Top Causes of Mortality in Children

Infants: congenital anomalies, RDS, LBW, weight r/t death (complications of prematurity), SIDS

What amount of children are living with complex chronic conditions in the US?

2.2 - 3.7 million

Children Dealing with Emotions r/t Dying

• Children decide to hide their emotions from parents and others
• Emotions don't differ from adults, but the ability to tolerate emotions can be of limited duration.
• Want to be like peers
• Isolation
• May see illness and procedures as punishment
• Young children are very egocentric
• KNOW THEY ARE DYING

Cognitive Concepts of Death

• Universality: everyone will die
• Finality: permanent cessation of bodily functions
• Nonfunctionality: once the body dies, all functions cease

If a child denies feeling any pain should you always take their word for it?

No, school-age children may deny pain because they don't want a shot or to be hospitalized (not seen in adolescents)

Possible Physical Signs/Symptoms of Pain

• Facial expression of discomfort
• Immobility or guarding of body area
• Elevated pulse or respirations
• Irritability or restlessness
• Decreased appetite
• Crying

Developmental Responses to Pain

• Infants: irritability, crying, poor feeding
• Toddlers: quiet, regressive behavior, uncooperative
• Preschoolers: procrastination/pain is punishment
• School-age: deny pain
• Adolescents: fears loss of control

At what age is self-reported pain valid?

Over the age of 4 years

COMFORT Scale

• ONLY tool recommended for use with unconscious and ventilated infants, children, and adolescents
• 8 indicators (score each between 1 and 5)
• Alertness; calmness/agitation; respiratory response
• Physical movement; BP; HR
• Muscle tone; facial tension
• Observe for 2 minutes and add the scores of each indicator
• Ranges from 8-40
• Score of 17-26 = adequate sedation and pain control

Poker Chip Tool

• Set of 4 red plastic poker chips
• Each chip = a "piece of hurt"
• Scores 0-4
• Good psychometric testing

FACES Pain Scale

• Wong-Baker FACES widely used in the US
• Wong-Baker 6 cartoon faces from "no pain" to "worst pain"
• Bieri Faces Revised: six faces 0-5; no smiling face at "no pain" and no tears at "most pain"; equivalent to 0-10 metric system

Numeric Pain Ratings

• For ages 8 and older
• 0-10 scale is widely used
• Easy to use

Verbal Analog Scale (VAS)

• "No hurt" to "biggest hurt" are more appropriate than "least pain" to "worst pain imaginable"
• Requires higher degree of abstract thinking than numeric pain scale (NRS)
• Recommended because of lack of supportive evidence through psychometric testing with the NRS in children and adolescents

Adolescent Pediatric Pain Tool (APPT)

• Assesses pain location, intensity, and quality
• Anterior and posterior body outline on one side; 100 mm word-graphing rating scale with a pain descriptor
• Facilitates assessments of pain quality and location

Pediatric Pain Questionnaire (PPQ)

• Assesses patient and parental perceptions of pain
• Cognitive and developmental considerations
• 8 ares of inquiry: pain history, pain language, colors associated with pain, experience, worst pain experiences, ways children cope with pain, positive aspects of pain, and location of child's current pain
• Three components of PPQ: VASs, color-coded rating scales, and verbal descriptors
• The child, parent, and physician each complete form separately

CRIES Neonatal Pain Scale

• Crying
• Requiring increased oxygen
• Increased vital signs
• Expression
• Sleeplessness

Premature Infant Pain Profile (PIPP)

• Specifically developed for premature infants
• Gives higher pain score to infants with lower gestational age
• Gives higher pain score to blunted behavioral response

Neonatal Pain, Agitation, and Sedation Scale (NPASS)

• Used in neonates from 23 weeks of gestation up to 100 days of age
• Measures 5 criteria in two dimensions: pain and sedation

Measuring Pain in Children with Communication and Cognitive Impairment

• Difficult to measure pain; high risk for inadequate tx of pain
• NCCPC: non-communicating children's pain checklist
• PICIC: pain indicator for communicatively impaired children

Cultural Influences on Pediatric Pain Scales

• Oucher Pain Scale: for 3-12 year olds; validated with African American and Caucasian children
• Hispanic versions of APPT scale available for children/adolescents with cancer

Nonpharmacologic Pain Interventions for Infants

• Containment
• Postioning
• Nonnutritive sucking
• Kangaroo holding

Patient-Controlled Analgesia (PCA) 

• Basal rate: continuous
• Bolus dose: can be administered by patient, parent, or nurse

Use of Morphine in Pain Management in Children

GOLD STANDARD; drug of choice for PCA

• 1 mg/ml typical of PCA usage

Other Opioids Used in Pain Management for Children

• Hydromorphone
• Fentanyl: side effect is rigid chest syndrome
• Meperidine (Demerol)
• Oxycodone: available with or without acetaminophen; total dose of acetaminophen considered

Use of Meperidine (Demerol) in Pain Management for Children

• May increase risk for seizures due to excitatory effects on nervous system; try not to use with sickle cell patients
• Recommended use for brief treatment of patients who have shown its effectiveness or who have allergies or uncorrectable intolerances to other opioids
• Maximum usage: 48 hours or 600 mg/24 hours

Coanalgesics or Adjuvant Analgesics

• Used alone or with opioids to control symptoms or side effects
• Anxiolytics, sedatives, amnesics: diazepam (Valium) or midazolam (Versed)
• TCAs and antiepileptics for neuropathic pain
• Stool softeners for diarrhea
• Antiemetics for n/v
• Diphenhydramine for itching

Placebo Use in Children

• Side effects similar to those of opioids
• May destroy trust; raises serious ethical/legal questions
• American Society of Pain Management Nursing is against use of placebos

Epidural Anesthesia

• Opioid: fentanyl, hydromorphone, or preservative-free morphine
• Local: bupivicaine or ropivicaine
• Instilled via single or intermittent bolus, continuous infusion, or PCEA

Nursing Care of Child with Epidural Anesthesia

• Careful monitoring for respiratory depression
• Skin care around catheter insertion site
• Assessment of pain

Transmucosal and Transdermal Analgesia

• Oral transmucosal fentanyl (Oralet)
• Fentanyl transdermal patch (Duragesic): chronically ill
• Anesthetic creams (EMLA, LMX): local for IV insertion
• Refrigerant sprays (ethyl chloride and fluorimethane)
• Numby stuff
• LidoSite activated patch

