The Child with Neuromuscular or Muscular Dysfunction



The Child with Neuromuscular or Muscular Dysfunction


Jean Stansbury, Barbara Montagnino and David Wilson



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evolve.elsevier.com/wong/essentials





Congenital Neuromuscular or Muscular Disorders


Cerebral Palsy


A new definition proposed in 2006 describes cerebral palsy (CP) as a “group of permanent disorders of the development of movement and posture, causing activity limitation, that are attributed to nonprogressive disturbances that occurred in the developing fetal or infant brain” (Rosenbaum, Paneth, Leviton, and others, 2007). In addition to motor disorders, the condition often involves disturbances of sensation, perception, communication, cognition, and behavior; secondary musculoskeletal problems; and epilepsy (Rosenbaum, Paneth, Leviton, and others, 2007). The etiology, clinical features, and course vary and are characterized by abnormal muscle tone and coordination as the primary disturbances. CP is the most common permanent physical disability of childhood, and the incidence is reported to be between 2.4 to 3.6 per every 1000 live births in the United States (Hirtz, Thurman, Gwinn-Hardy, and others, 2007; Yeargin-Allsopp, Van Naarden Braun, Doernberg, and others, 2008). Since the 1960s, the prevalence of CP has risen approximately 20%, which most likely reflects the improved survival of extremely low–birth-weight and very low–birth-weight infants.


Although the prevalent traditional hypothesis has been that CP results from perinatal problems, especially birth asphyxia, it is now believed that CP results more often from existing prenatal brain abnormalities; the exact cause of these abnormalities remains elusive but may include genetic factors, including clotting disorders as well as brain malformations. It has been estimated that as many as 80% of CP cases are attributable to unidentified prenatal factors (Krigger, 2006). Intrauterine exposure to maternal chorioamnionitis is associated with an increased risk of CP in infants of normal birth weight and preterm infants (Hermansen and Hermansen, 2006); however, not all term infants exposed to chorioamnionitis develop CP (Grether, Nelson, Walsh, and others, 2003; Wu, Escobar, Grether, and others, 2003). Perinatal ischemic stroke is also associated with a later diagnosis of CP (Golomb, Saha, Garg, and others, 2007). Additional factors that may contribute to the development of CP postnatally include bacterial meningitis, multiple births, viral encephalitis, motor vehicle crashes, and child abuse (shaken baby syndrome [traumatic brain injury]) (Krigger, 2006). One study found a higher risk of CP occurring among infants born at 42 weeks or later than among those born at 37 or 38 weeks’ gestation (Moster, Wilcox, Vollset, and others, 2010). A significant percentage (15% to 60%) of children with CP also have epilepsy. In summary, as many as 80% of the total cases of CP may be linked to a perinatal or neonatal brain lesion or brain maldevelopment, regardless of the cause (Krageloh-Mann and Cans, 2009).



Pathophysiology


It is difficult to establish a precise location of neurologic lesions on the basis of etiology or clinical signs because there is no characteristic pathologic picture. In some cases, there are gross malformations of the brain. In others, there may be evidence of vascular occlusion, atrophy, loss of neurons, and laminar degeneration that produce narrower gyri, wider sulci, and low brain weight. Anoxia appears to play the most significant role in the pathologic state of brain damage, which is often secondary to other causative mechanisms.


There are a few exceptions. In some cases, the manifestations or etiology are related to anatomic areas. For example, CP associated with preterm birth is usually spastic diplegia caused by hypoxic infarction or hemorrhage with periventricular leukomalacia in the area adjacent to the lateral ventricles. The athetoid (extrapyramidal) type of CP is most likely to be associated with birth asphyxia but can also be caused by kernicterus and metabolic genetic disorders such as mitochondrial disorders and glutaricaciduria (Johnston, 2011). Hemiplegic (hemiparetic) CP is often associated with a focal cerebral infarction (stroke) secondary to an intrauterine or perinatal thromboembolism, usually a result of maternal thrombosis or hereditary clotting disorder (Johnston, 2011). Cerebral hypoplasia and sometimes severe neonatal hypoglycemia are related to ataxic CP. Generalized cortical and cerebral atrophy often cause severe quadriparesis with cognitive impairment and microcephaly.



