1

Exam 3

PEDS study guide

Chap 34- Fractures p1159

Complete fracture- fragments are completely separated

Incomplete- remain intact

Transverse: crosswise, at right angles to the long axis of bone

Oblique-slanting by straight

Spiral: slanting and circular twisting around bone shaft

Simple/closed fracture- does not break skin

Compound-open wound, bone protrudes

Complicated- if bone fragments damages organs/tissues

lying in surrounding tissues

Comminuted- small fragments

Growth plate/ physeal- weakest point of long bones; frequent site of injury; close monitoring &
early treatment are essential to prevent long./angular deformities; may require surgical
treatment w/ open reduction & internal fixation; Healing is usually rapid in the epiphyseal plate
area. Complete immobilization is not necessary
Immediately after fracture occurs: muscle contract splinting the area; muscle
pulling/contracting pulls bones
out of alignment; muscle can be overcome by traction or complete relaxation with
anesthesia in order to realign
Salter-Harris classification of growth plate injuries
Type 1 slip/separation of GP w/o bone fracture
Type 2: separation of GP & broken metaphysic
Type 3: fracture of physis extending through the epiphysis into the joint surface
Type 4: fracture of growth plate, epiphysis, and metaphysic
Type 5: crushing, comminuted fracture of the physis
Cast purpose: joints above and below the site of injury are immobilized to eliminate the
possibility of movement that might cause displacement
Consist of gauze strips and bandages impregnated with: Plaster of Paris, Fiberglass,
polyurethane resin,
waterproof liners
Both types of cast produce heat from a chemical reaction activated by water
Soak caked skin in tub after cast removal
Plaster- used for molding closely to body part/heavier/not light weight
Cast removal- explain to pt. that heat and vibrations occur during removal
Plaster -24-48 hrs to dry; support with pillow in plastic; avoid indenting cast, creating pressure
points; turn plaster cast q2hr to help dry evenly; hair dryer cool setting; makes hollow sound
when dry

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Fiberglass-dries within minutes/must remain uncovered to dry from inside out
Chief concern- compartment syndrome: cast becomes tourniquet, cutting off circulation causing
neurovascular complications during first hr after applying cast
Mobilization devices- lighter better fitting devices leading to greater usage compliance
Orthotics- braces, fitting and fabrication; used to prevent deformity, increase energy
efficiency of gait and
control alignment
AFO-ankle foot
weakness,

SMO- supramalleolar

KAFO- knee ankle foot orthosis- use to prevent buckling of knee, marked quad
protect weak bone structure
Care of Skin: AFO, KAFO, HKAFO

decreased sensation in the legs, check the skin condition more frequently
than every 4 hours.
burning sensation under the brace, remove the brace and observe the skin
for any reddened areas. If the child complains of burning several times,
contact the provider or orthotist.
small blister or open area develops, cover it with a sterile bandage and check
the skin more often. Do not put alcohol on open areas; avoid wearing the
device until the skin heals.
If no sign of healing occurs after 3 days, contact the provider or orthotist.
Lotions and creams will soften the skin and should be used only if the skin is
dry.

TLSO

Because the device works by pressing against the body and is kept very snug
by straps and buckles, some skin pinkness is to be expected. The skin at the
brace edges and under the pads should be inspected carefully and
frequently, especially in the initial period when the child or adolescent is
getting accustomed to the device. Any red mark that does not fade within 20
minutes, or any area that appears raw and sore, should be reported to the
orthotist.
Have the child wear a cotton undershirt under the brace to protect the skin;
keeping it clean, dry, and free of wrinkles will help prevent skin problems.

Prosthetics- artificial limb fitting & fabrication

Sprain- joint trauma stretching ligament; often accompanied by vessel, muscle, tendon and
nerve damage
Joint laxity most valid indicator of the severity of sprain; feels loose, snap, pop, tearing
seldom are sprains
painful; rapid swelling immediate disability reluctance to use injured
joint

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Strain- microscopic tear to tendon; painful to touch swollen; happens over time; the more rapid
it occurs the more severe
Scoliosis- complex spinal deformity in three planes, usually involving lateral curvature, spinal
rotation causing rib asymmetry, and when in the thoracic spine, often thoracic hypokyphosis
Most common deformity
Congenital- fetal development Infantile-birth-3yrs Juvenile- 3-10yrs Adolescent 10+
May be caused by a number of conditions and may occur alone or in association with other
diseases, particularly neuromuscular conditions (neuromuscular scoliosis).
Idiopathic scoliosis: no apparent cause; identified during preadolescent growth spurt, abnormal
school screening, poor fitting clothes/jeans
AAOS & AAP scoliosis screening by school, physician office, & nurse clinic; screener must be
educated in the detection of spinal deformity
Girls screen at 10-12 yrs old Boys 13-14 yrs old
Therapeutic MNG of Scoliosis- observation w/ regular clinical, radiographic Eval, brace
(orthotic intrvnt.) and surgical spine infusion
Treatment based on magnitude, location type of curve age skeletal maturity & any
underlying disease processes
Bracing- moderate curves 25-45 degrees; not curative, slows progression to allow growth
& maturity
Nursing care of the adolescent who is facing scoliosis surgery, potential social isolation,
pain, and
uncertainty, not to mention misunderstood emotions and body image issues, must
be evaluated from the
adolescents perspective
The management involves the skills and services of a team of specialists, including the
orthopedist, physical therapist, orthotist (a specialist in fitting orthopedic braces), nurse,
social worker, and sometimes a thoracic or pulmonary specialist.
Developmental hip dysplasia

Abnormal development of the hip that may develop at any time during fetal life, infancy,
and childhood
Risk factors: breech birth, female gender, family history of DDH, left hip, first-born child,
and positive clicking hips with clinical examination
female gender, first pregnancy, family history, breech intrauterine position, high birth
weight, joint laxity, and postnatal positioning