Lidocaine

• Side effects: stinging and burning on injection
• Buffered lidocaine: decreased or eliminated stinging
• Warming lidocaine to body temp: decreased or eliminated stinging

Tolerance to Pain Medication

• Dose must be increased to achieve the same effect
• May develop after 10-21 days
• Treatment: increase dose; decrease duration between doses

Treatment of Physical Dependence

• Gradually reduce dose: one half dose q 6 hours for first 2 days; reduce by 25% q 2 days until totaly daily dose of 0.6 mg/kg/day of morphine (or equivalent) is reached; after 2 days on this dose, discontinue opioid
• May also switch to oral methadone, using 1/4 of equianalgesic dose as initial weaning dose and proceeding as described above

Consequences of Unrelieved Pain in Neonates

• Increased ICP, HR, RR, BP
• Decreased SaO2
• Muscle rigidity, facial expression, crying, withdrawal, and sleeplessness

Sedation for Nonpainful Procedures

• Chloral hydrate, nembutal
• Careful monitoring during and after procedure
• Decrease stimuli after procedure
• "Nembutal rage": agitation during emergence; nembutal severely depresses respiratory system
• Chloral hydrate: little effect on resp. and BP

"Windup Phenomenon"

• Studied in neonates
• Attributed to decreased pain threshold + chronic pain
• After exposure to pain, altered excitability at multiple levels of spinal cord
• Nonnoxious stimuli is perceived as noxious
• Same physiologic response to stress
• Results in chronic pain; biologic and clinical consequences

Conscious Sedation (Minimal Level of Sedation)

• Protective reflexes are maintained
• Able to maintain a patent airway independently and continuously
• Patient responds to physical stimulation or verbal command (ex. "open your eyes")
• Cognitive function may be impaired

Moderate Sedation 

• Patient not easily aroused but responsible purposefully to verbal commands, either alone or accompanied by light touch
• May return partial or no protective reflexes
• Airway, spontaneous ventilation, and cardiovascular function may or may not be adequate without interventions (ex. oxygen)
• NOT EASILY AROUSED

Deep Sedation/Anesthesia

• Patient cannot be easily aroused
• No protective reflexes
• Unable to maintain a patent airway; ventilatory and cardiovascular function independently may be impaired
• Continuous monitoring q 5 minutes

Emergency Equipment for Moderate and Deep Sedation

• Suction apparatus with catheters
• Oxygen source, oral and nasal airways, ETTs, laryngoscope/blades, Ambu bag/mask
• Medications for resuscitation and drug antagonism
• IV access
• Trained individual (MD, RN) whose sole responsibility is to monitor patient

Pain in Children with Sickle Cell Disease

• Opioids are considered the major therapy and are started in early childhood and continued throughout adult life
• Nonpharmacologic interventions
• Goals of treatment of acute episodes
• Pseudoaddiction

Pain Management for Childhood Cancers

• Sources/type of pain: bone, visceral, neuropathic, treatment-related
• Interventions
• Obstacles to successful analgesia

Ketamine Use in Children

• Often used for analgesia and sedation during procedures in kids
• Subanesthetic doses uses plus high dose opioids for uncontrolled cancer pain
• Appeared to improve pain control and to have opioid-sparing effect; dosage range (0.1 to 1 mg/kg/hr) lower than that used for anesthetic purposes
• Give lorazepam (0.025 mg/kg/12 hr) concurrently with ketamine
• Continuous monitoring (BP, RR, HR, SaO2)

Definition of Cognitive Impairment

Any type of intellectual disability

Diagnostic Criteria for CI

• Emphasis on functional strengths and weaknesses and supports needed
• Classifications describe real-world adaptiave skills
• Onset before age 18
• Subaverage intellectual functioning (IQ=70-75 or below)
• Functional impairment in at least 2 adaptive skill domains: communication; self-care; home living; social skills; use of community resources; self-direction; health and safety; functional academics; leisure; work

Developmental Delay (Definition)

• Any significant lag or delay in physical, cognitive, behavioral, emotional, or social development
• Routine developmental screening can assist in early identification
• Most common delays: language and cognitive skills; fine and gross motor skills

CI Emphasis

• Emphasizes abilities, environments, supports, and empowerment
• Classifications of support required: intermittent, limited, extensive, or pervasive
• Underlying assumption: function improves with support

Etiology of CI

• Familial, social, environmental, organic, and unknown causes
• Chromosomal disorders: Down Syndrome and Fragile X Syndrome
• Prenatal toxin exposure: FAS accounts for sizable proportion of causes
• Other identifiable disorders or syndromes: severe CP, microcephaly, and infantile spasms

Diagnostic Tests for CI

• Bayley Scales of Infant Development
• Mullen Scale of Early Learning
• The Harris Infant Neuromotor Test (HINT)
• Wechsler Intelligence Scale for Children for school-agers
• Stanford-Binet Intelligence Scale and Kaufman Assessment Battery from toddlerhood through school age
• Leiter International Performance Scale for children who speak a different language, nonverbal children, or those with significant language or motor impairment
• Individually tested by specially trained clinicians

Primary Prevention of CI 

• Avoidance of prenatal rubella infection - keep immunizations current
• Genetic counseling, especially regarding risk of Down syndrome or Fragil X syndrome
• Use of folic acid supplements 
• Education regarding dangers of smoking or ETOH use during pregnancy and ingesting lead during childhood
• Reduction of head injuries
• Future gene therapy for genetic disorders, such as PKU

Secondary Prevention of CI

• Early identification to initiate treatment to avert damage
• Prenatal diagnosis or carrier detection of disorders such as Down syndrome
• Newborn screening for treatable inborn errors of metabolism: congenital hypothyroidism, PKU, galactosemia

Tertiary Prevention of CI

• Tx to minimize LT consequence
• Early identification of therapies and rehabilitation services
• Treatment of coexisting problems; programs for infant stimulation, parent training, preschool education; counseling services to preserve the family unit

Goal for Caring for CI

• Promote optimum social, physical, cognitive, and adaptive development as individuals within a family and community

Early Behavioral Signs of Cognitive Impairment

• No response to contact, voice, movement
• Irritability
• Poor/slow feeding
• Poor eye contact during feeding
• Diminished spontaneous activity

