VARIATIONS IN PEDIATRIC ANATOMY AND PHYSIOLOGY
The neuromuscular system is the combination of the nervous system and the muscles working together to create movement. Anatomic and physiologic differences in infants and children, such as the immaturity of the neurologic and musculoskeletal systems, place them at increased risk for the development of a neuromuscular disorder.
Brain and Spinal Cord Development
Early in gestation, around 3 to 4 weeks, the neural tube of the embryo begins to differentiate into the brain and spinal cord. If the fetus suffers infection, trauma, malnutrition, or teratogen exposure during this criti- cal period of growth and differentiation, brain or spi- nal cord development may be altered. The premature infant’s central nervous system is less mature than the term newborn’s. Such immaturity in the preterm infant places him or her at a higher risk of central nervous insult within the neonatal period, which may result in delayed motor skill attainment or cerebral palsy. Com- pared with the adult, the child’s spine is very mobile, especially the cervical spine region, resulting in a higher risk for cervical spine injury.
Myelinization
Though development of the structures of the nervous system is complete at birth, myelinization is incomplete.
Myelinization continues to progress and is complete by about 2 years of age. Myelinization proceeds in a cepha- locaudal and proximodistal fashion, allowing the infant
to gain head and neck control before becoming able to control the trunk and the extremities. As myelinization proceeds, the speed and accuracy of nerve impulses increase.
Muscular Development
The muscular system, including tendons, ligaments, and cartilage, arises from the mesoderm in early embryonic development. At birth (term or preterm), the muscles, tendons, ligaments, and cartilage are all present and functional. The newborn infant is capable of spontaneous movement but lacks purposeful con- trol. Full range of motion is present at birth. Healthy infants and children demonstrate normal muscle tone;
hypertonia (increased muscle tone) or hypotonia (low muscle tone) is an abnormal inding. Deep ten- don relexes are present at birth and are initially brisk in the newborn and progress to average over the irst few months. Sluggish deep tendon relexes indicate an abnormality. As the infant matures and becomes mobile, the muscles develop further and become stronger. The adolescent boy, in response to testoster- one release, experiences a growth spurt, particularly in the trunk and legs, and develops bulkier muscles at that time.
COMMON MEDICAL TREATMENTS
A variety of medications as well as other medical treat- ments are used to treat neuromuscular disorders in chil- dren. Most of these treatments will require a physician’s or nurse practitioner’s order when the child is in the hospital. The most common treatments and medica- tions are listed in Common Medical Treatments 44.1 and Drug Guide 44.1. The nurse caring for the child with a neuromuscular disorder should become familiar with what these procedures are and how they work as well as common nursing implications related to use of these modalities.
NURSING PROCESS OVERVIEW FOR THE CHILD WITH A
NEUROMUSCULAR DISORDER
Care of the child with a neuromuscular disorder includes assessment, nursing diagnosis, planning, interventions, and evaluation. There are a number of general concepts related to the nursing process that may be applied to neuromuscular dysfunction in children. From a general understanding of the care involved for a child with a neuromuscular disorder, the nurse can then individualize the care based on speciics particular for that child.
COMMON MEDICAL TREATMENTS 44.1
Treatment Explanation Indications Nursing Implications
Skeletal or cervical traction
Traction is used to immobilize the cervi- cal spine. Halo traction will be applied in stable injuries and allows the child greater mobility. Cervical traction is applied by a jacket-like apparatus, and the child can get out of bed, use a wheelchair, and even ambulate.
To minimize or prevent trauma to the spinal cord
Monitor neurologic status closely.
Assess for signs and symptoms of infection or impaired skin integrity.
Provide appropriate pin site care.
Physical ther- apy, occupa- tional therapy, or speech therapy
Physical therapy focuses on attainment or improvement of gross motor skills.
Occupational therapy focuses on reinement of ine motor skills, feeding, and activities of daily living. Speech therapy is warranted for the child with a speech impairment or feeding dif- iculty related to oral muscular issues.
Cerebral palsy, spina biida, spinal cord injury, muscular dystrophy, spinal muscular atrophy
Provide follow-through with pre- scribed exercises or supportive equipment.
Success of therapy is dependent upon continued compliance with the prescribed regimen.
Ensure that adequate communication exists within the interdisciplinary team.
Orthotics, braces
Adaptive positioning devices specially itted for each child by the physical or occupational therapist or ortho- tist. Used to maintain proper body or extremity alignment, improve mobility, and prevent contractures
Cerebral palsy, spinal cord injury, spina biida, muscular dystrophy, spinal muscular atrophy
Provide frequent assessments of skin covered by the device to avoid skin breakdown.
Follow the therapist’s schedule of recommended “on” and “off” times.
Encourage families to comply with use.
DRUG GUIDE 44.1 COMMON DRUGS FOR NEUROMUSCULAR DISORDERS
Medication Actions/Indications Nursing Implications
Benzodiazepines (diazepam, lorazepam)
Anticonvulsant; enhance the inhibition of GABA Used adjunctively for relief of skeletal muscle
spasm associated with cerebral palsy and paralysis resulting from spinal cord injury
Monitor sedation level.
Assess for improvements in spasticity.
Baclofen (oral or intrathecal)
Central-acting skeletal muscle relaxant; pre- cise mechanism unknown
Used to treat painful spasms and decrease spasticity in children with motor neuron lesions, such as cerebral palsy and spinal cord injury
Assess motor function.