Pathophysiology of DDH- cause is unknown

Predisposing factors associated with DDH may be divided into three broad categories:

physiologic maternal hormone secretion, intrauterine positioning;

mechanical breech presentation, multiple fetuses, oligohydramnios, large infant size, as

well as continued maintenance of the hips in adduction and extension that with time can
cause dislocation
Genetic siblings of affected infants and even greater incidence of recurrence if a sibling
and one parent were affected. One study found a twelvefold increase in risk of DDH for
individuals with a first-degree relative affected

DDH can be categorized into two major groups:

Idiopathic, in which the infant is neurologically intact
Teratologic: neuromuscular defect such as arthrogryposis or myelodysplasia. The
teratologic forms usually occur in utero and are much less common
Three degrees of DDH:
1. Acetabular dysplasia: This is the mildest form of DDH, in which there is a delay in
acetabular development evidenced by osseous hypoplasia of the acetabular roof that is oblique
and shallow. The femoral head remains
in the acetabulum with no Subluxation or
dislocation.
2. Subluxation: The largest percentage/ most common of DDH, sublxation implies
incomplete dislocation. The
femoral head remains in contact with the acetabulum, but a
stretched capsule and ligamentum teres cause the head of the femur to be partially displaced.
Pressure on the cartilaginous acetabulum inhibits ossification and
produces a flattening of
the socket.
3. Dislocation: This is the most severe form of DDH. The femoral head loses contact with
the acetabulum
and is displaced posteriorly and superiorly.
DDH Diagnostic Eval
Treatment initiated before 2 months of age for highest success rate; DX in newborn period
if possible
Newborn- hip laxity=hip dysplasia
O & B tests for sublxation and the tendency to dislocate; most reliable from birth -4wks;
Adduction contractures occur at 6-10wks O sign disappears; then limited hip abduction test is
most sensitive

Ortolani test1.
2.
3.
4.
5.

6.

Infant supine on firm plat surface;

Hips and knees flexed at right angles
Place middle finger of each hand over the greater trochanter
Thumbs on the inner side of the thigh opposite of lesser trochanter
One at a time knees are carried to midabduction, and each hip joint is submitted, one at a
time, first to forward pressure exerted behind the trochanter and then to backward
pressure exerted from the thumbs in front as the opposite joint is held steady
If the femoral head can be felt to slip forward into the acetabulum on pressure from
behind, it is dislocated

Barlow test-

1. If, on pressure from the front, the femoral head is felt to slip out over the posterior lip of
the acetabulum, the hip is said to be dislocatable or unstable

Therapeutic Management of DDH

Treatment begins ASAP; treatment varies with age
Newborn 6months: pavlik harness worn continuously until stable clinically & via ultrasound;
6-12wks
6-24 months: surgical closed reduction; spica cast for 12wks

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Older children- operative reduction- preoperative traction, tenotomy of contracted muscles,
pelvic osteotomy

NSG Care Management- DDH

The primary nursing goal is teaching parents to apply and maintain the reduction device
Parents should be instructed not to adjust the harness.
Skin care is an important aspect of the care of an infant in a Pavlik harness. The following
instructions for preventing skin breakdown are stressed:
Check at least two to three times per day for red areas under the straps and at skin folds.
Gently massage healthy skin under the straps once a day to stimulate circulation.
In general, avoid lotions and powders because they can cake and irritate the skin.
Always place the diaper under the straps.

Clubfoot- talipes equinovarus- TEV- bone deformity and mal position/ soft tissue
contracture
Readily apparent at birth & prenatally; if you cant straighten it its deformed.
The affected foot (or feet) is usually smaller and shorter, with an empty heel pad and a
transverse plantar crease.
When the deformity is unilateral, the affected limb may be shorter, and calf atrophy is
present.
Generally, imaging such as radiographs or ultrasonography is not necessary for diagnosis.
A thorough hip examination should be performed for all infants with a clubfoot; an
increased risk of hip dysplasia is associated with clubfoot deformities

Therapeutic Management- TEV

Goal is to achieve painless, plantigrade and stable foot;
3 Stages:
1. Correct deformity
2. Maintenance of the correction until normal muscle balance is regained
3. Follow-up observation to avert possible recurrence of the deformity
NSG Care Management
Nursing responsibilities include
reinforcing and clarifying the orthopedic providers explanations and instructions,
teaching parents about care of the cast or bracing (including vigilant observation for
potential problems), and
Encouraging parents to facilitate normal development within the limitations imposed by
the deformity or therapy.

Osteogenesis Imperfecta OI- Brittle bones faulty bone

mineralization, abnormal
bone architecture, and increased susceptibility to fracture

Most common syndrome in children;

excessive fractures and bone deformity;
moderate/severe growth restriction

Clinical features include varying degrees of bone fragility, deformity, and fracture; blue sclera;
hearing loss; and dentinogenesis imperfecta (hypoplastic discolored teeth),
Autosomal dominant/recessive: COL1A1, COL1A2 genes- precollagen (collagen precursor)
Classification based on clinical

Type 1- most common; wide variability of bone fragility; some affected family members
have significant deformity and disability, whereas others lead agile, active lives
Type 2- variants are the most severe and are considered lethal in infancy
Type 3- multiple fractures, bone deformity, and severe disability; affected individuals
rarely live to 30 years of age
Type 4- similar to type I with blue or white sclera
Type 5- hyperplastic callus, a radiodense metaphyseal band, and calcification of the
interosseous membrane of the forearm; no collagen mutations are noted in this group

Therapeutic Management- OI- primarily supportive; bone marrow transplant,

Focus
1.
2.
3.
4.
5.

on
decreasing the number of fractures
decreasing pain
increasing growth
improve bone metabolism
optimizing function

