the biceps stretch; in spasticity they both contract at the same time). These may be an early indication of cere- bral palsy or another neuromuscular disorder.
Take Note!
In cases of trauma or suspected trauma, do not perform any assessment that involves movement of the head and neck until cervical injury is ruled out. Maintain complete immobilization of the cervical spine until that time.
AUSCULTATION
Auscultate the child’s lungs; adventitious sounds are often present when respiratory muscle function is impaired.
Laboratory and Diagnostic Testing
Common Laboratory and Diagnostic Tests 44.1 offers an explanation of the laboratory and diagnostic tests most commonly used in neuromuscular disorders. The tests can assist the physician or nurse practitioner in diagnosing the disorder and/or be used as guidelines in determining ongoing treatment. Laboratory or non- nursing personnel perform some of the tests, while the nurse might perform others. In either instance the nurse should be familiar with how the tests are performed, what they are used for, and normal versus abnormal re- sults. This knowledge will also be necessary when pro- viding child and family education related to the testing.
Nursing Diagnoses, Goals,
COMMON LABORATORY AND DIAGNOSTIC TESTS 44.1
Test Explanation Indications Nursing Implications
Cervical spine x-rays
Radiographic image of the cervical spine
Detection of spinal fractures
In the trauma victim, the cervical spine should remain immobilized until cleared after cervical spine radiographs.
Fluoroscopy Radiographic examination that uses continuous x-rays to show live, real- time images
Assessment of cervical spine instability during movement
Children may be afraid of the x-ray machine but will need to cooperate with lexion and extension of the neck.
Allow a parent or family member to accompany the child.
Myelography X-ray study of the spinal cord allowing visualiza- tion of the cord, nerve roots, and surrounding meninges
Detection of space-occu- pying lesions of the spi- nal cord; visualization of neural tube defects;
evaluation of traumatic injury
Involves injection of contrast medium into the CSF via lumbar puncture. Most common contrast used is water-soluble iodine, but there is also oil and air con- trast. Postprocedure interventions vary based on type of contrast medium;
therefore, determine which one was used.
After the procedure, bedrest is necessary for 4 to 24 hours; keep head of bed elevated for several hours.
Encourage hydration.
Observe for signs of meningeal irritation.
Ultrasound Use of sound waves to locate the depth and structure within soft tis- sues and luid
Assessment of spinal abnormalities
Better tolerated by nonsedated children than CT or MRI
Can be performed with a portable unit at bedside
Computed tomog- raphy (CT)
Noninvasive x-ray study that looks at tissue density and structures.
Images a “slice” of tissue
Evaluation of congenital abnormalities such as neural tube defects, fractures, demyeliniza- tion, or inlammation
Machine is large and can be frightening to children.
Procedure can be lengthy and child must remain still. If unable to do so, sedation may be necessary.
If performed with contrast medium, assess for allergy.
Encourage luids after procedure if not contraindicated.
Magnetic reso- nance imaging (MRI)
Based on how hydro- gen atoms behave in a magnetic ield when disturbed by radiofre- quency signals. Does not require ionizing radia- tion. Provides a 3D view of the body part being scanned
Assessment of inlam- mation, congenital abnormalities such as neural tube defects
Remove all metal objects from the child.
Child must remain motionless for entire scan; parent can stay in room with child. Younger children will require sedation in order to be still.
A loud thumping sound occurs inside the machine during the procedure, which can be frightening to children.
Creatine kinase Relects muscle damage:
it leaks from muscle into plasma as muscles deteriorate.
Diagnosis of muscular dystrophy, spinal mus- cular atrophy
Draw sample before electromyogram or muscle biopsy, as those tests may lead to release of creatine kinase.
Electromyography A recording electrode is placed in the skeletal muscle and electrical activity is recorded.
Differentiates muscular disorders from those that are neurologic in origin
Requires insertion of short needles into the muscles. Sedation or analgesia may be ordered.
• Urinary retention
• Constipation
• Self-care deicit (specify)
• Risk for impaired skin integrity
• Chronic sorrow
• Risk for injury
• Deicient knowledge (specify)
• Family processes, interrupted
COMMON LABORATORY AND DIAGNOSTIC TESTS 44.1 (continued)
Test Explanation Indications Nursing Implications
Nerve conduction velocity
Measures the speed of nerve conduction.
