1 PEDIATRIC NURSING Care of the Child and Family.

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PEDIATRIC NURSINGPEDIATRIC NURSINGPEDIATRIC NURSINGPEDIATRIC NURSINGCare of the Child Care of the Child

and Familyand Family

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Developmental Theorists

Maslow’s Hierarchy of Needs (1954)

Erik Erikson - Psychosocial Theory

Jean Piaget - Cognitive Theory

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Maslow’s Hierarchy of Needs

Principles: An individual’s needs are depicted in ascending levels on the hierarchy Needs at one level must be met before one can focus on a higher level need

Levels of Maslow’s Hierarchy of Needs: Physiologic/Survival Needs

Safety and Security Needs

Affection or Belonging Needs

Self-esteem/Respect Needs

Self-actualization Needs

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TRUST VS. MISTRUST

• Birth - 1 year– World/Self is good– Basic needs met

• Met = happy baby• Unmet = crying, tense,

clinging• Stranger Anxiety• Separation Anxiety Photo Source: Del Mar Image Library; Used

with permission

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AUTONOMY VS. SHAME & DOUBT

1 – 3 years– Sense of control– Exerts self/will– Pride in self-

accomplishment• Negativism• Ritualism/Routines• Parallel play

Photo Source: Del Mar Image Library; Used with permission

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INITIATIVE VS. GUILT

3 – 6 years– “Can-do” attitude– Behavior is goal-

directed and imaginative– Play is work– Be careful with criticism


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INDUSTRY VS. INFERIORITY

6 – 12 years– Mastery of skills– Peers in both play

and work– Rules important– Competition– Predictability


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IDENTITY VS. ROLE CONFUSION

12 -18 years– Sense of “I”– Peers are very

important– Independence

from parents– Self-image Photo Source: Del Mar Image Library; Used

with permission

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Piaget’s Cognitive Theory

Development of Thought Processes:

30 – 2 years:Sensorimotor

32 – 7 years: Preoperational

37 – 11 years: Concrete Operations

311 years + : Formal Operations

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SENSORIMOTOR

Birth - 2 years– Reflexive behavior

leads to intentional behavior

– Egocentric view of world– Cognitive parallels motor

development– Object Permanence

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PREOPERATIONAL THOUGHT

2 - 7 years– Egocentric thinking– Magical thinking– Dominated by self-

perception– Animism– No irreversibility– Thoughts cause actions


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CONCRETE OPERATIONS

7 - 11 years– Systematic/logical– Fact from fantasy– Sense of time– Problem solve– Reversibility– Cause & effect– Humor


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FORMAL OPERATIONS11 years - Adult– Abstract thinking– Analyze situations– New ideas created– Factors altering this:

• Poor comprehension

• Lack of education• Substance abuse


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Infant Physical TasksPhysical Tasks: 0 - 6 months: Fastest growth period Gains 5-7 oz (142-198 g) weekly for 6 months Grows 1 inch (2.5 cm) monthly for 6 months Head circumference is equal to or larger than chest circumference Posterior fontanel closes at 2-3 months* Obligate nose breathers* Vital signs: HR and RR faster and irregular* Motor: behavior is reflex controlled

sits with or without support at 6 mo* rolls from abdomen to back

Sensory: able to differentiate between light and dark

hearing and touch well developedTOYS = Mirror, Music, Mobile

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Infant Physical TasksPhysical Tasks 6 - 12 months: Gains 3-5 oz (84-140g) weekly for next 6 months

* triples weight by 12 months Gains 1/2 in (1.25 cm) monthly for next 6 months Teeth begin to come in Motor:

Intentional rolling over from back to abdomen*

Starts crawling and pulling to a stand*Develops pincer grasp*Sits without support by 9 months*

Sensory: Can fixate on and follow objectsLocalizes sounds

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Infant Psychosocial Tasks

Vocalizations:• Distinction in cry at 1 month• Coos at 3 months• Begins to imitate sound at 6

months – babbles• Verbalizes all vowels at 9 months• Can say 4–5 words at 12 months

Socialization:• Social smile at 2 months• Demands attention & social

interaction at 4 months• Stranger anxiety & comfort

habits begin at 6 months*• Separation anxiety develops at 9

months*Photo Source: Del Mar Image Library; Used with permission

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Infant Cognitive Tasks

Neonates Reflexes only

1-4 months Recognizes facesSmiles and shows pleasureDiscovers own body and surroundings

5-6 months Begins to imitate

7-9 months Searches for dropped objects *Object Permanence beginsResponds to simple commandsResponds to adult anger

10-12 months Recognizes objects by nameLooks at and follows pictures in books

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ToddlerPhysical Tasks:• Slow growth period• Gains 11 lbs (5 kg) • Grows 8 inches (20.3 cm)• Anterior fontanel closes at • 12 - 18 months*• Primary dentition (20 teeth)

complete by 2½ years• Develops sphincter control – toilet

training possible*

Motor Tasks: • Walks alone by 12 - 18 months*• Climbs and runs fairly well by 2

years• Rides tricycle well by 3 years


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Toddler Cognitive Tasks

Follows simple directions by 2 years

Uses short sentences by 18 months *favorite words “no” and “mine” = Autonomy

Knows own name by 12 months, refers to self

Achieves object permanence

Uses “magical” thinking

Uses ritualistic behavior

Repeats skills to master them and decrease anxiety

Egocentric thinking - thoughts cause actions

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Toddler Psychosocial Tasks

Increases independence Able to help with dressing self Temper tantrums (autonomy) Beginning awareness of ownership (me and mine) Shares possessions by 3 years Vocabulary increases to over 900 words Toilet training

Fears: separation anxiety, loss of control

TOYS = Push-pull toys, large blocks

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PreschoolerPhysical Tasks:• Slow growth rate continues• Weight increases 4-6 lbs (1.8–2.7

kg) a year• Height increases 2½ inches (5-6.25

cm) a year• Permanent teeth appear

Motor Tasks: • Walks up & down stairs• Skips and hops on alternate feet• Throws and catches ball, jumps

rope• Hand dominance appears• Ties shoes and handles scissors

well• Builds tower of blocks Photo Source: Del Mar Image Library; Used

with permission

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Preschooler Cognitive Tasks

Can only focus on one idea at a time

Begins awareness of racial and sexual differences

Develops an understanding of time• Learns sequence of daily events• Able to understand some time-oriented words

Begins to understand the concept of causality

Has 2,000 word vocabulary

Is very inquisitive and curious

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Preschooler Psychosocial Tasks Becomes independent

Gender-specific behavior is evident by 5 years

Egocentricity changes to awareness of others

Understands sharing

Aggressiveness and impatience peak at 4 years

Eager to please and shows more manners by 5 years

Behavior is goal-directed and imaginative

Play is work*

TOYS = Dolls, Dress-up, Imagination

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Preschooler Psychosocial Tasks

Fears: about body integrity (Fear & Injury) are common

Magical and animistic thinking allows illogical fears to develop*

Observing injuries or pain of others can precipitate fear

Able to imagine an event without experiencing it

Guilt and shame are common*

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School-agePhysical Tasks: Slow growth continues Weight doubles over this period Gains 2 inches (5 cm) per year At age 9, both sexes are the same

size At age 12, girls are bigger than

boys Very limber but susceptible to

bone fractures Develops smoothness & speed in

fine motor skills Energetic, developing large

muscle coordination, stamina & strength

Has all permanent teeth by age 12 Photo Source: Del Mar Image Library; Used

with permission

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School-Age Cognitive Tasks

Period of Industry:• Likes to accomplish or produce

• Interested in exploration & adventure

• Develops confidence

• Rules become important*

Concepts of time and space develop:• Understands causality, permanence of mass & volume

• Masters the concepts of conservation, reversibility, arithmetic and reading

• Develops classification skills

• Begins to understand cause and effect*

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School-Age Psychosocial Tasks

School occupies half of waking hours; has cognitive and social impact on child

Morality develops

Peer relationships start to be developed

Enjoys family activities

Has increased self-direction - tasks are important

Has some ability to evaluate own strengths & weaknesses

Enjoys organizational activities (sports, scouts, etc.)*

Modesty develops as child becomes aware of own body*

TOYS = Board games, computer

games, learning activities

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AdolescentPhysical tasks: Period of rapid growth Puberty starts Girls: height increases 3

inches/year Boys: growth spurt around 13-

yrs-old height increases 4 inches/yearweight doubles between 12-18

yrs Body shape changes:

Girls have fat deposits in thighs, hips & breast, pelvis broadensBoys become leaner with a broader chest


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AdolescentSexual Development

Girls Boys

Breasts develop Facial Hair growth

Menses begins Voice changes

First 1 –2 years infertile Enlargement of testes at 13 yrs

Nocturnal emission during sleep

Reaches reproductive maturity with viable sperm at 17 yrs

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Adolescent Cognitive Tasks

Develops abstract thinking abilities

Often unrealistic

Sense of invincibility = risk taking behavior*

Capable of scientific reasoning and formal logic

Enjoys intellectual abilities

Able to view problems comprehensively

ACTIVITIES = Music, video games, communication with

peers

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Adolescent Psychosocial TasksEarly Adolescent: Prone to mood swings

Needs limits and consistent discipline

Changes in body alter self-concept

Fantasy life, daydreams, crushes are normal

Middle Adolescent: Separate from parents

Identify own values and define self*

Partakes/conforms to peer group/values*

Increased sexual interest

May form a “love” relationship

Formal sex education begins

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Adolescent Psychosocial TasksLate Adolescent: Achieves greater independence*

Chooses a vocation

Finds an identity*

Finds a mate

Develops own morality

Completes physical and emotional maturity

Fears: Threats to body image – acne, obesity

Rejection

Injury or death, but have sense of “invincibility”

The unknown

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Let’s ReviewLet’s ReviewLet’s ReviewLet’s Review

A 10 month-old baby was admitted to the A 10 month-old baby was admitted to the pediatric unit. Each time the nurse enters pediatric unit. Each time the nurse enters the room the baby begins to cry. The most the room the baby begins to cry. The most appropriate action by the nurse would be to:appropriate action by the nurse would be to:

A. Complete all procedures quickly in order to A. Complete all procedures quickly in order to decrease the decrease the amount of time the baby will cry. amount of time the baby will cry. B. Ask another nurse to assist you with the B. Ask another nurse to assist you with the baby’s care.baby’s care.C. Distract the baby.C. Distract the baby.D. Encourage the parent to stay by the bedside D. Encourage the parent to stay by the bedside and assist with and assist with the care.the care.

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Let’s Review

A 6 month-old is admitted to the pediatric unit for a 3 week course of treatment. The infant’s parents cannot visit except on weekends. Which action by the nurse indicates an understanding of the emotional needs of an infant?

A. Telling the parents that frequent visits are unnecessary.B. Placing the infant in a room away from other children.C. Assigning the infant to different nurses for varied contacts.D. Assigning the infant to the same nurse as much as possible.

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Let’s Review

Which child is most likely to be frightened by hospitalization?

A. 4 month-old admitted with a diagnosis of bronchiolitis.B. 2 year-old admitted with a diagnosis of cystic fibrosis.C. 9 year-old admitted with a diagnosis of abdominal pain.D. 15 year-old admitted with a diagnosis of a fractured femur.