Bisphosphonate therapy with pamidronate, risedronate, olpadronate, neridronate, or

alendronate to promote increased bone density and prevent fractures has become standard
therapy for many children with OI. Bisphosphonate therapy reportedly is more beneficial for
increasing vertebral bone density; considered less effective for long bones. Others report
effectiveness of pamidronate in children with moderate to severe OI.
NSG Care Management- OI-Brittle bone-Supported when turned, moved, held, positioned;
never hold at the ankle gently lift buttocks or support with pillows; need compassion; obtain BP
with manual cuff
Upper resp. tract infections- includes rhinoviruses, RSV, adenovirus, influenza virus, and
parainfluenza virus, cause acute nasopharyngitis (the equivalent of the common cold
Clinical Manifestations-Nasopharyngitis more severe in infants & children; fever is common.
3 mo-3 years: sudden fever, irritability, restlessness, decreased appetite fluid intake,
decreased activity. Nasal inflammation may lead to obstruction of passages, producing openmouth breathing. Vomiting and diarrhea
may also be present
Older children dryness and irritation of nasal passages and the pharynx, followed by
chilling sensations,
muscular aches, an irritating nasal discharge, and, occasionally,
coughing or sneezing. Nasal inflammation may
lead to obstruction. Continual wiping away of
secretions causes skin irritation to nares.
Self limiting; resolves w/in 4-10 days without complications; prescribe cough suppressant w/
caution as coughing is encouraged
NSG Care Mang.
Small infants- most discomfort from nasal obstruction; elevate HOB to assist drainage;
saline drop & suction
prior to feeding, vaporization,
Maintain adq. Fluid intake, avoid contact with affected persons

Acute Strept. Pharyngitis- GABHS- group A beta-hemolytic streptococcus not serious itself
but can cause acute rheumatic fever (inflammation of the heart joints and CNS) and acute GN;
impetigo & pyoderma
Scarlet fever- S/S Pharyngitis, erythematous sandpaper-like rash
Clinical Manifestations: brief, subclinical- severe; abrupt onset, pharyngitis, headache,
fever, abdominal pain.
The tonsils and pharynx may be inflamed, covered with exudates by
day 2 (50% to 80% of cases), which usually appears by the second day of illness.
Therapeutic Management- 10days of penicillin if sore throat, 24hr response; Amoxicillin
for 10 days; must complete entire med therapy
NSG Care Management- swab throat for culture, Antibiotics, analgesic during acute
phase

Tonsillitis: mass of lymphoid tissue encircling the nasopharynx, oropharynx, known as the
Waldeyer tonsillar ring

Palatine, or faucial, tonsils are located on either side of the oropharynx, behind and
below the pillars of the fauces (opening from the mouth) surface of the palatine tonsils is
usually visible during oral examination.
Pharyngeal tonsils, (adenoids) are located above the palatine tonsils on the posterior
wall of the nasopharynx. Their proximity to the nares and eustachian tubes causes
difficulties in instances of inflammation.
Lingual tonsils at the base of the tongue.
Tubal tonsils- near the posterior nasopharyngeal opening of the eustachian tubes are not
part of the Waldeyer tonsillar ring.

Tonsils filter & protect the respiratory and alimentary tracts, antibody formation; children
generally have larger tonsils

Therapeutic Management- Tonsillitis self-limiting, treatment of viral pharyngitis is

symptomatic. Throat cultures positive for GABHS infection require antibiotic treatment. It is
important to differentiate between viral and streptococcal infection in febrile, exudative
tonsillitis. Because the majority of infections are of viral origin, early rapid tests can eliminate
unnecessary antibiotic administration.
Surgical treatment- Tonsillitis
Tonsillectomy (surgical removal of the palatine tonsils) may be indicated for massive
hypertrophy that results in
difficulty breathing or eating.
Adenoidectomy (the surgical removal of the adenoids) is recommended for children who
have hypertrophied
adenoids that obstruct nasal breathing; additional indications for
adenoidectomy include recurrent adenoiditis and sinusitis, OM with effusion, airway obstruction
and subsequent sleep-disordered breathing, and recurrent
rhinorrhea
NSG Care Management- providing comfort and minimizing activities or interventions that
precipitate bleeding A soft to liquid diet is generally preferred. A cool mist vaporizer keeps the
mucous membranes moist during periods of mouth breathing. Warm saltwater gargles, warm
fluids, throat lozenges, and analgesic-antipyretic drugs such as acetaminophen are useful to
promote comfort. Often opioids are needed to reduce pain for the child to drink. Combination
nonopioid and opioid elixirs such as hydrocodone (Lortab) relieve pain; every 4 hours while
symptoms persist. If surgery is needed, the need pain medication at regular intervals for at least
the first 24 to 48 hours; any longer theres another problem
Food and fluid are restricted until children are able to swallow them and are alert with no signs of
hemorrhage. Cool water, crushed ice, flavored ice pops, or diluted fruit juice is given; fluids with
a red or brown color are generally avoided to distinguish fresh or old blood in emesis from the
ingested liquid. Straws should be avoided, since these may damage the surgical site and cause
subsequent bleeding. Citrus juice may cause discomfort and is usually poorly tolerated. Milk, ice
cream, or pudding is not usually offered until clear fluids are retained because milk products coat
the mouth and throat, causing the child to clear the throat, which may initiate bleeding.

Otitis Media- ear infection; presence of fluid, inflammation in the middle ear along with acute
signs of illness; winter months, most prevalent in early childhood due to eustachian tubes
vertical wider shorter
Etiology- Bacteria: Streptococcus pneumoniae, H. influenzae, and Moraxella catarrhalis; Viruses:
RSV and influenza
Patho- primarily a result of a dysfunctioning eustachian tube(part of a contiguous system
composed of the nares, nasopharynx, eustachian tube, middle ear, and mastoid antrum and air
cells.
Eustachian tubes 3 functions relative to the middle ear:
1. protection of the middle ear from nasopharyngeal secretions,
2. drainage of secretions produced in the middle ear into the nasopharynx, and

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3. Ventilation of the middle ear to equalize air pressure within the middle ear and
atmospheric pressure in the external ear canal and replenishment of oxygen that has been
absorbed.
Clinical manifestations-OM
Therapeutic Management- OM-Treatment for AOM is one of the most common reasons for
antibiotic use in the ambulatory setting

Prescribe antibiotics to children 6 mos + older with severe otalgia for at least 48 hours or
temperature 102.2 F [39 C]).