Promoting Optimum Development

• Acceptable social behavior
• Personal feelings of self-esteem, worth, and security

Development of Communication

• Both receptive and expressive skills
• Sign language
• Message boards

Discipline in the child with CI

• Simple, consistent limit setting
• Must be appropriate for child's mental age
• Control measures based on specific behavior
• Behavior modification: reinforcement of desired actions; use of time-out

Socialization in the child with CI

• Acquisition of social skills
• Active rehearsal
• Positive reinforcement for desired behavior

Play and Exercise in the Child with CI

Benefits of exercise

• Development of coordination
• Cardiovascular fitness
• Weight management

Promoting Independence in the Child with CI

Parents may need assistance to promote independent self-help skills

• Feeding
• Toilet training
• Dressing
• Grooming

Classification of CI

• Educable/mild: IQ 50-75
• Trainable/moderate: IQ 36-49
• Severe: IQ 20-35
• Profound: IQ less than 20

Care of Child with CI During Hospitalization

• Detailed hx with special focus on self-care abilities
• Assess developmental age
• Communication with child appropriate to cognitive level

Down Syndrome (Definition)

Also called Trisomy 21

• Extra chromosome 21 in 97%
• Translocation of chromosome 21 in 3-6%
• Mosaicism in 1-3%
• Etiology unknown, likely multiple causes
• Most common chromosome abnormality; 1 in 800-1000 live births
• Most common genetic cause of CI

Down Syndrome (Possible Etiologies)

Maternal Age

• Overall incidence
• 80% of infants with Down syndrome born to mothers <35 years
• Age 35 + incidence approx. 1 in 350
• Age 40 + incidence approx. 1 in 100

Genetic Counseling

• Degree of physical and cognitive impairment is r/t percentage of cells with abnormal chromosomal makeup
• Nondisjunction: usually sporadic event; low risk for recurrence
• Hereditary translocation: higher risk of recurrence, testing of the parents is necessary to ID the carrier and offer genetic counseling

Down Syndrome (Manifestations)

• Head, face, eyes, musculoskeletal
• Chest, neck, abdomen
• Genitalia, skin
• Hands, feet

Down Syndrome IQ

• Wide variation from severely CI to low-average intelligence
• Generally mild to moderate CI
• Initial development may appear normal
• Social development may be 2-3 years > mental age

Down Syndrome Congenital Anomalies

• 40-45% heart defects: septal defects
• Renal, Hirschsprung, tracheoesophageal fistula
• Altered immune function
• Musculoskeletal: hypotonic muscles, hyperextensible joints, atlantoaxial instability (15-20%)
• Sensory
• Growth: weight v. height
• Sexual development

Down Syndrome Treatment and Prognosis

Atlantoaxial Instability

• Urgen if sudden signs of spinal compression
• If present, may need to avoid activities stressful to head and neck
• May require surgical intervention

Fragile X Syndrome (Definition)

• Most common inherited cause of CI
• Second most common genetic cause of CI after Down syndrome
• Incidence 1 in 2000-5000 live births
• Occurs more frequently in males, approx. 1 in 4000 males; approx. 1 in 8000 females
• Affects males more severely than females (only one X)

Fragile X Syndrome: Gender Differences

• Most males are mentally deficient
• 30% of females are mentaly deficient
• Males have only the nonfunctioning X
• Females have one normally functioning X and one nonfunctioning X

Etiology of Fragile X Syndrome

• Abnormal mutation on lower end of the long arm of the X chromosome; occurs in affected males and females, occurs in carrier females
• Fragile site fails to condense during mitosis, results in mutation
• Prenatal dx is possible
• Cognitively impaired individuals without known family hx need DNA testing to determine cause of CI

X-Linked Carrier Status r/t Fragile X Syndrome

Classic X-linked recessive pattern

• All carrier females are normal
• All affected males have sx of the disorder, and no males are carriers

Classic Physical Appearance of Fragile X Syndrome

• Large head circumference, long face, prognathism, large ears, long palpebral fissures, strabismus (improper eye alignment)
• High-arched palate
• Mitral valve prolapse
• Macroorchidism (large testicles)
• Hyperextensible finger joints, palmar crease
• Flat feet

Classic Behavioral Features of Fragile X Syndrome

• Mild to severe CI, normal IQ with learning difficulties
• Delayed speech and language
• Hyperactivity
• Autistic-like behaviors
• Aggressive behaviors

Therapeutic Management of Fragile X Syndrome

• Tegretol, Prozac: behavioral control
• Stimulants for hyperactivity (similar to ADHD management)
• Mimic behavior: "mainstream management"

Hearing Impairment (Overview)

• Incidence is 3 in 1000 births; if child in NICU 2-4 in 1000 births
• Ranges from slight to profound
• Deaf: a person whose hearing disability precludes processing linguistic information with or without hearing aid
• Hard of hearing: generally able to hear with hearing aid

Etiology of Hearing Impairments

• Anatomic malformation
• LBW
• Ototoxic drugs
• Chronic ear infections (otitis media)
• Perinatal asphyxia
• Perinatal infections
• Prenatal substance use/abuse
• Cerebral palsy

NICU: Potential Effects on Hearing

Sensorineural hearing loss

• From exposure to continuous humming sounds
• From high noise levels of equipment

Pathology of Hearing Impairments

• Conductive hearing loss: middle ear
• Sensorineural hearing loss: nerve deafness
• Mixed conductive-sensorineural loss: may follow recurrent OM with complications
• Central auditory interception: organic or functional

Symptom Severity of Hearing Impairment

• Measured in decibels (dB)
• Hearing threshold 
• Effect on speech

Therapeutic Interventions for Hearing Impairment

Conductive Defects

• Medication (antibiotics)
• Hearing aids to amplify sound

Manifestations of Hearing Impairment in Infancy

• Lack of startle reflex
• Absence of babbling by age 7 months
• General indifference to sound
• Lack of response to spoken word

Manifestations of Hearing Impairment in Childhood

• Profound deafness likely to be dx in infancy
• Entry into school
• Concerns with speech development

Promoting Communication with Hearing Impairment

• Lip reading
• Cued speech
• Sign language
• Speech language therapy
• Socialization
• Additional aids

Care for Hearing-Impaired Child During Hospitalization

• Reassess understanding of instructions given
• Supplement with visual and tactile media
• Communication devices: picture board, common words and needs (food, water, toilet)