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Infant NutritionBirth – 6 months: Breast milk is most complete diet Iron-fortified formulas are acceptable No solid foods before 4 months*

6 - 12 months: Breast milk or formula continues* Diluted juices can be introduced Introduction of solid foods*(4-6 mo): cereal, vegetables, fruits, meats Finger foods at 9-10 months Chopped table foods at 12 months Gradual weaning from bottle/breast No honey (risk for botulism)

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Toddler Nutrition Able to feed self – autonomy & messy! Appetite decreases- physiologic anorexia Negativism may interfere with eating Needs 16 – 20 oz. milk/day Increased need for calcium, iron, and

phosphorus – risk for iron deficiency anemia Caloric requirements is 100 calories/kg/day No peanuts under 3 years of age (allergies)* Do not restrict fats less than 2 years of age* Choking is a hazard (no nuts, hot dogs,

popcorn, grapes)*


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Preschooler Nutrition

Caloric requirements is 90 calories/kg/day May demonstrate strong taste preferences

• 4 years old – picky eaters• 5 years old – influenced by food habits of

others Able to start social side of eating More likely to try new foods if they assist in food

preparation Establish good eating habits - obesity

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School-Age Nutrition Caloric needs diminish, only need 85 kcal/kg

Foundation laid for increased growth needs

Likes and dislikes are well established

“Junk” food becomes a problem

Busy schedules – breakfast is important

Obesity continues to be a risk

Nutrition education should be integrated into the school program

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Adolescent NutritionNutritional requirements peak during years of

maximum growth: Age 10 – 12 in girlsAge 14 – 16 in boys Food intake needs to be balanced with energy expenditures

Increased needs for:Calcium for skeletal growthIron for increased muscle mass and blood cell developmentZinc for development of skeletal, muscle tissue and sexual

maturation


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Adolescent Nutrition (continued)

Eating and attitudes towards food are primarily family/peer centered

Skipping breakfast, increased junk food, decreased fruits, veggies, milk

Boys eat foods high in calories. Girls under-eat or have inadequate nutrient intake.

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Let’s Review

The nurse recommends to parents that popcorn and peanuts are not good snacks for toddlers. The nurse’s rationale for this action is:

A. They are low in nutritive value.B. They cannot be entirely digested.C. They can be easily aspirated.D. They are high in sodium.

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Let’s ReviewNutrition is an important aspect of health promotion for the infant. Priority information to give the parents concerning infant nutrition would include (check all that apply):

A. Restrict the fat intake of the infant to help reduce the chances of an obese child.B. Breast or infant formula must be continued for the first year.C. Encourage the use of a pacifier for non-nutritive sucking needs.D. Introduction of solid foods should begin at 4-6 months.

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Play is the work of Children

Enhances Motor Skills

Enhances Social Skills

Enhances Verbal Skills

Expresses Creativity

Decreases Stress

Helps Solve Problems

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Appropriate Play Activities

Infants - Solitary Play, stimulation of senses (music, mirror)

Toddler - Parallel Play, make believe, locomotion (push-pull toys), gross & fine motor, outlet for aggression & autonomy

Preschooler - Associative Play, Imaginary Playmate, dramatic & imitative, gross & fine motor

School Age - Cooperative Play, rules dominate play, team games/sports, quiet games/activities, joke books

Adolescent - Group activities predominate, activities involving the opposite sex in later years

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Preparation for Procedures

• Allow child to play with equipment

• Demonstrate procedure on doll for young child

• Use age-appropriate teaching activities

• Describe expected sensations• Use simple explanations• Clarify any misconceptions• Involve parents in comforting

child• Praise/reward child when

finished


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Communicating with Children

Provide a trusting environment

Get down to child’s eye level

Use words appropriate for age

Always explain what you are doing

Always be honest

Allow choices when possible

Allow child to show feelings/talk

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Let’s Review

The single most important factor for the nurse to recognize when communicating with a child is:

A. The child’s chronological age.B. Presence or absence of the child’s parents.C. Developmental level of the child.D. Nonverbal behaviors of the child.

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Health Promotion

Childhood Immunizations

Well child check-ups

Nutrition

Screenings throughout childhood(APGARS, newborn screenings, lead poisoning, vision/hearing, scoliosis)

Health Teaching

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Immunizations Primary prevention of many communicable

diseases Vaccines safety

• MMR vaccine and autism (no correlation)• Reactions (pre-medicate with Tylenol)

Live attenuated vaccines (MMR, Varicella)• Weakened form of disease• Body produces immune response• Contraindicated in immunosupressed

individuals Inactivated (killed virus/bacteria or

synthetic)• 1st dose only “primes” system- immunity

develops after 3rd

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Injury Prevention& Safety Issues

Accidents are the leading cause of death in infants and toddlers (falls, burns, poisons)

Toddlers and Preschoolers – drowningSchool-age and adolescents – motor

vehicle accidents and firearms90% of all accidents are preventable!Safety education is the answer

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Injury Prevention

Methods of Injury Prevention• Understanding and Applying Growth and

Developmental Principles• Anticipatory Guidance• Childproofing the environment• Educating caregivers and children• Legislation

Precipitating Factors

Potential Outcomes

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Pediatric PoisoningsHighest incidence occurs in children in 2-year-old age group and under 6 years of age

Major contributing factor – improper storage, allowing children to play with “bottles” – rattling of pills, “drink” syrups, toxic portion of plants.

Teach parents about proper storageKnowledge of plants in household, and keep away from infants and children who might “chew”

Emergency treatment depends on agent ingestedTeach parents to have poison control number availableRefer to appropriate method according to substance ingested

First Intervention is to call POISON CONTROL CENTER

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Types of Poisonings

Acetaminophen Ingestion

Lead Poisoning

Salicylate Poisoning

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Lead Poisoning Major environmental health concern Found in older homes (built before 1978),

lead-contaminated soil, water through lead pipes, lead-based paint in ceramics products, Mexican candies made in lead containers

Body rapidly absorbs lead – specially in periods of rapid growth – most harmful to children under 6 years

Absorbed in GI tract and accumulates in bones, brain, kidneys

Low levels in blood can cause behavioral/learning problems, mid-levels anemia-like symptoms and skeletal growth interference, and high levels can be fatal from CNS edema and encephalopathy

Diet high in fat, low in iron & calcium can increase lead absorption

Intervention=teaching for prevention. If blood level ≥ 45, chelation therapy is needed – monitor kidney function during treatment.

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Salicylate Poisoning

Can be acute or chronic ingestion S/S = nausea, disorientation, vomiting,

dehydration, hyperpyrexia, oliguria, coma, bleeding tendencies, tinnitus, seizures

Nursing interventions = activated charcoal, sodium bicarbonate for metabolic acidosis, external cooling measures for hyperpyrexia, anticonvulsant and seizure precautions (think patient safety!), vitamin K for bleeding, possible hemo (NOT peritoneal) dialysis

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Acetaminophen Poisoning

Most common drug poisoning in children Acute ingestion S/S start as nausea, vomiting, pallor, sweating »

hepatic involvement (jaundice, confusion, coagulation problems, RUQ pain)

Treatment is activated charcoal first, then the antidote N-acetylcysteine (Mucomyst) PO every 4 hours for 17 doses after a loading dose given

Always assess Level of Consciousness (LOC) before administering PO med!

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Let’s Review

Which would be the best approach for gastric emptying in a lethargic 18-month-old who ingested antihistamine tablets an hour ago?

A. Diluting toxic substance with water or milkB. Administering naloxone (Narcan)C. Gastric lavageD. Administering ipecac syrup

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Physical Assessment of Infant

Assessment is NOT in the head-to-toe manner When quiet, auscultate heart, lungs, abdomen Assess heart & respiratory rates before

temperature Palpate and percuss same areas Perform traumatic procedures last Elicit reflexes as body part examined Elicit Moro reflex last Encourage caretaker to hold infant during exam

Distract with soft voice, offer pacifier, music or toy

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Physical Assessment of Toddler

Inspect body areas through play – “count fingers and toes”

Allow toddler to handle equipment during assessment and distract with toys and bubbles

Use minimal physical contact initially Perform traumatic procedures last Introduce equipment slowly Auscultate, percuss, palpate when quiet Give choices whenever possible


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Physical Assessment of Preschooler

If cooperative, proceed with head-to-toe If uncooperative, proceed as with toddler Request self undressing and allow to wear

underpants Allow child to handle equipment used in

assessment Don’t forget “magical thinking” Make up “story” about steps of the procedure Give choices when possible If proceed as game, will gain cooperation


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Physical Assessment of School-Age Child

Proceed in head-to-toe May examine genitalia last in older children Respect need for privacy – remember modesty! Explain purpose of equipment and significance Teach about body function and care of body

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Physical Assessment of the Adolescent

Ask adolescent if he/she would like parent/caretaker present during interview/assessment Provide privacy Head-to-toe assessment appropriate Incorporate questions/assessment related to genitals/sexuality in middle of exam Answer questions in a straightforward, non- condescending manner Include the adolescent in planning their care

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Fever Causes – Often unknown, may be due to

dehydration, most often viral induced Danger in infants is febrile seizures – most

common between 3 months to five years. The seizure is a result of how quickly the temperature rises.

Hydration (20mls/kg is formula for bolus) Antipyretics – acetaminophen or ibuprofen Cooling measures – avoid shivering

• Tepid bath• Remove excess clothing and blankets• Cooling blankets/mattresses

NO ICE PACKS!

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Pediatric DifferencesFluid & Electrolyte

Percent Body Water compared to Total Body Weight:

• Premature infants: 90% water• Infants: 75 - 80% water• Child: 64% water

Higher percentage of water in extracellular fluid in infantsInfants and toddlers more vulnerable to fluid and electrolyte disturbancesConcentrating abilities of kidneys not fully mature until 2 yearsMetabolic rate is 2-3 times higher than an adultGreater body surface area per kg body weight than adults; dehydrates more quickly

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Dehydration• Types:

Isotonic – Most common; salt and water lost. Greatest threat – Hypovolemic Shock

Hypotonic – Electrolyte deficit exceeds water deficit- physical signs more severe with smaller fluid losses

Hypertonic – Water loss higher than electrolyte

Vomiting leads to metabolic alkalosisDiarrhea leads to metabolic acidosis

LAB WATCH: monitor sodium, potassium, chloride, carbon dioxide, BUN, and creatinine

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Assessment of Dehydration

• Skin gray, cold, mottled, poor to fair, dry or clammy

• Delayed capillary refill• Mucous membranes/lips dry• Eyes and fontanels sunken• No tears present when crying• Pulse and respirations rapid• Irritability to lethargy depending on cause

and severity, not responsive to parent and/or environment

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Dehydration:Nursing

Interventions Daily weight, I/O Assess hydration status Assess neurological status Monitor labs (electrolytes) Rehydrate with fluids both PO and IV (20 mls/kg

of NS) Diet progression: Pedialyte modified Bread-

Rice-Apple Juice-Toast (BRAT) Diet-for-age (DFA)

Skin care for diaper rash Stool output (Amount, Color, Consistency,

Texture - ACCT) HANDWASHING!

Priorities: fluid replacement & assess for S/S of shock

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Diarrhea

• Often specific etiology unknown, but rotavirus is most common cause of gastroenteritis in infants and kids

• Don’t forget contact precautions!!• Leading cause of illness in children younger

than 5• May result in fatality if not treated properly• History very important• Treatment aimed at correcting fluid

imbalance and treating underlying cause• Metabolic acidosis = blood pH < 7.35

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Vomiting

• Often result of infections, improper feeding techniques, GI blockage (pyloric stenosis), emotional factors

• Management directed toward detection, treatment of cause and prevention of complications

• Metabolic alkalosis = blood pH >7.45

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Let’s ReviewThe most appropriate type of IV fluid to infuse in treatment of extra-cellular dehydration in children is:

A. Isotonic solution.B. Hypotonic solution.C. Hypertonic solution.D. Colloid solution.

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Let’s Review

Which laboratory finding would help to identify that a child experiencing metabolic acidosis?

A. Serum potassium of 3.8B. Arterial pH of 7.32C. Serum carbon dioxide of 24D. Serum sodium of 136

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Pain Assessment: Infants

Assessment of pain includes the use of pain scales that usually evaluate indicators of pain such as cry, breathing patterns, facial expressions, position of extremities, and state of alertness

Examples: FLACC scale, NIPS scale

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Pain Assessment: Toddlers

Toddlers may have a word that is used for pain (“owie,” “boo-boo,” “ouch” or “no”); be sure to use term that toddler is familiar with when assessing.