Prescribe antibiotics for bilateral AOM > 24 months without severe otalgia for at least 48
hours or temperature<102.2 F [39 C]).
Either prescribe antibiotics or offer observation with close follow-up for unilateral AOM in
children 6 mo- 23 mo of age without severe otalgia for at least 48 hours or temperature
<102.2 F [39 C]); if child does not improve within 48 to 72 hours, begin antibiotic therapy.

Either prescribe antibiotics or offer observation with close follow-up for unilateral or
bilateral 24 mo old or older without severe signs and symptoms otalgia for at least 48
hours or temperature <102.2 F [39 C]).

NSG Care Management-AOM relieving pain, facilitating drainage when possible, preventing
complications or recurrence, educating the family in care of the child, and providing support to
the child and family.

Acute Epiglottitis- is a non infectious medical emergency; 2-5yrs but can occur to adulthood;
requires immediate medical attention; usually Caused by H. influenza, can be cause by hot
foods/liquids, smoke inhalation, foreign bodies, crack cocaine; LTB and epiglot. Do not occur
together
Clinical manifestations: abrupt, precede by sore throat, can rapidly progress to severe resp.
distress; children insist on sitting upright and leaning forward with chin out mouth open and
tongue protruding, drooling, difficulty and pain swallowing, irritable and extremely restless and
has an apprehensive frightened expression. The voice is thick and muffled, with a froglike
croaking sound on inspiration. The child is not hoarse. Suprasternal and substernal retractions
may be visible. The child seldom struggles to breathe, and slow, quiet breathing provides better
air exchange. The sallow color of mild hypoxia may progress to frank cyanosis if treatment is
delayed. The throat is red and inflamed, and a distinctive, large, cherry red, edematous epiglottis
is visible on careful throat inspection.
NSG Alert- Absence of spontaneous cough, drooling & agitation

Therapeutic Management- Epiglottitis- may develop suddenly, with respiratory

obstruction appearing rapidly. Progressive obstruction leads to hypoxia, hypercapnia, and
acidosis, followed by decreased muscular tone, reduced level of consciousness, and, when
obstruction becomes more or less complete, sudden death.

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Prevention: It is recommended that all children receive the H. influenza type B conjugate vaccine
beginning at 2 months of age since administration of the vaccine has become a routine part of
the immunization schedule; the incidence of epiglottitis has declined. Patients now tend to be
older and have disease caused by viral agents.
Nursing Care Management- Epiglottitis- serious and frightening disease for the child, family,
and health professionals it is important to act quickly but calmly and provide support without
unduly increasing anxiety. The child is allowed to remain in the position that provides the most
comfort and security and parents are reassured that everything possible is being done to obtain
relief for their child. Droplet isolation precautions are indicated for 24 hours after initiation of
effective antibiotic therapy to control spread of respiratory organisms. Prophylactic antibiotic
treatment of household and other contacts may be indicated
NSG ALERT: Nurses who suspect epiglottitis should not attempt to visualize the epiglottis
directly with a tongue
depressor or take a throat culture but should have the child seen by
the primary care provider immediately.
Resuscitation equipment and suction should be
immediately available and ready at the childs bedside

Acute Laryngotracheobronchitis- most common type of croup for children >5yrs,

Organisms responsible parainfluenza virus types 1 and 2, followed by parainfluenza virus

type 3, RSV, influenza types A and B, measles, and M. pneumoniae.
Usually proceeded by a URI, which gradually descends to adjacent structures.
gradual onset of low-grade fever, brassy cough, slow progressive
Parents often report that the child went to bed and later awoke with a barky, brassy cough
and at times inspiratory stridor.
Symptoms are typically worse at night
Agitation and crying tend to exacerbate the symptoms
Resp. acidosis/ resp failure
Inflammation of the mucosa lining the larynx and trachea causes a narrowing of the
airway

Therapeutic Management Acute Laryngotracheobronchitis

The major objective is maintaining an airway and providing for adequate

respiratory exchange.
Children with mild croup (no stridor at rest) are managed at home. Parents need to learn
the signs of respiratory
Cool mist provides relief for most children with mild croup, although there is no substantial
evidence to its efficacy; suggestions to provide cool air include taking the child outside to
breathe in cool night air, using a cold-water vaporizer or humidifier, standing in front of the
open freezer, and taking the child to a cool basement or garage.
Nebulized epinephrine (racemic epi) when that is not alleviated with cool mist. The onset
of action is rapid. Observe for 2-3 hours for resp. distress
Oral steroids (dexamethasone) (as a single dose) and are considered standard treatment
reduces edema
Severe cases- helium and oxygen (heliox) may reduce the work of breathing and relieve
the airway obstruction. Because helium has a lower density than room air, it forms a
respirable gas (with oxygen) that reduces airway turbulence.

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It is essential to allow children with mild croup to continue to drink beverages they like
and to encourage parents to use comforting measures with their child (e.g., holding,
rocking, walking, and singing, reading books). If the child is unable to take oral fluids, IV
fluid therapy and IV steroids may be indicated.

Nursing Care Management- The most important nursing function in the care of children
with LTB is continuous, vigilant observation and accurate assessment of respiratory status
RSV- respiratory syncytial virus
Bronchiolitis- acute viral infx; max affect at bronchiolar level, winter & spring, mostly caused by
RSV, adenoviruses & parainfluenza; human metaneurovirus by age 2 years most children have
been infected at least once.
Reinfection with RSV may occur in as many as three fourths of affected children in the second
year of life; the antibody response to the virus is inadequate to protect against subsequent
reinfection
Table 28-3 p.1188
Asthma
Bronchiolitis

Description

Age-group aff.

Predominant s/s

Treatment

Bronchitis

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Therapeutic Management- bronchiolitis- palivizumab, a humanized mouse

monoclonal antibody that is given once every 30 days (15 mg/kg); November and March; IM/ IV.

