increased incidence of accidents, partic- ularly motor vehicle accidents (Mayo Clinic Staff, 2009).
Spinal cord injury is a medical emergency and immediate medical attention is required. Cervical trac- tion is often used initially and surgical intervention is sometimes necessary. Ongoing medical treatment will be based on the child’s age and overall health and the extent and location of the injury. Therapeutic man- agement focuses on rehabilitation and prevention of complications. Spinal cord injury in children is managed similarly to that in adults.
Nursing Assessment
Symptoms vary based on the location and severity of the injury. Common signs and symptoms associated with spinal cord injury include:
• Inability to move or feel extremities
• Numbness
• Tingling
• Weakness
Paralysis depends on the location of the injury to the spinal cord; the higher the injury in the spinal cord, the more extensive the damage and the greater the loss of function. High cervical injury will result in damage to the phrenic nerve, which innervates the diaphragm. Damage to this nerve will leave the child unable to breathe with- out assistance.
The diagnosis of spinal cord injury is made by clini- cal signs and diagnostic tests, which may include radio- graphs, CT scans, and MRI.
Nursing Management
Any child who requires hospitalization due to trauma should be considered at risk for a spinal cord injury.
Immobilization of the spine is essential until full evalua- tion of the injury is complete and spinal cord damage is ruled out. Nursing management will be similar to manage- ment of the adult with a spinal cord injury and will focus on optimizing mobility, promoting bladder and bowel management, promoting adequate nutritional status, pre- venting complications associated with extreme immobil- ity such as contractures and muscle atrophy, respiratory complications, managing pain, and providing support and education to the child and family. Refer to the myelome- ningocele section of this chapter for information related to urinary and bowel elimination.
The nurse plays an important role not only in the acute care of children with spinal cord injury but also during rehabilitation. Recovery from a spinal cord injury requires long-term hospitalization and rehabilitation. An interdisciplinary team of physicians, nurses, therapists, social workers, and case managers will work to man- age the child’s complex and long-term needs. Promoting communication among the interdisciplinary team is essential and will be a key nursing function. Rehabilita- tion will need to focus on the ever-changing develop- mental needs of the child as he or she grows.
Prevention of spinal cord injuries is an important nursing consideration. Educate the public on vehicu- lar safety, including seat belt use and the proper use of age-appropriate safety seats. Additional education topics include bicycle, sports, and recreation safety; prevention of falls; violence prevention including gun safety; and water safety, including the risk of diving. This education can help decrease the incidence of spinal cord injury in children.
Guillain-Barré Syndrome
Guillain-Barré syndrome (also called acute inlamma- tory demyelinating polyradiculoneuropathy or polyneu- ropathy) is an uncommon disorder in which an immune response within the body attacks the peripheral nervous system but does not usually affect the brain or spinal cord. Guillain-Barré syndrome results in inlammation and demyelinization of the peripheral nerves. It is not fully understood why this occurs, but it is believed to be an autoimmune condition that most commonly is trig- gered by a previous viral or bacterial infection, usually described as an inluenza-like upper respiratory tract infection or an acute gastroenteritis with fever (NINDS, 2011b; Sivadon-Tardy et al., 2009). In rare cases it has
occurred after the child has had an immunization or sur- gery (NINDS, 2011b). In many cases the causative agent remains unidentiied (Sivadon-Tardy et al., 2009).
Therapeutic Management
Treatment of Guillain-Barré syndrome is symptomatic and focuses on lessening the severity and speeding recovery.
Management may include plasma exchange and admin- istration of intravenous immunoglobulins, especially in severe cases. The goal of treatment is to keep the body functioning until the nervous system recovers. Guillain- Barré syndrome is a life-threatening condition, and some children will die during the acute phase due to respira- tory failure. Most children will make a full recovery, but a few may have residual damage.
Nursing Assessment
Early diagnosis and prompt treatment are essential since the disorder can quickly lead to respiratory failure and death from muscle paralysis. For a full description of the assessment phase of the nursing process, refer to page 797. Assessment indings pertinent to Guillain- Barré syndrome are discussed below.
Health History
Elicit a description of the present illness and chief com- plaint. The clinical presentation of Guillain-Barré syn- drome is fairly similar in children and adults. It can occur a few days or weeks after the causative infection or event. Guillain-Barré syndrome has a quick onset and begins with muscle weakness and paresthesias such as numbness and tingling. Classically it initially affects the legs and progresses in an ascending manner. Occasion- ally it affects the arms or face irst and proceeds in a descending manner. Progression is usually complete in 2 to 4 weeks, followed by a stable period leading to the recovery phase, which lasts for a few weeks to months in most cases but can take years. Severity of the disor- der ranges from mild weakness to total paralysis.