Can also use FLACC scale, or Oucher scale (for older toddlers)

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Pain Assessment:Preschoolers

Think pain will magically go awayMay deny pain to avoid medicine/injectionsAble to describe location and intensity of painFACES scale, poker chips and Oucher scale may be used


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Pain Assessment:Older Children

Older children can describe pain with location and intensity

Nonverbal cues important, may become quiet or withdrawn

Can use scales like Wong’s FACES scale, poker chips, visual analog scales, and numeric rating scales

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Let’s ReviewThe nurse begins a full assessment on a 10 year-old patient. To ensure full cooperation from this patient it is most important for the nurse to:

A. Approach the assessment as a game to play.B. Provide privacy for the patient.C. Encourage the friend visiting to stay at the bedside to observe.D. Instruct the child to assist the nurse in the assessment.

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Let’s ReviewDuring a routine health care visit a parent asks the nurse why her 10 month-old infant is not walking as her older child did at the same age. Which response by the nurse best demonstrates an understanding of child development?

A. “Babies progress at different rates. Your infant’s development is within normal limits.” B. “If she is pulling up, you can help her by holding her hand.”C. “She’s a little behind in her physical milestones.”D. “You can strengthen her leg muscles with special exercises to make her stronger.”

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Let’s Review

When assessing a toddler identify the order in which you would complete the assessment:

1. Ear exam with otoscope2. Vital signs3. Lung assessment4. Abdominal assessment

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Let’s Review

When assessing pain in an infant it would be inappropriate to assess for:

A. Facial expressionsB. Localization of painC. CryingD. Extremity movement

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Genetic Disorders

Principles of Inheritance Autosomal Dominant Autosomal Recessive

Sex-linked (X-linked) Inheritance Chromosome Alterations

Down’s SyndromeTay-Sachs Disease

Nursing intervention is supporting parents and resources

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Down’s Syndrome• Most common cause of cognitive impairment (moderate to severe)

• 1 in 600 live births

• Risk factor- pregnancy in women over 35 yrs old

• Cause - extra chromosome 21 (faulty cell division)

• Causes change in normal embryogenesis process resulting in:

Cardiac defects, GI conditions, Endocrine disorders, Hematologic abnormalities, Dermatologic changes

• Physical features: small head, flat facial profile, broad flat nose, small mouth, protruding tongue, low set ears, transverse palmar creases, hypotonia

* Feeding is often a problem in infancy *

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Tay-Sachs Disease Occurs predominately in children of Eastern European Jewish ancestry

Fatal Disease - death usually occurs before age 4

Autosomal recessive inheritance

Degenerative brain disease

Caused by absence of hexosainidase A from body tissue

Symptoms: progressive lethargy in previously healthy 2-6 months old infants, loss of milestones, visual acuity, seizures, hyper-reflexia, posturing, malnutrition, dysphagia

Diagnosis: Classic cherry red spot on macula, enzyme measurement in

serum, amniotic fluid, white cells

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Let’s Review

The infant with Down’s Syndrome is closely monitored during the first year of life for which condition?

A. Thyroid complicationsB. Orthopedic malformationsC. Cardiac abnormalitiesD. Dental malformations

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Pediatric DifferencesNeurosensory System

Size and Structure:Rapid head growth in early childhood

Bones are not fused until 18-24 months

Function:Autonomic Nervous System is intact -

neurons are completely myelinized by 1 year

Infants behavior initially reflexive, but are replaced with purposeful movement by 1 year

Infants demonstrate a dominance of flexor muscles

Motor development occurs constantly in head to toe progression

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Pediatric DifferencesNeurosensory System

Eye and Vision:

Changes in development of eye and eye muscles

*strabismus normal until 6 months

Vision function becomes more organized

Papilledema rarely occurs in infants due to expansion of fontanels with increased ICP

Ear and Hearing:

Hearing fully developed at birth

Abnormal physical structures may indicate genetic problems

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The Neurosensory System

Disorders of the Nervous System

HydrocephalusSpina Bifida

Reyes SyndromeSeizuresCerebral Palsy (CP)Meningitis

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Hydrocephalus• Develops as a result of an imbalance of production

and absorption of CSF• The increase of CSF causes increased ventricular

pressure, leading to dilation of the ventricles, pressing on skull

• Signs/Symptoms of Increased ICP:• Poor feeding and vomiting• Bulging fontanel, head enlargement, separation of sutures• Lethargy, irritability, restlessness, not responsive to parents• CHILD - Headache, vomiting, diplopia, ataxia, papilledema• Seizures

A child’s head with an open fontanel (under 2 years old) has the ability to expand and better compensate for the increased intracranial pressure.

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Ventriculoperitoneal(VP) Shunts

• Relief of hydrocephalus• Prevention/treatment of

complications• Management of problems

related to psychomotor development

• Surgical intervention: ventriculoperitoneal (VP) shunt• One-way pressure valve

releasing CSF into peritoneal cavity where it is reabsorbed


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General Nursing Interventions

• Monitor Neuro Status Determine baseline Assess LOC Assess motosensory Pupil checks Vital signs, Head circ

• Provide Patient Safety Seizure precautions Fall precautions Possible restraints Determine LOC ac

• Decrease ICP Cluster care/ stress Quiet environment HOB 30-45

degrees Appropriate position

(head midline, no hip flexion, no prone)

Medications(pain meds,corticosteroids, diuretics, stool softeners, anti-infectives, anticonvulsants)

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General Nursing Interventions

• Maintain Adequate Cerebral Perfusion Maintain airway Monitor oxygenation

and apply O2 PRN Monitor temperature

and administer antipyretics PRN

Maintain normovolemia Monitor I/O Assess perfusion

• Maintain Nutritional & Fluid Needs Determine swallow

ability prior to PO’s NGT feedings may be

necessary Dietary consult PRN Daily weight Monitor lab results

• Psychosocial Support Child Life consult Teaching

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Spina Bifida: Occulta and Cystica

(meningocele and myelomeningocele)

• Etilogy is unknown, but genetic & environmental factors considered.– Maternal intake of folic acid– Exposure of fetus to teratogenic

drugs• The severity of clinical

manifestations depend on the location of the lesion.– T12 - flaccid lower extremities,

sensation, lack of bowel control and dribbling urine

– S 3 and lower - no motor impairment

• Other complications may occur.– Hydrocephalus (80-90%)– Orthopedic issues such as

scoliosis, kyphosis, club foot– Urinary retention – Skin breakdown/TraumaPhoto Source: Del Mar Image Library; Used

with permission

94

Spina BifidaNursing Interventions• Sterile dressing pre/post-op• Monitor VS, S/S infection• Use latex free items• Avoid stress on sac - prone position

only, especially pre-op; no supine until incision healed

• Monitor for S/S intracranial pressure (ICP)

• Interventions to ICP• Encourage touch & talk• Social service consult

95

Reye’s Syndrome• A true pediatric emergency - cerebral complications may reach an irreversible state. Vomiting & change in LOC to coma

• Acute encephalopathy with fatty degeneration of the liver causing fluid & electrolyte imbalances, metabolic acidosis, hypoglycemia, dehydration, and coagulopathies.

• Most frequently seen in children recovering from a viral illness during which salicylates were given.

• Therapeutic management is intensive nursing care and maintaining adequate cerebral perfusion, &↓ICP.

Increased ICP secondary to cerebral edema is major contributing factor to morbidity and mortality.

96

Seizures• Febrile seizures are the most common in children,

caused by by a RAPID elevation in temperature, usually above 102°.

• Most children do not have a second febrile seizure episode and only about 3% develop epilepsy.

• Focus of care is on patient safety, cause of fever and education of parents for home care.

• Remember basic CPR during seizures – airway before oxygen

• Seizure precautions: Suction, oxygen, padded rails

• Infants often have subtle seizures with only occular movements or some extremity movements.

97

Cerebral Palsy (CP)

1.5 - 5 in 1,000 live births

Neuromuscular disorder resulting from damage or altered structure of part of the brain

Caused by a variety of factors:• Prenatally - genetic, trauma, anoxia• Perinatally - fetal distress, drugs at delivery, precepitate or breech delivery with delay• Postnatally - kernicterus or head trauma

98

Cerebral Palsy (continued)

Spasticity - exaggerated hyperactive reflexes

Athetosis - constant involuntary, purposeless, slow writhing motions

Ataxia - disturbances in equilibrium

Tremor - repetitive rhythmic involuntary contractions of flexor and extensor muscles

Rigidity - resistance to flexion and extension

Associated Problems: Mental retardation, hearing loss, speech defect, dental & orthopedic anomalies, GI problems and visual changes

99

Cerebral Palsy: Nursing

Interventions• Safety

Feed in upright positionSeizure precautionsAmbulate with assistance if ableMedication administration

• Special NeedsNutritional needs include increased calories, assist with feeds, possible GT feeds. Speech, Occupational and Physical therapies

100

Bacterial Meningitis• Infectious process of CNS causing inflammation of

meninges and spinal cord.• ISOLATION IS MANDATORY• Signs and symptoms include those of increased ICP plus

photophobia, nuchal rigidity, joint pain, malaise, purpura rash, Kernig’s and Brudinski’s signs

• Can occur at any age, but often between 1 month-5 years• Most common sequele: hearing and/or visual

impairments, seizures, cognitive changes• Diagnostic confirmation is done by lumbar puncture and

CSF is cloudy with increased WBCs, increased protein, and low glucose

• Nursing Interventions include: appropriate IV antibiotics and meds for increased ICP as well as interventions to decrease ICP

101

Causes of Blindness

Tay-Sach’s disease

Inborn errors of metabolism

Perinatal: prematurity, retrolental fibroplasia

Postnatal: trauma, childhood infections,

Juvenile Arthritis

Genetic Disorders:

102

Causes of Deafness

Conductive:Interference in transmission from outer ear to middle ear from chronic OM

Sensorineural:Dysfunction of the inner ear

Damage to cranial nerve VIII from rubella, meningitis or drugs

103

Let’s Review

Which test would confirm a diagnosis of meningitis in children?

A. Complete blood countB. Bone marrow biopsyC. Lumbar punctureD. Computerized Tomography (CT) scan

104

Let’s Review

In performing a neurological assessment on a patient which data would be most important to obtain?

A. Vital signs.B. Head circumference.C. Neurologic “soft signs”.D. Level of consciousness (LOC).

105

Let’s ReviewA neonate born with myelomeningocele should be maintained in which position pre-operatively?

A. Prone.B. Supine.C. Trendelenberg.D. Semi-Fowler.

106

Let’s ReviewThe nurse witnesses a pediatric patient experiencing a seizure. The primary nursing intervention would be:

A. Careful observation and documentation of the seizure activity.B. Maintain patient safety.C. Minimize the patient’s anxiety.D. Avoid over stimulation and promote rest.

107

Let’s ReviewWhich assessment finding in an infant first day post-op placement of a ventriculoperitoneal (VP) shunt is indicative of surgical complications?

A. Hypoactive bowel sounds.B. Congestion in upper airways.C. Increasing lethargy.D. Incisional pain.

108

Cardiovascular System:

Pediatric Variances

Cardiac arrest is related to prolonged hypoxemia Heart Rate (HR) higher Cardiac Output depends on HR until heart muscle is fully developed (around 5 years of age) Innocuous (benign) murmurs Sinus arrhythmias normal in infants Congenital defects present at birth – the greater the defect, the more severe the clinical manifestations (S/S)

109

FETAL CIRCULATION


110

Cardiovascular System:Changes from Fetal

Circulation

Fetal Circulation - Pattern of Altered Blood Flow Normal Circulatory Changes at Birth:

Oxygenation takes place in LungsStructural changes occur: * Ductus venosus constricts by

3-7 daysbecomes ligamentum venosum

* Foramen ovale closes within first weeks

* Ductus arteriosus functional closure at

24 hours, anatomic closure 1-3 weeks

111

Cardiovascular System:Changes from Fetal

CirculationAbnormal Circulatory Patterns After Birth

Abnormal openings between the pulmonary

and systemic circulations can disrupt blood flow.

♥Blood will follow the path of least resistance -Left side of heart has greater pressure, so . . .