Clinical Classification


A revision of the Winter classification was proposed in 2005 to reflect the child’s actual clinical problems and their severity, an assessment of the child’s physical and quality-of-life status across time, and long-term support needs (Bax, Goldstein, Rosenbaum, and others, 2005; Nehring, 2010). The proposed new definition has four major dimensions of classification (Bax, Goldstein, Rosenbaum, and others, 2005):



Cerebral palsy has four primary types of movement disorders: spastic, dyskinetic, ataxic, and mixed (Nehring, 2010). The most common clinical type, spastic CP, represents an upper motor neuron muscular weakness (Box 32-1). The reflex arc is intact, and the characteristic physical signs are increased stretch reflexes, increased muscle tone, and (often) weakness. Early neurologic manifestations are usually generalized hypotonia or decreased tone that lasts for a few weeks or may extend for months or even as long as 1 year.



Box 32-1


Clinical Classification of Cerebral Palsy






Data from Nehring W: Cerebral palsy. In Allen PJ, Vessey JA, Schapiro NA, editors: Primary care of the child with a chronic condition, ed 5, St. Louis, 2010, Mosby; Jones MW, Morgan E, Shelton JE, and others: Cerebral palsy: introduction and diagnosis, part 1, J Pediatr Health Care 21(3):146–152, 2007; and National Institute of Neurologic Disorders and Stroke: Cerebral palsy: hope through research, 2006, retrieved July 9, 2007, from www.ninds.nih.gov/disorders/cerebral_palsy/detail_cerebral_palsy.htm.



Diagnostic Evaluation


image Infants at risk according to known etiologic factors associated with CP warrant careful assessment during early infancy to identify the signs of neuromotor dysfunction as soon as possible. The neurologic examination and history are the primary means for diagnosis. Neuroimaging of the child with suspected brain abnormality and CP is now recommended for diagnostic assessment, with MRI preferred to CT scan. Metabolic and genetic testing is recommended if no structural abnormality is identified by neuroimaging; laboratory tests are no longer recommended in the diagnostic process for CP.


image Case Study—Cerebral Palsy


Early recognition is made more difficult by the lack of reliable neonatal neurologic signs. However, nurses should monitor infants with known etiologic risk factors and evaluate them closely in the first 2 years of life. Because cortical control of movement does not occur until later in infancy, motor impairment associated with voluntary control is usually not apparent until after 2 to 4 months of age at the earliest. More often the diagnosis cannot be confirmed until the age of 2 years because motor tone abnormalities may be indicative of another neuromuscular illness. In addition, some children who show signs consistent with CP before 2 years do not demonstrate such signs after 2 years (Nehring, 2010). However, there is no consensus regarding an age cut-off for the onset of symptoms. Clinical manifestations of CP at the time of diagnosis are listed in Box 32-2; early warning signs are listed in Box 32-3, but these are not considered diagnostic.



Box 32-2


Clinical Manifestations of Cerebral Palsy (at Time of Diagnosis)









*May or may not be present.


From Nehring WM: Cerebral palsy. In Allen PJ, Vessey JA, Schapiro NA, editors, Primary care of the child with a chronic condition, ed 5, St. Louis, 2010, Mosby/Elsevier. Adapted from Jones MW, Morgan E, Shelton JE: Primary care of the child with cerebral palsy: a review of systems (part II), J Pediatr Health Care 21, 226–237, 2007.



Establishing a diagnosis may be easier with the persistence of primitive reflexes: (1) either the asymmetric tonic neck reflex or the persistent Moro reflex (beyond 4 months of age) and (2) the crossed extensor reflex. The tonic neck reflex normally disappears between 4 and 6 months of age. An obligatory response is considered abnormal. This is elicited by turning the infant’s head to one side and holding it there for 20 seconds. When a crying infant is unable to move from the asymmetric posturing of the tonic neck reflex when crying, it is considered obligatory and an abnormal response. The crossed extensor reflex, which normally disappears by 4 months, is elicited by applying a noxious stimulus to the sole of one foot with the knee extended. Normally, the contralateral foot responds with extensor, abduction, and then adduction movements. The possibility of CP is suggested if these reflexes occur after 4 months.


A number of assessment instruments are now available to evaluate muscle spasticity; functional independence in self-care, mobility, and cognition; self-initiated movements over time; and capability and performance of functional activities in self-care, mobility, and social function (Krigger, 2006).



Therapeutic Management


The goals of therapy for children with CP are early recognition and promotion of optimal development to enable affected children to attain normalization and their potential within the limits of their existing health problems. The disorder is permanent, and therapy is primarily preventive and symptomatic.


Therapy has five broad goals:



Each child is evaluated and managed on an individual basis. The scope of the child’s needs requires multidisciplinary planning and care coordination among professionals and the child’s family. The outcome for the child and family with CP is normalization and promotion of self-care activities that empower the child and family to achieve maximum potential.


