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Temperament
● Inborn in all of us
● A way of reacting to the world around us
Reaction Patterns:
● Activity level
○ Level of activity differs widely among children
● Rhythmicity
○ Have a regular rhythm is physiologic terms
● Approach
○ Child’s response to a new situation
● Adaptability
○ Is the child able to adapt to new situations?
● Intensity of reaction
● Distractibility
CYSTIC FIBROSIS
Epidemiology
● Affects approximately 30,000 children and adults in the US today or 1 in 2500
live births
● Occurs most commonly in whites, rarely in blacks and Asians
● More than 10 million Americans (1 in 31 or 3.3%) are symptom free carriers of
the defective CF gene
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Survival
● In 1955, few children with CF lived to attend elementary school. Today, the
median age of survival is 32 years.
● In this decade, many CF survive into their 40’s.
Etiology
● An inherited (genetic) disease
● CF is an autosomal-recessive disease (this means an individual must inherit 2
defective CF genes, one from each parent)
● The defective gene in CF occurs in Chromosome 7
● The gene causes the dysfunction of the exocrine glands
What are exocrine glands?
● Glands that secrete things such as:
○ Lungs
○ Pancreas
○ Sweat glands
○ Salivary glands
○ Digestive glands
Pathophysiology
● CF causes the body to produce an abnormally thick, sticky mucus which:
○ Clogs the lungs and leads to life-threatening lung infections;
○ Obstructs the pancreas, preventing enzymes from reaching the intestine to
help break down and digest food
● Primary symptoms:
○ Thick, sticky mucus
○ Salty taste on skin
● Marked electrolyte changes in sweat glands
○ Chloride in sweat is 2-5X above normal
Lung involvement
● Thick tenacious mucus polls in the bronchial tree and obstructs bronchioles
● Results in:
○ Bronchiectasis: Chronic dilation of the bronchi. Involves a chronic cough
that produces mucopurulent sputum. Over time results in destruction of
the bronchial walls.
○ Pneumonia: Staph Aureus, pseudomona’s, and H. Influenzae
● Symptoms over time include:
○ Clubbing of fingers
■ Clubbing is related to lack of tissue perfusion
Symptoms over time:
● Easily fatigued
● Physical growth stunted
● Chest may become enlarged from over inflation of alveoli because air cannot be
pushed past the thick mucus on expiration (barrel chested)
● Increased carbon dioxide in blood (hypercapnia and/or chronic acidosis)
Pancreas involvement
● Thick secretions mix with pancreatic enzymes and block the pancreatic duct.
● Therefore:
ERIKSONS STAGES
Infant
● One month to one year old
● Erikson’s developmental task: Trust vs. Mistrust
○ Task: Attachment to the mother
○ Resolution of crisis: Trust in persons; faith and hope about the
environment and future.
○ Unsuccessful resolution of crisis: General difficulties relating to person’s
effectively; suspicion; trust-fear conflict, fear of the future.
○ Developmental tasks: Learning to eat solid foods
Toddler
● Ages one to three years old
● Erikson’s developmental task: Autonomy vs. shame and doubt.
○ Task: Gaining some basic control over self and environment
○ Resolution of Crisis: Sense of self-control and adequacy; will power
○ Unsuccessful resolution of crisis: Independence-fear conflict; severe
feelings of self-doubt.
○ Developmental tasks:
■ Learning to walk
■ Learning to use fine muscles
■ Toilet training
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■ Learning to communicate
Preschool
● 3-6 years of age
● Erikson’s developmental task: Initiative vs. Guilt
○ Task: Becoming purposeful and directive
○ Resolution of Crisis: ability to initiate one’s own activities; sense of
purpose.
○ Unsuccessful resolution of crisis: aggression-fear conflict; sense of
inadequacy or guilt
○ Developmental tasks:
■ Independence of self-care
■ Learning sexual role identity
■ Forming reality concepts
■ Internalizing concepts of right and wrong
■ Learning to identify with family members and others.