Patch-like electrodes are attached to the skin at various nerve locations
Differentiation of muscu- lar disorders
Feels like mild electric shocks
Muscle biopsy Removal of a piece of muscular tissue either by needle or by open biopsy
Determination of type of muscular dystrophy or spinal muscular atrophy
Postbiopsy care is similar to that for other types of biopsy.
Involves a small incision with one or two sutures
Dystrophin A normal intracellular plasma membrane pro- tein in the muscle
Determination of speciic type of muscular dys- trophy
Absent in Duchenne muscular dystrophy, decreased in Becker muscular dystrophy
Genetic testing Tests for presence of the gene for the disease or for carrier status
Determination of disease or carrier status of inherited muscular disorder
Entire family should be tested, even those unaffected, because carrier status should be determined and genetic counseling related to reproduction provided.
Adapted from Fischbach, F. T., & Dunning III, M. B. (2009). A manual of laboratory and diagnostic tests (8th ed.). Philadelphia: Lippincott Williams & Wilkins.
Nursing goals, interventions, and evaluation for the child with neuromuscular dysfunction are based on the nursing diagnoses (see Nursing Care Plan 44.1).
The nursing care plan may be used as a guide in plan- ning nursing care for the child with a neuromuscular disorder. The care plan includes many nursing diagno- ses that are applicable to the child or adolescent. Chil- dren’s responses to neuromuscular dysfunction and its treatment will vary, and nursing care should be individ- ualized based on the child’s and family’s responses to illness; see Healthy People 2020. Additional information
H E A LT H Y P E O P L E 2020
Objective Nursing Significance Increase the proportion
of children with special health needs who have ac- cess to a medical home.
• Educate the family that specialist care (though often consisting of frequent and multiple visits) does not replace primary care through the medical home.
• Encourage families to ind a physician or nurse practitioner with whom they feel com- fortable for well-child check-ups and routine childhood illnesses.
Healthy People Objectives based on data from http://www .healthypeople.gov.
After completing an assessment of Fredrick, the nurse noted the following: he started walking at 2 years of age, he has difficulty jumping, his gait has a waddling appear- ance, and he does not rise from the loor in the usual fashion. Based on these assessment indings, what would your top three nursing diagnoses be for Fredrick?
about nursing management will be included later in the chapter as it relates to speciic disorders.
Based on your top three nursing diagnoses for Fredrick, describe appropriate nursing interventions.
NURSING CARE PLAN 44.1
Overview for the Child With a Neuromuscular Disorder
NURSING DIAGNOSIS: Impaired physical mobility related to muscle weakness, hypertonicity, im- paired coordination, loss of muscle function or control as evidenced by an inability to move extremities, to ambulate without assistance, to move without limitations
• Encourage gross and ine motor activities to facilitate motor development.
• Collaborate with physical therapy, occupational therapy, and speech therapy to strengthen mus- cles and promote optimal mobility to facilitate motor development.
• Use passive and active range-of-motion (ROM) exercises and teach child and family how to
perform them to prevent contractures, facilitate joint mobility and muscle development (active ROM), and help increase mobility.
• Praise accomplishments and emphasize child’s abilities to improve self-esteem and encourage feeling of confidence and competence.
Outcome Identification and Evaluation
Child will exhibit signs of adequate nutrition as evidenced by appropriate weight gain, intake and output within normal limits, and adequate ingestion of calories.
Intervention: Promoting Adequate Nutrition
• Monitor height and weight: insufficient intake will lead to impaired growth and weight gain.
• Monitor hydration status (moist mucous membranes, elastic skin turgor, adequate urine output): insufficient intake can lead to dehydration.
• Use techniques to promote caloric and nutritional intake and teach family about these techniques (e.g., positioning, modiied utensils, soft or blended foods, allowing extra time) to facilitate intake.
• Assess respiratory system frequently to assess for aspiration.
• Assist family to help child assume as normal a feeding position as possible to help increase oral intake.
NURSING DIAGNOSIS: Nutrition, imbalanced, less than body requirements, related to dificulty feeding secondary to deicient sucking, swallowing, or chewing; dificulty assuming normal feeding position;
inability to feed self as evidenced by decreased oral intake, impaired swallowing, weight loss, or plateau Outcome Identification and Evaluation
Child will be able to engage in activities within age parameters and limits of disease: child is able to move extremities, move about environment, and participate in exercise programs within limits of age and disease.
Interventions: Maximizing Physical Mobility
NURSING DIAGNOSIS: Urinary retention related to sensory motor impairment as evidenced by dribbling, inadequate bladder emptying
Outcome Identification and Evaluation
Child’s bladder will empty adequately, according to preestablished quantities and frequencies individualized for the child (usual urine output is 0.5 to 2 mL/kg/hour).