Ankle–foot orthoses (AFOs, braces) are worn by many of these children and are used to help prevent or reduce deformity, increase the energy efficiency of gait, and control alignment. Wheeled go-carts that provide sitting balance may serve as early “wheelchair” experience for young children. Manual or powered wheelchairs allow for more independent mobility (Figs. 32-1 and 32-2). Strollers can be equipped with custom seats for dependent mobilization.




Orthopedic surgery may be required to correct contracture or spastic deformities, to provide stability for an uncontrollable joint, and to provide balanced muscle power. This includes tendon-lengthening procedures, release of spastic muscles, and correction of hip and adductor muscle spasticity or contracture to improve locomotion. Hip dislocation often occurs in children with CP. Spinal fusion may be required for scoliosis. Computerized motion analysis, radiographs, and clinical findings are used to make decisions about orthopedic surgery. Selective dorsal rhizotomy provides marked improvement in some children with CP. The procedure involves selectively cutting dorsal column sensory rootlets that have an abnormal response to electrical stimulation. Achieving the benefits from the surgery requires intensive physical therapy and family commitment. Because the procedure results in flaccid muscles, the child must be retaught to sit, stand, and walk.


Surgical intervention is usually reserved for children who do not respond to the more conservative measures, but it is also indicated for children whose spasticity causes progressive deformities. Surgery is primarily used to improve function rather than for cosmetic purposes and is followed by physical therapy.


Intense pain may occur with muscle spasms in patients with CP. Pharmacologic agents given orally (dantrolene sodium, baclofen [Lioresal], and diazepam [Valium]) have had little effectiveness in improving muscle coordination in children with CP; however, they are effective in decreasing overall spasticity. The most common side effects of these agents include hepatotoxicity (dantrolene), drowsiness, fatigue, and muscle weakness; less commonly, diaphoresis and constipation may be seen with baclofen. Diazepam is used frequently but should be restricted to older children and adolescents.


Botulinum toxin A (Botox) is also used to reduce spasticity in targeted muscles. Botulinum toxin A is injected into a selected muscle (commonly the quadriceps, gastrocnemius, or medial hamstrings) after a topical anesthetic is applied. The drug acts to inhibit the release of acetylcholine into a specific muscle group, thereby preventing muscle movement. When it is administered early in the course of the condition, affected muscle contractures may be minimized, particularly in lower extremities, thus avoiding surgical procedures with possible adverse effects. The goal is to allow stretching of the muscle as it relaxes and permit ambulation with an AFO. The major reported adverse effects of botulinum toxin A injection are pain at the injection site and temporary weakness (Lukban, Rosales, and Dressler, 2009). Prime candidates for botulinum toxin A injections are children with spasticity confined to the lower extremities; the drug weakens spasticity so the muscles can be stretched and the child may walk with or without orthoses. The onset of action occurs within 24 to 72 hours, with a peak effect observed at 2 weeks and a duration of action of 3 to 6 months.


Children with CP may also experience pain as a result of surgical procedures intended to reduce contracture deformities, body position, gastroesophageal reflux, and physical therapy (McKearnan, Kieckhefer, Engel, and others, 2004). Therefore, pain management is an important aspect of care of children with CP.


The neurosurgical and pharmacologic approach to managing the spasticity associated with CP involves the implantation of a pump to infuse baclofen directly into the intrathecal space surrounding the spinal cord to provide relief of spasticity. Intrathecal baclofen therapy is best suited for children with severe spasticity that interferes with activities of daily living (ADLs) and ambulation. Patients may be screened before pump placement by the infusion of a “test dose” of intrathecal baclofen delivered via a lumbar puncture. Close monitoring for side effects (hypotonia, somnolence, seizures, nausea, vomiting, headache) occurs. Relief of spasticity occurs for several hours after the infusion. If a positive effect is noted, the patient is considered a candidate for pump placement. The implantation procedure is done in the operating room by a neurosurgeon. The pump, which is approximately the size of a hockey puck, is placed in the subcutaneous space of the midabdomen. An intrathecal catheter is tunneled from the lumbar area to the abdomen and connected to the pump. The pump is filled with baclofen and programmed to provide a set dose using a telemetry wand and a computer. Benefits of intrathecal baclofen include fewer systemic side effects than oral baclofen, dosage titration for maximizing effects, and reversibility of therapy with removal of the pump if so desired. The patient may remain hospitalized for 3 to 7 days to adjust the dosage and ensure proper healing. Outpatient visits to refill the pump and make dosage adjustments occur about every 3 to 6 months, depending on the patient’s response to the treatment. This procedure is most suited for a multidisciplinary setting where rehabilitation specialists are readily available and consistently involved in the patient’s ongoing care. Abrupt withdrawal of intrathecal baclofen may result in adverse effects such as rebound spasticity, pruritus, hyperthermia, rhabdomyolysis, disseminated intravascular coagulation, multiorgan failure, and death; in some cases, intrathecal baclofen withdrawal may mimic sepsis.