Prevention of Hearing Loss

• Treatment and management of recurrent OM
• Prenatal preventive measures
• Avoid exposure to noise pollution

Visual Impairment (Definition)

General term that refers to visual loss that cannot be corrected with regular prescription lenses

Visual Impairment Classification

Partially Sighted

• Acuity of 20/70 to 20/200
• Education usually in public school system

Etiology of Visual Impairments

• Perinatal or postnatal infections (gonorrhea, chlamydia, rubella, syphilis, toxoplasmosis)
• Retinopathy of prematurity
• Perinatal or postnatal trauma
• Other disorders
• Unknown causes

Refractive Errors in Visual Impairment

• Refraction: bending of light rays through the lens of the eye
• Myopia
• Hyperopia
• Astigmatism
• Strabismus (may or may not be refractive)

Other Visual Impairments

• Astigmatism (blurry vision)
• Amblyopia (lazy eye)
• Strabismus
• Cataracts
• Glaucoma

 Infections of the Eye

Conjunctivitis

• Ophthalmic antibiotics
• Systemic antibiotics in some cases
• Caution with use of steroids: may exacerbate viral infections
• Infection control concerns

Nursing Assessment with Visual Impairment

Infancy

• Response to visual stimuli
• Parental observations and concerns
• Expect binocularity by age 6 months

Promoting Child's Optimum Development with Visual Impairment

• Play and socialization
• Development of independence
• Education: braille, audio books and learning materials

Hospitalization of the Visually Impaired Child

• Safe environment
• Reassurance
• Orient child to surroundings
• Encourage independence
• Consistency of team members

Measures to Prevent Visual Impairment

• Prenatal care/prevention of prematurity
• Rubella immunizations for all children
• Safety counseling for preventing eye injuries

Periodic Recommended Screening for Visual Impairments

• Prenatal
• Newborns through preschoolers
• Children of all ages

Emergency Treatment for Eye Injuries

• Foreign body
• Chemicals
• UV burns
• Hematoma
• Penetrating injuries

Hearing and Visual Impairment in Children

• Historic cause: congenital rubella syndrome
• Profound effects on development
• Motor milestones usually achieved
• Other development often delayed
• "Finger spelling"
• Developing future goals for the child

Communication Impairment (Definition)

• Inability to receive, process, transmit, or represent symbol systems for the spoken word 
• Delayed language and speech is most common developmental delay in children
• Speech problems are more prevalent than language disorders
• Both types decline as children grow older

Autistic Spectrum Disorders (ASD)

• Complex brain dysfunction accompanied by broad range and severity of intellectual and behavioral deficits
• Etiology unknown; genetic basis
• ASD usually appears from 18-36 months 
• 4x more common in males
• Encompasses autistic disorder, Aspberger syndrome, and pervasive developmental disorder

Diagnostic Criteria for Autistic Disorder

• Qualitative impairment in social interaction
• Qualitative impairment in communication
• Restricted repetitive and stereotypical patterns of behavior, interests, and activities
• Delays or abnormal functioning with onset before 3 years

Nursing Considerations for Autism

• Wide variation in individual client response to tx efforts
• No cure for autism
• Most promising results seem to be through highly structured routines and intensive behavior modification programs

What is the critical time period for hearing loss?

Birth to 3.5 years

 Pediatric Differences in Musculoskeletal System

• Ossification: continues until 18-21
• Bones: less dense and more porous
• Epiphyseal plate
• Tendons and ligaments: stronger than bones
• Calcium intake

Assessment of Neurovascular Status (NVS)

Assess NVS q hour for 24 h, then 4-8 h

Compartment Syndrome

LIMB-THREATENING EMERGENCY

• May result in permanent neuromuscular disability (if not tx within 4-6 hours)
• Severe swelling and paresthesia occur in digits below affected compartment
• Treatment is immediate relief of pressure

Bryant's Traction

USED PRIMARILY IN CHILDREN

• Both legs are at 90 degree angle in bed
• Buttocks slightly lifted off mattress
• Used for fractured femur and dislocated hip
• Usually for children less than 3 years, weighing less than 30 lbs.

Forms of Skin Traction

• Buck Extension (affected leg flat on bed)
• Russell Traction (affected leg lifted at knee)
• Cervical Traction 

Forms of Skeletal Traction

• Crutchfield Tongs (top of head pulled up)
• Balanced Suspension (weights on both ends of bed)
• 90/90 Femoral Traction (knee bent up at 90 degree angle)
• Dunlop Traction

Distraction r/t Fractures (Definition)

The process of separating opposing bones to enhance bone regeneration in the created space.

Soft-Tissue Injury 

• Includes injuries to muscles, ligaments, and tendons
• Common in children
• Results from mishaps during play or sports

Soft-Tissue Injury Treatment

Rest - anywhere from several hours to several days

Ice - on for 30 minutes; off for 15

Compression - elastic bandage

Elevation - elevate injured part level with or above heart

Fractures in Infants

• RARE
• Usually result from birth trauma or non-accidental injury
• Nonmobile with broken extremity should be considered abuse
• Multiple fractures: osteogenesis imperfecta or abuse

Fractures in Children

• Increased mobility and inadequate or immature motor and cognitive skills
• Occur most often in play or sports or recreational activities
• Trauma: frequent cause of fractures
• Auto injury: Waddell's triad (fractured femoral shaft, intra-thoracic/intra-abdominal injuries, contralateral head injury)
• Falls: fractured wrists, elbows due to parachute reflex

Waddell's Triad

1. Fractured femoral shaft
2. Intra-thoracic/intra-abdominal injuries
3. Contralateral head injury

Clinical Manifestations of Fractures

Signs of Injury

• Generalized swelling
• Pain or tenderness
• Deformity
• Diminished functional use of affected part

Emergency Treatment for Fractures

• Assess the extent of injury - 5 Ps of vascular impairment
• Determine the mechanism of injury
• Avoid moving the injured part as much as possible
• Apply sterile or clean dressing to open wound
• Immobilize limb
• Reassess NVS
• Elevate
• Apply cold to area
• Call EMS

Therapeutic Management of Fractures

• Reduction: correct alignment of bony fragments
• Immobilization: to maintain alignment
• Restore to function
• Prevent further injury

Traumatic Amputation

• Severed part: wrapped lighlty in clean cloth or gauze saturated in saline and sealed in a watertight plastic bag, keep cool do NOT freeze
• Label bag: name, date, time
• Bag should be taken to the hospital with child

Osteomyelitis

• Etiology: older child - staph; younger child - h. influenzae
• Clinical Manifestations: very ill, irritable, localized tenderness

Osteomyelitis Nursing Management

• Position
• Contact precaution
• Medications (IV abx)
• VS, NVS
• Nutrition - increased protein and calories
• Diversional activity
• Additional assessments (psychosocial, etc.)