♥Blood normally shunted from left to right

Obstructions to pulmonary blood flow may cause right to left shunting of blood

112

NORMAL HEART ANATOMY BLOOD

FLOW


113

The Cardiovascular System

Care of the Child with Congestive Heart Failure

Congenital Heart Defects

Increased Pulmonary Blood FlowDecreased Pulmonary Blood Flow

Obstruction to Systemic Blood Flow

Acquired Heart Disease

114

Goals of Nursing Care with Congenital Heart

Disease

Reduce workload-Improve cardiac functionImprove respiratory functionMaintain nutrition to meet metabolic demands and promote growth Prevent infection and support/instruct parents

115

Congestive Heart Failure Review

• COMPENSATORY RESPONSES– Tachycardia, especially at rest– Diaphoresis– Fatigue– Poor Feeding– Failure to Thrive (FTT)– Exercise Intolerance– Decreased Peripheral Perfusion– Pallor and/or Cyanosis– Cardiomegaly

116

CLINICAL MANIFESTATIONS-CHF

• PULMONARY– Tachypnea– Dyspnea– Wheezes– Crackles

Retractions– Nasal Flaring– Cough

• SYSTEMIC– Edema (facial)– Sudden weight gain– Decreased Urine

Output– Hepatomegaly– Splenomegaly– Jugular Vein

Distention (JVD, children)

– Ascites

117

CHF: Focused Review Nursing Interventions

Therapeutic ManagementImprove cardiac function – Digitalization; Infant dose calculated 1000micrograms=1mg, ACE inhibitors

Diuretics, fluid restrictions, daily weights, I/O

Decrease tissue demands – Promote rest, minimize stress

Increase tissue oxygenation – Oxygen

Nutrition – Nipple feeds vs. gavage or GT, higher-calorie feeds

118

GENERAL NURSING INTERVENTIONS

• Improve Cardiac Function– Medicate

• Cardiac glycosides (Digoxin)

• Promote Fluid Loss– Medicate

• Furosemide• Spironolactone• Clorothiazide

– Fluid Restriction– Daily Weight– Monitor I/O

• Decrease Cardiac Demands– Promote rest– Minimize Stress– Monitor VS (temp)

• Reduce Respiratory Distress– HOB elevated– Possible supplemental

oxygen• Maintain Nutrition

– Nipple vs. Gavage/GTT– Higher-calorie feeds

(more than 20 cals/oz)

119

Increased Pulmonary Blood Flow (Acyanotic)

• Atrial Septal Defect (ASD)

• Ventricular Septal Defect (VSD)

• Patent Ductus Arteriosus (PDA)• CHF

• Feeding intolerance

• Activity intolerance

• Poor growth, failure to thrive

• Frequent Pulmonary Infections due to “boggy

lungs”

120


121

Decreased Pulmonary Blood Flow (Cyanotic)

• Pulmonary Stenosis • Tetralogy of Fallot• Transposition of the Great Vessels

Assessment findings/Compensatory mechanisms

• Oxygen desaturation• Varying degrees of cyanosis• Polycythemia

122

Decreased Pulmonary Blood Flow (Cyanotic)


123

Obstruction to Systemic Blood flow

• Aortic Stenosis

• Coarctation of the Aorta

• Think perfusion issues

-Diminished or unequal pulses

-Poor color

-Delayed capillary refill time

-Exercise intolerance

124

Obstruction to Systemic Blood flow


125

Rheumatic FeverAcquired Heart Disease

Inflammatory disorder involving heart, joints, connective tissue, and the CNS

Peaks in school-age children

Linked to environmental factors and family history

Thought to be an autoimmune disorder:Commonly preceded by a Strep Throat

Prognosis depends upon the degree of heart damage

Rest important in recovery – priority intervention in acute stage

Strep prophylaxis for 5 years or throughout adolescence

126

Hematologic System:Pediatric Variances

All bone marrow in a young child is involved in theformation of blood cells.

By puberty, only the sternum, ribs, pelvis, vertebrae,skill, and proximal epiphyses of femur andhumerus are involved in blood cell formation.

During the first 6 months of life, fetal hemoglobin is gradually replaced by adult hemoglobin.

127

The Hematologic System

Disorders of Red Blood Cells Iron Deficiency Anemia Sickle Cell Anemia

Disorders of Platelets/Clotting Factors

Idiopathic Thrombocytopenia Purpura (ITP) Hemophilia

128

IRON DEFICIENCY ANEMIA

• Most common nutritional anemia in childhood

• Severe depletion of iron stores resulting in a low HGB level

• Decreased O2 to tissues = fatigue, headache, pallor, increased heart rate

• Occurs after depletion of iron stores in body (6-9 mo of age)• Most likely to occur during rapid physical

growth and low iron intake

129

IRON DEFICIENCY ANEMIA

• Often occurs as a result of increased milk intake

• Lab results show low HGB, HCT, MCV, MCH, MCHC, iron, ferritin

• Teach parents proper nutrition• Meat, spinach, legumes, sweet

potatoes, egg yolks, seafood• Calcium inhibits iron, Vitamin C

enhances iron absorption

130

Sickle Cell Disease

PATHOLOGY Normal RBC has a flexible, round shape RBC w/HbS has a normal shape until it’s O2

delivered to tissue, then sickle shape occurs Stiff, non-pliable – can’t flow freely Trapped in small vessels = causes vaso-

occlusions, tissue ischemia and infarctions – painful episodes, most common area is joints

Hemolysis of RBC- lifespan down to 20 days Compensatory mechanism is increased

reticulocytes


131

Sickle Cell Disease

ACUTE FEBRILE ILLNESS

• High mortality rate <5 years old• Splenic dysfunction

begins at 6 mo old• Prophylactic PCN

– BID at 2-3 mo old• Monitor for Infection

– Temp > 101.5– Respiratory S/S

SPLENIC SEQUESTRATION

• Highly vascular• Susceptible to

injury/infarction• Occurs 6 mo-3yrs• Pallor, fatigue, abd

pain, splenomegaly, CV compromise

• Treatment: IV fluids, PRBC’s

132

Sickle Cell Disease:Nursing

InterventionsGENERAL NURSING CARE• Hydration is Priority!

– Fluid Bolus & maintenance + 1/2

• Oxygen - to decrease sickling of of cells

• Pain Management– Assess

frequently/appropriately– IV Morphine q3-4 hr, PCA– Non-pharmacological

methods

HOME MANAGEMENT

• Pain Control• Fluids• Teaching• Early

Identification of infection

• Immunizations• Avoid dehydration

133

Idiopathic Thrombocytopenic

Purpura (ITP) Acquired hemorrhagic disorder characterized by

thrombocytopenia and purpura Cause is unknown, but is to believed to be an

auto-immune response to disease-related antigens

Usually follows an URI, measles, rubella, mumps, chickenpox

Greatest frequency is between 2-8 years of age Platelet count is below 20,000 Therapeutic management is supportive with

safety concerns. Activity is usually restricted. Acute presentation therapy can include

prednisone, IV immunoglobulin, or Anti-D antibody (causes a hemolytic anemia to rid the body of the antibody-coated RBC’s)

Chronic ITP will involve a splenectomy.

134

Hemophilia• Group of genetic bleeding disorders of which

there is a deficiency of a clotting factor• Most common are Factor VIII (A) & Factor IX

(B)• Bleed LONGER not faster• Clinical manifestations: prolonged bleeding,

bruising, spontaneous hematuria• Management: replacement of missing

clotting factor (recombinant factor VIII concentrate), cryoprecipitate, DDAVP

• NSAIDS (aspirin, Indocin) are contraindicated, they inhibit platelet function

• Regular non-contact exercise/physical therapy is encouraged

135

Hemophilia

COMPLICATIONS• Bleeding into muscle

tissue• Hemarthrosis can

cause joint pain & destruction• Acute Treatment is rest,

ice, elevation, ROM


136

Let’s ReviewWhen assessing a child for any possible cardiac anomalies, the nurse takes the right arm blood pressure (BP) and the BP in one of the legs. She finds that the right arm BP is much greater than that found in the child’s leg. The nurse reacts to these findings in which way?A. Charts the findings and realizes they are normal.

B. Suspects the child may have coarctation of the aorta.C. Suspects the child may have Tetralogy of Fallot.

D. Notifies the physician and alerts the surgery team.

137

Let’s ReviewA 1-month-old infant is being admitted for complications related to a diagnosed ventricular septal defect (VSD). Which physician’s order should be questioned by the nurse?

A. Blood pressure every 4 hours.B. Serum digoxin level.C. Diet: Enfamil 20, nipple 6 oz q2H.D. Supplemental oxygen via nasal cannula prn maintain SaO2 >92%.

138

Let’s Review

A nursing intervention most pertinent for the child with hemophilia is:

A. Sedentary activities to prevent bleeding episodes.B. Meticulous oral care with dental floss to prevent infection.C. Warm compresses to bleeding areas to increase absorption.D. Active range of motion exercises for joint mobility.

139

Let’s ReviewWhich is the most appropriate information to teach a parent of a 14 month-old child with iron deficiency anemia?

A. Increase the child’s daily milk intake to a minimum of 24 ounces.B. Administer oral iron supplement for the child to drink in a small cup.C. Increase the amount of dark green, leafy vegetables and eggs in the child’s diet.D. Encourage the parents to let the child choose foods he prefers.

140

Let’s Review

Which strategy is appropriate when feeding the infant in congestive heart failure?

A. Continue the feeding until a sufficient amount of formula is takenB. Bottle feed no longer than 30 minutesC. Feed the infant every 2 hoursD. Rock and comfort the infant during feedings

141

Respiratory System Pediatric Variances

The airway is smaller and more flexible. The larynx is more flexible and more susceptible to

spasm. The lower airways are smaller with underdeveloped cartilage. The tongue is large. Infants < 6 months old are obligate nose breathers. Chest muscles are not well developed The diaphragm is the neonate’s major respiratory

muscle. Irregular breathing pattern and brief periods of apnea

(10 - 15 secs) are common Abdominal muscles are used for inhalation until age 5-

6 yrs. Respiratory rate is higher Increased BMR raises oxygen needs

142

The Respiratory System

Upper Airway DisordersTonsillitis CroupEpiglottisForeign Body Aspiration

Lower Airway DisordersBronchiolitisAsthmaCystic Fibrosis


143

Tonsillitis

CLINICAL MANIFESTATIONS

Sore throat Mouth breathing Sleep Apnea Difficulty swallowing Fever Throat C&S/Rapid Strep

IMPLEMENTATIONS

Ease Respiratory Efforts Provide Comfort

Warm saline garglesPain MedicationThroat lozenges

Reduce Fever Promote Hydration Administer Antibiotics Provide Rest Patient Teaching Tonsillectomy may be necessary

144

Tonsillectomy

Pre-operative Nursing CareMonitor Labs (CBC, PT, PTT)Age-appropriate Preparation/TeachingSurgical Consent

Post-operative Nursing CareFrequent site assessment - visualize!Monitor for S/S of ComplicationsPain ManagementDiet (push fluids-no citrus juices or red,

advance diet)Patient Teaching

145

Croup/Epiglottitis

• Infection and swelling of larynx, trachea, epiglottis, bronchi

• Often preceded by URI traveling downward

• Causative agent: Viral • Characterized by

hoarseness, barky cough, inspiratory stridor, and respiratory distress

• Most common ages 6 mo-3 yrs

• LTB form most commonPhoto Source: Del Mar Image Library; Used with permission

146

Acute Epiglottitis• Bacterial form of croup affecting epiglottis• LIFE-THREATENING EMERGENCY• Wellness to complete obstruction in 2-6 hours• Most common in ages 2-5 years• Do not examine throat!• Have functional emergency equipment at

bedside - Priority!• Often the child is intubated• 4 D’s - Drooling, Dysphagia, Dysphonia,

Distressed Inspiratory Effort• Lateral Neck X-ray shows “thumb sign”• HIB vaccine has reduced the cases

dramatically

147

Croup/Epiglottitis

Nursing Interventions Maintain Patent

Airway Assess and Monitor Ease Respiratory

Efforts Promote Hydration Reduce Fever Calm Environment Promote Rest

Nursing Interventions

Administer Meds Corticosteroids (HHN) Nebulizer

treatment of Racemic Epinephrine PRN stridor

Antibiotic for epiglottitis

148

Foreign Body Aspiration

• Occurs most often in small children• Choking, coughing, wheezing, respiratory

difficulty• Often it is round food, such as hot dogs,

grapes, nuts, popcorn• Bronchoscopy often needed for removal• Age-appropriate preparation needed for

procedure• Prevention and parent education is very

important

149

Bronchiolitis/RSV• Acute viral infection of the bronchioles

causing an inflammatory/obstructive process to occur

• Increased amount of mucus and exudates preventing expiration of air and overinflation of lungs

• Causative agent in 85% of cases is Respiratory Syncytial Virus (RSV). It is highly contagious - contact isolation must be enforced.