Antiepileptic drugs (AEDs) such as carbamazepine (Tegretol) and divalproex (valproate sodium and valproic acid; Depakote) are prescribed routinely for children who have seizures. Other medications include levodopa to treat dystonia; Artane for treating dystonia, and for increasing the use of upper extremities and vocalizations; and reserpine for hyperkinetic movement disorders such as chorea or athetosis (Johnston, 2011). All medications should be monitored for maintenance of therapeutic levels and avoidance of subtherapeutic or toxic levels.


Dental hygiene is essential in the care of children with CP. Regular visits to the dentist and prophylaxis, including brushing, fluoride, and flossing, should be started as soon as the teeth erupt. Dental care is especially important for children being given phenytoin because they often develop gum hyperplasia. Decreased oral intake can lead to more tartar buildup. Additional problems common among children with CP include constipation caused by neurologic deficits and lack of exercise, poor bladder control and urinary retention, chronic respiratory tract infections, problems with airway clearance, and aspiration pneumonia. These occur as a result of gastroesophageal reflux, abnormal muscle tone, immobility, and altered positioning, and skin problems may occur as a result of altered positioning, poor nutrition, and immobility.


A wide variety of technical aids are available to improve the functioning of children with CP. Airway clearance devices help mobilize secretions. Eye–hand coordination can also be enhanced by computerized toys and games. Toys may be operated by a head or hand switch. Microcomputers combined with voice synthesizers aid children with speech difficulties to “speak.” Smart phones with speech applications are appropriate for some children.


Many other electronic devices allow independent functioning. Sensors can be activated and deactivated by using a head stick or tongue or other voluntary muscle movement over which the child has control. Voice-activated computer technology may also allow increased mobility and ambulation with specially designed devices such as wheelchairs. The application of this technology makes it possible for persons with CP to function in their own residences and can be extended into the workplace.


There is some evidence that neuromuscular electrical stimulation (NMES) in addition to dynamic splinting may result in increased muscle strength, range of motion, and function of upper limbs in children with CP. Further studies are needed in children with CP to support the use of botulinum toxin A in conjuction with NMES to decrease muscle spasticity and improve function (Wright, Durham, Ewins, and others, 2012).


Behavior problems may occur and often interfere with the child’s development. Attention-deficit/hyperactivity disorder and other learning problems require professional attention. In addition, children with CP may have vision difficulties such as strabismus, nystagmus, and optic atrophy (Johnston, 2011). Speech-language therapy involves the services of a speech-language pathologist who may also assist with feeding problems.


Physical therapy is one of the most frequently used conservative treatment modalities. It requires the specialized skills of a qualified therapist with an extensive repertoire of exercise methods who can design a program to stimulate each child to achieve his or her functional goals.


An active therapy program involves the family; the physical therapist; and often other members of the health team, including the nurse. The most common approach uses traditional types of therapeutic exercises that consist of stretching, passive, active, and resistive movements applied to specific muscle groups or joints to maintain or increase range of motion, strength, or endurance.



Prognosis

The prognosis for the child with CP depends largely on the type and severity of the condition. Children with mild to moderate involvement (85%) have the capability of achieving ambulation between the ages of 2 and 7 years (Berker and Yalçin, 2008). If the child does not achieve independent ambulation by this time, chances are poor for ambulation and independence. Approximately 30% to 50% of individuals with CP have significant cognitive impairments, and an even higher percentage have mild cognitive and learning deficits. However, many children with severe spastic tetraplegic CP have normal intelligence. Growth is affected in children with spastic tetraplegia, and many children remain below the 5th percentile for age and sex.


As children with CP become adults, about 30% remain in the home and are cared for by a parent or caregiver; 50% of individuals with spastic tetraplegia live in independent settings and function at appropriate social levels considering their disability (Green, Greenberg, and Hurwitz, 2003). Vocational rehabilitation and higher education are possible for adults with CP. Children with severe CP mobility impairment and feeding problems often succumb to respiratory tract infection in childhood. The few survival rate studies on children or adults with CP show that survival is influenced by existing comorbidities (Nehring, 2010).


Prevention of some cases of CP may become a reality in the near future. Studies indicate that early neuroprotection in term infants with the use of therapeutic hypothermia (head cooling or whole-body cooling) within 6 hours of birth improved survival without CP by approximately 40% (Johnston, Fatemi, Wilson, and others, 2011).