Legg-Calve-Perthes Disease (LCP)

(Definition, Stages and Etiology)

AVASCULAR NECROSIS OF FEMORAL HEAD; ages 3-12 years

• Primarily affects boys; Caucasians
• Self-limiting
• Worse prognosis in older children

LCP Clinical Manifestations, Diagnosis and Management

Clinical Manifestations

• Insidious onset
• Limp
• Hip soreness, ache, or stiffness
• Pain

Slipped Femoral Capital Epiphysis (SFCE)

(Definition, Etiology and Clinical Manifestations)

Spontaneous displacement of proximal femoral epiphysis; occurs in older children

• Incidence is greatest during rapid growth spurt during adolescence
• Twice as frequent in males
• Unknown cause

SFCE Management

• Therapeutic goals: avoid further damage

Scoliosis 

LATERAL CURVATURE OF THE SPINE

Diagnosis

• Screening techniques; x-ray

Juvenile Idiopathic Arthritis

(Definition and Diagnosis)

Types

• Systemic
• Pauclarticular
• Polyarticular

Juvenile Idiopathic Arthritis (JIA) Management

Drug Therapy

• NSAIDs: ibuprofen, naproxen, tolectin sodium (DOC)
• Cytotoxic drugs (methotrexate), corticosteroids, immunologic modulators
• Slow-acting anti-rheumatic drugs (SAARDs): sulfasalazine, gold, D-penicillamine
• Assess for hearing loss: high dose aspirin can be ototoxic

Osteogenesis Imperfecta (OI)

(Definition and Clinical Manifestations)

Group of 4 types of heterogenous inherited disorders - characterized by connective tissue and bone defects

• Abnormal precollagen type 1 OI is most common and mildest
• Type II is most severe and often leads to early death
• Most common genetic bone disease - usually autosomal dominant, may be recessive

OI Diagnosis and Management

Diagnosis

• X-rays: indicate multiple fracture healing sites
• US can detect some types
• Rule out all other possibilities before diagnosis

Systemic Lupus Erythematosus (SLE)

(Definition and Clinical Manifestations)

Disease of connective tissues and blood vessels; characterized by inflammation

• Variable and unpredictable
• Unknown cause; suspected triggers; autoimmune

SLE Diagnosis, Clinical and Nursing Management

Diagnosis

• Established when 4 of 11 diagnostic criteria are met

Muscular Dystrophy

PROGRESSIVE, TERMINAL ILLNESS

Clinical Manifestations

• Muscle weakness at approx. age 3 (preschool age)
• Calf muscles hypertrophy
• Waddle gait
• Gower's sign (use hands/arms to "walk up" body)

Cerebral Palsy (CP)

(Definition, Clinical Manifestations and Associated Complications)

Non-progressive motor (upper motor neuron) and posture dysfunction

• Anoxic damage
• Most common chronic childhood disorder

CP Etiology, Types, Diagnosis and Prognosis

Etiology

• Trauma
• Infection
• Lesion

CP Medical and Nursing Management

Medical Management

• Help child develop maximum potential
• Referrals: PT, OT, speech therapy, special education
• Surgical intervention: achilles tendon, hamstrings

Guillain-Barre Syndrome (GBS)

(Definition and Clinical Manifestations)

Often follows respiratory or GI viral infection

• Cause is unknown; affects children and adults
• Most often in school-age children and adolescents

GBS Diagnosis, Intervention and Nursing Implications

Diagnosis

• Clinical history
• Significant signs: paresthesia, paralysis, CSF findings
• Major dx tests: exam of CSF, nerve conduction tests

Congenital Clubfoot

Foot twisted out of normal position

• Occurs approx. in 1-3 in 1000 births
• Affects boys x2 as often 
• Bilateral in about 50% of cases
• Positive family hx

Developmental Dysplasia of Hip (DDH)

(Definition, Clinical Manifestations and Diagnosis)

Abnormal development of the hip

• May develop at any time during fetal life, infancy, or childhood
• Commonly involve: left hip, girls, Caucasian children

DDH Therapeutic and Nursing Management

Therapeutic Management

• Newborn-6 months: abduction device; worn 24 h/day
• Ages 6-18 months: traction; open reduction; spica cast
• Older child: correction is more difficult

Renal Development and Function in Infancy

• Glomerular filtration and absorption low in infancy until age 1-2
• Newborn unable to concentrate urine effectively
• Newborn unable to reabsorb sodium and water
• Newborn produces very dilute urine
• More liable to develop severe metabolic acidosis

Normal Urinalysis

• pH 5-9
• Specific gravity 1.001 to 1.035
• Protein <20 mg/dL
• Urobilinogen up to 1 mg/dL
• NONE of the following: glucose, ketones, Hgb, WBCs, RBCs, casts, nitrites

Normal Characteristics of Urine

• Clear
• Newborn production about 1-2 mL/kg/hr
• Child production about 1 mL/kg/hr

Urinary Tract Infection 

(Definition, Causes and Classification)

Second most common bacterial disease

• UTIs account for more than 8 million office visits per year
• >15% patients who develop gram-negative bacteria die
• 1/3 of gram-negative infections originate in urinary tract

Complicated UTI Infections

• Stones
• Obstruction
• Catheters
• Diabetes or neurologic disease
• Recurrent infections

Types of UTIs

Recurrent 

• Reinfection in person whose prior infection was successfully eradicated
• Unresolved bacteriuria - bacteria resistant or drug discontinued before bacteriuria was eradicated

Etiology and Pathophysiology of UTIs

• Physiologic and mechanical defense mechanisms maintain sterility
• Emptying bladder - stasis most important factor in UTI
• Normal antibacterial properties of urine and tract
• Ureterovesical junction competence
• Peristatic activity
• Alteration of defense mechanisms increases risk
• Organisms usually introduced via ascending route from urethra; less common routes: bloodstream and lymphatic system