Intervention: Promoting Successful Bladder Emptying
Overview for the Child With a Neuromuscular Disorder
(continued)NURSING CARE PLAN 44.1
NURSING DIAGNOSIS: Risk for constipation related to immobility, loss of sensation
Outcome Identification and Evaluation
Child will demonstrate adequate stool passage: will pass soft, formed stool every 1 to 3 days without straining or other adverse effects.
Intervention: Promoting Appropriate Bowel Elimination
• Assess usual pattern of stooling to determine baseline and identify potential problems with elimination.
• Palpate for abdominal fullness and auscultate for bowel sounds to assess for bowel function and presence of constipation.
• Encourage iber intake to increase frequency of stools.
• Ensure adequate luid intake to prevent formation of hard, dry stools.
• Encourage activity within child’s limits or restrictions: even minimal activity increases peristalsis.
• Administer medications or enemas as ordered to promote bowel training/evacuation (especially in child with myelomeningocele or spinal cord injury).
NURSING DIAGNOSIS: Self-care deicit related to neuromuscular impairments, cognitive deicits as evidenced by an inability to perform hygiene care and transfer self independently
Outcome Identification and Evaluation
Child will demonstrate ability to care for self within age parameters and limits of disease:
child is able to feed, dress, and manage elimination within limits of disease and age.
• Assess child’s ability to empty bladder via his- tory focused on character and duration of lower urinary symptoms to establish baseline.
• Assess for history of fecal impaction or consti- pation, as alterations in bowel elimination may hinder urinary elimination.
• Assess for bladder distention by palpation or urinary retention by postvoid residual obtained via catheterization or bladder ultrasound to de- termine extent of retention.
• Maintain adequate hydration to avoid irritating effects that dehydration has on the bladder.
• Schedule voiding to decrease bladder overdistention.
• Teach the family (and the child if old enough) with signiicant urinary retention the technique of clean intermittent catheterization to allow regular, complete bladder emptying.
Intervention: Maximizing Self-Care
• Introduce child and family to self-help methods as soon as possible to promote indepen- dence from the beginning.
• Encourage family and staff to allow child to do as much as possible to allow child to gain confidence and independence.
• Teach speciic measures for bowel and urinary elimination as needed to promote independence and increase self-care abilities and self-esteem.
• Collaborate with physical therapy, occupational therapy, and speech therapy to provide child and family with appropriate tools to modify environment and methods to promote transferring and self-care to allow for maximum functioning.
• Praise accomplishments and emphasize child’s abilities to improve self-esteem and encour- age feelings of confidence and competence.
• Balance activity with periods of rest to reduce fatigue and increase energy for self-care.
(continued)
Overview for the Child With a Neuromuscular Disorder
(continued)NURSING CARE PLAN 44.1
NURSING DIAGNOSIS: Risk for impaired skin integrity related to immobility, use of braces or adaptive devices
Outcome Identification and Evaluation
Child’s skin will remain intact, without evidence of redness or breakdown.
Intervention: Promoting Skin Integrity
NURSING DIAGNOSIS: Chronic sorrow related to presence of chronic disability as evidenced by child’s or family’s expression of sadness, anger, disappointment, or feeling overwhelmed
Outcome Identification and Evaluation
Child and/or family will accept situation: child/family will appropriately identify feelings, function at a normal developmental level, and plan for the future.
Intervention: Easing Sorrow
• Assess degree of sorrow to provide baseline for intervention.
• Identify problems with eating or sleeping, often affected when grief or sorrow is present.
• Spend time with the child and family: an empathetic presence is valued by suffering families.
• Encourage the use of positive coping techniques: taking action, expressing feelings, and intentional attempts at coping are helpful techniques.
• Refer to appropriate support groups: can be helpful to talk to others in similar situations.
• Refer for spiritual counseling as desired: many families experience grief resolution in a more timely fashion if spiritual needs are addressed.
NURSING DIAGNOSIS: Risk for injury related to muscle weakness
Outcome identification and evaluation
Child will remain free from injury: child will not fall or experience other injury.
Intervention: Preventing Injury
• Ensure that side rails of bed are elevated when caregiver is not directly at bedside to prevent fall from bed.
• Use appropriate safety restraints with adaptive equipment and wheelchairs to prevent fall or slipping from equipment.