In children, pain, especially of the lower extremi- ties, as the initial presentation preceding motor involve- ment has been reported. Other symptoms seen during the course of the illness include:
• Fairly symmetric laccid weakness or paralysis
• Ataxia
• Sensory disturbances
Physical Examination and Laboratory and Diagnostic Tests
Physical examination indings may include decreased or absent tendon relexes. Facial weakness or dificulty swallowing may also be present. Diagnosis is usually
based on clinical indings of paralysis. CSF analysis may reveal an increased level of protein, but this may not be evident until after the irst week of the illness.
Take Note!
Tickling may be a successful technique for assessing the level of paralysis in the child with Guillain-Barré syndrome, either initially or in the recovery phase.
Nursing Management
Nursing management is supportive. In severe cases the child may require intensive nursing care along with mechanical ventilation. Observe the child closely for the extent of paralysis and monitor for respiratory involve- ment. Nursing care focuses on the same concerns as in any child with extreme immobility or paralysis.
Prevention of complications associated with immobility is a central concern and involves maintain- ing skin integrity, preventing respiratory complications and contractures, maintaining adequate nutrition, and managing pain. Interventions include turning and repo- sitioning every 2 hours, assessing the skin for redness or breakdown, performing range-of-motion exercises, keeping the skin clean and dry, encouraging intake of luids to maintain hydration status, and encourag- ing coughing and deep breathing every 2 hours and as needed. Enteral feeding or parenteral nutrition may be indicated if swallowing becomes impaired. Physi- cal therapy may be helpful in preventing complications and promoting motor skill recovery. Provide support and education to the parent and child. The rapid onset and long recovery can be dificult and can cause strain on the family and its inances. If residual disability occurs, the family will need help adjusting and caring for their child.
Take Note!
Serial measurement of tidal volumes may reveal respiratory deterioration in the child with Guillain- Barré syndrome.
Myasthenia Gravis
Myasthenia gravis is an autoimmune disease that may be inherited as a rare genetic disease, may be acquired by infants born to mothers with myasthenia gravis, or may develop later in childhood. The cause is unclear but the thymus gland sometimes plays a role and it is suspected that viral or bacterial infection can trigger it (Muscular Dystrophy Association, 2009d). The acetylcholine recep- tor at the neuromuscular junction is affected, inhibiting normal neuromuscular transmission. The result is pro- gressive weakness and fatigue of the skeletal muscles.
Though no gene has been discovered as responsible for myasthenia gravis, autoimmune diseases do demonstrate a genetic predisposition.
Myasthenia gravis usually reaches maximum severity within 1 to 3 years of onset, and with proper treatment children can remain physically active (Muscular Dystro- phy Association, 2009d). However, there is no cure for myasthenia gravis. Symptoms can be controlled, but it is a lifelong condition, with early detection being the key to managing the disorder successfully. The disease may be aggravated by stress, exposure to extreme tem- peratures, and infections, resulting in a myasthenic crisis.
Myasthenic crisis is a medical emergency with symptoms including sudden respiratory distress, dysphagia, dysar- thria, ptosis, diplopia, tachycardia, anxiety, and rapidly increasing weakness.
Therapeutic management generally involves the use of anticholinesterase medications such as pyridostig- mine, which blocks the breakdown of acetylcholine at the neuromuscular junction and enhances neuromus- cular transmission. If weakness is not controlled, addi- tional medications may include corticosteroids and other immunosuppressants. Other treatments include plas- mapheresis, which removes antibodies from the blood;
intravenous immunoglobulin, and thymectomy (how- ever, the role of the thymus gland in the disease pro- cess is unclear; therefore, this procedure may or may not improve the child’s symptoms).
Nursing Assessment
Note history of fatigue and weakness; dificulty chew- ing, swallowing, or holding up the head; or pain with muscle fatigue. In the verbal child, note complaints of double vision. Observe the child for ptosis (droopy eyelids) or altered eye movements from partial paral- ysis. The neonate may display inadequate suck, weak cry, loppy extremities, and, possibly, respiratory insuf- iciency. Note increased work of breathing. Laboratory testing may involve the edrophonium (Tensilon) test, in which a short-acting cholinesterase inhibitor is used.
Acetylcholine receptor (AchR) antibodies may be present in elevated quantities in the serum.
Nursing Management
The goals of nursing management include prevention of respiratory problems and providing adequate nutri- tion. Administer anticholinergic or other medications as ordered, teaching children and families about the use of these drugs. Anticholinergic drugs should be given 30 to 45 minutes before meals, on time and exactly as ordered.
Encourage families to seek prompt medical treatment for suspected infections. Encourage appropriate stress man- agement and avoidance of extreme temperatures. Teach families that physical activities should be performed
Nursing Management
Administer medications as ordered and teach families about their use; instruct them to monitor for side effects.
Educate the family about the importance of maintain- ing the medication regimen in order to prevent calcino- sis (calcium deposits) and joint deformity in the future.
Encourage compliance with physical therapy regimens.
Ensure that children are excused from physical educa- tion classes while the disease is active.