School age child
● 6-12 years old
● Erikson’s developmental task: Industry vs. Inferiority
○ Task: developing social, physical, and school skills
○ Resolution of Crisis: competence, ability to learn and work
○ Unsuccessful resolution of crisis: Sense of inferiority; difficulty learning
and working
○ Developmental tasks:
■ Acquiring game skills
■ Learning to relate positively with peers
■ Building a wholesome self-concept
■ Refining communication skills
Adolescent
● Ages 12-20 years
● Erikson’s developmental task: Identity vs. Role confusion
○ Task: developing sense of identity
○ Resolution of Crisis: sense of personal identity
○ Unsuccessful resolution of crisis: confusion about who one is; identity
submerged in relationships or group memberships
○ Developmental tasks:
■ Forming peer relationships
■ Responding to an appropriate sexual role
■ Attaining emotional independence
■ Achieving a sense of economic independence
Hypoxemia
● Color is not a great indicator
● SaO2 of 80-85%-- Saturation of Oxygen (arterial blood/Hemoglobin)
● Polycythemia (increase of the RBC’s d/t chronically low oxygen saturations)
● Clubbing
● Squatting to increase venous return
● Hypercyanotic spells
Surgical intervention
● Early intervention prior to hypoxic episodes preferred
● Mortality rates vary from 2% to 25%
● Surgery should be done in major centers
Physical assessment
● Skin
○ Color indicates the state of perfusion
○ As extracellular fluid volume decreases (as with dehydration), peripheral
circulation decreases.
○ Note the following:
■ Warmth
■ Skin color: from pink to pale as peripheral circulation decreases
(cyanosis is a late sign)
■ Hands will get cold before core temperature
■ Cap refill increases <2 seconds
● Turgor:
○ In dehydrated states:
■ Skin is unusually dry
■ Tenting of skin (is a later sign)
■ The best place to check skin turgor is the abdomen and medial area
of the thighs
● Skin temperature
○ Decreases in fluid status; results in vasoconstriction of small arterioles
○ Vasoconstriction causes a decrease in skin temperature
○ Extremities usually remain cold even if the child has a fever
● Mucous membrane
○ Mouth is the most mucous membrane to assess for fluid deficit.
○ Note:
■ Amount of saliva in mouth
■ Lips and nares may be dry
■ Mucous membranes may be cracked.
■ Wrinkled tongue can indicate dehydration
■ Be sure dryness is not due to excessive mouth breathing—look at
other variables as well.
● Head Assessment
Parasites
● Giardia lamblia
● Cryptosporidium
Etiology
● Spread by contaminated food or water
● Also from person to person
● More common in crowded conditions, such as day care centers and schools
● Infants immune system plays a role (the younger the infant, the less developed the
immune system).
Assessment of mild diarrhea
● Fever of 101-102 degrees F may be present
● Anorexia
● Irritable
● Appear unwell
● Episodes of diarrhea consist of 2-10 stools per day
● Mucous membranes are dry
● Pulse rapid
● Skin feels warm
● Skin turgor is not yet decreased at this time
● Urine output usually normal
● Skin color is pale
Therapeutic management of mild diarrhea
● Is not serious at this stage—children can be treated at home
● Rest the GI tract for at least one hour; then offer water or oral hydration solutions
such as pedialye.
○ Approximately 1 tablespoon every 15 minutes x 4
○ Then 2 tablespoons every ½ hour x 4
○ If retained, give small sips of fluid; avoid giving large amounts of fluid.
Give in small doses
○ Do not give OTC drugs such as Imodium or Kaopectate because they’re
too strong for little tummies
○ Depended on developmental age of child; instruct on good handwashing
technique.