For a kidney infection to occur from hematogenous transmission, there must be ____

prior injury to urinary tract (obstruction of ureter, damage from stones, renal scars)

Pediatric patients with significant bacteriuria may have no symptoms or nonspecific symptoms with UTI like ___ or ____.

fatigue or anorexia

Pediatric Manifestations of UTI

• Frequency of urination
• Fever in some cases, hypothermia in newborn
• Odiferous urine
• Blood or blood-tinged urine
• Sometimes no sx except generalized sepsis
• Squirming
• Irritability
• Vomiting, diarrhea and other GI signs

UTI Diagnostic Studies

• Dipstick: identify presence of nitrates, WBCs and leukocyte esterase
• Microscopic urinalysis
• Imaging studies for suspected obstruction (IVP or abdominal CT)

Culture: indicated in complicated or nosocomial, persistent bacteria, or frequently recurring (>2 episodes annually)

• Clean catch preferred; U-bag from child
• Specimen obtained by catheterization or suprapubic needle aspiration has more accurate results

UTI Collaborative Care Drug Therapy

(Antibiotics)

Trimethoprim-sulfamethoxazole (TMP-SMX) or nitrofurantoin

• Used to tx empiric uncomplicated or initial UTI
• Inexpensive
• Take twice daily

UTI Collaborative Care Drug Therapy for Repeated UTIs

• Prophylactic or suppressive antibiotics
• TMP-SMX administered every day to prevent recurrence or single dose before events likely to cause UTI

Vesicoureteral Reflux (VUR)

Acute Pyelonephritis 

(Etiology and Pathophysiology)

Inflammation caused by bacteria, fungi, protozoa, or viruses infecting kidneys

• Urosepsis - systemic infection from urologic source (can lead to septic shock and death in 15% of cases)
• Usually infection is via ascending urethral route
• Frequent causes - E. coli, proteus, Klebsiella, enterobacter
• Preexisting factors - VUR; dysfunction of lower urinary tract (obstruction or structure)
• Commonly starts in renal medulla and spreads to adjacent cortex
• Recurring episodes lead to scarred, poorly functioning kidneys and chronic pyelonephritis

Clinical Manifestations of Acute Pyelonephritis

• Vary from mild to "classic" to very severe
• Costovertebral tenderness
• Sx often subside in a few days, even without therapy (bacteriuria and pyuria still persist)

Diagnostic Studies of Acute Pyelonephritis

• Urinalysis - shows pyuria, bacteriuria, and hematuria
• WBC casts - indicate involvement of renal parenchyma
• CBC - show leukocytosis with increase in immature bands
• Imaging studies (IVP or CT)
• Ultrasound
• Prompt recognition and treatment of septic shock may prevent irreversible damage or death

Collaborative Care of Acute Pyelonephritis

• Hospitalization - patients with severe infections and complications such as n/v with dehydration
• Parenteral antibiotics - to establish high serum levels
• Relapses treated within 6-week course of antibiotics
• Reinfections treated as individual episodes or managed with long-term therapy (prophylaxis may be used for recurrent infections)

Acute Glomerulonephritis

(Definition and Types)

• May be primary event or evidence of systemic disorder
• Range from minimum to severe

Glomerulonephritis Symptoms

• Generalized edema r/t decreased GFR (begins with periorbital, progresses to lower extremities and then to ascites)
• HTN r/t increased ECF
• Oliguria
• Hematuria (bleeding in upper urinary tract = smoky urine)
• Proteinuria (increased amount of protein = increase in severity of renal disease)

Acute Poststreptococcal Glomerulonephritis (APSGN)

(Definition and Etiology)

NONINFECTIOUS RENAL DISEASE (autoimmune)

• Onset 5-12 days after other type of infection
• Often group A beta-hemolytic streptococci
• Most common in children 6-7 years old
• Uncommon in children younger than 2 years old
• Can occur at any age
• Winter and spring for poststreptococcal pharyngitis
• Summer and fall for impetigo

APSGN Clinical Manifestations and Prognosis

Clinical Manifestations

• Edema (periorbital, facial edema more pronounced in the AM)
• Anorexia
• Urine - cloudy, smoky-brown, decreased volume
• Pallor
• Irritability
• Lethargy
• Headaches
• Abdominal discomfort
• Dysuria
• Mild to moderate increased blood pressure

APSGN Complications and Diagnosis

Complications

• Hepatic encephalopathy - headache, dizziness, abdominal discomfort, vomiting, loss of vision, hemiparesis, disorientation, seizures
• Acute cardiac decompensation
• Acute renal failure

APSGN Treatment and Management

Treatment

• Primarily symptomatic
• Diet - no salt added to foods
• Fluids and daily weight
• Electrolytes
• HTN - antihypertensives

Nephrotic Syndrome

(Definition and Types)

Most common presentation of glomerular injury in children

Characteristics

• Proteinuria
• Hypoalbuminemia
• Hyperlipidemia
• Edema
• Massive urinary protein loss

Nephrotic Syndrome

(Changes and Prognosis)

Changes

• Glomerular membrane - normally impermeable to large proteins; becomes permeable to albumin; albumin lost in urine; fluid shifts from plasma to interstitial spaces 
• "Edema phase"
• "Remission phase"

Nephrotic Syndrome

(Clinical Manifestations, Management and Family Issues)

Clinical Manifestations

• Edema (eyes, face, abdomen, and lower limbs)
• Weight gain r/t fluid retention, weight loss from poor appetite
• Diarrhea, loss of appetite, and poor intestinal absorption
• Decreased urine
• Pallor
• Irritability, fatigue

Nephrogenic Diabetes Insipidus (NDI)

Major disorder associated with a defect in ability to concentrate urine

• Distal tubules and collecting ducts are insensitive to action of ADH (vasopressin)
• X-linked recessive inheritance

Hemolytic-Uremic Syndrome

(Clinical Features and Associated Organisms)

Clinical Features

• Hemolytic anemia
• Thrombocytopenia
• Renal injury
• CNS symptoms
• Hypertension

Hemolytic-Uremic Syndrome

(Clinical Manifestations and Diagnosis)