• Nasal swab or nasal washing obtained for viral panel, including RSV

• CXR shows hyperinflation and consolidation if atelectasis present

• Primarily seen in children under 2 years of age

• Most common in winter and early spring• Palivizumab (Synagis)

150

Bronchiolitis/RSVCLINICAL

MANIFESTATIONS Nasal Congestion Cough Rhonchi, Crackles,

Wheezes Increased RR & SOBRespiratory Distress Fever Poor Feeding

IMPLEMENTATIONS Suction – priority Bronchodilator via HHN CPT Promote fluids Monitor VS , SaO2, lung sounds & respiratory effort Supplemental oxygen Reduce fever Promote rest HANDWASHING!

151

Asthma


Tachypnea SaO2 below 95% on RA Wheezes, crackles Retractions, nasal

flaring Non-productive cough Silent chest Restlessness, fatigue Orthopnea Abdominal pain CXR = hyperinflation

INTERVENTIONS Monitor VS (HR, RR) Monitor SaO2 Auscultate lung

sounds Monitor respiratory

effort Humified oxygen Calm environment Ease respiratory

efforts Promote hydration Promote rest Monitor labs/x-rays Patient teaching

152

AsthmaAdminister Medications

Bronchodilator via HHN or MDI with spacer (Albuterol) -Peak flows should always be done before and after Tx

Mast cell inhibitor via HHN or MDI (Cromolyn Sodium - Intal)

Corticosteroid IV or PO (Solu-medrol or Decadron) Antibiotic if precipitated from a respiratory

infection

Home Medication Management Bronchodilator via HHN or MDI with spacer

(Albuterol -Proventil, Levalbuterol - Xopenex) Inhaled steroids (Beclamethasone - Vanceril) Mast cell inhibitor via HHN or MDI (Cromolyn

Sodium - Intal) Leukotriene modifiers PO for long-term control -

Singular

153

Cystic Fibrosis1 in 1,500-2,000 live births

Dysfunction of the exocrine gland (mucus producing)

Multi-system disorderSecretions are thick and cause obstruction and fibrosis of tissue.The clinical manifestations are the result of the obstructive process.

Sweat has a characteristic high sodium- Sweat Chloride Test

Pancreatic involvement in 85% of CF patients

Disease is ultimately fatal. Average age at death: 32 years

154

Cystic FibrosisPULMONARY

MANIFESTATIONS• Initial

• Wheezing• Dry, non-productive

cough

• Eventual & Progressive• Repeated lung infections• Wet & paroxysmal cough• Emphysema/Atelectasis• Barrel-chest

- Clubbing- Cyanosis

GI MANIFESTATIONS• Large, loose, frothy

and foul-smelling stools

• Increased appetite (early)

• Loss of appetite (later)

• Weight loss• FTT• Distended abdomen• Thin extremities• Deficiency of A,D, E, K• Anemia

155

Cystic FibrosisMANAGEMENT/INTERVENTIONS

– Airway Clearance - Chest physiotherapy (CPT) Priority

– Drug Therapy• Bronchodilators - via HHN• Mucolytic Agent (Dnase-Pulmozyme) - via HHN• Antibiotics - via HHN, IV, or PO• Digestive enzymes

Nutrition - needs are at 150%• Increased calories and protein - TPN or GT feedings at

night• Additional fat soluble vitamins• Additional salt with vigorous exercise and hot weather

Exercise Patient Teaching

156

Otitis Media Most common childhood illness Inflammation of middle ear Impaired eustachian tube causes decreased ventilation and drainage Acute otitis media (AOM)

Infectious process by pathogen Infection can spread leading to meningitis S/S: pain, pulling on ears, fever, irritability,

vomiting, diarrhea, ear drainage, full/bulging tympanic membrane

Otitis media with effusion (OME) Inflammation of middle ear with fluid behind tympanic

membrane-no infection Peaks spring and fall (allergies)

Chronic otitis media Inflammation of middle ear > 3 mo Can lead to hearing loss/delayed speech


157

Otitis MediaRISK FACTORS

Secondary smoke Formula feeding

(positioning) Day care Pacifier > 6 mo old

TREATMENT Antibiotics (for AOM) Myringotomy with Pressure

Equalizing (PE) tubesINTERVENTIONS

Teaching No bottle propping Feeding techniques Medication regimen

PAIN MANAGEMENT Fever management Surgery prep if needed


158

Let’s ReviewThe nurse’s first action in responding to a child with tachypnea, grunting, and retractions is to:

A. Place the child in an upright, semi-fowler’s position. B. Apply a pulse oximeter to determine oxygen saturation. C. Assess for further symptoms. D. Call for a stat respiratory nebulizer treatment (HHN).

159

Let’s ReviewA 3-year-old child is brought to the emergency room with a sore throat, anxiety, and drooling. The priority nursing action is to:

A. Inspect the child’s throat for infection.B. Prepare intubation equipment and call the physician.C. Obtain a throat culture for respiratory syncytial virus (RSV).C. Obtain vital signs and auscultate lung sounds.

160

Let’s ReviewAn assessment finding in a child with asthma requiring immediate action by the nurse is:

A. Diminished breath sounds. B. Wheezing in bronchi. C. Crackles in lungs. D. Refusal to take PO fluids.

161

Let’s Review

Which sign is indicative of air hunger in an infant?

A. Nasal flaring. B. Periods of apnea lasting 15 seconds. C. Irregular respiratory pattern. D. Abdominal breathing.

162

Let’s ReviewThe priority nursing intervention in caring for the infant with Respiratory Syncytial Virus (RSV) induced bronchiolitis is:

A. Nasopharyngeal suctioning. B. Coughing and deep breathing exercises. C. Administration of intravenous antibiotic. D. Administration of antipyretics for fever.

163

Gastrointestinal System

• Many GI issues require surgical intervention

• Nursing interventions will often include general pre and post-op care

• Bilious vomiting is a sign of GI obstruction and requires immediate intervention

• Assess stools!• Assess hydration

status


164

Gastrointestinal System

Pediatric Variances• Mechanical functions of digestion are immature at birth• Liver functions are immature throughout infancy• Production of mucosal-lining antibodies is decreased• Infants have decreased saliva• Infant’s stomach lies transversely• Peristalsis is faster in infants• Digestive processes are mature as a toddler• The child’s liver and spleen are large and vascular• Infants and children who vomit bile-colored emesis require immediate attention

• Gastric acidity is low at birth

165

The Gastrointestinal System

Altered Connections Esophageal

Atresia/Tracheoesophageal Fistula Cleft Lip and Palate

Gastrointestinal Disorders Gastroesophageal Reflux Pyloric Stenosis Hirschsprung’s Disease Imperforate Anus Intussusception

Acquired Gastrointestinal Disorders Celiac Disease

Appendicitis Parasitic Worms

166

ESOPHAGEAL ATRESIA & TRACHEOESOPHAGEAL

FISTULA

• Congenital defects of esophagus• EA is an incomplete formation of esophagus• TEF is a fistula between the trachea and

esophagus• Classic 3 “C’s” - coughing,choking,cyanosis


167

ESOPHAGEAL ATRESIA & TRACHEOESOPHAGEAL

FISTULASIGNS/SYMPTOM

• Copious, frothy oral secretions

• Abdominal distension from air in stomach

• Look for 3 C’s• Confirmed with

radiographic studies

TREATMENT• Surgery: either a one-

or two-stage repair • Pre-op care focuses on

preventing aspiration and hydration

• Post-op care focus is a patent airway, prevent incisional trauma

168

Cleft Lip/Palate May present as single defect or

combined Non-union of tissue and bone of upper

lip and hard/soft palate during fetal development

CL-failure of nasal & maxillary processes to fuse at 5-8 weeks gestation

CP-failure of palatine planes to fuse 7-12 weeks gestation

Cleft interferes with normal anatomic structure of lips, nose, palate, muscles – depending on severity and placement

Open communication between mouth and nose with cleft palate

169

Cleft Lip/Palate

Multidisciplinary care throughout childhood and early adulthood

Nutrition is a challenge in infancy ESSR method (enlarge, stimulate,

swallow, rest) Risk for aspiration Respiratory distress

Altered bonding is a possibilityPhoto Source: Del Mar Image Library; Used with permission

170

CLEFT LIP & CLEFT PALATE:

Operative Care Cleft lip surgery by 4 weeks & again at 4-5

yrs Cleft palate surgery at 6-24 months of age,

usually done by 1 year so speech will not be affected

Protect suture lines- priority Monitor for infection

Clean Cleft Lip incision Pain Management Cleft Palate starts feedings 48-hour post-op:

Clear and advance to soft diet No straws, pacifiers, spouted cups Rinse mouth after feeding

171

GASTROESOPHAGEAL REFLUX

Regurgitation of gastric contents back into esophagus - 50% healthy term babies affected

Related to inappropriate relaxation of Lower Esophageal Sphincter (LES) making the LES pressure less than the intra abdominal pressure

GER may predispose patient to aspiration and pneumonia

Apnea has been associated with GER

chance of GER after 12-18 mo old related to growth due to elongation of esophagus and the LES drops below the diaphragm Photo Source: Del Mar Image Library; Used

with permission

172

GASTROESOPHAGEAL REFLUX

SIGNS/SYMPTOMS• Vomiting/spitting

up• Gagging during

feedings• Irritability• Arching/posturing• Frequent URI’s/OM• Anemia• Bloody stools

DIAGNOSTIC EVAL• History of feedings/PE• Upper GI/Barium

swallow to eliminate anatomical problems

• Upper GI endoscopy to visualize esophageal mucosa

• pH probe study

173

GASTROESOPHAGEAL REFLUX:

Therapeutic Management• Positioning

• Prone HOB 30°• Right side

• Dietary modifications• Small, frequent

feedings, burp often• Possibly thicken

formula• Avoid fatty, spicy

foods caffeine, & citrus

• Teach

• Medications• Prokinetic agents:

LES pressure & gastric motility

• Histamine H-2 antagonists are added if esophagitis : acid

• Proton Pump Inhibitors if H-2 ineffective:acid

• Mucosal Protectants

• Surgery: fundoplication

174

HIRSHSPRUNG’S• Aganglionic megacolon

No ganglion cells at affected area usually at rectum/proximal portion of lower intestineAbsence of peristalsis leads to intestinal distension, ischemia & maybe enterocolitis

• Treatment Mild-mod: stool softeners & rectal irrigationsMod-severe: single or 2-step surgeryColostomy with later pull-through Photo Source: Del Mar Image Library; Used

with permission

175

HIRSHSPRUNG’SSIGNS/SYMPTOMS

Infants Unable to pass

meconium stool within 24 hours of life

Abdominal distention Bilious vomiting Refusal to feed Failure to thrive Children Chronic constipation Pellet or ribbon-like

stools (foul-smelling) Vomiting/FTT

NURSING INTERVENTIONS

• Surgery prep: bowel cleansing, antibiotics, NPO, IVF’s, therapeutic play for surgery preparation

• Infection & Skin Integrity: monitor ostomy/anus

• Nutrition & Hydration: NGT, NPO then advance to Diet as tolerated, assess bowel function and abdominal status

176

INTUSSUSCEPTION• Prolapse or

“telescoping” of one portion of the intestine into another

• Abrupt onset• Usually occurs in 3-24

months of age• Sudden abdominal pain• Vomiting• Red, current jelly stool• Abd distention/tender• Lethargy• Can lead to septic

shock Photo Source: Del Mar Image Library; Used with permission

177

INTUSSUSCEPTION

DIAGNOSTIC STUDY• Barium or air enema• Abdominal ultrasound

TREATMENT• Hydrostatic reduction:

force exerted using water-soluble contrast and air to push the affected intestine apart

• Surgical reduction if hydrostatic reduction is unsuccessful

NURSINGINTERVENTIONS

• Monitor for infection, shock, pain

• Maintain hydration - assess status!