Uncomplicated cases of bronchiolitis are treated symptomatically with supplemental

oxygen as required, adequate fluid intake, airway maintenance, and medications.
Mostly managed at home.
Hospitalization for respiratory distress/cannot maintain adequate hydration.
The American Academy of Pediatrics practice oxygen if the infant fails to
maintain oxygen saturation of at least 90% after nasal suctioning and
repositioning
Routine chest physiotherapy (CPT) is not recommended
Candidates for this drug include: palivizumab:
> 32 weeks gestation
> 2 yrs (24mo) with bronchopulmonary dysplasia who have received supplemental
oxygen, bronchodilator, diuretic, or corticosteroid for the condition w/in 6 months before RSV
season
Between 32 - > 35 weeks:
> 3 months before the onset of or during the RSV season
1 out of 2 risk factors present: attends day care, at least one other child younger than
age 5 in
the household)
> 3 months of age

Nursing Care Management

separate rooms or rooms with other RSV-infected children.

Use Droplet, Contact, and Standard Precautions
Hand washing, not touching the nasal mucosa or conjunctiva, and using gloves, gowns,
and masks when entering the patients room.
Reduce contact with patient hospital personnel, visitors, uninfected

FBI-Foreign Body Ingestion & Aspiration

1st degree- obstruction allows air to pass in both directions
2nd degree- air can pass only in one direction
Complete Obstruction- FB & edematous mucosa block passage
NSG Alert- Signs of truly choking: cant speak, cyanotic, collapses; requires immediate action;
child can die w/in 4min; Follow-up care after the FB is removed includes monitoring for
respiratory distress and educating the parents.

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1. Cannot speak 2. Cyanotic 3. Collapses

Asthma a chronic inflammatory disorder w/ recurring symptoms, airway obstruction, and bronchial
hyper responsiveness
Wheezing, breathlessness, chest tightness, and cough, especially at night or in the early
morning.
airflow limitation or obstruction that is reversible either spontaneously or with treatment;
increase in bronchial hyperresponsiveness to a variety of stimuli

Asthma triggers
Allergens: OutdoorTrees, shrubs, weeds, grasses, molds, pollens, air pollution, spores
IndoorDust or dust mites, mold, cockroach antigen
IrritantsTobacco smoke, wood smoke, odors, sprays
Exposure to occupational chemicals, Exercise, Cold air: Changes in weather or temperature
Environmental changemoving to new home, starting new school
Colds and infections
AnimalsCats, dogs, rodents, horses
MedicationsAspirin, nonsteroidal anti-inflammatory drugs, antibiotics, beta blockers
Strong emotionsFear, anger, laughing, crying
ConditionsGastroesophageal reflux, tracheoesophageal fistula
Food additivesSulfite preservatives
FoodsNuts, milk and dairy products
Endocrine factorsMenses, pregnancy, thyroid disease
Pathophysiology- inflammation causes heightened airway reactivity in asthma; It is unlikely
that asthma is caused by either a single cell or a single inflammatory mediator.
Chronic inflammation may also cause permanent damage (airway remodeling) to airway
structures; this remodeling cannot be prevented by and is not responsive to current
treatments
Clinical Manifestations-Asthma: dyspnea, wheezing, coughing.

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Children: acute episodes of shortness of breath, wheezing, and cough followed by a quiet period
to a relatively continuous pattern of chronic symptoms that fluctuate in severity.
Diagnostic Evaluation: Pulmonary Function Test- objective eval of the presence and degree
of lung disease & the response to therapy
Spirometry: for children 5-6yrs, traditional/simple mechanical done at the time of initial
assessment, after treatment when symptoms are stabilized every 1-2 years to assess airway
funx. Maintenance
PEFR- Peak expiratory flow rate (max air flow forcefully exhaled in 1 sec and measured in
L/min) can be measured using PEFM peak expiratory flow meter
Green (80% to 100% of personal best) signals all clear. Asthma is under reasonably good
control. No symptoms are present, and the routine treatment plan for maintaining control can be
followed.
Yellow (50% to 79% of personal best) signals caution. Asthma is not well controlled. An acute
exacerbation may be present. Maintenance therapy may need to be increased. Call the
practitioner if the child stays in this zone.
Red (below 50% of personal best) signals a medical alert. Severe airway narrowing may be
occurring. A short-acting bronchodilator should be administered. Notify the practitioner if the
peak expiratory flow rate does not return immediately and stay in yellow or green zone
Asthma drug therapy
ACUTE:
Anticholinergic (atropine and Ipratropium) help relieve acute bronchospasm. However,
these drugs have adverse side effects that include drying of respiratory secretions, blurred
vision, and cardiac and central nervous system stimulation. The primary anticholinergic drug
used is Ipratropium: which does not cross the BBB, therefore elicits no CNS effects (as does
atropine).
Magnesium sulfate, a potent muscle relaxant that acts to decrease inflammation and improves
pulmonary function and peak flow rate, may be used in pediatric patients treated in the
emergency department or ICU with moderate to severe asthma. The drug is administered
intravenously at 25 to 75 mg/kg
-Adrenergic agonists (short acting) (primarily albuterol, levalbuterol [Xopenex], and
terbutaline) are used for treatment of acute exacerbations and for the prevention of EIB. Given
first In emergency treatment
Long term treatment:
Corticosteroids are anti-inflammatory; reduce bronchial hyperresponsiveness in chronic
asthma. Inhaled corticosteroids should be used as first-line therapy in children over 5 years of
age.

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Cromolyn sodium maintenance therapy age 2 years+; stabilizes mast cell membranes; inhibits
activation, release of mediators from eosinophil and epithelial cells; inhibits the acute airway
narrowing after exposure to exercise; cold, dry air; and sulfur dioxide. NO immediate relief of
symptoms; minimal side effects (occasional cough from inhalation of the powder formulation);
only available as an oral preparation or via nebulizer. Cromolyn sodium and nedocromil sodium
inhaler preparations discontinued 2010
Leukotrienes: mediators of inflammation that cause increases in airway hyperresponsiveness.
zafirlukast [Accolate] 5yr+ & montelukast sodium (singular) 12mo+ block inflammatory
and bronchospasm effects. These drugs are NOT used to treat acute episodes but are given
orally in combination with -agonists and steroids to provide long-term control and prevent
symptoms in mild persistent asthma.
Pharm. Book Info
Anti inflammatory- glucocorticoids- given on fixed scheduled
Bronchodilators- beta-2 agonists- acute bronchospasm; PRN/fixed/long term schedule
1. Prophylaxis & maintenance 12mos+
2. Prevention of EIB 15yr+
3. Relief of allergic rhinitis

Cystic Fibrosis- CF

Estimated that 1 in 29 Caucasians in the United States is a symptom-free carrier.