Nursing Care Management


image Because children with CP are being identified and treated at an earlier age, parents are participating earlier in treatment programs for their children with disabilities. They are taught the proper handling and home care of young children with CP and need a carefully planned program so that their change of role from parent to caregiver can be melded into the already established relationship. Close work with other multidisciplinary team members is essential. Nurses reinforce the therapeutic plan and assist the family in devising and modifying equipment and activities to continue the therapy program in the home. The nursing process in the care of the child with CP is outlined in the Nursing Care Plan.



image Nursing Care Plan


The Child with Cerebral Palsy

















































































































Nursing Diagnosis Patient Outcomes Nursing Interventions Rationale


Carry out and teach family to perform stretching exercises on affected muscles. To prevent muscle contractures
Use assistive devices such as wheelchair, AFOs, and wrist splints. To increase mobility and prevent contractures
Administer medications (specify) intended to decrease muscle spasticity. To minimize pain and decrease spasticity
Encourage and teach parent(s) to use jaw control during feedings. To facilitate eating
Position child semiupright during feedings. To decrease chance of aspiration and facilitate mobilization of food and fluids through esophagus
Encourage play exercises that involve joint movement and promote fine and gross motor skill acquisition and repetition. To promote joint movement
To promote achievement of developmental milestones

 


Educate family regarding child’s physical limitations that place him or her at greater risk for injury. To prevent accidental injury during mobilization
Instruct family in steps to avoid injury, including padded furniture, lowered bed or side rails as appropriate, gates on stairs, avoidance of throw rugs, thick carpeting. To promote family involvement in injury prevention
Position child in semiupright position after feedings. To prevent aspiration
Assess child’s ability to manage (chewing and swallowing) oral feedings To determine appropriate feeding method and prevent aspiration
Use jaw support as needed during feedings. To prevent choking and possible aspiration
Use appropriate mobilization devices and ensure they are safe for child’s age. To prevent muscle contractures
    Encourage mobilization and play activities that stretch muscles. To promote personal safety
    Teach child which ADLs are safe and appropriate to perform without assistance of another person. To promote self-care
   
 


Administer medications to control spasticity (specify). To prevent muscle spasm pain
Perform stretching exercises after pain medication has been administered (60 minutes for oral medications). To manage pain impulses during exercises
Administer pain medications (specify) such as NSAIDs. To minimize pain
For treatments such as botulinum toxin A (Botox) injections, assist with administration of appropriate pain medications and monitoring child for pain sensation (specify). To decrease pain of injection at site
    For postoperative pain, administer pain medications on an around-the-clock schedule for 48 to 72 hours; use PCA pump as child’s cognitive and motor skills allow. To promote personal physical comfort
    Use objective pain scale to assess pain level. To provide objective measure of pain for intervention
    Encourage child to verbalize effects of pain on ADLs. To provide outlet for frustration related to chronic pain experience
    Use assistive devices such as AFOs or KAFOs. To decrease muscle spasticity and contractures.
    Teach parent(s) and child appropriate positions to assume while sitting and recumbent to minimize effects of muscle spasticity. To promote self-care
   
 


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ADLs, Activities of daily living; AFO, ankle–foot orthosis; KAFO, knee–ankle–foot orthoses; NIC, Nursing Interventions Classification; NOC, Nursing Outcomes Classification; NSAID, nonsteroidal antiinflammatory drug; PCA, patient-controlled analgesia.



image Nursing Care Plan—The Child with Cerebral Palsy


Because children with CP expend so much energy in their efforts to accomplish ADLs, more frequent rest periods should be arranged to avoid fatigue. The diet should be tailored to the child’s activity and metabolic needs. Gastrostomy feedings may be necessary to supplement regular feedings and ensure adequate weight gain, particularly in children at risk for growth failure and chronic malnutrition, those with severe CP and subsequent oral feeding difficulties, and children whose well-being is affected by illness and decreased fluid or medication intake (Rogers, 2004). Oral feedings may be continued to maintain oral motor skills. Weight gain is perceived as an important measure of adequate oral feeding efficiency.


Parents may need assistance and advice with medication administration through a gastrostomy tube to prevent clogging. A skin-level gastrostomy is particularly suited for children with CP. Because jaw control is often compromised, more normal control can be achieved if the feeder provides stability of the oral mechanism from the side or front of the face. When directed from the front, the middle finger of the nonfeeding hand is placed posterior to the body portion of the chin, the thumb is placed below the bottom lip, and the index finger is placed parallel to the child’s mandible (Fig. 32-3). Manual jaw control from the side assists with head control, correction of neck and trunk hyperextension, and jaw stabilization. The middle finger of the nonfeeding hand is placed posterior to the bony portion of the chin, the index finger is placed on the chin below the lower lip, and the thumb is placed obliquely across the cheek to provide lateral jaw stability (Fig. 32-4).