Clinical Manifestations

• Vomiting
• Irritability
• Lethargy
• Marked pallor
• Hemorrhagic manifestations (bruising, petechiae, jaundice, etc.)
• Oliguria or anuria
• CNS (seizures, stupor, coma)
• Signs of acute heart failure (fluid volume overload)

Hemolytic-Uremic Syndrome

Therapeutic Management

• Supportive care
• Dialysis
• Fresh frozen plasma
• Packed cells

Renal Trauma

• Kidneys in children are more mobile than in adults, and the outer borders of kidneys of children are less well protected
• Injuries usually are from blunt trauma (falls, sports injuries, etc.)
• Amount of hematuria is not r/t degree of injury

Renal Failure

Acute Renal Failure (ARF)

• Response to inadequate perfusion
• Kidney disease
• Urinary tract obstruction

Acute Renal Failure (ARF)

(Definition, Patho and Clinical Manifestations)

Kidneys suddenly unable to regulate volume and composition of urine

• Not common in children
• Principal feature is oliguria (assoc. with azotemia, metabolic acidosis and electrolyte disturbances)
• Most common pathologic cause: transient renal failure from severe dehydration

ARF Diagnosis, Management and Complications

Diagnosis

• BUN
• Creatinine
• pH
• Electrolytes - Na, K, calcium
• Dx of underlying disorder

Hyperkalemia

MEDICAL EMERGENCY

• Serum potassium above 7 mEq/L

Chronic Renal Failure (CRF)

(Definition, Etiology and Patho)

Begins when diseased kidneys cannot maintain normal chemical structure of body fluids; clinical syndrome called uremia

Etiology

• Congenital renal and urinary tract malformations
• VUR associated with recurrent UTIs
• Chronic pyelonephritis
• Chronic glumerulonephritis
• SLE

CRF Clinical Manifestations

Clinical Manifestations

• Early signs: loss of energy, increased fatigue on exertion, pallor, elevated BP
• With progression: decreased appetite esp. at breakfast, less interest in normal activities, increased or decreased urine output, sallow, muddy appearance of skin
• Headache
• Muscle cramps
• Nausea
• Weight loss
• Facial edema
• Bone or joint pain
• Growth retardation
• Dryness or itching of skin
• Bruised skin
• Sensory or motor loss
• Amenorrhea

Untreated Uremic Syndrome

GI Symptoms

• Anorexia; N/V

CRF Diagnosis, Management and Considerations

Diagnosis

• Initial signs vague and nonspecific

Types of Dialysis

• Hemodialysis
• Peritoneal dialysis
• Hemofiltration

Hemodialysis

• Requires creation of vascular access and special dialysis equipment
• Best suited for children who can be brought to facility 3x a week for 3-5 hours
• Achieves rapid correction of fluid and electrolyte abnormalities
• Adolescents and hemodialysis
• Provide diversional activities during hemodialysis to lessen boredom

Peritoneal Dialysis

• Abdominal cavity acts as semipermeable membrane for filtration
• Can be managed at home in some cases
• Warmed solution enters peritoneal cavity by gravity; remains for period of time before removal
• Peritonitis - potential complication

Continuous Venovenous Hemofiltration

• Uses technique for ultrafiltration of blood continuously at a very slow rate
• Works with fluid overload in postoperative period
• Successful alternative for critically ill children who might not survive rapid volume changes of hemodialysis and/or PD

Transplantation

• Can be from living, related donor or cadaver donor
• Primary goal is long-term survival of grafted tissue
• Immunosuppressant therapy

Phimosis

• Narrowing or stenosis of preputial opening of foreskin, preventing retraction of foreskin over glans
• Normal in infants and young children
• Usually disappears as child grows
• Balanitis - inflammation of foreskin

Foreskin Hygiene

• Uncircumcised infants - external cleansing of phimotic foreskin during routine bathing
• Never forcibly retract foreskin
• Expect natural retraction of foreskin in later childhood (5-10 years old)

Hydrocele

• Presence of fluid in processus vaginalis; fluid filled sac in spermatic cord
• Scrotum appears enlarged, especially after activity in childhood
• May need surgical repair

Cryptorchidism

• Up to 45% in preterm males; 3-4% in full-term males
• Undescended, retractile, or absence of testes

Hypospadias

• Urethral opening of male located below the glans or underneath the penile shaft
• 1 in 300 live births
• Familial tendency
• Repair at 6-12 months before body image develops

Chordee

• Ventral curvature of the penis
• Often accompanies more severe forms of hypospadias
• Foreskin may be absent ventrally
• Hooded or crooked appearance of penis
• Surgical repair

Epispadias Exstrophy Complex

• Bladder exstrophy is a severe defect with externalization of bladder and associated tissues
• Results from failure of abdominal wall to fuse in utero
• May be assoc. with epispadias, undescended testes, and/or inguinal hernia

Obstructive Uropathy

Ureterohydronephrosis; hydronephrosis (unilateral or bilateral, congenital or acquired)

Clinical Manifestations

• Depend on location of obstructing lesion, its severity, and underlying cause
• May be asx; may cause UTIs
• May have abdominal mass
• May cause "renal colic"

Etiologies of Ambiguous Genitalia

• Abnormalities of chromosomal complement
• Defects of embryogenesis
• Hormonal or biochemical abnormalities

Conditions Producing Ambiguous Genitalia in Newborn

• Masculinized female
• Incompletely masculinized male
• Presence of both male and female sexual organs
• Mixed gonadal dysgenesis

Congenital Adrenogenital Hyperplasia (CAH)

MOST COMMON type of ambiguous genitalia

• Inherited deficiency of adrenal-cortical hormones
• Masculinization of external genitalia with normal internal female anatomy
• Life-threatening alteration of adrenal-cortical hormones

Incompletely Masculinized Male

Potential Causes

• Deficient production of fetal androgen
• Deficiency of enzymes needed for testosterone biosynthesis
• Unresponsiveness of genitalia to testosterone

Mixed Gonadal Dysgenesis

• Infants are sex chromosome mosaics
• Genitalia vary greatly
• Those appearing predominately female but with dysplastic testes may experience masculinization at puberty

Nursing Considerations of Ambiguous Genitalia

• Decision for gender assignment is often difficult
• Need family support: dealing with feelings of guilt and shame; concern for child's future

When does the foramen ovale close?

At birth in most individuals

How long does it take the ductus arteriosus to close?