Autosomal recessive trait;

Pathophysiology
Several clinical features characterize CF:

increased viscosity of mucous gland secretions, a

striking elevation of sweat electrolytes, an
increase in several organic and enzymatic constituents of saliva, and
Abnormalities in autonomic nervous system function
primarily a result of abnormal chloride movement; the CFTR appears to function as a
chloride channel

Primary factor, responsible for many of the clinical manifestations of the disease: is
mechanical obstruction caused by the increased viscosity of mucous gland secretions
The goals of CF therapy are to prevent or minimize pulmonary complications, ensure
adequate nutrition for growth, encourage appropriate physical activity, and promote
a reasonable quality of life for the child and the family
Pancreas issues
Diagnosed via: sweat test- positive 60mEq/L; fat in stools, patchy atelectasis/obstructive
emphysema on Chest X-ray
Clinical Manifestations: The most common symptoms are

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(1) Progressive chronic obstructive lung disease associated with infection
(2) Maldigestion from exocrine pancreatic insufficiency
(3) Growth failure from malabsorption and anorexia
(4) Diabetes symptoms of hyperglycemia, polyuria, glycosuria,
S/S: growth failure (failure to thrive), increased weight loss despite increased appetite, gradual
deterioration of the respiratory system. may not be readily apparent, especially when there is no
familial evidence of CF
Respiratory Tract- Initial pulmonary manifestations are often wheezing respirations and a
dry, nonproductive cough.
Gastrointestinal Tract-The earliest postnatal manifestation of CF is meconium ileus, Thick,
putty-like, tenacious, usually at or near the ileocecal valve- intestinal obstruction, abdominal
distention, vomiting, failure to pass stools, rapid development of dehydration with associated
electrolyte imbalance.
Reproductive System-Delayed puberty in girls with CF is common even when their nutritional
and clinical status is good. Pregnant w/ CF have an increased incidence of premature labor & low
birth weight infant. Favorable nutritional status and pulmonary function are positively correlated
with favorable pregnancy outcomes.
High-frequency chest compression (HFCC) vest
Therapeutic Management- Improved survival attributable to: antibiotic therapy &
improved nutritional /respiratory management. Give bronchodilator BEFORE CPT
The goals of CF therapy are
to prevent or minimize pulmonary complications
ensure adequate nutrition for growth
encourage appropriate physical activity
Promote a reasonable quality of life for the child and the family.
A multidisciplinary system approach is needed to accomplish these goals.
Transition to Adulthood- Males with CF are sterile; harmful effects of pregnancy on resp.;
children will be carriers,
Adolescents should take personal ownership and management of the illness; Anticipatory grief;
hospice treatments for pain and dyspnea

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Leukemia- p 1398
broad term for malignant diseases of the bone marrow & lymphatic system

Morphology- In children, two forms are generally recognized:

Acute lymphoblastic leukemia (ALL) three subtypes: L1- most common L2, and L3.
Acute myelogenous leukemia (AML) 8 subtypes
Chromosome Studies-Chromosome analysis of leukemic cells has become an important tool in
the diagnosis and management of patients with ALL and AML. For example, children with trisomy
21 have 20 times the risk of other children for developing ALL
Pathologic and Related Clinical Manifestations Leukemia: unrestricted proliferation of
immature white blood cells in the blood-forming tissues of the body; not a tumor but the
leukemic cells have the neoplastic properties of solid cancers in any tissue of the body with
nonfunctional leukemic cells; highly vascular organ, spleen& liver most severely affected.
To understand the pathophysiology of the leukemic process, it is important to clarify two
common misconceptions.
First, although leukemia is an overproduction of white blood cells, most often the leukocyte
count is because of greatly elevated counts of immature cells, or blasts.
Second, these immature cells do not deliberately attack and destroy the normal blood cells or
vascular tissues. Cellular destruction occurs through the process of infiltration and subsequent
competition for metabolic elements.
Onset: acute to insidious; few symptoms; may be diagnosed when a minor infection, such as a
cold, fails to completely disappear, The child continues to be pale, listless, irritable, febrile, and
anorexic; weight loss, petechiae, bruising without cause, and continued complaints of bone and
joint pain: massive production of WBC in bone marrow causes pressure & pain
Signs and symptoms mimicking such conditions as RA or mononucleosis. In some cases the
diagnosis of leukemia accompanies some totally unrelated event, such as a routine physical
examination or injury.
Therapeutic Management- Leukemia
Treatment: IV and intrathecal (spinal column) chemotherapeutic agents and Radiation for
resistant CNS disease/testicular relapse
Typically leukemia treatment is divided into phases:
(1) Induction- which achieves a complete remission or clinical disappearance of leukemic cells
(2) Intensification / consolidation therapy, which further decreases the total tumor burden

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(3) Maintenance- which consists of further chemotherapy to ensure the disease stays in
remission.

Bone Marrow Transplantation: successfully in treating some children with ALL and AML.