• Prepare child/parent for hydrostatic reduction - teach, consent, NPO, NGT

• Monitor stools pre & post procedure

• If surgery: general pre & post-op care

178

PYLORIC STENOSIS

Hypertrophy of pyloric sphincter, causing a narrowing/ obstruction (bands pylorus)

Usually occurs between 2-8 weeks of age Infant presents with non-bilious projectile

vomiting, and is “always hungry” Can lead to dehydration and

hypochloremic metabolic alkalosis Weight loss


179

PYLORIC STENOSIS

DIAGNOSTIC EVAL• History/PE: “olive”

palpated in epigastrum

• Upper GI (string sign)• Abdominal

Ultrasound

TREATMENT• Surgical Intervention:

Pyloromyotomy

INTERVENTIONS• Pre-op: NPO, NGT to

LIS, hydration, I/O, monitor electrolytes

• Post-op: Start feedings in 4-6 hrs. Progressive feeding schedule begin w/5cc GW half strength formula Full strength formula

180

IMPERFERATE ANUS• Anorectal malformations• No obvious anal opening• Fistula may be present from distal rectum to

perineum or GU system• Diagnostic Eval: patency of anus in newborn,

passage of meconium; ultrasound is suspected• Therapeutic Management: manual dilatation

for anal stenosis, surgical treatment for malformations

• Nursing Implementations: pre and post-op care – IV fluids, consent, assessing surgical site for infection and monitoring for complications, possible NGT, diet progression, possible colostomy and teaching; preferred post-op condition is side-lying.

181

Celiac Disease• Malabsorption syndrome characterized by intolerance of

gluten (rye, oats, wheat and barley)

• Familial disease - more common in Caucasians

• Thought to be an inborn error of metabolism or an immunological disorder

• Reduced absorptive surfaces in small intestine which causes marked malabsorption of fats (frothy, foul-smelling stools)

• Child has diarrhea, abdominal distention, failure to thrive

• Treatment is lifelong low-gluten diet; corn and rice are substituted grain foods

182

APPENDICITIS• Inflammation and

infection of vermiform appendix, usually related to an obstruction

• Cause may be bacteria, virus, trauma

• Ischemia can result from the obstruction, leading to necrosis causing perforation

• S/S: periumbilical painRLQ pain (McBurney’s point), fever, vomiting, diarrhea, lethargy, irritability, WBC’s

• Surgery is necessary• If ruptured, often

child will receive IV antibiotics for 24 hrs prior to OR

• Pre-op Care: NPO, pain management, hydration, prep & teaching, consent

• Post-op Care: routine post-op care, IVF/antibiotics, NPODAT, ambulation, positioning, pain management, wound care, possible drains.

183

PINWORM (enterobiasis)

Transmission: oral-fecal Persist in indoors for up to 3 weeks contaminating

anything they contact (toilets, bed linens) S/S: intense perianal itch, sleeplessness, abd pain,

vomiting Scotch tape test – collects eggs laid by female

outside of anus. Must be obtained in am prior to bath or BM.

Treatment:*mebendazole (Vermox) for over 2 years of

age. Under 2 years of age treatment may be pyrvinium pamoate (Povan) which stains stool and emesis red

*All family members must be treated.

184

Let’s Review

Which intervention would have the highest priority for the nurse assisting in the feeding of a child post cleft palate repair?

A. Permiting the child to choose the liquids desired. B. Providing diversional activities during feeding. C. Applying wrist restraints. D. Cleansing the mouth with water after each feeding.

185

Let’s Review

Which food choice by a parent of a child with celiac disease indicates a need for further teaching?

A. OatmealB. Rice C. CornbreadD. Beef

186

Let’s Review

Which assessment finding would the nurse find in a child with Hirschsprung’s Disease?

A. Current jelly stoolB. DiarrheaC. Constipation

D. Foul-smelling, fatty stool

187

Let’s ReviewChildren with gastroenteritis often receive intravenous fluids to correct dehydration. How would you explain the need for IV fluids to a 3 year-old child?

A. “The doctor wants you to get more water, and this is the best way to get it.”B. “Your stomach is sick and won’t let you drink anything. The water going through the tube will help you feel better.”C. “See how much better your roommate is feeling with his IV! You will get better, too.”D. “The water in the IV goes into your veins and replaces the water you have lost from vomiting and diarrhea.”

188

Let’s Review

The nurse caring for a child with suspected appendicitis would question which physician order?

A. NPO statusB. Start IV fluids of D5 ½ NS at 50 mls/hourC. Complete Blood Count (CBC)D. Apply heating pad to abdomen for comfort

189

Genitourinary SystemAnatomy & Physiology

Review• The GU system

maintains homeostasis of the body (water & electrolytes)

• Responsible for the excretion of waste products

• Nephron is the workhorse of the kidney (filter blood at the rate of 125mL/minute)-GFR

• Renin helps maintain Na & water balance (and B/P)

• Kidneys produce erythropoeitin which stimulates RBC production in marrow


190

Pediatric VariancesGenitourinary System

• Infants & young children excrete urine at a higher rate related to the increased BMR producing more waste

• Infant kidneys have function if under stress

• Infant can’t concentrate urine well until 3-6 mo

• In infants, kidney & bladder are abdominal organs

• Infant kidneys are less protected because of unossified ribs, less fat padding & large size

• Young children have shorter urethras• Nephrons continue to develop after birth

191

The Genitourinary System

Minimum urine outputs by age groups:• INFANTS & TODDLERS

– 2-3 ml/kg/hr• PRESCHOOLERS & YOUNG SCHOOL-AGE

– 1-2 ml/kg/hr• SCHOOL-AGE & ADOLESCENTS

– 0.5-1 ml/kg/hr

• TIP: Bladder capacity in ounces: AGE in years + 2Example: a 2-year-old’s bladder can hold up to 4 ounces or 120 mls

192

The Genitourinary System

Disorders of the Genitourinary System

Enuresis Nephrotic Syndrome

Acute Glomerulonephritis Hemolytic Uremic Syndrome

(HUS)

193

Glomerulonephritis

• Group of kidney disorders that show main focus of injury is the glomerulus

• It is characterized by inflammation of the glomerular capillaries

• Acute disorders occur suddenly and resolve completely

• Acute poststreptococcal glomerulonephritis (APSGN) is the most common type

• History, presenting symptoms, and lab results establishes the diagnosis of APSGN

194

GlomerulonephritisPATHOPHYSIOLOGY

ACUTE RENALFAILURE

Kidneys Enlargewith sodium, water, waste

EDEMA

Ineffective Filtration

Proteins Pass ThroughDecreased GFR

InflammatoryResponse

Injury to Capillary Walls

Bacterial Antigens plus Antibodies formImmune Complexes& trap in Glomerulus

StreptococcalInfection

Producing Antibodies

Photo Source: Teresa Simbro, RN, Santa Ana College, Used with permission.

195

GlomerulonephritisASSESSMENT• Hematuria• Proteinuria• Edema: periorbital,

ankles Urine Output• Hypertension• Fatigue• Possible fever• Abdominal discomfort• Labs: +ASO,

Bicarb,K BUN, Creat, H & H

INTERVENTIONS• Monitor Urine

(Dipstick)• Monitor fluid overload• Assess lung

sounds/Resp effort• Possible fluid & salt

restriction• Monitor I/O, Daily

Weights• Monitor VS • Antibiotic, diuretic &

antihypertensive medications

• Promote & provide rest • Provide comfort

measures• Monitor labs

196

Nephrotic Syndrome

• Kidney disorder characterized by proteinuria, hypoalbuminemia, and edema.

• There is primary (involving kidney only) and secondary (caused by systemic disease or heavy metal poisoning) NS. Primary is the most common (MCNS).

• Cause not fully understood-may have an immunologic component.

• Primary age affected is 2-6 years (boys 2:1)• There is no occlusion of glomerular vessels.• Loss of immunoglobulins also occur (IgG)• Hypovolemia and the severe proteinuria put the

child in a hypercoagulable state• Treatment is prednisone (2mg/kg/day) for about 4-

6 weeks. Remission is obtained when the urine protein is 0-tr for 5-7 days

• Albumin followed by furosemide may be given for the edema

197

Nephrotic Syndrome

PATHOPHYSIOLOGY

Decreased RenalBlood Flow

Triggers Renin ProductionCausing Increased Aldosterone

EDEMA

Reabsorption of Sodiumand Water retention

Hyperlipidemia

Fluid ShiftIntravascular to

InterstitialHYPOVOLEMIA

Proteinuria(Hypoalbuminemia)

Damage toBasement Membrane

of glomerulus(increased permeability)

Alterationin

Glomerulus


198

Nephrotic Syndrome ASSESSMENT

• Proteinuria (3-4+), frothy urine

• Edema (pitting):periorbital, genitals, lower extremities, abdominal

Urine Output (Hypovolemia)

• Normotensive or hypotensive

• Fatigue • Recent URI, Pneumonia• Abdominal Pain/Anorexia• Labs:

Albumin Platelets H & H Cholesterol Triglycerides

INTERVENTIONS• Monitor Urine

(Dipstick)• Monitor

edema/dehydration• Assess skin

integrity/turn often• Possible fluid & salt

restriction• Monitor I/O, Daily

Weights• Monitor VS & S/S of

infection • Administer medications• Promote & provide rest • Monitor labs• HANDWASHING/monitor

visitors

199

Hemolytic Uremic Syndrome (HUS)

• It is the most common cause of acute renal failure (ARF) in children.

• HUS is characterized by the triad of anemia, thrombocytopenia, and ARF.

• Most children have associated GI symptoms- almost all are caused by e. coli 0157.

• Treatment is supportive and based on symptoms.• No antibiotics are given; more damage can be

caused. • Serum electrolytes may be outside of normal limits.• Blood transfusions and/or dialysis may be

necessary.• More than 90% of the children recover with good

renal function.

200


Thrombocytopenia

Acute Renal Failure

Decreased GFR

Occlusion of Vessels

(Glomerular Vessels)

Fragmented RBC'sCausing Anemia

Inflammatory ResponseCollection of

FibrinLipids

Platelet Fragments

Damages Capillary Walls

Bacteria Adheres toGI MucosaMultiplies

Releases Toxins

GASTROENTERITIS

e. coli #0157


201


ASSESSMENT• History: emesis, bloody

diarrhea, abd pain, Urine

• Petechiae, bruises, purpura

• Edema (possible CHF)• Hepatosplenomegaly• Altered LOC, seizure• Hypertension• Fatigue• Abdominal discomfort• Labs: Lytes may be

abnormal BUN Creatinine H & H Platelets

INTERVENTIONS• Monitor I/O, Daily

Weights• Evaluate for signs of

bleeding• Monitor fluid

overload/edema• Assess for dehydration• Monitor VS with neuro

checks• Seizure Precautions,

HOB • Diuretic &

antihypertensive medications

• Provide rest/calm environment

• Provide comfort measures

• Monitor labs closely

202

Enuresis

• Involuntary passage of urine in children whose chronological or developmental age is at least 5 years of age

• Voiding occurs at least twice a week for minimum 3 months

• More common in boys• Alteration in neuromuscular bladder function• Often benign and self-limiting• Organic factor could be the cause• Familial tendency• Emotional factor could be considered• Therapeutic techniques include: bladder training,

night fluid restriction, drugs (imipramine, oxybutynin, DDAVP)

203

Let’s Review

A clinical finding that warrants further intervention for a child with acute post-streptococcal glomerulonephritis is:

A. Weight loss to 1 pound of pre-illness weight.B. Urine output of 1 ml/kg per hour.C. A normal blood pressure.D. Inspiratory crackles.