Safety precautions are implemented, such as having children wear protective helmets if they are subject to falls or capable of injuring their heads on hard objects. Because children with CP are at risk for altered proprioception and subsequent falls, the home and play environments should be adapted to their needs to prevent bodily harm. Appropriate immunizations should be administered to prevent childhood illnesses and protect against respiratory tract infections such as influenza or pneumonia. Dental problems may be more common in children with CP, which creates a need for meticulous attention to all aspects of dental care. Transportation of the child with motor problems and restricted mobility may be especially challenging for the family and child. Attention must be given to the child’s safety when riding in a motor vehicle; a federally approved safety restraint should be used at all times. It is recommended that children with CP ride in a rear-facing position as long as possible because of their poor head, neck, and trunk control (Lovette, 2008). Car restraints especially designated for children with poor head and neck control are available and should be used.*


The involvement of physical therapy, speech therapy, and occupational therapy is particularly important in establishment and maintenance of muscle function, development of adequate speech and phonation, and identification of modifications necessary for the child’s environment so that ADLs can be performed to the child’s satisfaction.


As in all aspects of care, educational requirements are determined by the child’s needs and potential. Children with mild to moderate cognitive involvement are generally able to participate in regular classes. Resource rooms are available in most schools to provide more individualized attention. Integration of children with CP into regular classrooms should be the initial goal. For those who are unable to benefit from formal education, a vocational training program may be appropriate. At adolescence, prevocational and vocational counseling and guidance are arranged. At any phase or in any setting, education is geared toward the child’s assets.


Recreation and after-school activities should be considered for children who are unable to participate in the regular athletic programs and other peer activities. Some children can compete in athletic and artistic endeavors, and many games and pastimes are suited to their capabilities. Competitive sports are also becoming increasingly available to children with disabilities and offer an added dimension to physical activities. Recreational activities serve to stimulate children’s interest and curiosity, help them adjust to their disability, improve their functional abilities, and build self-esteem. Any accomplishment that helps children approach a normal way of life enhances their self-concept.



Support the Family

Probably the nursing interventions most valuable to the family are support and help in coping with the emotional aspects of the disorder, many of which are discussed in relation to the child with a disability (see Chapter 18). Initially, the parents need supportive counseling directed toward understanding the meaning of the diagnosis and all of the feelings that it engenders. Later they need clarification regarding what they can expect from the child and from health professionals. Educating families in the principles of family-centered care and parent–professional collaboration is essential. The family may require help in modifying the home environment for care of the child (see also Chapter 20). Transportation to the practitioner’s office and other health care agencies often requires special arrangements.


Care coordination for the child and family with CP is an important nursing role. The home health nurse or care manager has an important role in the support and encouragement for families who assume the primary care of a child with CP. Having a child with CP implies numerous problems of daily management and changes in family life, and the nurse can help with education, assessment, and mobilization of resources.


The nurse needs to support the parents in their frustration, problem solving, concerns, approaches to helping the child, and the positive approaches they use. Parents and other family members may need support and counseling. Siblings of a child with a disability are affected and may respond to the child’s presence with overt or less evident behavioral problems. The family needs a relationship with nurses who can provide continued contact, support, and encouragement through the long process of habilitation.


Parents may also find help and comfort from parent groups, with whom they can share problems and concerns and from whom they can derive comfort and practical information. Parent support groups are most helpful through sharing experiences and accomplishments. For example, parents can learn from others what it is like to have a child with CP, which is generally not possible from professionals (see Family-Centered Care box). The national organization United Cerebral Palsy* has branches in most communities. The association provides a variety of services for children and families. A number of excellent books also are available to guide parents and nurses who work with children with CP.




Support Hospitalized Child

Cerebral palsy is not a disorder that requires ongoing hospitalization; therefore, when children with CP are hospitalized, they are usually admitted for illness or corrective surgery. Nursing care of the child with CP similar to that of any child with a disability, and children with CP should be approached as would any child in the hospital. Speech impairment is common in children with CP, but this may not correlate with their ability to understand. Therapy programs should be continued, when appropriate, during the time they are hospitalized. Encouraging the parent to room-in and actively participate in the child’s care helps promote family-centered care. However, it is also important to remember that hospitalization may be the first time a parent can defer care to a nurse and not be the primary caregiver. Discuss this with the family to find the right level of involvement for them.