15 hours, but can take 1-2 weeks

Pediatric Indicators of Cardiac Dysfunction

• Poor feeding
• Tachypnea/tachycardia
• Failure to thrive/poor weight gain/activity intolerance
• Developmental delays
• Prenatal hx
• Family hx of cardiac disease

Tests of Cardiac Function

• Chest x-ray: can see heart size and vasculature
• ECG
• Echocardiography: under 3 may need sedation (use chloral hydrate), monitor q 15 minutes
• Cardiac catheterization: moderate IV sedation; can also fix some defects in cath lab

Interventional Cardiac Catheterization Procedures in Children

• Transposition of great vessels repair
• Some complex single-ventricle defects
• ASD repair
• Pulmonary artery stenosis repair
• Valvular pulmonic stenosis - balloon dilation
• Recurrent coarctation of aorta - balloon dilation
• Congenital mitral stenosis - balloon dilation

Murmurs

• Heart sounds that reflect flow of blood within heart
• May occur in systole, diastole, or both (diastolic very concerning)
• Can occur during normal periods of stress (anemia, fever, etc.)
• Innocent murmurs: normal cardiac anatomy and function; occur in up to 50% of all children at some time

Two Types of Cardiac Defects

Congenital

• Anatomic: abnormal function

Causes of Congenital Heart Disease

Maternal or environmental (1-2%)

• FAS - 50% have CHD
• Rubella in first 7 wks = 50% risk of defects including PDA and pulmonary branch stenosis
• CMV, toxoplasmosis, other viral illnesses = cardaic defects
• IDMs = 10% risk of CHD (VSD, cardiomyopathy, TGA most common)

CHD Overview/Incidence

• 5-8 per 1000 live births; about 2-3 are sx in first year of life
• Major cause of death in first year of life (after prematurity)
• Most common anomaly is VSD
• 28% of children with CHD have another recognized anomaly (trisomy 21, 13, 18)

CHF in Children

Impaired myocardial function

• tachycardia, fatigue, weakness, restlessness, pale extremities, decreased urine output

New Classifications of CHD

By hemodynamic characteristics

• Increased pulmonary blood flow
• Decreased pulmonary blood flow
• Obstruction of blood flow from the heart
• Mixed blood flow

Increased Pulmonary Blood Flow Defects

• Abnormal connection between 2 sides of the heart; either the septum or the great vessels
• Increased blood volume on the R side of the heart
• Increased pulmonary blood flow
• Decreased systemic blood flow
• Atrial septal defect (ASD)
• Ventricular septal defect (VSD)
• Patent ductus arteriosus (PDA)

Atrial Septal Defect (ASD)

• Hole between atriums; closed in cath lab usually
• Blood flows over to R side, pumping high on L
• Kids usually tolerate this well
• Increased risk for thrombi/emboli
• May have dysrhythmias

Vetricular Septal Defect (VSD)

• Hole between ventricles; can sometimes repair in cath lab
• Most common CHD
• Usually children outgrow this; just watch it for worsening
• R ventricle becomes hypertrophic
• Murmur will be heard
• CHF and bacterial endocarditis can occur

Patent Ductus Arteriosus (PDA)

• Hole between pulmonary artery and aorta
• LOUD machinery murmur
• Blood flows into pulmonary vessels
• Prostaglandin keeps PDA open; many premies have PDA
• Indomethacin usually closes; or fix in cath lab

Obstructive Defects of Heart

• Coarctation of the aorta
• Aortic stenosis
• Pulmonic stenosis

Coarctation of the Aorta (COA)

• Narrowing along the arch (usually near ductus arteriosus)
• Headaches, dizziness, pulses dec. in lower extremities, high BP
• CHF; aortic aneurysm; stroke; aortic rupture
• Can fix in the cath lab
• Possible complication of HTN

Aortic Stenosis (AS)

• Hypotension, faint pulses, murmur, chest pain, poor feeding, etc.
• Can fix with angioplasty in cath lab
• Blood pools in lungs; L ventricle becomes hypertrophic
• Pulmonary HTN; coronary arteries don't get a lot of blood = at risk for ischemic MI

Pulmonic Stenosis (PS)

• R ventricle becomes hypertrophic, blood backs into it
• Foramen ovale can open back up
• Cyanotic, may have CHF
• Often have PDA also
• Usually repaired in cath lab

Decreased Pulmonary Blood Flow Defects

• Tetralogy of Fallot
• Tricuspid atresia

Tetralogy of Fallot (TOF)

1. Pulmonary stenosis
2. R ventricle hypertrophy
3. VSD
4. Overriding aorta

Tricuspid Atresia

• No tricuspid valve; need PDA and ASD
• Give prostaglandin to keep PDA open
• Evident at birth
• Cath lab: tear hole between atria, done in NICU right after birth
• GLENN shunt

Mixed Defects

• Transposition of great vessels
• Total anomalous pulmonary venous connection
• Hypoplastic heart syndrome (L or R)

Transposition of Great Vessels

• Pulmonary artery and aorta are switched
• Have PDA and ASD
• Cath lab: open btwn atria; surgically switch aorta and pulmonary artery
• Very delicate surgery because coronary arteries come off of the aorta

Total Anomalous Pulmonary Venous Connection (TAPVC)

• Must be repaired surgically
• ASD; lots of mixing of blood

Hypoplastic Left Heart Syndrome

• Aortic valve stenosis resulting in narrowed aorta
• HAS to have PDA; sx may not be seen until PDA closes
• Repaired in 3 stages

Postoperative Care for the Child with CHD

• Monitor VS and arterial/venous pressures
• Intraarterial monitoring of BP
• Intracardiac monitoring
• Respiratory needs
• Rest, comfort, and pain management
• Fluid management
• Progression of activity

Chest Tubes After Cardiac Surgery

• Monitor chest tube drainage q hour for color: immediate postop may be bright red
• Monitor chest tube drainage for quantity: notify surgeon if chest tube drainage >3 ml/kg/hr x3 consecutive hours OR 5-10 ml/kg in any 1 hour (possible hemorrhage)
• Be alert for cardiac tamponade (rapid onset)
• Look for bleeding in chest tubes

Postpericardiotomy Syndrome

• Symptoms: fever, rub, pericardial and pleural effusion
• Occurs in immediate postoperative period; also can occur later (7-21 days postop)
• Unknown cause
• May require pericardiocentesis or pleurocentesis

Endocarditis