204

Let’s Review

A 3 year-old is scheduled for surgery to remove a Wilms tumor from one kidney. The parents ask the nurse what treatments, if any, will be necessary after recovery from surgery. The nurse’s explanation is based on knowledge that:

A. No additional treatments are necessary.B. Chemotherapy may be necessary.C. Chemotherapy is indicated.D. Kidney transplant is indicated.

205

Let’s Review

Fluid balance in the child who has acute glomerulonephritis is best estimated by assessing:

A. Intake and outputB. Abdominal circumferenceC. Daily weightsD. Degree of edema

206

Let’s Review

In evaluating the effectiveness of nursing actions when caring for a child with nephrotic syndrome, the nurse expects to find:

A. A recurrence of pneumonia.B. Weight gain.C. Increased edema.D. Decreased edema.

207

Pediatric VariancesMusculoskeletal

SystemBone Growth:

Linear growth results from skeletal development

Bone circumference growth occurs as new bone tissue is formed beneath the periosteum

Skeletal maturity is reached by age 17 in boys and 2 years after menarche in girls (14 yrs)

Bone growth affected by Wolff’s Law - bone grows in the direction in which stress is placed on it

Certain characteristics of bone affect injury and healing

Children’s bones are softer and are easily fractured

208

Pediatric VariancesMusculoskeletal

System

Muscle Growth:

Responsible for a large part of increased body weight

The number of muscle fibers is constant throughout life

Results from increase in size of fibers and increased number of nuclei per fiber

Most apparent in adolescent period

209

The Musculoskeletal System

Disorders of the Musculoskeletal System

Developmental Dysplasia of the HipTalipes (Clubfoot)Osteogenesis ImperfectaScoliosisMuscular DystrophyJuvenile Rheumatoid Arthritis

210

Developmental Dysplasia of the Hip

(DDH)Variety of hip abnormalities – shallow acetabulum, subluxation or dislocationOften made in newborn period – often appears as hip joint laxity rather than dislocationOrtolani click if < 4 weeks old, older ultrasound needed to diagnoseTreatment is Pavlik Harness (abducted position) for newborn to 6 months old – monitor for Avascular Necrosis6-18 months – traction followed by spica castOlder children – operative reductionPriority nursing interventions are skin care and facilitating normal growth and development

211

Talipes (Clubfoot)

Most common type is when foot is pointed downward and inward

Often associated with other disorders May be due to decreased movement in

utero Treatment requires surgical intervention Serial casting is begun shortly after birth

and usually lasts for 8-12 weeks Priority nursing interventions are skin

care and facilitating normal growth and development

212

Osteogenesis Imperfecta (OI)

Inherited disorder of connective tissue and excessive fragility of bones

Pathologic fractures occur easily Incidence of fractures decrease at puberty related

to increased hormones making bones stronger Treatment is supportive: careful handling of

extremities, braces, physical therapy, weight control diet, stress on home safety

Surgical techniques for correcting deformities and for intermedullary rodding

213

Scoliosiso Abnormal curvature of the spine (lateral)o Congenital or develops later, most common during

the growth spurt of early adolescence (idiopathic)o Diagnosis is made by physical exam and x-rayso Treatment for curvatures < 40 degrees is bracingo Surgical intervention is for severe curvatures –

internal fixation and instrumentation (Harrington)o Postoperative care includes logrolling, neurologic

assessments, pain management, skin care, assessing for paralytic ileus and possible mesenteric artery syndrome

o Don’t forget the developmental needs of the adolescent

214

Muscular Dystrophy• Duchenne’s Muscular Dystrophy most common• Gradual degeneration of muscle fibers• S/S begin to show about 3 years of age –

difficulties in running and climbing stairs• Changes to having difficulty moving from a

sitting/supine position• Profound muscular atrophy continues, wheelchair

by 12 yrs• Respiratory and cardiac muscles affected and

death is usually respiratory or cardiac in nature• Diagnosis made with physical exam, muscle

biopsy, EMG, serum studies: AST (SGOT), aldolase, creatine phosphokinase high first 2 years of life

• Nursing care is to maintain optimal level of functioning and to help the child and family cope with the progression and limitations of the disease

215

Juvenile (Rheumatoid)

Arthritis• Inflammatory disease with an unknown cause• Occurs in children < 16 years; lasts > 6 weeks• Clinical manifestations: stiffness, swelling, and

loss of motion in affected joints, tender to touch• Therapeutic management includes drug therapy

(NSAID’s, SAARD’s, cytoxic drugs, corticosterioids), physical and occupational therapy, exercise (swimming), moist heat for pain and stiffness, general comfort measures

216

General Nursing Interventions for Children

with Musculoskeletal Dysfunctions (immobility)

• Maintain optimal level of functioning• Promote general good health• Facilitate compliance• Facilitate optimal growth and

development• Maintain skin integrity• Safety considerations at home• Pain management• Support child and family

217

Let’s Review

An infant is being treated non-surgically for clubfoot. Which describes a major goal of care for this patient? Prevention of:

A. Skin breakdownB. Calf atrophyC. Structural ankle deformitiesD. Thigh atrophy

218

Let’s ReviewThe nurse is helping parents create a plan of care for their child with osteogenesis imperfecta. A realistic outcome is for this child to:

A. Have a decreased number of fracturesB. Demonstrate normal growth patternsC. Participate in contact sportsD. Have no fractures after infancy

219

Let’s Review

During acute, painful episodes of juvenile arthritis, a priority intervention is initiating:A. A weight-control diet to decrease stress on the joints.B. Proper positioning of the affected joints to prevent musculo-skeletal complications.C. Complete bedrest to decrease stress to the joints.D. High-resistance exercises to maintain muscular tone in the affected joints.

220

Pediatric VariancesEndocrine SystemGrowth Hormone:

Does not effect prenatal growth

Main effect on linear growth

Maintains rate of body protein synthesis

Thyroid-stimulating hormone (TSH):

Important for growth of bones, teeth, brain

Secretion decreases throughout childhood and

increases at puberty

Adrenocorticotrophic Hormone (ACTH):

Activated in adolescent

Stimulates adrenals to secrete sex hormones

Influences production of gonadotropic hormone

221

The Endocrine System

Disorders of the Endocrine System

Type 1 Diabetes Mellitus Congenital HypothyroidismGrowth Hormone DeficiencyPrecocious Puberty

222

Type 1 Diabetes MellitusPediatric

Considerations INSULIN

• Most children are well-controlled with BID dosing of fast acting (Lispro) short acting (regular) and intermediate acting (NPH, Lente) insulin. There is also Lantis, an insulin that acts a “basal.”

• U-20 insulin is also available for infants• Insulin pump, pen• “Honeymoon” phase • Stress, infection, illness and growth at

puberty can increase insulin needs

223


Considerations• HYPOGLYCEMIC EPISODES

• In small children it is more difficult to determine and may just be a behavior change.

• Treatment is the same – simple sugar – assess LOC first!

• NUTRITION• Carb counting – most children’s calories should

not be restricted; meal plan might change as child grows.

• Some sweets may be incorporated into the diet and may help with compliance.

• 3meals with 3 snacks per day

224


Considerations EXERCISE

• Important for normal growth and development

• Assists with daily utilization of dietary intake• Enhances insulin absorption, so may

decrease amount needed• Add 15-30 grams of carbs for each 45-60

minutes of exercise• Watch for hypoglycemia with strenuous

exercise

225


ConsiderationsDEVELOPMENTAL ISSUES

• Infant/Toddler• Autonomy & choices, rituals, hypoglycemia

identification difficult• Preschooler

• Magical thinking-let them know they did not cause it• Use dolls for teaching• Urine testing may be done• Can choose finger to use for testing

• School-age• Very busy with school and activities• Likes tasks and explanations• Can do self blood testing; injections at age 8-10 years

• Adolescents• Peers and body image preoccupation• High risk for non-compliance• Collaborative health care with parent involvement very

important

226

Congenital Hypothyroidism

• Thyroid is not producing enough thyroid hormone to meet needs of the body (resulting in↓oxygen consumption, BMR and protein synthesis)

• Clinical manifestations: cool, mottled skin, bradycardia, large tongue, large fontanel, hypothermic, hypotonia, lethargy, feeding problems - THINK SLOW!

• Labs: High TSH, low T4• Decreased brain development will result with

cognitive impairments• Part of newborn screening• Therapeutic management is life-long thyroid

hormone replacement (levothyroxine)

227

Growth Hormone (GH) Deficiency

• Deficient secretion of growth hormone• Definitive diagnosis is made with GH levels

(using stimulation testing) under 10mg/ml and x-rays of hand and wrist for ossification levels

• Treatment is replacement of GH (subcutaneous daily injections) until goals met

• Nursing care is directed at child and family support

• Remember to interact and speak to the child at her appropriate developmental level!

228

Precocious Puberty• Manifestations of sexual development in boys

younger than 9 years and girls younger that 8 yrs

• Causes also an early acceleration of growth with closure of growth plates

• Therapeutic management is directed toward the specific cause, if known

• The early secretion of sex hormones will be treated with monthly subcutaneous injections of leuteinizing hormone-releasing hormone (LHRH)

• Priority interventions are directed at psychological support of child and family – encourage play with same age peers

229

Let’s ReviewA child weighing 25 kilograms is being

treated with synthetic growth hormone. The recommended dosage range is 0.3 – 0.7 mg/kg/week. The mother informs the nurse that her child receives 1.25 mg subcutaneously at bedtime 6 times per week. The proper response from the nurse would be:

A. “That dose is too high, the doctor needs to be notified.”

B.“You are doing a great job, that is the correct dose for your child.”

C. “The injection should be given intramuscular, not subcutaneous.”

D.“That dose is too low based on your child’s new weight.”

230

Let’s ReviewThe nurse should include which information in teaching the parents of a recently diagnosed toddler with Type 1 diabetes mellitus?

A. Allow the toddler to choose which finger to use for blood glucose monitoringB. Allow the toddler to assist with the daily insulin injectionsC. Test the toddler’s blood glucose every time she goes out to playD. Let the toddler determine meal times

231

Let’s ReviewWhich is the most appropriate teaching intervention for a nurse to give parents of a 6-year-old with precocious puberty?

A. Advise the parents to consider birth control for their childB. Inform the parents there is no treatment currently availableC. Explain the importance for the child to foster relationships with peersD. Assure the parents there is no increased risk for sexual abuse.

232

Let’s Review

Number in order of priority the following interventions needed while caring for a patient in diabetic ketoacidosis.

_____ Hydration_____ Electrolyte replacement_____ Dietary intake _____ IV Insulin_____ Subcutaneous insulin

233

Pediatric VariancesIntegumentary

System

Evaporative water loss is greater in infants/small children

Skin more susceptible to bacterial infections

More prone to toxic erythema

More susceptible to sweat retention and maceration

234

The Integumentary System

Disorders of the Integumentary System

Impetigo Roseola Diaper Rash

235

Impetigo

• Superficial bacterial skin infection, often secondary from insect bite

• Highly contagious• Late summer outbreak• Toddlers &

preschoolers• Rash is bullous or

honey-colored crusted lesions

• Treatment: topical & systemic antibiotics, comfort measures, teaching, preventing comps


236

Roseola

• Transmission: contact with secretions (saliva)

• Virus• 6 - 18 months• Fever »flu symptoms »

rose-pink macular rash• Fades with pressure• Treatment is supportive


237

Diaper Rash

• Cause could be fungal in nature; assess mucous membranes for thrush

• Cause could be due to infrequent diaper changes, an allergic reaction to the diaper product or diarrhea

• Skin care includes appropriate skin barrier cream/ointment, keeping area dry

• Teach parents appropriate skin care

238

Medication Administration

Oral Medication Hold infant with head elevated to

prevent aspiration

Slowly instill liquid meds by dropper along side of the tongue

Crush pills and mix with sweet-tasting liquid if permitted, but don’t add too much liquid!