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Severe diarrhea
● May result from mild or may appear on it’s own
● Temp: 103-104F
● Pulse and respirations are weak and rapid
● Skin is pale and cool
● Infants may appear apprehensive, listless, lethargic (appears as a hypovolemic
state)
● Obvious signs of dehydration
○ Depressed fontanel
○ Poor skin turgor
○ Sunken eyes
● Bowel movement every few minutes
● Stool is liquid green, may be mixed with mucus and blood (because the intestines
are inflamed and irritated)
● Stool may be passed with explosive force
● Urine output scanty and concentrated
● Lab findings:
○ Increased hematocrit, hemoglobin, serum protein levels
○ Falsely high because the percentage is low
● Loss of 2.5-5% of body weight- mild dehydration
● Severe diarrhea can cause of loss of 5-15% of body weight quickly
● Any infant who has lost more than 10% if body weight requires immediate
treatment
Therapeutic measures
● Assessment of the fluid and electrolyte imbalance
● Rehydration
● Maintenance of fluid therapy
● Reintroduction of adequate diet
● Antimicrobial agents if necessary
Nursing considerations: assessment
● Observe general appearance and behavior
● Assess for dehydration
○ Decreased urine output
○ Decrease in weight
○ Dry mucous membranes
○ Poor skin turgor
○ Sunken fontanel in infant
● More severe dehydration
○ Increase in pulse
○ Increase in respirations
○ Decreased blood pressure
○ Prolonged cap refill time
○ All signs of impending hypovolemic shock
○ Also assess for septic shock
Nursing goals
● Urine output is more than 1ml/kg/hr
● Bowel movements are formed and fewer than 4 per day
Treatment of intussusception:
● Surgery
● Reduction by fluid/air/barium (done in radiology)
Motility disorders
● Hirschsprung’s disease (once known as Congenital Aganglionic megacolon)
● GERD (low esophageal and gastric mobility)
Hirschsprung’s Disease (Congenital Aganglionic megacolon)
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● Absence of ganglionic innervation to a portion of the bowel
● Peristalsis does not occur in non-innervated bowel areas
● Patient’s have chronic constipation or ribbon-like stools
Treatment of Hirschsprung’s disease
● Surgery to remove the agangilionic colon segment
● May be done in 2 stages, with a temporary colostomy for 6-8 months to allow
bowel to rest.
Meckel’s Diverticulum
● In embryonic life, the intestine is attached for the umbilicus by a duct
● This duct becomes a ligament near term
● In 2-3% of infants, this duct remains open as a pouch off of the ileum
Problems associated with Meckel’s diverticulum
● Some gastric mucosa (which secretes acidic fluids) may be displaced
● This may allow acidic, irritating secretions to flow into the intestine
● This irritates the bowel, leading to ulceration and bleeding
● Appendicitis symptoms
● Can serve as a lead point causing intussusception (from weakened area)
● A fibrous band extending from the diverticulum to the umbilicus can act as a
constricting band around the bowel
Treatment of Meckel’s diverticulum
● The diverticulum (pouch) is surgically resected.
Structural defects
● Cleft lip and palate
● Esophageal atresia
● Tracheoesophageal fistula
Cleft lip and palate
● Cleft lip occurs when there is a failure of the fusion of the maxillary and median
nasal processes.
● Cleft palate occurs when there is a failure of the fusion of the palatal process (roof
of the mouth)
Associated Nursing Diagnoses for Cleft lip and palate
● Risk for fluid volume deficit
● Risk for altered nutrition; less than body requirements
● Risk for aspiration
Treatment for cleft lip and palate
● Special nipples before surgery and while recovering
● Cleft lip surgery is usually done between birth and 10 months of age
● Cleft palate repairs are done at 18-24 months, so that anatomic changes in the
palate contour are complete.
● Recovery is usually excellent
● Remember, these are typically stages surgeries
Tracheoesophageal Fistula and Esophageal atresia
● Failure of the esophagus to develop normally, will end in a blind pouch or
connect directly into the trachea instead of being a discrete passage.