BMT is not recommended for children with ALL during the first remission because of
the excellent results possible with chemotherapy. Best result w/ BMT during second
remission.
poorer prognosis with AML, transplantation considered during the first remission
Prognosis- The majority of children with newly diagnosed leukemia who receive effective
multiagent chemotherapy will survive; reported overall survival is more than 80%
Nursing Care Management- Nursing care of the child with leukemia is directly related to the
regimen of therapy. Myelosuppression, drug toxicity, and leukemic infiltration cause secondary
complications that necessitate supportive physical care

Hemophilia- p1357

The term hemophilia refers to a group of bleeding disorders resulting from congenital
deficiency, dysfunction, or absence of specific coagulation proteins or factors
80% of all cases X-linked recessive inheritance pattern
Most common- factor VIII 8 deficiency (hemophilia A, or classic hemophilia) most prevalent
1/5000; abnormal PTT labs
factor 9 IX deficiency (hemophilia B, or Christmas disease); 1/20-30,000
The most frequent pattern of transmission is through the union of an unaffected male
with a trait-carrier female
Modes of Transmission

X-linked recessive disorder, but only about 60% of affected children have a positive family
history for the disease.
Up to 1/3 gene mutation.
The most frequent pattern of transmission is through the union of an unaffected male with
a trait-carrier female.
B/c treatments for hemophilia have improved the results of a union between an affected
male and a normal or carrier female must also be considered. For example, the chances
are equal (e.g., 1 in 4) that an offspring of an affected male and a carrier female will be an
affected son, an affected daughter, a carrier daughter, or a normal son. Such parentage is
one of the few ways in which a female inherits the disorder. Female carriers may have low
levels of factor VIII and be symptomatic.

Pathophysiology and Clinical Manifestations- hemophilia

The basic defect of hemophilia A is a deficiency of factor VIII (antihemophilic factor).

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Factor VIII is produced by the liver and is necessary for the formation of thromboplastin in
phase I of blood coagulation.
The less factor VIII that is found in the blood, the more severe the disease
Subcutaneous and intramuscular hemorrhages are common
Hemarthrosis, bleeding into the joint cavities, especially the knees, elbows, and ankles, is
the most frequent form of internal bleeding.
Bony changes and crippling deformities occur after repeated bleeding episodes over
several years.
Early signs of hemarthrosis: stiffness, tingling, or ache in the affected joint, followed by
a decrease in the ability to move the joint. Obvious signs and symptoms are warmth,
redness, swelling, and severe pain, with considerable loss of movement.

Sickle Cell Trait-p1340

Pathophysiology and Clinical Manifestations
The clinical manifestations of SCA are primarily the result of:
(1) Obstruction caused by the sickled RBCs
(2) Vascular inflammation
(3) Increased RBC destruction.
The abnormal adhesion, entanglement, and enmeshing of rigid sickle-shaped cells accompanied
by the inflammatory process intermittently blocks the microcirculation, causing vasoocclusion;
The resultant absence of blood flow to adjacent tissues causes local hypoxia, which leads to
tissue ischemia and infarction (cellular death)
Normal adult hemoglobin is partly or completely replaced by abnormal hemoglobin
Autosomal recessive
Sickle Cell Crises- Vasoocclusive crisis (VOC), preferably called a painful episode or event, is
the most common type of non-life-threatening crisis. It is characterized by ischemia that causes
mild to severe pain that may last from minutes to days.
Screening of Newborns-Screening for SCD in the newborn period can identify children with
hemoglobinopathies. Universal screening of newborns for SCD has become standard in all 50 U.S.
states and territories
Therapeutic Management- The aims of therapy are to
1. prevent the sickling phenomenon, which is responsible for the pathologic sequelae
2. To treat the medical emergency of sickle cell crisis.
The successful achievement of these aims depends on prompt nursing interventions and
medical therapies, child and family preventive measures, and innovative treatment
interventions.
Nursing Care Management- Minimize Tissue Deoxygenation, promote hydration, minimize
crises, promote supportive therapy

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Minimize Tissue Deoxygenation-Anything that increases cellular metabolism also results in
tissue hypoxia.
(1) Taking frequent rest breaks during physical activities
(2) Avoiding contact sports if the spleen is enlarged
(3) Avoiding environments with low oxygen concentration, such as high altitudes or
nonpressurized airplane flights; and (4) avoiding known sources of infection. If the child has even
a mild infection, the parents must seek medical attention at once.
Promote Hydration- importance of adequate hydration to prevent sickling and delay the
vasoocclusion and hypoxia ischemia cycle. (Approximately 1600 ml/m2/day), which is the
minimum daily fluid intake. The nurse also assesses the childs usual fluid consumption to
evaluate its adequacy and makes adjustments based on this knowledge. It is not sufficient to
advise parents to force fluids or encourage drinking. They need specific instructions on how
many glasses or bottles of fluid are required; check mucus membranes
Minimize Crises-Because infection is the major cause of death due to the bodys inability to
resist infection, the nurse stresses to parents the importance of adequate nutrition, frequent
medical supervision, proper hand washing, and isolation from known sources of
infection. Keep in mind that children also need to live a normal life. Overprotection can be as
devastating emotionally as an infection is physically. Parents need to be aware of the need to
seek prompt medical care at the first sign of any infection. Teach the family the signs and
symptoms of crises and advise them to seek medical attention immediately when any are
present. Teaching parents spleen palpation for earlier detection of splenic sequestration can
reduce mortality from this serious complication.
Promote Supportive Therapies The success of many of the medical therapies relies heavily
on nursing implementation. Management of pain is an especially difficult problem and often
involves experimenting with various analgesics, including opioids, and various schedules before
relief are achieved. Unfortunately, these children tend to be under medicated, which results in
clock watching and demands for additional doses sooner than might be expected. Often this
incorrectly raises suspicion of drug addiction, when in fact the problem is one of inadequate pain
control
Drug Alert - Meperidine (pethidine [Demerol]) is not recommended. Normeperidine, a
metabolite of meperidine, is a central nervous system stimulant that produces anxiety, tremors,
myoclonus, and generalized seizures when it accumulates with repetitive dosing. Patients with
SCD are particularly at risk for normeperidine-induced seizures

Childrens Understanding and Reactions to Dying-p797

Ages and stages may be helpful as a general guide, but communication should be based
on the cognitive
developmental capabilities of the individual child and his or her readiness for
such discussion
By apprx. 7yrs most children understand the key bio-scientific components of death; 412yrs have an adult like understanding of death
Infants and toddlers:

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Concept of Death: Toddlers are egocentric and can only think about events in terms of
their own frame of reference: living. Their egocentricity and vague separation of fact and fantasy
make it impossible for them to comprehend absence of life. Instead of understanding death,
this age-group is affected more by any change in
lifestyle.