Allow choices for the child such as which med to take first

Flush following gastrostomy or NG tube

239

Factors to consider when selecting IM sites

Age Weight Muscle development Amount of subcutaneous fat Type of drug Drug’s absorption rate

240

IM and SQ Meds

Select needle length according to muscle size for IM

Infant - should use 1 inch needle Preemies can use 5/8 inch needle

• Use Z-track for iron and tissue-toxic meds• Apply EMLA or other topical anesthetic 45-60 minutes prior to injection• May mix medication with lidocaine • Some medications may be need to be separated into 2 injections depending on amount

241

Peds IM Injection Sites

Vastus lateralis for infants Ventrogluteal and dorsogluteal

Don’t inject into dorsogluteal until age 3 years - muscle not well developed until child walks and sciatic occupies a larger portion of the area.

Deltoid after 3 years

242

IV Meds

Site may be peripheral or central Administer IV fluids cautiously Always use infusion pumps with infants and small children Inspect sites frequently (q 1-2 hours) for signs of infiltration Cool blanched skin, puffiness( infiltration) Warm and reddened skin (inflammation)

243

Nose Drops

Instill in one nare at a time in infants because they are obligate nose breathers.

Suction nare with bulb syringe prior to administration if nasal congestion present

244

Ear Meds

Pull the ear down and back to instill eardrops in infants/toddler (↓3 years pull ↓)

Pull the ear up and out to instill in older children (↑ 3 years pull ↑)

Have medication at room temperature

245

Rectal Medication

Insert the suppository past the anal sphincter

Hold buttocks together for a few seconds after insertion to prevent expulsion of medication

It is a very stressful route for children, and the school-age and adolescent have issues with modesty.

246

Inhalers and Spacers Shake the inhaler for 2-5 seconds. Position inhaler into spacer (with mask or

mouthpiece). After normal exhale, place mask on face or

mouthpiece in mouth – both with a good seal. Have child inhale slowly after canister is pressed

down . Have child take a few breaths with a spacer and

without a spacer have them hold breath for few seconds after medication released.

Inhalers without spacers aren’t placed in the mouth because spacers require a seal around mouthpiece; masks with spacers can be used for infants.

247

MDI with Spacer MDI with Spacer and

Mask


248

Let’s Review

The nurse would prepare which site for an intramuscular injection to a 11 month-old?

A. DorsoglutealB. DeltoidC. Vastus lateralisD. Ventrogluteal

249

Pediatric Oncology

Cancer is the leading cause of death from disease in children from 1 - 14 years.

Incidence: 6,000 children develop cancer per year

2,500 children die from cancer annually

Boys are affected more frequently

Etiologic factors: environmental agents, viruses, host factors, familial/genetic factors

Leukemia is the most frequent type of childhood cancer followed by tumors of the CNS system.

250

Oncology Stressful Events

“Treatment is worse than the disease.”

1. Diagnosis

2. Treatment - multimodal

3. Remission

4. Recurrence

5. Death

251

Oncology Interventions

Surgery

Radiation Therapy

Chemotherapy

Bone Marrow Transplant

252

Stages of Cancer Treatment

1. Induction

2. Consolidation

3. Maintenance

4. Observation

5. Late Effects of Treatment• Impaired growth & development

• CNS damage

• Psychological problems

253

Pediatric Oncology

Types of Childhood Cancers

Leukemia Brain Tumors Wilm’s Tumor Neuroblastoma Osteogenic Sarcoma Ewing’s Sarcoma

254

LeukemiasMost common form of childhood cancer

Peak incidence is 3 to 5 years of age

Proliferation of immature WBCs (blasts)

May spread to other sites (CNS, testes)

Types of Leukemia: Acute lymphocytic leukemia (ALL)

• 80-85% of childhood leukemia• 95% chance of remission

Acute nonlymphocytic Leukemia (ANLL)

• 60-80 % chance of remission

Treatment is chemotherapy: prednisone, allupurinol, selected chemotherapeutic agents

255

Leukemias


• Purpura, Bruising• Pallor• Fever Unknown

Origin• Fatigue, Malaise• Weight loss• Bone pain• Hepatosplenomegaly• Lymphadenopathy

LABS & DIAGNOSTIC TESTS

↑ WBC’s (50-100) orVery low WBC’s ↓Hgb, Hct, PlateletsBlast cells in

differentialBONE MARROW

ASPIRATIONLUMBAR PUNCTURE BONE SCAN possible

256

Brain Tumors

Second most prevalent type of cancer in children

Males affected more often

Peak age 3 - 7 years

Types: Medulloblastoma

Astrocytoma

Brain Stem glioma

Look for S/S of increased ICP and area of brain affected

257

Wilm’s Tumor

Also known as Nephroblastoma

Large, encapsulated tumor that develops in the renal parenchyma (do not palpate abdomen!)

Peak age of occurrence: 1 - 3 years

Prognosis is good if no metastases- lungs first

Treatment is surgery, chemotherapy and sometimes radiation

258

Neuroblastoma

Highly malignant tumor – extracranial

Often develop in adrenal gland, also found in head, neck, chest, pelvis

Incidence: One in 10,000

Males slightly more affected

From infancy to age 4

Often diagnosed after metastasis occurs

Treatment includes surgery, chemotherapy and radiation

259

Bone TumorsOsteogenic Sarcoma:

Occurs most often in boys between 10-20 yrs

10-20% 5 year survival rate

Primary bone tumor of mesenchymal cell

Treatment:surgery (amputation or salvage) and chemo

Ewing’s Sarcoma:

Occurs in boys between 5 - 15 years

Primary tumor arising from cells in bone marrow

Treatment is radiation and chemotherapy

260

Pediatric Oncology:Nursing

Interventions

CHEMOTHERAPY SIDE EFFECTS

• Leukopenia (Nadir)

• Thrombocytopenia• Stomatitis• Nausea/Vomiting• Alopecia• Hepatotoxicity• Nephrotoxicity

NURSING INTERVENTIONS

• HANDWASHING!• Monitor visitors• Monitor for

infection• Meticulous oral care• Antiemetics ATC• Monitor Labs• Support/Teaching

261

Pediatric Oncology:Nursing Interventions

• Supportive care for radiation treatment, focusing on skin care

• Surgical interventions are based on location and type of surgery• Basic pre and postoperative care

• Psychosocial care for patient and family – utilize Child Life and Social Services

262

Pediatric Oncology

• Teach, teach, teach!

• Support the child and family

• Provide resources• Be honest• Include the child in

the care planning


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Let’s ReviewIn caring for the child with osteosarcoma, it is important for the nurse to inform the child and family of the treatment plan. Which would be appropriate?

A. The affected extremity will have to be amputated.B. The child will only need chemotherapy.C. Both surgery and chemotherapy are indicated.D. Only palliative measures are taken.

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Let’s Review

The nurse assessing a child who is undergoing chemotherapy finds the child to be suffering from mucositis. Which intervention would be the highest priority?

A. Meticulous oral care.B. Obtain dietician consult. C. Place the child on a full liquid diet only.D. Medicate for pain around the clock.

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Let’s Review

The priority nursing intervention in caring for a child with acute lymphocytic leukemia (ALL) during the child’s nadir period is:

A. Handwashing.B. Monitoring lab results.C. Administering antiemetics.D. Monitoring visitors.

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Death & DyingChild’s Response to Death:

Infants & Toddlers:

Do not understand

Viewed as a form of separation

Can sense sadness in others

Preschooler:

Death is temporary

Viewed as sleep or separation

Feel guilty and blames self

Dying children may regress in behavior

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Death & Dying

School-Age: Have concept of irreversibility of death

Fear, pain, mutilation and abandonment

Ask many questions

Feel death is a punishment

May personify death (bogeyman)

Will ask directly if they are dying

Interested in the death ceremony

Comforted by having parents and loved ones with them

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Death & Dying

Adolescent:

Have an accurate understanding of death

Death as inevitable and irreversible

May express anger at impending death

May find it difficult to talk about death

May wish to leave something behind to remember them by

May wish to plan own funeral

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Death & Dying

Parental responses to death:

Major life stress

Experience grief at potential loss of child

Related to circumstances regarding child’s death (denial, shock, disbelief, guilt)

Confronted with major decisions regarding care

May have long term disruptive effects on family

Bereaved parents experience intense grief of long duration

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Communicating with the Dying Child and Family

•Use child’s own language

•Don’t use euphemisms

•Don’t expect an immediate response

•Communicate through touch

•Encourage questions and expressions of feelings

•Strengthen positive memories

•Listen, touch, cry

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Impending Death Care Guidelines

Do not leave child alone

Do not whisper in the room

Touching the child is very important

Let the child and family talk and cry

Let parents participate in care as much as they are emotionally capable of doing

Continue to read favorite stories or play the child’s favorite music

Be aware of the needs of the siblings

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Let’s ReviewWhich intervention would be most helpful in supporting a dying child’s family as they cope with the various decision-making periods of a lengthy terminal illness?

A. Encouraging the parents to take their child home to die.B. Encouraging the parents to go through all of the Kubler- Ross stages of dying as quickly as possible.C. Referring the child’s family to the hospital clergy service as soon as possible.D. Using active listening to identify specific fears and concerns of the child’s family members.

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Types of Child Abuse Neglect: Intentional or unintentional

omission of basic needs and support

Physical Abuse: Is non-accidental injury to a child

by an adult

Sexual Abuse: Forced involvement of children in

sexual activities by an adult

Emotional Abuse:Withholding of affection, use of cruel and degrading language towards a child by an adult

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Child Abuse

Reports of violence against children has almost tripled since 1976.

Many of the abused children are infants.

“Red Flags”Fractures in infants Spiral fractures Injuries do not match story told

NURSES ARE MANDATED NURSES ARE MANDATED REPORTERSREPORTERS

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Child AbuseNeglect

Physical or emotional maltreatment Failure to thrive Contributing factors may be ignorance or lack of

resources

Physical Abuse Minor or major physical injury (bruising, burns,

fractures) May cause death Munchausen by Proxy (MSP) Shaken baby syndrome (SBS)

Sexual Incest, molestation, child porn, child

prostitution

Emotional May be suspected, but difficult to substantiate Impairs child’s self-esteem and competence

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Child AbuseWarning Signs

Incompatibility between history of event and injuries Conflicting stories from various people involved History inconsistent with developmental level of child Repeated visits to emergency rooms Inappropriate response from child and/or caregiver

Nursing Interventions Assess: Physical assessment and history of event, observe

and listen to caregiver’s and child’s verbal and non-verbal communication

Documentation: Complete CAR form and contact Child Protective Services, hospital documentation

Support family and child: Social services, resources, teaching

THE CHILD’S SAFETY COMES FIRST AND IS THE PRIORITY!

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Let’s Review

In caring for a 4 year-old with a diagnosis of suspected child abuse, the most appropriate intervention for the nurse is:

A. Avoid touching the child.B. Provide the child with play situations that allow for disclosure of event.C. Discourage the child from speaking about the event.D. Give the child realistic choices to feel in control.

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Let’s Review

Which pediatric patient would most necessitate further investigation by the community-based nurse?

A. An adolescent who prefers to spend time with friends rather than family.B. A toddler with dark bruises located on both legs.C. An infant with numerous insect bite marks and diaper rash.D. A preschooler with dirty knees and torn pants.

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Photo Acknowledgement:All unmarked photos and clip art contained in this module

were obtained from the 2003 Microsoft Office Clip Art

Gallery.

1 PEDIATRIC NURSING Care of the Child and Family.

Documents

Transcript of 1 PEDIATRIC NURSING Care of the Child and Family.