● These defects may occur separately or in combination
Fracture complications
● Circulatory impairment
Cast care
● Risk for altered peripheral tissue perfusion related to pressure from cast
● Expected outcomes:
○ No pain or numbness in extremity
○ Distal nail beds blanches and refills in less than 3 seconds
○ Pedal pulses palpable
MUSCULAR DYSTROPHIES
The largest group of muscle disorders of childhood
Three types:
● Congenital myotonic dystrophy
○ Begins in utero
○ Newborn may already have significant myotonia (muscle weakness)
○ Usually die before one year of age due to respiratory failure
■ Assessory muscles for respiration fail
Facioscapulohumeral Muscular dystrophy
● Begins after 10 years of age
● Facial weakness is the predominant symptom
● Very slow progression of symptoms
● Less disability than the other types
● Normal lifespan is possible.
Pseudohypertrophic Muscular Dystrophy/ Duchenne’s Disease
● Most common form of MD
● Symptoms usually appear by age 3 years
● Occurs only in boys
● Late in meeting motor milestones
● Muscle weakness gradually becomes more pronounced
● Usually wheelchair-dependent by adolescence
● Heart and respiratory failure may occur by early adulthood
Cerebral Palsy
Cerebral Palsy
● A group of non-progressive disorders of upper motor neuron impairment that
result in motor dysfunction.
● Can happen before, during, or after birth
● Occurs 2:1000 births
● Most common permanent disability of childhood
Incidence and Causes of CP
● Most frequently associated with brain anoxia that leads to cell destruction
○ Symptoms can range from very mild to quite severe, depending on the
extent of brain damage
● Also can be caused by:
○ Kernicterus (a form of jaundice from hyerbilirubinemia; staining of the
brain with bilirubin)
○ Meningitis (viral is the most common)
● Occurs most frequently in very low weight infants (born prematurely), or those
small for their age.
○ Their lungs haven’t been fully developed
Febrile convulsions
Febrile convulsions
● Seizures associated with high fever (102-104 degrees F)
● Most common in preschool children or between 5 months and 5 years of age
● Usually no more than 5-7 of these episodes occur in a child’s life
Seizure activity
● Seizure usually lasts 15-20 seconds
● Shows an active tonic-clonic pattern (alternately contracting and relaxing the
muscles)
● EEG tracing usually normal
● Usually a family history
● Seizures subside once the fever is gone
Prevention of Febrile convulsions
● Give Tylenol to keep fever below 101
● Often fever develops during the night when parent/caregiver is not with child
● If child has one febrile seizure, no further treatment given other than to advise
parents to administer Tylenol to keep fever below 101
● If more than one seizure, child may be put on Phenobarbital (controversial)
Therapeutic management of seizures
● Teach parents that after the seizure subsides, they should:
○ Sponge the child with tepid water
■ Do not put child in bathtub
■ Do not use rubbing alcohol or cold water
■ Do not give Tylenol right after the seizure (not awake enough to
swallow)
● If unable to decrease temperature by sponging, advise parents to:
○ Put a cool washcloth on child’s forehead, axillary, and groin area’s (which
are temperature receptors)
Healthcare facility will:
● Determine underlying cause
● Lumbar puncture to rule out meningitis
● Antipyretic drugs
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● Antibiotic therapy if needed
● Assure parents that febrile convulsions do not lead to brain damage and child will
be well.
BACTERIAL MENINGITIS
Infections or inflammation of the cerebral meninges (the membranes covering the
brain and spinal cord)
● 90% of cases are between 1 month and 5 years
● Peak incidence is in the winter.
● Causative organisms (95% of cases)
○ H. Influenzae (type B)
○ Streptococcus pneumoniae
○ Neisseria meningitis- epidemic form; droplet from nasophargeal secretions
■ MUST be put on droplet isolation
Pathophysiology of Bacterial meningitis
● Pathologic organism spreads to the meninges from upper respiratory tract or by
lymphatic drainage from the sinuses.
● Once pathogen enter the meningeal space, they spread rapidly
● Produces an inflammatory effect that leads to thick exudates that blocks CSF
flow.
● Brain becomes edematous, covered with purulent exudate.
● Spreads VERY quickly through CNS
Clinical signs in Children and Adolescents
● Usually have 2-3 days of a cold, upper respiratory infection and occasionally and
ear infection.