Reaction to dying: regression due to change in routine; Toddlers may perceive the
seriousness from parents
reaxn, sadness, depression or anger. With the death of someone
else, they may continue to act as though the person is alive. As children grow older, they will be
increasingly able and willing to let go of the dead person. Ritualism is important; a change in
lifestyle could be anxiety producing. This age-group reacts more to the pain and discomfort of a
serious illness than to the probable fatal prognosis. This age-group also reacts to parental
anxiety and sadness.
Interventions: Help parents deal with their feelings, allowing them more emotional
reserve to meet the needs
of their children. Encourage parents to remain as near to the
child as possible, yet be sensitive to the parents
needs. Maintain as normal an environment
as possible to retain ritualism. If a parent has died, encourage the
establishment of a
consistent caregiver for the child. Promote primary nursing.
Preschool children: egocentric, believe their thoughts because events can lead to feelings of
guilt, shame & punishment
Concept of death: Children of this age believe their thoughts are sufficient to cause
death; the consequences a
feeling of guilt, shame, and punishment. Their egocentricity
implies a tremendous sense of self-power and
omnipotence. They usually have some sense
of the meaning of death. Death is seen as a departure, a kind of sleep. They may recognize the
fact of physical death but do not separate it from living abilities. Death is seen as
temporary
and gradual; life and death can change places with one another. There is no understanding of the
universality and inevitability of death.
Reactions to Dying: If they become seriously ill, they conceive of the illness as a
punishment for
their thoughts
or actions. They may feel guilty and responsible for the
death of a sibling. Their greatest fear concerning
death is
separation from their parents.
They may engage in activities that seem strange or abnormal to adults. With fewer defense
mechanisms to deal with loss, young children may react to a less significant loss with more
outward grief than to the loss of a significant person. The loss is so deep, painful, and
threatening that the child
must deny it for the time being to survive its overwhelming
impact. Behavioral reactions such as giggling, joking,
attracting attention, or regressing to
earlier developmental skills indicate childrens need to distance themselves from tremendous
loss
Intervention: Help parents deal with their feelings, allowing them more emotional
reserve to meet the needs of
their children. Help parents to understand behavioral reactions
of their children. Encourage parents to remain
near the child as much as possible, to
minimize the childs great fear of separation from parents. If a parent has
died, encourage
establishment of a consistent caregiver for the child. Promote primary nursing.

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School age: may still associate misdeeds or bad thoughts with causing death and feel intense
guilt and responsibility but because of their higher cognitive abilities, they respond well to logical
explanations and comprehend the figurative meaning of words better
Concept of Death: These children still associate misdeeds or bad thoughts with causing
death and feel intense
guilt and responsibility for the event. Because of their higher cognitive
abilities, they respond well to logical explanations and comprehend the figurative meaning of
words. They have a deeper understanding of death in a
concrete sense. They particularly fear
the mutilation and punishment they associate with death. They personify
death as the devil,
a monster, or the bogeyman. They may have naturalistic-physiologic explanations of death. By
age 9 or 10, children have an adult concept of death, realizing that it is inevitable,
universal, and irreversible
Reaction to death: Because of their increased ability to comprehend, they may have
more fearsor example:
The reason for the illness
Communicability of the disease to themselves or others
Consequences of the disease
The process of dying and death itself
Fear of the unknown, which is greater than their fear of the known
The realization of impending death is a tremendous threat to their sense of security and
ego strength.
They are likely to exhibit fear through verbal uncooperativeness rather than actual
physical aggression.
They are interested in post death services. They may be inquisitive about what happens to
the body.
Intervention: Help parents deal with their feelings, allowing them more emotional
reserve to meet the needs of
their children. Encourage parents to remain near the child as
much as possible, yet be sensitive to the parents needs. Because of childrens fear of the
unknown, anticipatory preparation is very important. Because the
developmental task of
this age is industry, interventions of helping children maintain control over their bodies
and
increasing their understanding allow them to achieve independence, self-worth, and self-esteem
and avoid
a sense of inferiority. Encourage children to talk about their feelings and provide
aggressive outlets. Encourage parents to honestly answer questions about dying rather than
avoiding questions or fabricating euphemisms.
Encourage parents to share their moments of
sorrow with their children. Provide preparation for post death
services
Adolescents:
Concept of death: Adolescents have a mature understanding of death. They are still very
much influenced by remnants of magical thinking and are subject to feelings of guilt and

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shame. They are likely to see deviations
illness.

from accepted behavior as the reason for their

Reaction to Death: Adolescents straddle transition from childhood to adulthood. They

have the most difficulty in coping with death. They are least likely to accept the cessation of
life, particularly if it is their own. Concern
for the
present is much greater than for the
past or the future. They may consider themselves alienated from
their peers and unable to
communicate with their parents for emotional support, feeling alone in their struggle.
Adolescents orientation to the present compels them to worry about physical changes
even more than the
prognosis. Because of their idealistic view of the world, they may
criticize funeral rites as barbaric, money
making, and unnecessary.
Intervention: Help parents deal with their feelings, allowing them more emotional
reserve to meet the needs of
their children. Avoid alliances with either parent or child.
Structure hospital admission to allow for maximum
self-control and independence. Answer
adolescents questions honestly, treating them as mature individuals and respecting their
needs for privacy, solitude, and personal expressions of emotions. Help the parents understand
their childs reactions to death and dying, especially that concern for present crises, such as loss
of
hair, may be much greater than concern for future ones, including possible death.

Congenital Heart defects

Defects that allow blood flow from the high-pressure left side of the heart to the lower-pressure
right side (left-to-right shunt) result in increased pulmonary blood flow and cause HF.
Obstructive defects impede blood flow out of the ventricles; obstruction on the left side of the
heart results in HF, whereas severe obstruction on the right side causes cyanosis.
Defects that cause decreased pulmonary blood flow result in cyanosis.
Mixed lesions present a variable clinical picture based on the degree of mixing and amount of
pulmonary blood flow; hypoxemia (with or without cyanosis) and HF usually occur together.