• Do not leave child unattended in tub as weakness may cause the child to slip under the water.
• Avoid restraint use if at all possible: close observation is more appropriate.
• Monitor condition of entire skin surface at least daily to provide baseline and allow for early identification of areas at risk.
• Avoid excessive friction or harsh cleaning products that may increase risk of breakdown in child with susceptible skin.
• Keep child’s skin free from stool and urine to decrease risk of breakdown.
• Change child’s position frequently to decrease pressure to susceptible areas.
• Monitor skin condition affected by braces or adaptive equipment frequently to prevent skin breakdown related to poor fit.
NURSING DIAGNOSIS: Deicient knowledge related to lack of information regarding complex medical condi- tion, prognosis, and medical needs as evidenced by verbalization, questions, or actions demonstrating lack of understanding regarding child’s condition or care
Overview for the Child With a Neuromuscular Disorder
(continued)NURSING CARE PLAN 44.1
Outcome Identification and Evaluation
Child and family will verbalize accurate information and understanding about condition, prog- nosis, and medical needs: child and family demonstrate knowledge of condition and prognosis and medical needs, including possible causes, contributing factors, and treatment measures.
Intervention: Providing Child and Family Teaching
NURSING DIAGNOSIS: Family processes, interrupted, related to child’s illness, hospitalization, diagnosis of chronic illness in child, and potential long-term effects of illness as evidenced by family’s presence in hospital, missed work, demonstration of inadequate coping
Outcome Identification and Evaluation
Family will maintain functional system of support and demonstrate adequate coping and ad- aptation of roles: parents are involved in child’s care, ask appropriate questions, express fears and concerns, and are able to discuss child’s care and condition calmly.
Intervention: Promoting Appropriate Family Functioning
• Encourage parents and family to verbalize concerns related to child’s illness, diagnosis, and prognosis: allows the nurse to identify concerns and areas where further education may be needed; demonstrates family-centered care.
• Explain therapies, procedures, child’s behaviors, and plan of care to parents:
understanding of the child’s current status and plan of care helps decrease anxiety.
• Encourage parental involvement in care to allow parents to feel needed and valued and to have a sense of control over their child’s health.
• Identify support system for family and child: support system is often needed by stressed families to assist with coping.
• Educate family and child on additional resources available to help them develop a wide base of support.
• Assess child’s and family’s willingness to learn:
child and family must be willing to learn for teaching to be effective.
• Provide family with time to adjust to diagnosis to facilitate adjustment and ability to learn and participate in child’s care.
• Repeat information to allow family and child time to learn and understand.
• Teach in short sessions: many short sessions are more helpful than one long session.
• Gear teaching to the level of understanding of the child and family (depends on age of child, physical condition, memory) to ensure understanding.
• Provide reinforcement and rewards to facilitate the teaching/learning process.
• Use multiple modes of learning involving many senses (provide written, verbal, demonstration, and videos) when possible: child and family are more likely to retain information when it is presented in different ways using many senses.
ommended to take a higher dosage and should consult with their physician or nurse practitioner (Centers for Disease Control and Prevention, 2011). Prenatal screen- ing of maternal serum for α-fetoprotein (AFP) and ultra- sound examination at 16 to 18 weeks’ gestation can help identify fetuses at risk. Neural tube defects primarily affecting spinal cord development include spina biida occulta, meningocele, and myelomeningocele (Fig. 44.4).
Neural tube defects primarily affecting brain develop- ment are discussed in Chapter 38.
Spina Bifida Occulta
Spina bifida is a term that is often used to refer to all neural tube disorders that affect the spinal cord. This can be confusing and a cause of concern for parents. There are well-deined degrees of spinal cord involvement, and it is important for health care professionals to use the correct terminology.
Spina biida occulta is a defect of the vertebral bod- ies without protrusion of the spinal cord or meninges.
This defect is not visible externally and in most cases has no adverse affects (see Fig. 44.4). Spina biida occulta is a common anomaly. It is estimated that it affects 10% to 20% of otherwise healthy people (Spina Biida Association, 2011a). Children with spina biida occulta need no imme- diate medical intervention. Complications are rare, but may include more signiicant abnormalities of the spinal cord such as tethered cord, syringomyelia, or diastematomyelia.
Nursing Assessment
In most cases, spina biida occulta is benign and asymp- tomatic and produces no neurologic signs. In fact, it may be considered a normal variant (Spina Biida Associa- tion, 2011a). The defect, which is usually present in the