● Become VERY irritable due to headache
● May have convulsions
● photophobia
● As the disease progresses, more signs of meningeal irritability occurs:
○ Positive Brudzinski’s (image on page 674)
■ When child’s head is flexed forward (while laying on back), both
hips, knees and ankles flex. This shows meningeal irritation
○ Positive Kernig’s (image on page 674)
■ Flex child’s hips and knee (while laying on back)
■ Then extend leg—this will cause pain, resistance and spasm which
indicate irritation.
○ Nuchal rigidity occurs (neck stiffness)
○ In the newborn—poor sucking, weak cry, lethargy
Diagnostic Evaluation—Lumbar Puncture
● Obtained by history and analysis and CSF via Lumbar puncture
○ Culture and gram stain identify causative organism
● Blood cell count—WBC elevated
● Lowered glucose
○ Increased metabolic rate due to the body and brain trying to fight off
infection; draws glucose out of blood for energy.
● Protein content increased
○ Due to extra cells and metabolism occurring in the CNS
BRAIN TUMORS
● The most common solid tumor that occurs in children, secondary to leukemia
● Tends to occur between 1 and 10 years of age (5-years is the peak)
● In children, tends to occur in areas of the brain where they are difficult to remove.
● Signs and symptoms due to increased intracranial pressure (ICP)
Treatment of Brain tumors
● 4-6 months may pass from first symptom before diagnosed
● diagnostic tests:
○ skull films
○ bone scan
○ MRI
○ Cerebral angiography (difficulty getting dye through due to abnormal
vascularization)
○ CT scan
Therapeutic management of brain tumors
● Includes a combination of surgery, radiotherapy, and chemotherapy
● Preoperative care
○ Will usually receive a stool softener—do not want child to strain after
their brain is operated on
○ A portion of the head is shaved; very traumatic for adolescent
○ If going to the ICU after surgery, have child meet the ICU staff
Postoperative care
● Positioning
○ Depends on location of tumor; usually on the opposite side of incision
○ Bed is flat or slightly elevated
○ DO NOT lower the head of the bed
○ Child will be very lethargic due to brain swelling
● Assess VS every 15 minutes initially until stable. Eventually will decrease to
every 4 hours
● Monitor IV fluids very carefully. Too much fluid can cause edema in the brain
○ We don’t want the child to be dehydrated, but we do want them on the
“drier” side
● Prevent nausea and vomiting
Therapeutic management of ICP
● Identify source and remove ASAP
● Keep coughing, vomiting, and sneezing to a minimum
● Place child in a semi-fowler’s position (infant seat for infants)
● Monitor IV fluids very carefully
● Meds:
○ Corticosteriods (Decadron)—reduces inflammation
○ Osmotic diuretic (mannitol)—pulls fluid out of tissues
DOWNS SYNDROME
● A generalized syndrome—1:800 to 1:1000 live births
● Etiology unclear
● 90% + cases attributable to an extra chromosome 21 (trisomy 21)
● Statistically greater risk if mother is over 35, but 80% born to women under the
age of 35.
● Paternal age may also be a factor
● Degree of physical and cognitive development impairment related to the
percentage of cells with abnormal chromosomal makeup
Clinical manifestations of Down syndrome
● Intelligence varies from severely affected to near-normal intelligence
● Social: 2-3 years behind mental age, especially in childhood
● Congenital anomalies: 30-40% has a congenital heart disease, especially septal
defects. May also have GI and ortho alterations.
● Respiratory—infections very prevalent
○ Due to hypotonia; swallowing muscles are weak—prone to aspiration
● Growth—rate reduced in height and weight as children; but often overweight as
teens/adults
● Sexual development—may be delayed, incomplete, or both
Physical manifestations
● Head—separated sagital suture
● Face: flat profile
● Eyes: upward, outward slant
● Nose: small and depressed
● Ears: small, sometimes low set
● Mouth: high-arched palate, downward curve, especially when crying
● Hands: broad, short, transverse palmar crease (simian line)
● Feet: wide space between great and first toes, plantar crease between great and
second toes.