Neural Tube Defects (Myelomeningocele)


Abnormalities that derive from the embryonic neural tube (neural tube defects [NTDs]) constitute the largest group of congenital anomalies that are consistent with multifactorial inheritance. Normally, the spinal cord and cauda equina are encased in a protective sheath of bone and meninges (Fig. 32-5, A). Failure of neural tube closure produces defects of varying degrees (Box 32-4). They may involve the entire length of the neural tube or may be restricted to a small area.




In the United States, rates of NTDs have declined from 1.3 per 1000 births in 1970 to 0.3 per 1000 births after the introduction of mandatory food fortification with folic acid in 1998. One concern is that NTD rates have not decreased among Hispanic and non-Hispanic white mothers since 1999 (Centers for Disease Control and Prevention [CDC], 2009). In 2005, the rates for spina bifida (SB) were estimated by the CDC to be 17.96 per 100,000 live births, thus making this one of the most common birth defects in the United States (Matthews, 2009; Wolff, Witkop, Miller, and others, 2009). Increased use of prenatal diagnostic techniques and termination of pregnancies have also affected the overall incidence of NTDs. (see also Prevention, p. 1102).


Anencephaly, the most serious NTD, is a congenital malformation in which both cerebral hemispheres are absent. The condition is usually incompatible with life, and many affected infants are stillborn. For those who survive, no specific treatment is available. The infants have a portion of the brainstem and are able to maintain vital functions (e.g., temperature regulation and cardiac and respiratory function) for a few hours to several weeks but eventually die of respiratory failure.


Myelodysplasia refers broadly to any malformation of the spinal canal and cord. Midline defects involving failure of the osseous (bony) spine to close are called spina bifida (SB), the most common defect of the central nervous system (CNS). SB is categorized into two types, SB occulta and SB cystica.


Spina bifida occulta refers to a defect that is not visible externally. It occurs most frequently in the lumbosacral area (L5 and S1) (Fig. 32-5, B). SB occulta may not be apparent unless there are associated cutaneous manifestations or neuromuscular disturbances.


Spina bifida cystica refers to a visible defect with an external saclike protrusion. The two major forms of SB cystica are meningocele, which encases meninges and spinal fluid but no neural elements (Fig. 32-5, C), and myelomeningocele (or meningomyelocele), which contains meninges, spinal fluid, and nerves (Fig. 32-5, D). Meningocele is not associated with neurologic deficit, which occurs in varying, often serious, degrees in myelomeningocele. Clinically, the term spina bifida is used to refer to myelomeningocele.



Pathophysiology


The pathophysiology of SB is best understood when related to the normal formative stages of the nervous system. At approximately 20 days of gestation, a decided depression, the neural groove, appears in the dorsal ectoderm of the embryo. During the fourth week of gestation, the groove deepens rapidly, and its elevated margins develop laterally and fuse dorsally to form the neural tube. Neural tube formation begins in the cervical region near the center of the embryo and advances in both directions—caudally and cephalically—until by the end of the fourth week of gestation, the ends of the neural tube, the anterior and posterior neuropores, close.


Most authorities believe the primary defect in neural tube malformations is a failure of neural tube closure. However, some evidence indicates that the defects are a result of splitting of the already closed neural tube as a result of an abnormal increase in cerebrospinal fluid (CSF) pressure during the first trimester.



Etiology


There is evidence of a multifactorial etiology, including drugs, radiation, maternal malnutrition, chemicals, and possibly a genetic mutation in folate pathways in some cases, which may result in abnormal development. There is also evidence of a genetic component in the development of SB; myelomeningocele may occur in association with syndromes such as trisomy 18, PHAVER (limb pterygia, congenital heart anomalies, vertebral defects, ear anomalies, and radial defects) syndrome, and Meckel-Gruber syndrome (Shaer, Chescheir, and Schulkin, 2007). Additional factors predisposing children to an increased risk of NTDs include prepregnancy maternal obesity, maternal diabetes mellitus, low maternal vitamin B12 status, maternal hyperthermia, and the use of AEDs in pregnancy. The genetic predisposition is supported by evidence of the risk of recurrence after one affected child (3%–4%) and a 10% risk of recurrence with two previously affected children (Kinsman and Johnston, 2011).


The degree of neurologic dysfunction depends on where the sac protrudes through the vertebrae, the anatomic level of the defect, and the amount of nerve tissue involved. The majority of myelomeningoceles (75%) involve the lumbar or lumbosacral area (Fig. 32-6). Hydrocephalus is a frequently associated anomaly in 80% to 90% of the children. About 80% of patients with myelomeningocele develop a type II Chiari malformation (Kinsman and Johnston, 2011).