Monitor for a decrease in spasticity.
Observe for mental confusion, depression, or hallucinations.
Dosage must be tapered before discontinuing because withdrawal symptoms may occur.
Corticosteroids Anti-inlammatory and immunosuppressive action
Duchenne muscular dystrophy, myasthenia gravis, dermatomyositis
Administer with food to decrease GI upset.
May mask signs of infection
Do not stop treatment abruptly or acute adrenal insuficiency may occur.
Monitor for Cushing syndrome.
Dosage may be tapered over time.
Botulin toxin Neurotoxin produced by Clostridium botulinum that blocks neuromuscular conduction Relief of spasticity in cerebral palsy,
occasionally for torticollis
Injected into the muscle by an advanced provider. May cause dry mouth
GABA, γ-aminobutyric acid; GI, gastrointestinal.
Adapted from Karch, A. (2010). 2010 Lippincott’s nursing drug guide. Philadelphia: Wolters Kluwer Health/Lippincott Williams & Wilkins and Taketomo, C. K., Hodding, J. H., & Kraus, D. M. (2010). Lexi-comp’s Drug Reference Handbook:
Pediatric & Neonatal Dosage Handbook (18th ed.). Hudson, OH: Lexi-comp, Inc.
cranial nerve, cerebellar, and motor function. Observe the child’s general appearance, noting any asymmetry in muscle development. When extremities are not used, muscular atrophy develops, so a shortened limb may indicate chronic hemiparesis. Note symmetry of sponta- neous movement of extremities as well as facial muscles.
Determine cranial nerve function (refer back to Chapter 38 for a complete description of assessment of cranial nerves). Inspect the spine for cutaneous abnormalities such as dimples or hair tufts, which may be associated with spinal cord abnormalities. Observe the child’s level of consciousness (LOC), noting a decrease or signiicant changes. Note the presence of lethargy. Refer to Chapter 38 for a complete description of evaluation of LOC.
Motor Function. Observe spontaneous activity, pos- ture, and balance, and assess for asymmetric move- ments. In the infant, observe resting posture, which will normally be a slightly lexed posture. The infant should be able to extend extremities to a normal stretch. Note position of comfort of the infant’s or child’s neck.
Relexes. Note sluggish or brisk deep tendon relexes.
Note persistence of primitive relexes in the older infant or child, such as Moro or tonic neck. Assess for devel- opment of protective relexes, which is often delayed in infants with motor disorders.
Sensory Function. Alterations in sensory function accompany many neuromuscular disorders. Assess sen- sory function in a similar fashion to that used in the adult. The sensory functions of light touch, pain, vibra- tion, heat, and cold are distinguishable by a child. In the infant, assess for response to light touch or pain.
The usual response to pain will be withdrawal from the stimulus. Always prepare the child for the sensory examination in order to gain cooperation. The pinprick test may be particularly frightening, but most children will cooperate if educated appropriately.
PALPATION
Assess muscle strength and tone in the infant or child.
Compare strength and tone bilaterally. Evaluate neck tone by pulling the infant from a supine position to a sitting position (Fig. 44.1). By 4 to 5 months the in- fant should be able to maintain the head in a neu- tral position. Perform passive range of motion of the neck. Alterations in range of motion may indicate a neuromuscular disorder or torticollis. Note trunk tone in the infant by holding the infant under the axillae and palpating for trunk tone. The hypotonic infant will feel as though he or she is slipping through the exam- iner’s hands. Generalized hypotonia is a common sign of neuromuscular disease in the infant and young child (Sarnat, 2011). The hypertonic infant will feel rigid, ex- tending the trunk and legs. Assess leg tone in the infant
Assessment
Assessment of neuromuscular dysfunction in children includes health history, physical examination, and labo- ratory and diagnostic testing.
Health History
The health history consists of the past medical history, including the mother’s pregnancy history; family his- tory; and history of present illness (when the symp- toms started and how they have progressed), as well as treatments used at home. The past medical history might be signiicant for prematurity, dificult birth, in- fection during pregnancy, changes in gait, falls, delayed development, or poor growth. Family history might be signiicant for neuromuscular disorders that are genetic.
When eliciting the history of the present illness, inquire about the following:
• Changes in gait
• Recent trauma
• Poor feeding
• Lethargy
• Fever
• Weakness
• Alteration in muscle tone
Determine the child’s history of attainment of de- velopmental milestones. Note the age that milestones such as sitting, crawling, and walking were attained, and determine whether the pace of attainment of milestones has decreased. Some children may progress normally at irst and then demonstrate decreased velocity of devel- opment of achievements or even loss of abilities. Obtain a clear description of weakness; is it fatigue, or is the child truly not as strong as he or she was in the past?
Physical Examination
Physical examination of the nervous and musculoskel- etal systems consists of inspection, observation, and palpation. It should also include auscultation of the heart and lungs, as the function of these organs may be affected by certain neuromuscular conditions.
INSPECTION AND OBSERVATION
Observe the infant or child playing with toys, crawl- ing, or walking to obtain signiicant information about
Remember Frederick, the 4-year-old who has been falling and having dificulty climb- ing stairs and who seems to tire easily when playing with his sister? What additional health history and physical examination assessment information should the nurse obtain?
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.