● Hypotonia
Prognosis with Down syndrome
● Improved in recent years
● Significantly lower than for the general population
● Survival at one year with CHD: 76%; at 20 years of age: 53%
● Survival at one year without CHD: 91%; at 20 years of age: 82%
● Dramatic increase in mortality after the age of 44, virtually all have neuro changes
similar to Alzheimer’s disease
Possible nursing diagnoses for DS
● Potential for infection related to hypotonia, increased susceptibility to respiratory
infection
● Impaired swallowing related to hypotonia, large tongue, cognitive impairment
● Altered family processes related to having a child with Down syndrome
● Altered growth and development related to impaired cognitive functioning
● Potential for injury due to hypotonia/cognitive impairments high risk for falls
HYDROCEPHALUS
● Caused by imbalance in production and absorption of CSF
● CFS accumulates within ventricular system of brain, producing dilation of
ventricles.
Mechanisms of Fluid imbalance in Hydrocephalus
● Tumor of choroid plexus (the area that produces CSF in brain) may cause
increased secretion of CFS.
● Choroid tumors are rare, but structural malformations may cause impaired
absorption or obstruction to outflow of CSF.
● Imbalance of secretion and absorption of CFS causes CFS to accumulate in the
ventricles, which dilate and compress against cranium
● Skull also enlarged
● Most are a result of developmental malformations (in ventricular system)
● Usually presents in infancy, but can also be up to early childhood
● Other causes: infections, neoplasms, trauma, brain damage
Clinical manifestations of hydrocephalus
● Influenced by acuity of onset and presence of pre-existing structural lesions
● In infancy, head grows at an abnormal rate
● Anterior fontanel tense, bulging, dilated scalp veins (due to the skin stretching)
Manifestations of Hydrocephalus in childhood
● Caused by increased ICP
● Headache upon awakening with improvement following emesis or upright posture
● Papilledema (edema and inflammation of optic nerve)
● Strabismus
● Ataxia
● Irritability/lethargy
● Confusion
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● Incoherence
Diagnosis
● Head circumferences
● Associated Neuro signs
● CT, MRI, skull x-ray
● Dye inserted into ventricle through anterior fontanel—will not appear in CSF
from lumbar puncture if non-communicating
Therapeutic management
● Relief of hydrocephalus
● Treatment of complications
● Management of issues related to psychomotor alterations
Surgical treatment
● Direct removal of obstruction if present (cyst, neoplasm, Hematoma)
● A shunt is inserted under the skin the drain ventricles, may include a reservoir to
add medications and remove fluid.
More on Shunts…
● Valves open at a predetermined intraventricular pressure and close when the
pressure falls below that level (prevents backflow)
● Ventriculo-Peritoneal (VP) shunt is preferred for infants and young children
● Ventriculo-Atrial (VA) shunt (ventricle to right atrium) reserved for older
children who have attained most of their growth and for children with abdominal
pathology (perforation of bowel, etc.)
Complications of shunts
● Mechanical obstruction within ventricles from tissue or exudates, displacement
related to growth, thrombus (clot)
● Often presents as an emergency; increased ICP and worsening of neuro status
● Infection—the most serious complication. May occur at any time but the greatest
risk is 1-2 months after placement. (tubing colonized with bacteria)
Postoperative care after shunt placement
● Position on un-operated side
● May need to keep flat to avoid too rapid reduction of intracranial fluid
● Observe for signs of increased ICP
○ If increased ICP occurs, elevated the HOB to 15-30 degrees to enhance
gravity flow through the shunt
● Monitor I & O’s carefully, may be on a fluid restriction
● Presence of bowel sounds determined before feeding infant with VP shunt (in
case the bowel was perforated at the time of placement—do not want shit leaking
into the tube)
Signs of CSF infection
● Elevated vital signs
● Poor feeding
● Decreased LOC
● Seizures
Associated nursing diagnoses
● Potential for injury related to increased ICP
● Potential for infection related to presence of mechanical drainage system