Diagnostic Evaluation


The diagnosis of SB is made on the basis of clinical manifestations (Box 32-5) and examination of the meningeal sac. Diagnostic measures used to evaluate the brain and spinal cord include MRI, ultrasonography, and CT. A neurologic evaluation will determine the extent of involvement of bowel and bladder function as well as lower extremity neuromuscular involvement. Flaccid paralysis of the lower extremities is a common finding with absent deep tendon reflexes.




Prenatal Detection

It is possible to determine the presence of some major open NTDs prenatally. Ultrasonographic scanning of the uterus and elevated maternal concentrations of α-fetoprotein (AFP, or MS-AFP), a fetal-specific γ-1-globulin, in amniotic fluid may indicate anencephaly or myelomeningocele. The optimum time for performing these diagnostic tests is between 16 and 18 weeks of gestation before AFP concentrations normally diminish and in sufficient time to permit a therapeutic abortion. It is recommended that such diagnostic procedures and genetic counseling be considered for all mothers who have borne an affected child, and testing is offered to all pregnant women (Kirkham, Harris, and Grzybowski, 2005). Chorionic villus sampling is also a method for prenatal diagnosis of NTDs; however, it carries certain risks (skeletal limb depletion) and is not recommended before 10 weeks of gestation.



Therapeutic Management


Management of the child who has a myelomeningocele requires a multidisciplinary team approach involving the specialties of neurology, neurosurgery, pediatrics, urology, orthopedics, rehabilitation, physical therapy, occupational therapy, and social services, as well as intensive nursing care in a variety of specialty areas. The collaborative efforts of these specialists focus on (1) the myelomeningocele and the problems associated with the defect—hydrocephalus, paralysis, orthopedic deformities (e.g., developmental dysplasia of the hip, clubfoot; scoliosis), and genitourinary abnormalities; (2) possible acquired problems that may or may not be associated, such as Chiari II malformation, meningitis, seizures, hypoxia, and hemorrhage; and (3) other abnormalities, such as cardiac or gastrointestinal (GI) malformations. Many hospitals have routine outpatient care by multidisciplinary teams to provide the complex follow-up care needed for children with myelodysplasia.


Many authorities believe that early closure, within the first 24 to 72 hours, offers the most favorable outcome. Surgical closure within the first 24 hours is recommended if the sac is leaking CSF (Kinsman and Johnston, 2011).


A variety of neurosurgical and plastic surgical procedures are used for skin closure without disturbing the neural elements or removing any portion of the sac. The objective is satisfactory skin coverage of the lesion and meticulous closure. Wide excision of the large membranous covering may damage functioning neural tissue.


Associated problems are assessed and managed by appropriate surgical and supportive measures. Shunt procedures provide relief from imminent or progressive hydrocephalus (see Chapter 28). When diagnosed, ventriculitis, meningitis, urinary tract infection, and pneumonia are treated with vigorous antibiotic therapy and supportive measures. Surgical intervention for Chiari II malformation is indicated only when the child is symptomatic (i.e., high-pitched crowing cry, stridor, respiratory difficulties, oral-motor difficulties, upper extremity spasticity).


Early surgical closure of the myelomeningocele sac through fetal surgery has been evaluated in relation to prevention of injury to the exposed spinal cord tissue and the improvement of neurologic and urologic outcomes in the affected child. The Management of Myelomeningocele Study, a clinical trial supported by the National Institute of Health, found that prenatal surgery for myelomeningocele reduced the need for shunting (for hydrocephalus), evaluated at 12 months, and there was an improvement in mental and motor function scores at 30 months in the children who had prenatal surgery (compared with children who had postnatal surgery) (Adzick, Thom, Spong, and others, 2011). Outcome data for urologic and bowel function are not available at this time.



Infancy

Initial care of the newborn involves preventing infection; performing a neurologic assessment, including observing for associated anomalies; and dealing with the impact of the anomaly on the family. Although meningoceles are repaired early, especially if there is danger of rupture of the sac, the philosophy regarding skin closure of myelomeningocele varies. Most authorities believe that early closure, within the first 24 to 72 hours, offers the most favorable outcome. Early closure, preferably in the first 12 to 18 hours, not only prevents local infection and trauma to the exposed tissues but also avoids stretching of other nerve roots (which may occur as the meningeal sac expands during the first hours after birth), thus preventing further motor impairment. Broad-spectrum antibiotics are initiated, and neurotoxic substances such as povidone–iodine are avoided at the malformation.


Improved surgical techniques do not alter the major physical disability and deformity or chronic urinary tract and pulmonary infections that affect the quality of life for these children. Superimposed on these physical problems are the disorder’s effects on family life and finances and on school and hospital services.

Jan 16, 2017 | Posted by in NURSING | Comments Off on The Child with Neuromuscular or Muscular Dysfunction
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